169 results on '"Piotr Czauderna"'
Search Results
2. Case Report: Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy Application in Intraperitoneally Disseminated Inflammatory Myofibroblastic Tumor and in the Youngest Patient in the World: New Indication and Modification of Technique
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Hanna Garnier, Maciej Murawski, Tomasz Jastrzebski, Katarzyna Pawinska-Wasikowska, Walentyna Balwierz, Katarzyna Sinacka, Wojciech Gorecki, Ewa Izycka-Swieszewska, and Piotr Czauderna
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HIPEC (heated intraperitoneal chemotherapy) ,cytoreductive surgery ,inflammatory myofibroblastic tumor ,pediatric oncology ,oncological surgical treatment ,Surgery ,RD1-811 - Abstract
Introduction: Peritoneal metastases occur in cancers that spread to the peritoneal cavity and indicate the advanced stage of the disease. In children they are mainly seen in sarcomas, Gastrointestinal Stromal Tumors and primary disseminated ovarian tumors. Inflammatory Myofibroblastic Tumor (IMT) is a very rare lesion, characterized by an unpredictable clinical course. The absorption of chemotherapeutic agents through the peritoneal-plasma barrier (PPB) is minimized, thus HIPEC procedure limits the systemic exposure to chemotherapy and permits the administration of its higher doses. The main purpose of HIPEC is to remove the visible macroscopic disease in order to achieve complete cytoreduction (CRS).HIPEC Procedure in Children: Several papers deal with the CRS and HIPEC in children and adolescents, however pediatric experience is still limited. Thus far, the HIPEC procedure has been carried out on patients over 2 years old. The most common indication for the surgery and the best outcome was experienced by patients with desmoplastic small round cell tumor (DSRCT). Most patients received intraperitoneal cisplatin.HIPEC Modification: A 5-month-old infant was admitted to the Department of Pediatric Oncology due to the abdominal distention and blood in the stool. The Computed Tomography (CT) revealed a solid-cystic mass in the right abdominal area. The primary tumor and numerous peritoneal metastasis were removed and the Inflammatory Myofibroblastic Tumor (IMT) was diagnosed. The patient underwent subsequently CRS and modified HIPEC procedure. To avoid overheating of the infant, the intraperitoneal normothermic chemoperfusion was performed. Due to the low body weight a modified dosage of intraperitoneal doxorubicin was used. The child underwent standard postoperative chemotherapy and received crizotinib therapy. At 12 months follow-up since treatment completion the patient remains in complete remission. To our knowledge this is the youngest patient, the only infant and the first pediatric patient with IMT who underwent the modified HIPEC procedure in the world.Conclusions: CRS and HIPEC is technically possible also in infants. For its safe course patients selection and technique modification are necessary. Use of HIPEC should be also considered in intraperitoneally disseminated IMT. A complete cytoreductive surgery as the first HIPEC step seems to be the key factor in survival.
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- 2021
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3. Hepatoblastoma: current understanding, recent advances, and controversies [version 1; referees: 3 approved]
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Piotr Czauderna and Hanna Garnier
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Medicine ,Science - Abstract
Introduction: Hepatoblastoma (HB) is the most common primary malignant liver neoplasm in children. Its increasing survival rate is related to the progress in modern imaging, surgical techniques, and new chemotherapy regimens. Clinical approach: One of the past achievements was the development of the pretreatment extension of disease (PRETEXT) system. Gradually, the HB therapeutic approach has become more individualized with better stratification of patients. Controversies: These include the need for preoperative chemotherapy and its optimal duration; intensity of preoperative chemotherapy required for locally advanced cases (PRETEXT 4); optimal surgical treatment for locally advanced tumors: aggressive hepatic resections versus liver transplantation; the role of postoperative chemotherapy in the post-transplant setting; the timing and role of metastasectomy in patients with disseminated disease who undergo partial liver resection; and the prognostic significance of several HB pathology variants. Hepatoblastoma biology: Beta-catenin mutations and the beta-catenin/Wnt pathway play an important role in HB development. There have been at least two molecular signatures in HB published. Unluckily, all of these findings are based on relatively small clinical series and require confirmation. Conclusion: The treatment of HB started from one and the same therapy for all patients and aimed at increased treatment individualization, but the future seems to lie in biology-driven patient-tailored therapies.
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- 2018
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4. The treatment of hepatoblastoma: Its evolution and the current status as per the SIOPEL trials
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Daniel C Aronson, Piotr Czauderna, Rudolf Maibach, Giorgio Perilongo, and Bruce Morland
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Chemotherapy ,hepatoblastoma ,liver transplantation ,PRETEXT ,risk stratification ,SIOPEL-studies ,surgical resection ,Pediatrics ,RJ1-570 ,Surgery ,RD1-811 - Abstract
Aim of the Review: To describe the significant improvement in the diagnosis, treatment and outcome of children diagnosed with hepatoblastoma (HB) that has occurred in the past four decades. Recent findings are mainly focused on lessons learned from the experiences of the Childhood Liver Tumors Strategy Group (SIOPEL). Important milestones were the risk stratification of HB that allowed to tailor down therapy for standard-risk HB and intensify treatment for high-risk HB. The multi-institutional international cooperative SIOPEL trials are reviewed and current treatment guidelines are given. Intensified cooperation between the SIOPEL and the Children′s Oncology Group (COG) and the national study groups from Germany (GPOH) and Japan (JPLT) led to the acceptance and use of one staging system (PRETEXT) and the formation of a single robust database containing data of 1605 HB patients. This will allow analysis with enough statistical power of treatment directing factors that will form one of the bases of the next-generation clinical trial that is currently designed by all four collaborating study groups. Summary: Successive SIOPEL trials and increasing international collaboration have improved survival rates of patients with HB through risk stratification, advances in chemotherapy and increased complete resection rates including liver transplantation as a surgical option.
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- 2014
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5. Using Acetaminophen's Toxicity Mechanism to Enhance Cisplatin Efficacy in Hepatocarcinoma and Hepatoblastoma Cell Lines
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Alexander J. Neuwelt, Y. Jeffrey Wu, Narcyz Knap, Marcin Losin, Edward A. Neuwelt, Michael A. Pagel, Steven Warmann, Joerg Fuchs, Piotr Czauderna, and Michal Wozniak
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Neoplasms. Tumors. Oncology. Including cancer and carcinogens ,RC254-282 - Abstract
Background/Aims: Acetaminophen overdose causes hepatotoxicity mediated by toxic metabolites generated through the cytochrome P450 enzyme. The objective of this study was to investigate whether acetaminophen (AAP) can enhance cisplatin (CDDP) cytotoxicity against human hepatocarcinoma and hepatoblastoma cells in vitro and whether this effect can be prevented by N-acetylcysteine (NAC). Methods: In vitro studies (glutathione [GSH] level, cell viability, and immunoblot assays) were performed using human hepatocarcinoma and hepatoblastoma cells cultured in AAP, CDDP, and the combination of both with or without delayed NAC administration. The pharmacology and toxicology of high-dose AAP in rats were also examined. Results: Acetaminophen decreased GSH levels in liver cancer cells in a dose- and time-dependent manner. Acetaminophen combined with CDDP had enhanced cytotoxicity over CDDP alone. The cytotoxicity caused by AAP plus CDDP was decreased by NAC, with the effectiveness being time-dependent. The GSH level was lowered in the liver but not in the blood or the brain in rats treated with a high dose of AAP (1000 mg/kg). The expression of CYP2E1 protein, a key cytochrome P450 enzyme, varies among species but is not correlated to AAP sensitivity in liver cancer cells. Conclusions: Our results suggest that a chemotherapeutic regimen containing both AAP and CDDP with delayed NAC rescue has the potential to enhance chemotherapeutic efficacy while decreasing adverse effects. This would be a promising approach particularly for hepatoblastomas regardless of cellular CYP2E1 protein level but could also be beneficial in other malignancies.
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- 2009
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6. Single incision laparoscopic surgery cholecystectomy in children – preliminary experience
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Andrzej Gołębiewski, Piotr Czauderna, and Marcin Łosin
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cholecystectomy ,laparoscopy ,SILS ,paediatric surgery ,Medicine - Abstract
Introduction: Laparoscopic cholecystectomy has become the gold standard for cholecystectomy, since Mouret introducedit in 1987. In 1997 Navarra described “one incision” cholecystectomy, but only recently has single incisionlaparoscopic surgery (SILS) gained wider acceptance, mostly due to technological developments. The primary goals ofSILS are avoidance of visible scarring and minimizing surgical trauma.Aim: We present our first experience of 3 cases of children (2 females, 1 male; ages 2.5-17 years) treated with SILScholecystectomy.Methods and results: One child had undergone previous open left adrenalectomy for neuroblastoma. We used a CovidienSILS Port in one case and 3 single-use low-profile ports in the others. Percutaneous stay suture was used to suspendthe gallbladder, and standard cholecystectomy was performed using 1 straight and 1 disposable articulatinginstrument. There were no postoperative complications. Average operating time was 70 min. Hospital stay varied from2 to 4 days.Conclusion: Our early experience with SILS cholecystectomy in children suggests that it is safe and effective, but furtherstudies and greater numbers will be required to investigate the potential benefits of this approach.
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- 2010
7. Single incision laparoscopic adrenalectomy – initial experience
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Marcin Łosin, Piotr Czauderna, Andrzej Gołębiewski, and Joanna Stefanowicz
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adrenalectomy ,laparoscopy ,SILS ,paediatric surgery ,Medicine - Abstract
A laparoscopic approach to the adrenal gland was introduced in 1992. Since then many studies on adult populationsevaluating the safety and results of laparoscopic surgery have been published. A laparoscopic approach to adrenalsurgery in children still remains challenging. During the last 20 years we have observed a tendency to minimize surgicaltrauma that challenged many surgeons to seek new approaches in laparoscopic surgery. Single incision laparoscopicsurgery (SILS), in which all instruments are placed through a single, small incision, might be a step towards lessinvasive surgical procedures. We present two cases of paediatric patients treated in our department with single incisionlaparoscopic adrenalectomy (SILA). According to our best knowledge this report includes the first such procedureperformed in Poland.
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- 2010
8. Identification of IGF2 as Genomic Driver and Actionable Therapeutic Target in Hepatoblastoma
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Jordi Abril-Fornaguera, Laura Torrens, Carmen Andreu-Oller, Juan Carrillo-Reixach, Alex Rialdi, Ugne Balaseviciute, Roser Pinyol, Carla Montironi, Philipp K. Haber, Álvaro Del Río-Álvarez, Montserrat Domingo-Sàbat, Laura Royo, Nicholas K. Akers, Catherine E. Willoughby, Judit Peix, Miguel Torres-Martin, Marc Puigvehi, Stefano Cairo, Margaret Childs, Rudolf Maibach, Rita Alaggio, Piotr Czauderna, Bruce Morland, Bojan Losic, Vincenzo Mazzaferro, Ernesto Guccione, Daniela Sia, Carolina Armengol, and Josep M. Llovet
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Cancer Research ,Oncology - Abstract
Management of hepatoblastoma (HB), the most frequent pediatric liver cancer, is based on surgical resection and perioperative chemotherapy regimens. In this study, we aimed to identify actionable targets in HB and assess the efficacy of molecular therapies in preclinical models of HB. Paired tumor and adjacent tissues from 31 HBs and a validation set of 50 HBs were analyzed using RNA-seq, SNP, and methylation arrays. IGF2 overexpression was identified as the top targetable HB driver, present in 71% of HBs (22/31). IGF2high tumors displayed progenitor cell features and shorter recurrence-free survival. IGF2 overexpression was associated in 91% of cases with fetal promoter hypomethylation, ICR1 deregulation, 11p15.5 loss of heterozygosity or miR483-5p overexpression. The antitumor effect of xentuzumab (a monoclonal antibody targeting IGF1/2) alone or in combination with the conventional therapeutic agent cisplatin was assessed in HB cell lines, in PDX-derived HB organoids and in a xenograft HB murine model. The combination of xentuzumab with cisplatin showed strong synergistic antitumor effects in organoids and in IGF2high cell lines. In mice (n = 55), the combination induced a significant decrease in tumor volume and improved survival compared with cisplatin alone. These results suggest that IGF2 is an HB actionable driver and that, in preclinical models of HB, the combination of IGF1/2 inhibition with cisplatin induces superior antitumor effects than cisplatin monotherapy. Overall, our study provides a rationale for testing IGF2 inhibitors in combination with cisplatin in HB patients with IGF2 overexpression.
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- 2023
9. Data from Identification of IGF2 as Genomic Driver and Actionable Therapeutic Target in Hepatoblastoma
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Josep M. Llovet, Carolina Armengol, Daniela Sia, Ernesto Guccione, Vincenzo Mazzaferro, Bojan Losic, Bruce Morland, Piotr Czauderna, Rita Alaggio, Rudolf Maibach, Margaret Childs, Stefano Cairo, Marc Puigvehi, Miguel Torres-Martin, Judit Peix, Catherine E. Willoughby, Nicholas K. Akers, Laura Royo, Montserrat Domingo-Sàbat, Álvaro Del Río-Álvarez, Philipp K. Haber, Carla Montironi, Roser Pinyol, Ugne Balaseviciute, Alex Rialdi, Juan Carrillo-Reixach, Carmen Andreu-Oller, Laura Torrens, and Jordi Abril-Fornaguera
- Abstract
Management of hepatoblastoma (HB), the most frequent pediatric liver cancer, is based on surgical resection and perioperative chemotherapy regimens. In this study, we aimed to identify actionable targets in HB and assess the efficacy of molecular therapies in preclinical models of HB. Paired tumor and adjacent tissues from 31 HBs and a validation set of 50 HBs were analyzed using RNA-seq, SNP, and methylation arrays. IGF2 overexpression was identified as the top targetable HB driver, present in 71% of HBs (22/31). IGF2high tumors displayed progenitor cell features and shorter recurrence-free survival. IGF2 overexpression was associated in 91% of cases with fetal promoter hypomethylation, ICR1 deregulation, 11p15.5 loss of heterozygosity or miR483-5p overexpression. The antitumor effect of xentuzumab (a monoclonal antibody targeting IGF1/2) alone or in combination with the conventional therapeutic agent cisplatin was assessed in HB cell lines, in PDX-derived HB organoids and in a xenograft HB murine model. The combination of xentuzumab with cisplatin showed strong synergistic antitumor effects in organoids and in IGF2high cell lines. In mice (n = 55), the combination induced a significant decrease in tumor volume and improved survival compared with cisplatin alone. These results suggest that IGF2 is an HB actionable driver and that, in preclinical models of HB, the combination of IGF1/2 inhibition with cisplatin induces superior antitumor effects than cisplatin monotherapy. Overall, our study provides a rationale for testing IGF2 inhibitors in combination with cisplatin in HB patients with IGF2 overexpression.
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- 2023
10. Supplementary Data from Identification of IGF2 as Genomic Driver and Actionable Therapeutic Target in Hepatoblastoma
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Josep M. Llovet, Carolina Armengol, Daniela Sia, Ernesto Guccione, Vincenzo Mazzaferro, Bojan Losic, Bruce Morland, Piotr Czauderna, Rita Alaggio, Rudolf Maibach, Margaret Childs, Stefano Cairo, Marc Puigvehi, Miguel Torres-Martin, Judit Peix, Catherine E. Willoughby, Nicholas K. Akers, Laura Royo, Montserrat Domingo-Sàbat, Álvaro Del Río-Álvarez, Philipp K. Haber, Carla Montironi, Roser Pinyol, Ugne Balaseviciute, Alex Rialdi, Juan Carrillo-Reixach, Carmen Andreu-Oller, Laura Torrens, and Jordi Abril-Fornaguera
- Abstract
Supplementary Figures and Tables
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- 2023
11. Surgical management in hepatoblastoma: points to take
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Maciej Murawski, Viola B. Weeda, and Piotr Czauderna
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Pediatrics, Perinatology and Child Health ,Surgery ,General Medicine - Abstract
Hepatoblastoma is the most common primary malignant paediatric liver tumour and surgery remains the cornerstone of its management. The aim of this article is to present the principles of surgical treatment of hepatoblastoma. All aspects of surgery in hepatoblastoma are discussed, from biopsy, through conventional and laparoscopic liver resections, to extreme resection with adjacent structures, staged hepatectomy and transplantation.
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- 2023
12. Transition and management of Ukrainian war refugee children on kidney replacement therapy
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Michal Maternik, Roman Andrunevych, Dorota Drożdż, Piotr Czauderna, Ryszard Grenda, and Marcin Tkaczyk
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Nephrology ,Pediatrics, Perinatology and Child Health - Published
- 2023
13. Outcomes of Patients Treated for Hepatoblastoma with Low Alpha-Fetoprotein and/or Small Cell Undifferentiated Histology: A Report from the Children's Hepatic Tumors International Collaboration (CHIC)
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Angela D. Trobaugh-Lotrario, Rudolf Maibach, Daniel C. Aronson, Arun Rangaswami, Beate Häberle, Allison F. O’Neill, Irene Schmid, Marc Ansari, Tomoro Hishiki, Sarangarajan Ranganathan, Rita Alaggio, Ronald R. de Krijger, Yukichi Tanaka, Soo-Jin Cho, Christian Vokuhl, Rebecca Maxwell, Mark Krailo, Eiso Hiyama, Piotr Czauderna, Milton Finegold, James H. Feusner, Marcio H. Malogolowkin, Rebecka L. Meyers, and Dolores Lopez-Terrada
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Hepatoblastoma ,Cancer Research ,Oncology ,SCU (small cell undifferentiated) ,rhabdoid ,AFP ,SMARCB1 ,Liver Disease ,Oncology and Carcinogenesis ,SCU ,Digestive Diseases ,Cancer - Abstract
Small cell undifferentiated (SCU) histology and alpha-fetoprotein (AFP) levels below 100 ng/mL have been reported as poor prognostic factors in hepatoblastoma (HB); subsequent studies reported SMARCB1 mutations in some SCU HBs confirming the diagnosis of rhabdoid tumor. The Children’s Hepatic tumors International Collaboration (CHIC) database was queried for patients with HB who had AFP levels less than 100 ng/mL at diagnosis or were historically diagnosed as SCU HBs. Seventy-three of 1605 patients in the CHIC database were originally identified as SCU HB, HB with SCU component, or HB with low AFP levels. Upon retrospective review, they were re-classified as rhabdoid tumors (n = 11), HB with SCU component (n = 41), and HB with low AFP (n = 14). Seven were excluded for erroneously low AFP levels. Overall survival was 0% for patients with rhabdoid tumors, 76% for patients with HB with SCU component, and 64% for patients with HB with AFP less than 100 ng/mL. Patients with HB with SCU component or low AFP should be assessed for SMARCB1 mutations and, if confirmed, treated as rhabdoid tumors. When rhabdoid tumors are excluded, the presence of SCU component and low AFP at diagnosis were not associated with poor prognosis in patients diagnosed with HB.
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- 2023
14. Impact of COVID-19 pandemic on paediatric surgical training across Europe
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Elisa Zambaiti, Salvatore Cascio, Gloria Pelizzo, Alexander Siles Hinojosa, Juan Carlos de Augustin Asensio, Caroline Fortmann, Martin Lacher, Maria Malowiecka, Piotr Czauderna, Christoph Arneitz, Holger Till, Mathilde Glenisson, Arnaud Bonnard, Anders Telle Hoel, Kristin Bjørnland, Maria Hukkinen, Godosis Demetrios, Nikolaos Zavras, Helena Reusens, Henri Steyaert, Andrea Pevere, Ophelia Aubert, and Luca Pio
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Europe ,SARS-CoV-2 ,Surveys and Questionnaires ,Humans ,COVID-19 ,Surgery ,Child ,Pandemics - Published
- 2022
15. Liver Tumors in Pediatric Patients
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Piotr Czauderna, Greg Tiao, Eiso Hiyama, and Rebecka L. Meyers
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Hepatoblastoma ,Pediatrics ,medicine.medical_specialty ,Carcinoma, Hepatocellular ,education ,Extent of disease ,Mesenchymal hamartoma ,03 medical and health sciences ,0302 clinical medicine ,Multicenter trial ,Humans ,Multicenter Studies as Topic ,Medicine ,Child ,Staging system ,health care economics and organizations ,business.industry ,Liver Neoplasms ,Focal nodular hyperplasia ,medicine.disease ,digestive system diseases ,Oncology ,030220 oncology & carcinogenesis ,Hepatocellular carcinoma ,030211 gastroenterology & hepatology ,Surgery ,business ,Global risk - Abstract
The most recent advance in the care of children diagnosed with hepatoblastoma and hepatocellular carcinoma is the Pediatric Hepatic International Tumor Trial, which opened to international enrollment in 2018. It is being conducted as a collaborative effort by the pediatric multicenter trial groups in North America, Europe, and the Far East. This international effort was catalyzed by a new unified global risk stratification system for hepatoblastoma, an international histopathologic consensus classification for pediatric liver tumors, and a revised 2017 collaborative update of the PRE-Treatment EXTent of disease radiographic based staging system.
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- 2021
16. Pediatric Gastrointestinal Stromal Tumors and Neuroendocrine Tumors
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Caitlyn Loo, Hanna Garnier, Sanjeev A. Vasudevan, and Piotr Czauderna
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medicine.medical_specialty ,Stromal cell ,Surgical approach ,GiST ,business.industry ,Neuroendocrine tumors ,medicine.disease ,03 medical and health sciences ,0302 clinical medicine ,Oncology ,030220 oncology & carcinogenesis ,Pediatric surgery ,Pediatric oncology ,Medicine ,030211 gastroenterology & hepatology ,Surgery ,Management principles ,business ,Intensive care medicine ,Pediatric population - Abstract
Gastrointestinal stromal tumors and neuroendocrine tumors in adult and pediatric populations differ immensely. Despite these established differences, the extreme rarity of gastrointestinal stromal tumors and neuroendocrine tumors in the pediatric population has resulted in the lack of consensus management guidelines, making optimal surgical approaches unclear. Comprehensive management principles to guide surgical approaches in adult literature are extensive. However, these are still lacking for pediatric patients. International cooperation to develop standardized pediatric-specific guidelines is urgently warranted in the future. This article highlights the vast differences between adult and pediatric parameters and provides recommendations on optimal and novel surgical approaches in children.
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- 2021
17. Palliative surgery in a patient diagnosed with trisomy 18 and rare neurological comorbidities
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Piotr Czauderna, Paula Szenejko, and Stefan Anzelewicz
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Edwards syndrome ,Pediatrics ,medicine.medical_specialty ,Corpus Callosum Agenesis ,business.industry ,Prenatal diagnosis ,General Medicine ,medicine.disease ,Palliative surgery ,Hydrocephalus ,Schizencephaly ,Intervention (counseling) ,medicine ,business ,Trisomy - Abstract
Introduction: The authors describe a case of a patient with a prenatal diagnosis of Trisomy 18 with atypical manifestation, rare anomalies of the central nervous system and subsequent surgical treatment. Case presentation: The infant was diagnosed with holoprosencephalia, schizencephalia, corpus callosum agenesis and severe hydrocephalus. No significant heart, gastrointestinal or kidney defects were found. Being in otherwise good condition, the patient was qualified for a ventriculi-peritoneal shunt surgery due to rapidly increasing head circumference. Following the uneventful operation patient’s parents reported a general improvement in their child condition. Conclusions: Bearing in mind the possibility of complications and the child’s poor prognosis, the reasons for surgical intervention were evaluated, as well as the possible outcomes of withholding invasive treatment. Discussion over the benefits of palliative surgery and the risk of ‘palliative harm’ illustrate how selected children benefit from an interventionist approach.
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- 2021
18. Laparoscopic resection of liver tumors in children
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Maciej Murawski, Andrzej Gołębiewski, Piotr Czauderna, Ewa Iżycka-Świeszewska, Katarzyna Sinacka, Irena Zabolska, and Marcin Łosin
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Adult ,Male ,medicine.medical_specialty ,medicine.medical_treatment ,Benign tumor ,03 medical and health sciences ,0302 clinical medicine ,030225 pediatrics ,Laparotomy ,Biopsy ,medicine ,Hepatectomy ,Humans ,Hamartoma ,Laparoscopic resection ,Child ,Laparoscopy ,medicine.diagnostic_test ,business.industry ,Liver Diseases ,Liver Neoplasms ,Vascular malformation ,Focal nodular hyperplasia ,Infant ,General Medicine ,medicine.disease ,Surgery ,030220 oncology & carcinogenesis ,Pediatrics, Perinatology and Child Health ,Female ,business - Abstract
Background Laparoscopy for the resection of liver tumors in children has remained undeveloped in comparison to adults. Most of the indications for pediatric laparoscopic hepatic surgery have been limited to diagnostic laparoscopy (biopsy). Over the past ten years, however, laparoscopic liver resections for pediatric hepatic diseases have been performed successfully, and many case reports have been published. Methods The authors report 6 cases of laparoscopic hepatic resection of benign tumors in children. The most important aspects of surgical technique are presented. There were 3 boys and 3 girls, with age between 4 months and 16 years. The lesions were located in the following segments: II and III (4 patients), I (1), V (1). The maximum tumor size was 7 cm. Results One anatomical (left bisegmentectomy) and 5 nonanatomical resections were performed. Conversion to laparotomy was necessary in 1 patient owing to bleeding from the posterior branch of the right hepatic artery. There were no postoperative complications and patients were discharged on postoperative day 4, 5, 5, 5, 7 and 3 accordingly. The postoperative pathology of the specimens confirmed their benign nature: infantile hemangioendothelioma (1), nested stromal epithelial tumor (1), focal nodular hyperplasia (3), mixed benign tumor (hamartoma + vascular malformation) (1). Conclusions This report demonstrates the feasibility of a laparoscopic hepatic resection in children. On the other hand, laparoscopic liver resection is challenging and teamwork and specific training are necessary.
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- 2021
19. UEMS Section and Board of Paediatric Surgery—A Historical Perspective
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Jacob Williams, Piotr Czauderna, Gian Battista Parigi, Udo Rolle, and Salvatore Cascio
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Medical education ,medicine.medical_specialty ,Standardization ,business.industry ,media_common.quotation_subject ,Specialty ,Harmonization ,History, 20th Century ,History, 21st Century ,Pediatrics ,Specialties, Surgical ,Europe ,Section (archaeology) ,Service (economics) ,Pediatrics, Perinatology and Child Health ,Pediatric surgery ,medicine ,Humans ,media_common.cataloged_instance ,Surgery ,European union ,business ,Societies, Medical ,media_common ,Accreditation - Abstract
The European Union of Medical Specialists (UEMS) Section and Board of Pediatric surgery was founded more than 40 years ago. Since then major activities have been related to the improvement of quality of care of pediatric surgery in Europe. Remarkable success was achieved in the development of pediatric surgery as an independent specialty all over Europe. Other major successful activities of the UEMS Section and Board of Pediatric Surgery consisted of the development of a high-quality European examination and delineating a minimal common standard in pediatric surgery training in the form of European training requirements. Recommendations drawn from examination experience support that candidates who achieve weaker passes in part 1 may wish to consider more practice before attempting part 2 due to the weak correlation between the two scores. It may be helpful for candidates to consider having some experience working in an English-speaking clinical setting, if not truly fluent in English, to improve their chances of being successful in the part 2 examination. Other achievements of the Section were accreditation of the training centers in Europe and European Census in pediatric surgery project. All the aforementioned activities led to standardization and harmonization of pediatric surgery, as well as contributed to increasing quality of pediatric surgical service throughout Europe.
- Published
- 2020
20. Guidelines from the Polish Surgical Society and Polish Society of Oncological Surgery Concerning Quality Assurance for Centres Performing Cytoreductive Procedures and HIPEC Procedures in the Treatment of Primary and Secondary Peritoneal Tumours
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Wojciech Polkowski, Wojciech M. Wysocki, Piotr Czauderna, Wojciech Biernat, Jerzy Mielko, Arkadiusz Jeziorski, Tomasz Polec, Grzegorz Wallner, Lucjan Wyrwicz, Wojciech Zegarski, Radosław Owczuk, Marek Jackowski, Krzysztof Szewczyk, Piotr Richter, Dariusz Wydra, Tomasz Olesiński, Michał Studniarek, Anna Sommer, Tomasz Jastrzębski, Adam Dziki, and Marek Bębenek
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Cancer Research ,medicine.medical_specialty ,Treatment quality ,Oncology ,Peritoneal cancer ,business.industry ,General surgery ,medicine ,Oncological surgery ,Hyperthermic intraperitoneal chemotherapy ,Surgical treatment ,business ,Quality assurance - Abstract
Surgical treatment of patients with peritoneal metastases in combination with Hyperthermic intraperitoneal Chemotherapy (HIPEC) and systemic treatments is applied with increasing frequency and, with correct patient qualification, allows for obtaining 5-year survival at a level of 32–52%. The conditions necessary for positive results of such treatment include the high experience of a given centre, its appropriate infrastructure, and appropriate patient qualification for the procedure. As a result of the debate connected with the need to evaluate treatment quality and results, at the request of the Peritoneal Cancer Section of the Polish Society of Oncological Surgery, the conditions for quality assurance were worked out and a Quality Assurance Commission was set up for the centres performing cytoreductive procedures and HIPEC procedures in the treatment of primary and secondary peritoneal tumours.
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- 2020
21. Pediatric Surgical Oncology
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Christophe Chardot, Sabrine Ben Youssef, Carmen Capito, Piotr Czauderna, Jan Godzinski, Alexander Rokitansky, and Zacharias Zachariou
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- 2022
22. Health centres 75+ as a new model to improve care for older people in Poland
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Anna Sagan, Małgorzata Gałązka-Sobotka, Piotr Czauderna, Aldona Frączkiewicz-Wronka, Katarzyna Badora-Musiał, Natalia Petka, and Iwona Kowalska-Bobko
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Aging ,Geriatrics ,Health, Toxicology and Mutagenesis ,RA0421 Public health. Hygiene. Preventive Medicine ,Public Health, Environmental and Occupational Health ,Humans ,Poland ,Ambulatory Care Facilities ,Aged - Abstract
According to a recent national audit, the cost of treating patients in geriatric wards is 20–30% less compared to those treated in internal medicine wards. Yet, geriatric care remains largely underdeveloped in Poland, with few human, material, and financial resources. Despite numerous attempts to raise the profile of geriatrics over the years, little progress has been achieved. In 2019, experts under the President of Poland proposed the creation of a network of Health Centres 75+ as the first pillar of geriatric care. These are meant to provide ambulatory services for older people and coordinate provision of other health and social care services at the county level. The goal is to create a community model of care, whereby older people would receive needed services close to their place of residence, allowing them to live independently for as long as possible. Although the proposal has been welcomed by the geriatric community and the patients, the acute shortages of human, physical, and financial resources raise concerns about its feasibility. However, the new strategic plans for the health system propose solutions that appear to be supportive of the new proposal, and the Office of the President is discussing joining forces with the Ministry of Health to improve its chances of implementation. Given the increasing pace of population ageing and underdeveloped provision of geriatric services, these efforts are very much needed.
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- 2022
23. Case Report: Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy Application in Intraperitoneally Disseminated Inflammatory Myofibroblastic Tumor and in the Youngest Patient in the World: New Indication and Modification of Technique
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Maciej Murawski, Walentyna Balwierz, Wojciech Górecki, Katarzyna Sinacka, Piotr Czauderna, Hanna Garnier, Katarzyna Pawinska-Wasikowska, Ewa Izycka-Swieszewska, and Tomasz Jastrzębski
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Cisplatin ,medicine.medical_specialty ,Chemotherapy ,Crizotinib ,Desmoplastic small-round-cell tumor ,RD1-811 ,business.industry ,medicine.medical_treatment ,Case Report ,pediatric oncology ,medicine.disease ,Primary tumor ,HIPEC (heated intraperitoneal chemotherapy) ,Surgery ,Peritoneal cavity ,medicine.anatomical_structure ,medicine ,cytoreductive surgery ,Hyperthermic intraperitoneal chemotherapy ,Doxorubicin ,inflammatory myofibroblastic tumor ,oncological surgical treatment ,business ,medicine.drug - Abstract
Introduction: Peritoneal metastases occur in cancers that spread to the peritoneal cavity and indicate the advanced stage of the disease. In children they are mainly seen in sarcomas, Gastrointestinal Stromal Tumors and primary disseminated ovarian tumors. Inflammatory Myofibroblastic Tumor (IMT) is a very rare lesion, characterized by an unpredictable clinical course. The absorption of chemotherapeutic agents through the peritoneal-plasma barrier (PPB) is minimized, thus HIPEC procedure limits the systemic exposure to chemotherapy and permits the administration of its higher doses. The main purpose of HIPEC is to remove the visible macroscopic disease in order to achieve complete cytoreduction (CRS).HIPEC Procedure in Children: Several papers deal with the CRS and HIPEC in children and adolescents, however pediatric experience is still limited. Thus far, the HIPEC procedure has been carried out on patients over 2 years old. The most common indication for the surgery and the best outcome was experienced by patients with desmoplastic small round cell tumor (DSRCT). Most patients received intraperitoneal cisplatin.HIPEC Modification: A 5-month-old infant was admitted to the Department of Pediatric Oncology due to the abdominal distention and blood in the stool. The Computed Tomography (CT) revealed a solid-cystic mass in the right abdominal area. The primary tumor and numerous peritoneal metastasis were removed and the Inflammatory Myofibroblastic Tumor (IMT) was diagnosed. The patient underwent subsequently CRS and modified HIPEC procedure. To avoid overheating of the infant, the intraperitoneal normothermic chemoperfusion was performed. Due to the low body weight a modified dosage of intraperitoneal doxorubicin was used. The child underwent standard postoperative chemotherapy and received crizotinib therapy. At 12 months follow-up since treatment completion the patient remains in complete remission. To our knowledge this is the youngest patient, the only infant and the first pediatric patient with IMT who underwent the modified HIPEC procedure in the world.Conclusions: CRS and HIPEC is technically possible also in infants. For its safe course patients selection and technique modification are necessary. Use of HIPEC should be also considered in intraperitoneally disseminated IMT. A complete cytoreductive surgery as the first HIPEC step seems to be the key factor in survival.
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- 2021
24. Abstract PO011: Proteomic profiling of childhood liver cancer: identification of novel diagnostic and prognostic biomarkers
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Álvaro Del Río-Álvarez, Juan Carrillo-Reixach, Laura Royo, Montse Domingo-Sàbat, Mikel Azkargorta, Roland Kapler, Stefano Cairo, Christian Vokuhl, Ronald de Krijger, Rita Alaggio, Marta Garrido, Gabriela Guillen, Constantino Sábado, Laura Guerra, Francisco Hernandez, Maria Elena Mateos, Manuel López-Satamaría, Barbara Torres, Maria Pilar Abad, Bajčiová Viera, Piotr Czauderna, Marie Annick Buendia, Felix Elortza, Keith Wheatley, Bruce Morland, and Carolina Armengol
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Cancer Research ,Oncology - Abstract
Childhood liver cancers hepatoblastoma (HB) and hepatocellular carcinoma (HCC) are rare diseases but with a rising incidence. HEMNOS (Hepatocellular malignant neoplasm, not otherwise specified) is a recent entity with histopathological features of HB and HCC. Current chemotherapy treatments are effective to shrink tumor before surgery, nonetheless can cause severe lifelong adverse effects and are not effective for patients with aggressive and metastatic HB or HCC (~20% die due to the disease). The HB and HCC diagnosis is key to assign therapeutic regimens in the ongoing Pediatric Hepatic International Tumor Trial (PHITT); however, differential diagnosis for some patients is challenging due to the lack of specific biomarkers. Nowadays, there is an urgent need to identify diagnostic and prognostic biomarkers to improve the clinical management of childhood liver cancer. Herein, we aimed to uncover the proteomic profiles of different types of childhood liver cancer and identify new biomarkers for improving tumor diagnosis and early detection of the most aggressive cases. For this, 99 frozen tissue samples from 71 patients (mean age: 54.5 months, 60.6% boys, 40.9% metastasis, 23.7% deaths) including 70 primary tumors (57 HB, 8 HCC and 5 HEM-NOS), 22 non-tumors and 7 metastases were analyzed by label free mass spectrometry analysis. A total of 5,417 proteins were sequenced in the different samples. Through supervised analysis, we identified a total of 1302 differential expressed proteins in tumors as compared to non-tumor livers (FC +2 and FDR 2) was defined for HB, HCC and HEM-NOS, respectively. Hierarchical unsupervised clustering and principal component analysis showed two main cluster of tumors: one including HB and HEM-NOS and another one with a mixture of HB, HEM-NOS and HCC. Interestingly, this second cluster was associated with clinical and molecular features of aggressive tumors such as multifocality (p = 0.029), Epigenetic Epi-CB subclass (p = 0.006), high-risk Molecular Risk Stratification (p = 0.009) and poor survival (log rank test=0.012). A total of 2082 proteins were found to be differently expressed between the two prognostic clusters (FC +2 and FDR < 0.01); the GEO enrichment analysis revealed that these proteins were significantly enriched in post-transcriptional gene silencing and RNA splicing mechanisms (FDR 6 and FDR < 10−14). In conclusion, we identified a list of potential diagnostic and prognostic protein biomarkers that after a validation in large patient cohort, could be used to improve the clinical management of childhood liver cancer. Citation Format: Álvaro Del Río-Álvarez, Juan Carrillo-Reixach, Laura Royo, Montse Domingo-Sàbat, Mikel Azkargorta, Roland Kapler, Stefano Cairo, Christian Vokuhl, Ronald de Krijger, Rita Alaggio, Marta Garrido, Gabriela Guillen, Constantino Sábado, Laura Guerra, Francisco Hernandez, Maria Elena Mateos, Manuel López-Satamaría, Barbara Torres, Maria Pilar Abad, Bajčiová Viera, Piotr Czauderna, Marie Annick Buendia, Felix Elortza, Keith Wheatley, Bruce Morland, Carolina Armengol. Proteomic profiling of childhood liver cancer: identification of novel diagnostic and prognostic biomarkers [abstract]. In: Proceedings of the AACR Special Conference: Advances in the Pathogenesis and Molecular Therapies of Liver Cancer; 2022 May 5-8; Boston, MA. Philadelphia (PA): AACR; Clin Cancer Res 2022;28(17_Suppl):Abstract nr PO011.
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- 2022
25. Abstract PO009: Identification of IGF2 as genomic driver and actionable therapeutic target in hepatoblastoma
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Jordi Abril-Fornaguera, Laura Torrens, Juan Carrillo-Reixach, Alex Rialdi, Ugne Balaseviciute, Júlia Huguet-Pradell, Carla Montironi, Philipp Haber, Álvaro Del Río-Álvarez, Montserrat Domingo-Sàbat, Laura Royo, Nicholas Akers, Catherine E Willoughby, Judit Peix, Miguel Torres-Martin, Marc Puigvehi, Roser Pinyol, Stefano Cairo, Margaret Childs, Rudolf Maibach, Rita Alaggio, Piotr Czauderna, Bruce Morland, Bojan Losic, Vincenzo Mazzaferro, Ernesto Guccione, Daniela Sia, Carolina Armengol, and Josep M Llovet
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Cancer Research ,Oncology - Abstract
Background and Aims: Management of hepatoblastoma (HB), the most frequent pediatric liver cancer, is based on surgical resection and perioperative chemotherapy regimens, commonly cisplatin. Here, we aimed to identify actionable targets in HB and assess the efficacy of molecular therapies in preclinical models of HB.Methods: Paired tumor and adjacent tissues from 31 resected HBs and a validation set of 50 HBs were analyzed at the transcriptomic, genomic and epigenomic level using RNAseq, SNP and methylation arrays, respectively. The main targetable driver in HB was identified by gene co-expression network analysis (GCN) and its overexpression was confirmed by qRT-PCR. The anti-tumor effect of driver inhibition with molecular therapies alone or in combination with cisplatin was assessed in cell lines, patient-derived HB organoids and in a HB xenograft murine model.Results: Seven network modules were significantly deregulated in tumors compared to non-tumoral samples, including IGF2 signaling pathway, cell cycle and survival and immune response. IGF2 overexpression (FC> 4 vs adjacent tissue) was identified as the top targetable HB driver (study cohort: 71%, 22/31; independent validation cohorts: 78% and 76%). IGF2high tumors displayed progenitor cell features and were significantly enriched in molecular classes with aggressive phenotypes and CTNNB1 mutations, while IGF2low tumors were enriched in inflammatory and TGF-β signaling. IGF2high tumors correlated with shorter recurrence-free survival after resection (median 34 months vs not reached for IGF2low; p = 0.02). IGF2 overexpression correlated in most cases (86%) with fetal promoter hypomethylation (50%), 11p15.5 loss of heterozygosity (LOH, 57%) or overexpression of miR483 (55%). Xentuzumab (anti-IGF1/2 mAb) alone or combined with cisplatin reduced proliferation and clonogenic capacity in IGF2high cell lines. The combination of xentuzumab and cisplatin exhibited synergistic effects in terms of cell viability in organoids derived from IGF2high human HBs. The combination treatment induced apoptosis and reduced IGF2 pathway activation in vitro. In mice (n = 13-14 per arm), this combination induced a significant decrease in the viable tumor volume (p < 0.01), extended survival compared to cisplatin alone (p < 0.05) and inhibited tumor angiogenesis (p < 0.05).Conclusion: IGF2 is an actionable driver in HB and its overexpression was associated with fetal promoter hypomethylation, LOH or miR483 overexpression. The combination of a mAb against IGF1/2 (xentuzumab) with cisplatin led to remarkable anti-tumoral effects in pre-clinical models, providing the rationale for exploring this regimen in IGF2high HB patients. Citation Format: Jordi Abril-Fornaguera, Laura Torrens, Juan Carrillo-Reixach, Alex Rialdi, Ugne Balaseviciute, Júlia Huguet-Pradell, Carla Montironi, Philipp Haber, Álvaro Del Río-Álvarez, Montserrat Domingo-Sàbat, Laura Royo, Nicholas Akers, Catherine E Willoughby, Judit Peix, Miguel Torres-Martin, Marc Puigvehi, Roser Pinyol, Stefano Cairo, Margaret Childs, Rudolf Maibach, Rita Alaggio, Piotr Czauderna, Bruce Morland, Bojan Losic, Vincenzo Mazzaferro, Ernesto Guccione, Daniela Sia, Carolina Armengol, Josep M Llovet. Identification of IGF2 as genomic driver and actionable therapeutic target in hepatoblastoma [abstract]. In: Proceedings of the AACR Special Conference: Advances in the Pathogenesis and Molecular Therapies of Liver Cancer; 2022 May 5-8; Boston, MA. Philadelphia (PA): AACR; Clin Cancer Res 2022;28(17_Suppl):Abstract nr PO009.
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- 2022
26. Abstract PO010: Molecular characterization of pediatric hepatocellular carcinoma: genomic, methylomic and transcriptomic analysis
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Juan Carrillo-Reixach, Álvaro del Río-Álvarez, Laura Royo, Montserrat Domingo-Sàbat, Rita Alaggio, Ronald de Krijger, Christian Vokuhl, Marta Garrido, Laura Guerra, Constantino Sábado, Francisco Hernández, Manuel López-Santamaría, Viera Bajčiová, Rudolf Maibach, Margaret Childs, Piotr Czauderna, Keith Wheatley, Roland Kappler, Stefano Cairo, Bruce Morland, Margarita Sala, Maria Rosa Sarrias, and Carolina Armengol
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Cancer Research ,Oncology - Abstract
Background: The main pediatric liver tumors are hepatoblastoma (HB) and, to a lesser extent, pediatric hepatocellular carcinoma (HCC). HB appears at an early age (8 years) or adolescents and it is usually associated to underlying metabolic diseases. HEM-NOS (Hepatocellular malignant neoplasm, not otherwise specified) is a recent entity with histopathological features of HB and HCC. Due to the rarity of the disease ( Citation Format: Juan Carrillo-Reixach, Álvaro del Río-Álvarez, Laura Royo, Montserrat Domingo-Sàbat, Rita Alaggio, Ronald de Krijger, Christian Vokuhl, Marta Garrido, Laura Guerra, Constantino Sábado, Francisco Hernández, Manuel López-Santamaría, Viera Bajčiová, Rudolf Maibach, Margaret Childs, Piotr Czauderna, Keith Wheatley, Roland Kappler, Stefano Cairo, Bruce Morland, Margarita Sala, Maria Rosa Sarrias, Carolina Armengol. Molecular characterization of pediatric hepatocellular carcinoma: genomic, methylomic and transcriptomic analysis [abstract]. In: Proceedings of the AACR Special Conference: Advances in the Pathogenesis and Molecular Therapies of Liver Cancer; 2022 May 5-8; Boston, MA. Philadelphia (PA): AACR; Clin Cancer Res 2022;28(17_Suppl):Abstract nr PO010.
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- 2022
27. Endometrial glial polyp and peritoneal gliomatosis: Neuropathological lesions in gynecological biopsy
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Katarzyna Czarnota, Sebastian Goertz, Jacek Gulczyński, Piotr Czauderna, Blanka Hermann-Okoniewska, Elżbieta Adamkiewicz-Drożyńska, and Ewa Iżycka-Świeszewska
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Pathology ,medicine.medical_specialty ,Adolescent ,Biopsy ,Neuropathology ,Pathology and Forensic Medicine ,Lesion ,Young Adult ,Immunophenotyping ,Peritoneal Gliomatosis ,medicine ,Humans ,Sampling (medicine) ,Young adult ,Pathological ,medicine.diagnostic_test ,business.industry ,Teratoma ,Glioma ,General Medicine ,Neurology ,Female ,Neurology (clinical) ,Nervous System Diseases ,medicine.symptom ,business ,Neuroglia - Abstract
The wide scope of neuropathology is also connected to different systemic pathology findings. Neuroectodermal-type female genital tract lesions are not frequent. This article presents a short review of such entities in gynecological pathology and reports on two rare cases. The first described lesion is an endometrial glial polyp in a young woman. The second is a mature cystic teratoma accompanied by a peritoneal gliomatosis in an adolescent girl. Both presented entities posed diagnostic difficulties from the clinical and pathological perspective. They demanded careful sampling, immunophenotyping, as well as neuropathological consultation.
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- 2019
28. A Prospective Randomized Controlled Trial of Single-Port and Three-Port Laparoscopic Appendectomy in Children
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Andrzej Gołębiewski, Stefan Anzelewicz, Piotr Czauderna, Dominika Lubacka, and Agnieszka Wiejek
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Male ,medicine.medical_specialty ,Time Factors ,Adolescent ,Operative Time ,Surgical Wound ,law.invention ,Port (medical) ,Randomized controlled trial ,law ,Appendectomy ,Humans ,Medicine ,Postoperative Period ,Prospective Studies ,Child ,Intraoperative Complications ,Laparoscopy ,Pain, Postoperative ,medicine.diagnostic_test ,Interleukin-6 ,business.industry ,Length of Stay ,Appendicitis ,Conversion to Open Surgery ,Surgery ,Analgesics, Opioid ,C-Reactive Protein ,Wound Infection ,Female ,business - Abstract
Background: This study aimed to evaluate single-port laparoscopic appendectomy (SPLA) in comparison with three-port laparoscopic appendectomy (3PLA) in children about the extent of surgica...
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- 2019
29. Abstract 4005: Identification of IGF2 as genomic driver and actionable therapeutic target in hepatoblastoma
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Jordi Abril-Fornaguera, Laura Torrens, Juan Carrillo-Reixach, Alexander Rialdi, Ugne Balaseviciute, Carla Montironi, Philipp Haber, Álvaro Del Río-Álvarez, Montserrat Domingo-Sàbat, Laura Royo, Nicholas Akers, Catherine E. Willoughby, Judit Peix, Miguel Torres-Martin, Marc Puigvehi, Roser Pinyol, Stefano Cairo, Margaret Childs, Rudolf Maibach, Rita Alaggio, Piotr Czauderna, Bruce Morland, Bojan Losic, Vincenzo Mazzaferro, Ernesto Guccione, Daniela Sia, Carolina Armengol, and Josep M. Llovet
- Subjects
Cancer Research ,Oncology - Abstract
Background and Aims: Management of hepatoblastoma (HB), the most frequent pediatric liver cancer, is based on surgical resection and perioperative platin-based regimens, commonly cisplatin. Here, we aimed to identify actionable targets in HB and assess the efficacy of molecular therapies in HB cell lines, organoids and murine models. Methods: Paired tumor and adjacent tissues from 31 resected HBs and a validation set of 50 HBs were analyzed at the transcriptomic, genomic and epigenomic level using RNAseq, SNP and methylation arrays, respectively. The main targetable driver in HB was identified by gene co-expression network analysis (GCN) and its overexpression was confirmed by qRT-PCR. The anti-tumor effect of driver inhibition with molecular therapies alone or in combination with cisplatin was assessed in cell lines, patient-derived HB organoids and in a HB xenograft murine model. Results: IGF2 overexpression (FC> 4 vs adjacent tissue) was identified as the top targetable HB driver (71%, 22/31). IGF2high tumors displayed progenitor cell features and were significantly enriched in molecular classes with aggressive phenotypes and CTNNB1 mutations, while IGF2low tumors were enriched in inflammatory and TGF-β signaling. IGF2high tumors correlated with shorter recurrence-free survival after resection (median 34 months vs not reached for IGF2low; p = 0.02). IGF2 overexpression correlated in most cases (86%) with fetal promoter hypomethylation (50%), 11p15.5 loss of heterozygosity (LOH, 57%) or overexpression of miR483 (55%). Xentuzumab (anti-IGF1/2 mAb) alone or combined with cisplatin reduced proliferation and clonogenic capacity in IGF2high cell lines. The combination of xentuzumab and cisplatin exhibited synergistic effects in terms of cell viability in organoids derived from IGF2high human HBs. In mice, this combination induced a significant decrease in the viable tumor volume and extended survival compared to cisplatin alone (n = 13-14 per arm, p = 0.04). Conclusion: IGF2 is an actionable driver in HB and its overexpression was associated with fetal promoter hypomethylation, LOH or miR483 overexpression. The combination of a mAb against IGF1/2 (xentuzumab) with cisplatin led to remarkable anti-tumoral effects in pre-clinical models, providing the rationale for exploring this regimen in IGF2high HB patients. Citation Format: Jordi Abril-Fornaguera, Laura Torrens, Juan Carrillo-Reixach, Alexander Rialdi, Ugne Balaseviciute, Carla Montironi, Philipp Haber, Álvaro Del Río-Álvarez, Montserrat Domingo-Sàbat, Laura Royo, Nicholas Akers, Catherine E. Willoughby, Judit Peix, Miguel Torres-Martin, Marc Puigvehi, Roser Pinyol, Stefano Cairo, Margaret Childs, Rudolf Maibach, Rita Alaggio, Piotr Czauderna, Bruce Morland, Bojan Losic, Vincenzo Mazzaferro, Ernesto Guccione, Daniela Sia, Carolina Armengol, Josep M. Llovet. Identification of IGF2 as genomic driver and actionable therapeutic target in hepatoblastoma [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2022; 2022 Apr 8-13. Philadelphia (PA): AACR; Cancer Res 2022;82(12_Suppl):Abstract nr 4005.
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- 2022
30. Pediatric Gastrointestinal Stromal Tumors and Neuroendocrine Tumors: Advances in Surgical Management
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Hanna, Garnier, Caitlyn, Loo, Piotr, Czauderna, and Sanjeev A, Vasudevan
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Adult ,Neuroendocrine Tumors ,Consensus ,Gastrointestinal Stromal Tumors ,Humans ,Child - Abstract
Gastrointestinal stromal tumors and neuroendocrine tumors in adult and pediatric populations differ immensely. Despite these established differences, the extreme rarity of gastrointestinal stromal tumors and neuroendocrine tumors in the pediatric population has resulted in the lack of consensus management guidelines, making optimal surgical approaches unclear. Comprehensive management principles to guide surgical approaches in adult literature are extensive. However, these are still lacking for pediatric patients. International cooperation to develop standardized pediatric-specific guidelines is urgently warranted in the future. This article highlights the vast differences between adult and pediatric parameters and provides recommendations on optimal and novel surgical approaches in children.
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- 2021
31. Preoperative planning in paediatric liver tumour surgery - a literature review
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Agnieszka Niemirycz-Makurat, Stefan Anzelewicz, Dominika Kościuszko, Piotr Czauderna, and Andrzej Gołębiewski
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Computer-assisted surgery ,medicine.medical_specialty ,Preoperative planning ,Tumour surgery ,business.industry ,General surgery ,medicine.medical_treatment ,medicine ,business - Published
- 2021
32. Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study
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Naomi Jane Wright, Andrew J.M. Leather, Niyi Ade-Ajayi, Nick Sevdalis, Justine Davies, Dan Poenaru, Emmanuel Ameh, Adesoji Ademuyiwa, Kokila Lakhoo, Emily Rose Smith, Abdel Douiri, Maria Elstad, Marcus Sim, Cristiana Riboni, Bruno Martinez-Leo, Melika Akhbari, Stephen Tabiri, Ashrarur Mitul, Dayang Anita Abdul Aziz, Camila Fachin, Alliance Niyukuri, Muhammad Arshad, Fowzia Ibrahim, Natalie Moitt, Mohamed Fahmy Doheim, Hannah Thompson, Harmony Ubhi, Isabelle Williams, Sophia Hashim, Godfrey Sama Philipo, Laura Herrera, Aayenah Yunus, Dominique Vervoort, Samuel Parker, Yousra-Imane Benaskeur, Osaid H. Alser, Nana Adofo-Ansong, Ahmad Alhamid, Hosni khairy Salem, Mahmoud Saleh, Safa Abdal Elrais, Sadi Abukhalaf, Patricia Shinondo, Ibrahim Nour, Emrah Aydin, Agota Vaitkiene, Kelly Naranjo, Andile Maqhawe Dube, Sodumisa Ngwenya, Mina A. Yacoub, Henang Kwasau, Gabriella Hyman, Shrouk Mahmoud Elghazaly, Ibrahim Al-Slaibi, Intisar Hisham, Helena Franco, Hana Arbab, Lubna Samad, Aqil Soomro, Muhammad Amjad Chaudhry, Safina Karim, Muhammad Adnan Khan Khattak, Shireen Anne Nah, Doris Mae Dimatatac, Candy SC Choo, Niveshni Maistry, Ashrarur Rahman Mitul, Samiul Hasan, Sabbir Karim, Hina Yousuf, Taimur Qureshi, Ibrahim Rabi Nour, Raed Nael Al-Taher, Osama Abdul Kareem Sarhan, Luis Garcia-Aparicio, Jordi Prat, Eva Blazquez-Gomez, Xavier Tarrado, Martí Iriondo, Paolo Bragagnini, Segundo Rite, Lars Hagander, Emma Svensson, Sheila Owusu, Alhassan Abdul-Mumin, Dominic Bagbio, Vijay Anand Ismavel, Ann Miriam, Shajin T, Marlene Anaya Dominguez, Monica Ivanov, Andreea Madalina Serban, Miliard Derbew, Mahmoud Elfiky, Maricarmen Olivos Perez, Marcia Abrunhosa Matias, Alexis P Arnaud, Ahmed Negida, Sebastian King, Mohamad Rafi Fazli, Nadia Hamidi, Souhem Touabti, Rossana Francisco Chipalavela, Pablo Lobos, Brendan Jones, Damir Ljuhar, Georg Singer, Annelien Cordonnier, Lorena Jáuregui, Zlatan Zvizdic, Janice Wong, Etienne St-Louis, Qiang Shu, Yang Lui, Catalina Correa, Lucie Pos, Elvyn Alcántara, Erick Féliz, Luis Enrique Zea-Salazar, Liza Ali, Matthieu Peycelon, Nzanzu Kipata Anatole, Cherno S. Jallow, Judith Lindert, Dhruv Ghosh, Cathline Freya Adhiwidjaja, Ahmad Khaleghnejad Tabari, Saran Lotfollahzadeh, Haidar Mohammad Mussein, Fabrizio Vatta, Noemi Pasqua, David Kihiko, Hetal Gohil, Ibrahim R. Nour, Muhammed Elhadi, Suad Ahmed Almada, Gilvydas Verkauskas, Toni Risteski, Alejandro Peñarrieta Daher, Oumaima Outani, James Hamill, Taiwo Lawal, Jack Mulu, Benjamin Yapo, Lily Saldaña, Beda Espineda, Krystian Toczewski, Eugene Tuyishime, Isaac Ndayishimiye, Enaam Raboe, Philip Hammond, Gregor Walker, Ivona Djordjevic, Milind Chitnis, Joonhyuk Son, Sanghoon Lee, Muaad Hussien, Sawazen Malik, Enas Musa Ismail, Ampaipan Boonthai, Nesrine Ben Hadj Dahman, Nigel Hall, Fabiola Ruth Castedo Camacho, Helena Sobrero, Marilyn Butler, Aliev Makhmud, Nathan Novotny, Ahmad G. Hammouri, Maisara Al-Rayyes, Bruce Bvulani, Qais Muraveji, Muhammad Yousuf Murzaie, Ajmal Sherzad, Sayed Aman Haidari, Abdul Baqi Monawar, Dr. Ahmad Zia Samadi, Jesh Thiessen, Ntakarutimana Venant, Sonia Inamuco Hospital, Niyonkuru Jérémie, Jean Claude Mbonicura, Butoyi Jean Marie Vianney, Amezene Tadesse, Samuel Negash, Charles A. Roberts, John N. Jabang, Abdoulie Bah, Kajali Camamra, Armandou Correa, Babucarr Sowe, A. Gai, Musa Jaiteh, Kwizera Jean Raymond, Jean Paul Mvukiyehe, Innocent Itangishaka, Emmanuel Kayibanda, Emery Manirambona, Joseph Lule, Ainhoa Costas-Chavarri, Ian Shyaka Gashugi, Albert Ndata, Georges Gasana, Yves Castar Nezerwa, Turatsinze Simeon, Jean De Dieu Muragijimana, Sakina Rashid, David Msuya, Joseph Elisante, Meghna Solanki, Emmanuel Manjira, Jay Lodhia, Mubashir Jusabani, Murad Tarmohamed, Sengua Koipapi, Touabti Souhem, Nabti Sara, Brahimi Sihem, Bouguermouh Dania, Iaiche Achour Toufik, Baghdadi Nour el islam Mounira, Alouani Habiba, Liliana Aragão, Victor Gonçalves, Marcelo Mauricio Lino Urquizo, Maria Florencia Varela, Pedro Mercado, Bonavia Horacio, Andrea Damiani, Carlos Mac, Daniel Putruele, Karen Liljesthrom, Marianela Bernaus, Cesar Jauri, Alejandrina Cripovich, Ezequiel Bianchin, Maria Gabriela Puig, Lorna Andreussi, Susana Iracelay, Dolores Marcos, Carina Herrera, Nelly Palacios, Romina Avile, Belen Serezo, Debora Montoya, Rodrigo Cepeda, Justo Vaquila, Sofficci Veronica, Liliana Pardo, Pelussi Valeria, Lapalma Julio, Aranda Diego Martin, Palazzi Lucio, Comba Gabriel, Depetrini Marianella, José Alfredo Calderón Arancibia, Enrique Huespe, Gabriela Natalia Losa, Elsa Arancibia Gutiérrez, Humberto Scherl, Daniel Emilio Gonzalez, Valentina Baistrocchi, Yanina Silva, Marcelo Galdeano, Pablo Medard, Ines Sueiras, Enrique Romero Manteola, Victor Hugo Defago, Carlos Mieres, Carlos Alberto, Fabio Cornelli, Marcelo Molina, Pablo Ravetta, Celeste Carolina Patiño Gonzalez, Maria Belen Dallegre, Maria Tatiana Szklarz, Marcos Federico Leyba, Nahuel Ignacio Rivarola, Maria Delia Charras, Adriana Morales, Paloma Caseb, Luzia Toselli, Carolina Millán, Maria del Carmen Junes, Oscar Di Siervi, Jose Gilardi, Soledad Simon, Carla Sofia Contreras, Nair Rojas, Lucia Beatriz Arnoletto, Otilia Eva Blain, Mauro Nicolas Bravo, Nancy Sanchez, Luciana Martina Herrera Pesara, Maria Eugenia Moreno, Carlos Ariel Sferco, Umama Huq, Tamanna Ferdousi, Abdullah Al-Mamun, Sadia Sultana, Refoyez Mahmud, Khalid Mahmud, Fatema Sayeed, Alexander Svirsky, Denisse Sempertegui, Amalia Negrete, Araceli Teran, Mariana Sadagurschi, Nusret Popovic, Kenan Karavdic, Emir Milisic, Asmir Jonuzi, Amira Mesic, Sabina Terzic, Nejra Dendusic, Elna Biber, Anesa Sehic, Nada Zvizdic, Emina Letic, Adna Saracevic, Ajla Hamidovic, Nejra Selak, Dzan Horozic, Lamija Hukic, Amila Muhic, Nedim Vanis, Emir Sokolovic, Adnan Sabic, Karin Becker, Elis Novochadlo Klüppel, André Iván Bradley dos Santos Dias, Miguel Angelo Agulham, Cristiano Bischoff, Stella Sabbatini, Rachel Fernandes de Souza, Ana Beatriz Souza Machado, Juliana Werneck Raposo, Maria Lucia da Silva Augusto, Bianca M.R. Martins, Mariana de Souza Santos Ferreira, Darli Fernandes de Oliveira, Carla Silva dos Santos, Fernanda Ribeiro de Fernández y Alcázar, Érika Alves Dutra da Silva, Mariana Furtado, Horácio Tamada, Marília Silva Ferreira dos Santos, Thayná Lopes de Almeida, Susy Oliveira de Andrade, Antonio Cipriano Gurgel do Amaral, Lais Sartori Giovanoni, Kamila de Deus Passos Leles, Eduardo Corrêa Costa, Leticia Feldens, Luciano Ferraz Schopf, José Carlos Soares de Fraga, Felipe Colombo de Holanda, Paola Maria Brolin Santis Isolan, Julia Loyola Ferreira, Carla Luisa Bruxel, Danielle Lopes Teixeira Ferdinando, Fabricio Zottis Barcelos, Natalia Baseggio, Nicole Knorr Brenner, Rafael Trindade Deyl, Carolina Dure, Iuri Nunes Kist, Rafael Bueno Mazzuca, Sarah Bueno Motter, Yna Ramos, Cristine Suzana Trein, Bianca Rezende Rosa, Murilo de Assis Silva, Flavio Augusto Menin, Isabela Cristina Semensato Carloni, Juliana Antinarelli Norberto da Silva, Adriano Luis Gomes, Mariana Girão Tauffer, Paulo César Bassan Gonçalves, Geraldo Magela Nogueira Marques, Eliane Moriya, Carla Labonia, Ana Lucia Carrasco, Karine Furtado Meyer, Luiz Farion-Aguiar, Fernando Amado, Amanda Antunes, Elisângela Silva, Leila Telles, Giovana Almeida, Aluísio Augusto Belmino Gadelha, Flavia de Azevedo Belesa, Acimar Gonçalves da Cunha, Jr, Beatriz Souza Barros, Josiane Bernartt Zanellato, Patricia Guimarães, Karina Ilheu da Silva, Bianca Ribas, Cristina Reuter, Francis Tanise Casado, Mila Torii Correa Leite, Daniela Testoni, Ruth Guinsburg, Simone de Campos Vieira Abib, Edson Khodor Cury, Suely Dornellas do Nascimento, Arthur Almeida Aguiar, Rodrigo Melo Gallindo, Carolina Gonçalves Borges, Yang Liu, Cai Duote, Jinhu Wang, Zhigang Gao, Liang Liang, Wenjuan Luo, Xiaoxia Zhao, Rui Chen, Peng Wang, Yijiang Han, Ting Huang, Hu Donglai, Guo Xiaodong, Chen Junjie, Libin Zhu, Guowei Wu, Xiaozhou Bao, Haijing Li, Junying Lv, Zhongrong Li, Feng Yong, Zhou Chong Gao, Qiang Bai, Weibing Tang, Hua Xie, Jethishka Motee, Jianming Zhu, Gang Wen, Weiwei Ruan, Shungen Li, Lulu Chen, Shungen Huang, Zhibao Lv, Jinjing Lu, Liuming Huang, Mengnan Yu, Wang Dajia, Yu Zuo Bai, Luis Carlos Rincon, Juliana Mancera, Edgar Alzate Gallego, Laura Torres-Canchala, Nathalia Silva Beltrán, Ghordana Osorio Fory, Daniela Castaño Avila, Angelica Maria Forero Ladino, Juanita Gomez, Martha Jaramillo, Otto Morales, Beatriz Sanchez, Nestor Julien Tinoco Guzmán, Sergio Castañeda Espinosa, Osbaldo Prieto Vargas, Lina Maria Pardo, Eliana Toral, Freud Cáceres Aucatoma, Daniel Hinostroza, Santiago Valencia, Vicente Salinas, Enrique Landivar Cino, Gabriela Yulissa Ponce Fajardo, Miguel Astudillo, Virginia Garcia, Guillermo Muñoz, Leonardo Verduga, Ivan Verduga, Ericka Murillo, Elena Bucaram, Marisol Guayelema, Monica Marmol, Janina Sanchez, Carolina Vergara, Adriana Mena, Junior Velaña, Karla Salazar, Sandra Lara, Elena Chiriboga, Julian Silva, Dalia Gad, Doaa Samy, Menan Ahmed Elsadek, Hanan Mahmoud Mohammed, Mohamed Abouheba, Karim Osamy Ali, Hayssam Rashwan, Omar Moustafa Fawzy, Tarek mohamed Kamel, Rawan Nemer, Mohamed Abada Hassan, Eyad Hassan Falah, Dina Sobhy Abdelhady, Mostafa Zain, Eman Abouzeid Abouzeid Ibrahim, Omar Ossama Elsiraffy, Ahmed Aboelela, Eman mohamed Farag, Ahmed Mohamed Oshiba, Omar Sameh Emam, Alaa Mobarak Attia, Moustafa A. Laymouna, Islam Abdelmonem Ghorab, Mansour Mkayed Mohammed, Nourhan Akram Soliman, Khaled Abd elrahman Ghaly, Kareem Sadek, Mohamed Elsherbiny, Amr Saleh, Hesham Sheir, Tamer Wafa, Mohamed Abd Elmenam, Sherif Abdelmaksoud, Ahmed Reda, Islam Mansour, Mohamed Elzohiri, Basma Waseem, Mohamed Elewaily, Mohammed El-Ghazaly, Ahmad Elhattab, Amr Shalaby, Adham Elsaied, Ahmad Adawy, Mirna Sadek, Mahmoud Abdelfattah Ahmed, Mohamed Omar Herdan, Gena Mohamed Hamed Elassall, Azhar Arabi Mohammed, Mohammed Hamada Takrouney, Tarek Mohamed Essa, Ahmed Mokhtar Mahmoud, Alshaimaa M. Saad, Mariam Albatoul Nageh Fouly, Mahmoud abdelshakour Ibrahim, Mohammad Nageh, Mahmoud M. Saad, Helmy Badr, Mohamed Fayez Fouda, Ahmed Hassan Nofal, Hisham Almohamady, Mohamed Ahmed Arafa, Mohamed Amad, Mohamed Awad Mansour, Jennifer O'Connor, Zachary O'Connor, Nzanzu Anatole, Elysé Nkunzimana, Solomon Machemedze, Lemfuka Dieudonné, William Appeadu-Mensah, Theophilus Teddy Kojo Anyomih, Priscilla Alhassan, Francis A. Abantanga, Vishal Michael, Roshine Mary Koshy, Ankit Raj, Vijay Kumar, Sundeep PT, P Santosh Prabhu, Armin Vosoughi, Ali Farooq Al-Mayoof, Muhamed Jassim Fadhle, Ali Egab Joda, Hayder Nadhim Obaid Algabri, Sultan S. Abdelhamid, Hashem M. Al-Momani, Marzouq Amarin, Louay Y. Zaghlol, Nijmeh Nasser Alsaadi, Yasmeen Z. Qwaider, Hibah Qutishat, Ahmad Hasan Aliwisat, Esraa Arabiat, Isam Bsisu, Raghad M. Murshidi, Mohammad S. Jabaiti, Ziad A. Bataineh, Husam Aldean Abuhayyeh, Thekraiat M. Al Quran, Faris J. Abu Za'nouneh, Mohanad Mutasem Alebbini, Hamzah Abullah Qudah, Omar Ghazi Hussein, Amir M.I. Murad, Justin Z. Amarin, Haya H. Suradi, Sayel H. Alzraikat, Rand Y. Omari, Bashar M. Matour, Layana Al-Halbouni, Rajai O. Zurikat, Ahmad H. Yanis, Sara Al Hussein, Ali Shoubaki, Waleed H. Ghanem, Kuria David, Soita Wycliffe Chitiavi, Moraa Mose, Robert Mugo, James Ndungu, Timothy Mwai, Swaleh Shahbal, Janan Malik, Nirav Chauhan, Francisa Syovata, Kevin Ochieng, Polycarp Omendo Liyenzero, Syeda Ra'ana Hussain, Stanley Mugambi, Roseline Ochieng, Ebtesam Othman Abdulsalam Elkhazmi, Ala Khaled, Aya Albozidi, Manal Ben Enbaya, Mala Elgammudi, Enas Soula, Wegden ibrahim almabrouk Khalel, Yasmine Ali Elhajjaji, Nouriyah Ali Alwaggaa, Sumayyah Ghayth, Dafer abdulhakim .S. Zreeg, Sara Abobaker Tantush, Fatma Bibas, Tesneem Layas, Randa Alamen M Sharif, Wesal Omar F. Saied Aljadidi, Ahmed Tarek, Hazem Ahmed, Kamila Almabrouk Mohammed Essamilghi, Mabroka Alfoghi, Ma'aly A. Abuhlega, Saddam Arrmali, Fatima Mousa Abduljawad, Hasan Mustafa Alosta, Abdulsalam Abuajaila, Fakereldeen Abdelmutalib, Fatma Bashir, Inas Almengar, Mohammad hasan Annajjar, Abdelaziz Deyab, Fathi Elzowawi, Yousef Krayem, Weam Drah, Asma Meftah, Abobaker Mohammed, Lina Ali Arrmalli, Hajir Aljaboo, Abdallah Elayeb, Mohamed Altomi, Ahmed Altaweel, Mohamed Tumi, Hana Milad Bazozi, Aisha Shaklawoon, Mohammed Meftah Alglaib, Abdullahn Abdousalam Elkaloush, Sara Trainba, Hisham Swessi, Ali Alnaeri, Aya Essam Shnishah, Hamassat Mustufa, Sondas Ali Gargum, Sara Ali Tarniba, Hawa Ahmed Shalluf, Hajer Ali Shokri, Taher L. Sarkaz, Osama Tababa, Ahmed Elhadi, Vesna Cvetanovska Naunova, Laze Jovcheski, Marjan Kamilovski, Aleksandra Gavrilovska-Brzanov, Zarina Abdul Latiff, Siti Farhan Moh Pauzi, Marjmin Osman, Felicia Lim, Ainal Huda Abu Bakar, Azrina SK Zaman, Shareena Ishak, Rufinah Teo, Dr. Tammy Teoh Han Qi, Mohd Yusran Bin Othman, Dato' Dr Zakaria bin Zahari, Zulfitri bin Md Hassan, Cheah Hui Shan, Abhirrami Lechmiannandan, Hafatin Fairos bt Tamaddun, Mohd Fitri Shukri bin Mohamed Adanan, Mohd Yusof bin Abdullah, Wang Junyi, Mohd. Tarmizi Mohd Nor, Wan Ruzaimie Noor, Mohd Razin bin Hassan, Noor Fa'izatul Rahil Ambok Dalek, Hidayah Hayati binti Hashim, Ahmad Zulhisyam bin Zarwawi, V Muthualhagi M Vellusamy, Quah Soong Yuen, Hemasutha a/p Kannessan, Najua binti Ramli, Ahmad Shafiee bin Bujarimin, Jessmine Anntinea, Anthony Dass, Hazlina Mohd. Khalid, Nur Atiqah binti Mohd Hanifah, Keily Wong Yue Jyun, Rahilah binti Abd Razak, Nur Atifah binti Mohd Naim, Siti Nur Aien binti Hamid Hamzah, Cristian R. Zalles Vidal, Eduardo Bracho Blanchet, Roberto Dávila Perez, Emilio Fernandez Portilla, Raúl Villegas Silva, Daniel Ibarra, Antonio Calderon Moore, Cesar Carrasco-Ortega, Monica Noguez Castillo, Dorihela Herappe Mellado, Guillermo Yanowsky Reyes, Luis Fernando Gonzalez Cortez, Rafael Santana Ortiz, Jamie Orozco Perez, Jorge Román Corona C.Rivera, Juan Jose Cardenas Ruiz Velasco, Moises Quiles Corona, Christian Peña Padilla, Lucina Bobadilla Morales, Alfredo Corona Rivera, Izabel Maryalexandra Rios Flores, Cristian Irela Aranda Sánchez, Gabriela Ambriz-González, Nestor Martínez Hernández Magro, Francisco Javier León Frutos, José de Jesús Cárdenas Barón, Alejandro González Ojeda, Jessica Yarza Fernández, Juan Domingo Porras, Pastor Aguirre-Lopez, Vicente Sánchez Paredes, Arturo Montalvo Marin, Jose Manuel Diaz Gomez, Lorenzo Juvencio Caamal, David Bulnes Mendizabal, Pablo Sanchez Valladares, Humberto Garcia Martinez, Opeoluwa Adesanya, Moses Olanrewaju, Rilwan Adegboyega, Nurudeen Abdulraheem, Anuoluwapo Aremo, Florence Dedeke, Anyanwu Lofty-John Chukwuemeka, Mohammad Aminu Mohammad, Abdullahi Lawalbarau, Nwokoro Collins, Ogundele Ibukunolu, Amo Shonubi, Oluwaseun Ladipo-Ajayi, Olumide Abiodun Elebute, Justina Seyi-Olajide, Felix Alakaloko, George Ihediwa, Kayode Olayade, Christopher Bode, Olakayode Ogundoyin, Dare I. Olulana, Ifeanyichukwu Kelvin Egbuchulem, Felix O. Kumolalo, Ikechukwu Ulasi, Uchechukwu Obiora Ezomike, Sebastian Okwuchukwu Ekenze, Elochukwu Perpetua Nwankwo, Emmanuel Ifeanyi Nwangwu, Isaac Chukwu, Christopher Chim Amah, Nene Elsie Obianyo, Omolara Williams, Roland Iheanyichukwu Osuoji, Omolara Moronkeji Faboya, Olalekan Temitope Ajai, Moruf Adekunle Abdulsalam, Titiloye Hannah Agboola, Bolarinwa Bolanle Temilade, Maryrose Osazuwa, Morayo Monsurat Salawu, Eze Chukwuemeka Ejinkeonye, Mariya Mukhtar Yola, Amsa B. Mairami, Adekunle T. Otuneye, Matthias Igoche, Adebayo Gbenga Tanimola, Emmanuel Akinlabi Ajao, Efeturi Agelebe, Samson Olori, Philip Mari Mshelbwala, Olabisi Osagie, Adewale Oyinloye, Auwal M Abubakar, Lateef Oyebanji, Ibrahim Shehu, Cyril Cletus, Ahmed Bamanga, Faruk Suleiman, Sani Adamu, David C.Nwosu, Yahya S.Alkali, Iliya Jalo, Aliu Rasaki, Yusuf T.Sambo, Kalakwa A.Mohammed, Abubakar M.Ballah, Victor Modekwe, Okechukwu Hyginus Ekwunife, Ugochukwu S Ezidiegwu, Andrew N Osuigwe, Jideofor O Ugwu, Chuka A Ugwunne, Nadeem Akhter, Mudassir Fayaz Gondal, Rafee Raza, Ali Raza Chaudary, Hassan Ali, Muhammad Umar Nisar, Muhammad Umer Jamal, Ghuri Shankar Pandit, Uzma Mumtaz, Muhammad Bin Amjad, Nabila Talat, Wajeeh ur Rehman, Muhammad Saleem, Muhammad Bilal Mirza, Imran Hashim, Naveed Haider, Soban Hameed, Ayesha Saleem, Sohail Dogar, Muhammad Sharif, Muhammad Kashif Bashir, Fatima Naumeri, Zarqa Rani, Muath A.M. Baniowda, Basheer Ba'baa', Majd Yousef Mohammed Hassan, Ammar Darwish, Abrar Shaheen Sehwiel, Mohammed Shehada, Abrar ghassan Balousha, Yara Ajrami, Ainaa Ata Mohammad Alzamari, Bashar Yaghi, Hasan Subhi Hasan Abu Al-saleem, Mervat Sufian Abu Farha, Mohammad Omar Mohammad Abdelhafez, Firas Anaya, Asef belal Qadomi, Abd Al-Naser Bany Odi, Muath Abdelrahem Fuad Assi, Fadwa Sharabati, Ahmad Abueideh, Doha mustafa saleh Beshtawi, Hasan Arafat, Lara Zahi Adel Khatatba, Safa' Jamal Abatli, Hiba Al-Tammam, Dania Jaber, Yara Imad Omar Kayed, Ali Abdelhay Abumunshar, Rami Anwar Misk, Asmahan Mohammad Suliman Alzeer, Mutassem Sharabati, Ihsan Ghazzawi, Osama Majed Darras, Mahmoud M.Qabaja, Ma'alem sameer Hajajreh, Yasmeen Ahmad Samarah, Dua Hasan Yaghi, Moradallah Asad Fahmi Qunaibi, Abdelrazzaq Abu Mayaleh, Sharehan Joubeh, Annan Ebeido, Samer Adawi, Ihda Adawi, Mohammad Omar Ibrahim Alqor, Ahmad Samih Arar, Hadeel Awad, Fawzi Abu-Nejmah, Osaid Shaher Shabana, Firas Alqarajeh, Tareq Z. Alzughayyar, Jomana Madieh, Mahmoud Fuad Sbaih, Raghad mohammad abdu Alkareem, Raghad abdullateef Lahlooh, Yasmeen Adly Halabi, Wisam Baker, Tasneem Fathi Hasan Almusleh, Abdulraheem Adnan Abdulraheem Tahyneh, Yazid yousef mahmoud Atatri, Najlaa Abu Jamie, Nasrallah Ashraf Al Massry, Walaa Lubbad, Ayoub A.Nemer, Mohammed Alser, Aya Azmi Shehda Salha, Khaled Alnahhal, Aya Mahmoud Elmzyyen, Amir Talat Sheda Ghabayen, Abdulwhhab Ayman Abu Alamrain, Samar H. Al-Shwaikh, Omar Adly Elshaer, Nureddin Shaheen, Jehad Fares, Hisham Dalloul, Anas Qawwash, Mustafa abu Jayyab, Dina Ayman Ashour, Ahmad Ashraf Shaheen, Samy Rafat Ramadan Naim, Eman Abu Shiha, Nagham Mohammed Al Dammagh, Walaa Almadhoun, Ashraf Ayman Al-Salhi, Abdalkarim Yhya Hammato, Jamal Mohammed Salim, Doaa Khalil Hasanain, Soha Marwan Salem Alwadia, Ismail Nassar, Hala M. Al-Attar, Haya Abdulnasser Ali Alshaikhkhalil, Yasmin Mohammed Khalil Abu Jamie, Yara shareef Ashour, Sharif S. Alijla, Mohamed Anwer El Tallaa, Adham Ashraf Abuattaya, Bisan D.M. Wishah, MOHAMMED A.M. ALDIRAWI, Ahmed S Darwish, Sulaiman T. Alzerei, Nidal Wishah, Sharif Alijla, Isidora Garcia, Marlene Diaz Echegaray, Veronica Raquel Cañapataña Sahuanay, Fernando Trigoso Mori, Jackelyne Alvarado Zelada, Juan Jose Salinas Barreto, Porfirio Rivera Altamirano, Cesar Torres Miranda, Rocio Anicama Elias, Julio Rivera Alvarez, Juan Pedro Vasquez Matos, Fernando Ayque Rosas, Jesmarina Ledesma Peraza, Andrea Gutarra Palomino, Stephany Vega Centen, Victor Casquero, María Rosa Ortiz Argomedo, Francisco Lapouble, Genaro Llap Unchón, Florangel Patricia Delgado Malaga, Luis Ortega Sotelo, Segundo Gamboa Kcomt, Araceli Villalba Villalba, Nancy Rossana Mendoza Leon, Loreley Raquel Cardenas Alva, Maria Susana Loo Neyra, Cathy Lee Alanguia Chipana, Cintya Maria de Jesus Torres Picón, Natalia Huaytalla Quiroz, Danny Dominguez, Carlos Segura Calle, Jenny Arauco, Luis Ormeño Calderón, Ximena Ghilardi Silva, Miriam Daniela Fernandez Wilson, Joan Elizabeth Gutierrez Maldonado, Cesar Diaz Leon, Waldo Berrocal Anaya, Patricia Chavez Galvez, Prince Pamela Aguilar Gargurevich, Flor de Maria Diaz Castañeda, Carmen Guisse, Erika Ramos Paredes, Jose Luis Apaza Leon, Faye Aguilar Aguilar, Raul Ramirez De La Cruz, Lenny Flores Carbajal, Carlos Mendoza Chiroque, Gladys Johana Sulca Cruzado, Natalia Tovar Gutierrez, Jennifer Sotelo Sanchez, Carolina Paz Soldan, Karina Hernández Córdova, Edgar Fernando Delgado Quinteros, Luz Mery Brito Quevedo, Juan Jose Mendoza Oviedo, Angel Samanez Obeso, Patricia Paredes Espinoza, Johann de Guzman, Raisa Yu, Vlad Cosoreanu, Sebastian Ionescu, Aurel Mironescu, Lucian Vida, Adrian Papa, Roxana Verdeata, Bogdan Gavrila, Liviu Muntean, Marija Lukac, Miona Stojanovic, Djordje Toplicic, Milan Slavkovic, Andjelka Slavkovi, Dragoljub Zivanovic, Ana Kostic, Maja Raicevic, Delphine Nkuliza, Daniel Sidler, Corné de Vos, Elmarie vd Merwe, David Tasker, Omar Khamag, Cecilia Rengura, Thozama Siyotula, Uzair Jooma, Dirk von Delft, Marion Arnold, Hansraj Mangray, Shamaman Harilal, Sanele Madziba, Naveen Wijekoon, Tharanga Gamage, Benedict Paul Bright, Alaa Abdulrahman, Ola Ahmed Abdulmjeed Mohammed, Mohammed Salah, Ahmad Elian Abu Ajwa, Mohammed Morjan, Mohammad Mohannad Batal, Vivian Faks, Mohamad Bassel Mouti, Ahmadfateh Assi, Ahmad Al-Mouakeh, Ahmad Sankari Tarabishi, Ziad Aljarad, Aos Alhamid, Jiraporn Khorana, Wannisa Poocharoen, Sirima Liukitithara, Anan Sriniworn, Wasun Nuntasunti, Monawat Ngerncham, Ratiyaporn Phannua, Kanokrat Thaiwatcharamas, Patchareeporn Tanming, Lassaad Sahnoun, Nahla Kchiche, Roua Abdelmoumen, Egemen Eroğlu, Mehmet Ali Ozen, Hatice Sonay Yalçın Cömert, Mustafa İmamoğlu, Haluk Sarıhan, Şebnem Kader, Mehmet Mutlu, Yakup Aslan, Ahmet Beşir, Şükran Geze, Bahanur Çekiç, Ali Yalcinkaya, Kaan Sönmez, Ramazan Karabulut, Zafer Türkyılmaz, Kıvanç Şeref, Merve Altın, Merve Aykut, M.Eren Akan, Melisa Erdem, Ebru Ergenekon, Canan Türkyılmaz, Elif Keleş, Ali Canözer, Aslı Öztürk Yeniay, Elif Eren, İlknur Banlı Cesur, Zerrin Özçelik, Gökmen Kurt, Mustafa Kurthan Mert, Hatice Kaya, Müge Çelik, Suleyman Cuneyt Karakus, Nazile Erturk, Alev Suzen, Nilay Hakan, Fatih Akova, Mehmet Pasaoglu, Shukurali Eshkabilov, Rustam Z. Yuldashev, Dekhkonboev Avazjon Abdunomonovich, Aliev Makhmudjan Muslimovich, Azad Patel, Chisengo Kapihya, Nicholas Ensar, Ramesh M Nataraja, Mithila Sivasubramaniam, Matthew Jones, Warwick Teague, Sharman Tan Tanny, Gordon Thomas, Kiera Roberts, Soundappan Sannappa Venkatraman, Holger Till, Manon Pigeolet, Martine Dassonville, Anas Shikha, Win Sabai Phyu Win, Zahidah Adlynee Haji Ahmad, Léamarie Meloche-Dumas, Louise Caouette-Laberge, Dickens St-Vil, Ann Aspirot, Nelson Piché, Shahrzad Joharifard, Nadia Safa, Jean-Martin Laberge, Sherif Emil, Pramod Puligandla, Kenneth Shaw, Hussein Wissanji, Eileen Duggan, Elena Guadagno, Maria Consuelo Puentes, Paola Osses Leal, Carolina Mendez Benavente, Michal Rygl, Barbora Trojanová, Klára Berková, Tereza Racková, Ladislav Planka, Jan Škvařil, Radek Štichhauer, Shahad Sabti, Alex Macdonald, Nordeen Bouhadiba, Dorothy Kufeji, Caroline Pardy, Simon Mccluney, Alireza Keshtgar, Rebecca Roberts, Hannah Rhodes, Kate Burns, Robin Garrett-Cox, Kat Ford, Hannah Cornwall, Krithi Ravi, Felicity Arthur, Paul Losty, Tony Lander, Ingo Jester, Suren Arul, Oliver Gee, Giampiero Soccorso, Michael Singh, Max Pachl, Benjamin Martin, Afnan Alzubair, Arun Kelay, Jonathan Sutcliffe, Thomas Middleton, Amy Hughes Thomas, Merina Kurian, Fraser Cameron, Jayaram Sivaraj, Mark C Thomas, Dean Rex, Ceri Jones, Kate Bradshaw, Arnaud Bonnard, Xavier Delforge, Camille Duchesne, Caroline Le Gall, Coralie Defert, Samia Laraqui Hossini, Florent Guerin, Géraldine Hery, Virginie Fouquet-Languillat, Jules Kohaut, Aline Broch, Thomas Blanc, Luke Harper, Thomas Delefortrie, Quentin Ballouhey, Laurent Fourcade, Céline Grosos, Benoit Parmentier, Guillaume Levard, Maria Giovanna Grella, Mariette Renaux Petel, Lucie Grynberg, Olivier Abbo, Sofia Mouttalib, Mélodie Juricic, Aurelien Scalabre, Elodie Haraux, Anke Rissmann, Hardy Krause, Peter Goebel, Ludwig Patzer, Udo Rolle, Andrea Schmedding, Alexandra Antunez-Mora, Bernd Tillig, Sylvester von Bismarck, Patricia Reis Barbosa, Christian Knorr, Domitille Stark, Marco Brunero, Luigi Avolio, Francesco Manni, Matilde Molinelli, Marinella Guazzotti, Alessandro Raffaele, Piero Giovanni Romano, Silvia Cavaiuolo, Gian Battista Parigi, Laszlo Juhasz, Anna Rieth, Arunas Strumila, Rūta Dagilytė, Arunas Liubsys, Pranas Gurskas, Dalius Malcius, Agne Mikneviciute, Asta Vinskaite, Vidmantas Barauskas, Liam Vierboom, Timothy Hall, Spencer Beasley, Lucy Goddard, Mark Stringer, Naveen Weeratunga, Stephen Adams, Jitoko Cama, Marilyn Wong, Sridharan Jayaratnam, Askar Kukkady, Udaya Samarakkody, Sylwester Gerus, Dariusz Patkowski, Agnieszka Wolny, Tomasz Koszutski, Szymon Tobor, Marta Osowicka, Piotr Czauderna, Dariusz Wyrzykowski, Hanna Garnier, Stefan Anzelewicz, Osowicka Marta, Agata Knurowska, Alicja Weiszewsk, Andrzej Grabowski, Wojciech Korlacki, Michal Pasierbek, Przemyslaw Wolak, Aneta Piotrowska, Anna Roszkiewicz, Piotr Kalicińsk, Agata Trypens, Grzegorz Kowalewsk, David Sigalet, Amer Alsaied, Mansour Ali, Ameen Alsaggaf, Alaa Ghallab, Yazeed Owiwi, Ali Zeinelabdeen, Mohamed Fayez, Ahmed Atta, Mazen Zidan, Asaad saleh Radwan, Hanin Shalaby, Reem Abdelbaqi, Khalid Alattas, Yar Kano, Omar Sindi, Abdullah Alshehri, Tariq Altokhais, Fahad Alturki, Mohammad Almosaibli, Dasha Krisanova, Wisam Abbas, Hee-Beom Yang, Hyun-Young Kim, Joong Kee Youn, Jae Hee Chung, Seok Hyeon Cho, In ji Hwang, Ju yeon Lee, Eung song Song, Jenny Arboleda, Mercedes Ruiz de Temiño Bravo, Alexander Siles Hinojosa, Miriam García, Isabel Casal Beloy, Detlef Oliu San Miguel, Maria Elena Molina Vazquez, Verónica Alonso, Alberto Sanchez, Oscar Gomez, Isabel Carrillo, Tomas Wester, Carmen Mesas Burgos, Martin Salö, Erik Omling, Niclas Rudolfson, Christina Granéli, Helena Arnadóttir, Emma Grottling, Kate Abrahamsson, Vladimir Gatzinsky, Michaela Dellenmark Blom, Daniel Borbonet, Paul Puglia, Vinicio Jimenez Morejon, Gaston Acuna, Mario Moraes, Jonathan Chan, Pavan Brahmamdam, Alan Tom, Karen Sherer, Brandy Gonzales, Aaron Cunningham, Sanjay Krishnaswami, Reto Baertschiger, Mary Leech, Regan Williams, Lauren Camp, Ankush Gosain, Maria Mora, Bailey D. Lyttle, Jeremy Chang, Lydia McColl Makepeace, Kathryn L Fowler, Sara Mansfield, Erica Hodgman, Chukwubinyelum Amaechi, Alana Beres, Mark N. Pernik, Luke J. Dosselman, Murad Almasri, Sunil Jain, Varun Modi, Marianelly Fernandez Ferrer, John Coon, Joann Gonzalez, Medhavi Honhar, Nensi Ruzgar, Griffin Coghill, Sarah Ullrich, Maija Cheung, Katrine Løfberg, Jodie Greenberg, Kate Davenport, Samir Gadepalli, Sarah Fox, Stephanie Johnson, Mercedes Pilkington, April Hamilton, Nicole Lin, Juan Sola, Yang Yao, Jenna Kylene Davis, Monica Langer, Jonathan Vacek, Fizan Abdullah, Julie Khlevner, William Middlesworth, Marc Levitt, Hira Ahmad, Sabina M Siddiqui, Alex Bowder, Terry Derks, Afua Amoabin Amoabin, Brooke Pinar, Frank Owusu-Sekyere, Benmanseur Saousen, Rasika Naidoo, Azra Karamustafic, Danielle Paula de Oliveira, Jerhy Andrade, Antonín Šafus, Jason Langley, Alexandra Wilke, Corazone Deya, Habib Mansour Murtadi, Mindaugas Berzanskis, Nwachukwu Calistus, Olalekan S. Ajiboye, Michael Felix, Osagie O Olabisi, Seçil Erçin, Teymursha Muradi, Stephen S. Burks, Sergio Lerma, Jillian Jacobson, Calin Calancea, Rafael Valerio-Vazquez, Guigui Sikwete, Owusu Sekyere, Akhona Mbonisweni, Shahnoor Syed, Cho Seok Hyeon, Fatemeh Pajouhandeh, Sheba Mary Pognaa Kunfah, Global PaedSurg Research Collaboration, and Tıp Fakültesi
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Male ,Pediatrics ,medicine.medical_specialty ,Adolescent ,Gastrointestinal Diseases ,Psychological intervention ,Disease ,Global Health ,Specialties, Surgical ,Congenital Abnormalities ,Cohort Studies ,medicine ,Humans ,Prospective Studies ,Prospective cohort study ,Child ,Developing Countries ,business.industry ,Gastroschisis ,Developed Countries ,Intestinal atresia ,Infant, Newborn ,Gestational age ,Congenital diaphragmatic hernia ,Infant ,Articles ,General Medicine ,medicine.disease ,Gastrointestinal Tract ,Atresia ,Child, Preschool ,Female ,business - Abstract
Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung's disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung's disease) from 264 hospitals (89 in high-income countries, 166 in middle-income countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p
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- 2020
33. Does massive bowel resection in newborns affect further immunity in children ?
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Agnieszka Jankowska, Maciej Zieliński, Katarzyna Sznurkowska, Magdalena Malanowska, Piotr Trzonkowski, Agnieszka Szlagatys Sidorkiewicz, Maciej Zagierski, Anna Borkowska, Marcin Łosin, and Piotr Czauderna
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medicine.medical_specialty ,business.industry ,Immunity ,Internal medicine ,medicine.medical_treatment ,medicine ,Bowel resection ,Affect (psychology) ,business ,Gastroenterology - Abstract
Background Short bowel syndrome (SBS) is defined as the a malabsorptive condition most often caused by massive resection of the small intestine. In children most cases of SBS originate in the newborn period and result from congenital anomalies or necrotizing enterocolitis. Loss of gut mucosa during resection does not only mean loss of absorption surface, but also deprives organism of many immunocompetent cells concentrated in gut associated lymphoid tissue, which is regarded the largest immune organ in humans. Aim of the study: We have aimed to access the influence of bowel resection on adaptive immunity in children, basing on peripheral lymphocyte populations and serum immunoglobulins. Patients and methods: 18 children, who underwent bowel resection in the first month of life and required further home parenteral nutrition were enrolled into the study. 12 healthy children, constituted control group. Based on flow cytometry the following subpopulations of lymphocytes were evaluated: T, B, NK, CD4+, C8 + and activated T cells. Serum immunoglobulins were determined with the use of immunoturbidimetric method. Results The percentage of B lymphocytes was reduced, while the rates of lymphocytes T and CD8 + lymphocytes were higher compared to healthy children. We documented significantly lower absolute count and proportion of NK cells in SBS group than in the control group. Absolute counts of lymphocytes, lymphocytes B, T, CD4 + and percentages of lymphocytes CD4+, and activated T cells inversely correlated with the time after resection. No statistically significant differences were found between the levels of IgA, IgM and IgG in the studied and the control group Conclusions Children with SBS do not present with clinical signs of immunodeficiency as well as deficits in peripheral lymphocyte populations and serum immunoglobulins. Lower number of NK cells in SBS patients compared to healthy children needs to be verified in larger cohort. The tendency of the lymphocyte subpopulations to decrease over time after resection points out the necessity for longer follow- up.
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- 2020
34. Arterial constriction after resection of neuroblastic tumors in children: Two-center retrospective study
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Piotr Czauderna, Przemysław Mańkowski, Ewelina Gowin, Danuta Januszkiewicz-Lewandowska, Patrycja Sosnowska-Sienkiewicz, and Marcin Łosin
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medicine.medical_specialty ,medicine.medical_treatment ,Constriction ,03 medical and health sciences ,Neuroblastoma ,0302 clinical medicine ,030225 pediatrics ,Medicine ,Humans ,Child ,Paresis ,Retrospective Studies ,Chemotherapy ,business.industry ,Medical record ,Infant, Newborn ,Infant ,Vasospasm ,Retrospective cohort study ,General Medicine ,medicine.disease ,Neuroblastic Tumor ,Surgery ,Infarction ,030220 oncology & carcinogenesis ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,medicine.symptom ,business ,Complication - Abstract
Background/purpose Neuroblastic tumors are the most common pediatric extracranial solid tumors in infants and very young children. Although, especially in newborns, there is an increasing number of situations in which observation alone is used, surgery remains an important step in the treatment of neuroblastoma but can be complicated by arterial vasospasm in the surgical field. The aim of this two-center retrospective study was to analyze the occurrence of arterial constriction as a complication of neuroblastic tumors resection. Methods Medical records of patients who were treated surgically for neuroblastic tumors in the years 2012–2019 were reviewed. Results For 8 years, 113 children were treated for neuroblastic tumors. The treatment included both primary and delayed surgery after initial chemotherapy carried out according to SIOPEN protocols. In 11 out of 113 cases (9.7%) local arterial constriction was observed. In 6 out of 11 cases an attempt was made to save the organ, however, two patients suffered from kidney atrophy, two other partial kidney infarctions, one child suffered from partial limb paresis, and another from brain ischemia and death. Conclusions Local arterial constriction constitutes a relatively common (10% of cases) and severe complication of neuroblastic tumors resection. Efficacy of local application of papaverine based on our experience remains unproven hence further research is warranted.
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- 2020
35. Epigenetic footprint enables molecular risk stratification of hepatoblastoma with clinical implications
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Cristina Beléndez, Viera Bajčiová, Nicholas K. Akers, Aroa Soriano, David Piñeyro, Manuel López Santamaría, Michael A. Grotzer, Carolina Armengol, José Antonio Salinas, Lara Nonell, Mar Mallo, Jordi Abril-Fornaguera, Bruce Morland, Roland Kappler, Monique Fabre, Josep M. Llovet, Ramon Planas, Helena Masnou, Piotr Czauderna, María Elena Mateos, Constantino Sábado, Genevieve Laureys, Catherine Guettier, Ricardo López-Almaraz, Claudia Paris, Maria Rosa Sarrias, Montserrat Domingo-Sàbat, Yasmina Mozo, Olga Kuchuk, Marta Garrido, José Javier Uriz, Laura Torrens, Stefano Cairo, Julià Blanco, Gabriela Guillén, Blanca López-Ibor, Sophie Branchereau, Francisco Andrés Pérez Hernández, Daniela Sia, Bojan Losic, Bárbara Torres, Magdalena Arnal, Laura Guerra, Margarita Sala, Laura Royo, Maria Vázquez-Vitali, Gema Ramírez, Núria Villalmanzo, Alberto Villanueva, Ariadna Clos, Mireia Jordà, Nagore García de Andoin, Marina Simon-Coma, Marie Annick Buendia, Juan Carrillo-Reixach, Lauro Sumoy, and Sonia Ragull
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Hepatoblastoma ,Male ,0301 basic medicine ,RNA editing ,Molecular risk stratification ,Epigenesis, Genetic ,Transcriptome ,0302 clinical medicine ,Drug Discovery ,Choline Kinase ,beta Catenin ,Epigenomics ,Hepatoblastoma (HB) ,Liver Neoplasms ,Prognosis ,Phenotype ,Neoplasm Proteins ,3. Good health ,Female ,030211 gastroenterology & hepatology ,Antioncogenes ,Liver cancer ,Prognostic biomarker ,Choline kinase alpha ,BLCAP ,Risk Assessment ,Càncer de fetge ,03 medical and health sciences ,Biomarkers, Tumor ,medicine ,Humans ,Epigenetics ,14q32 ,Hepatology ,business.industry ,Gene Expression Profiling ,Calcium-Binding Proteins ,Infant ,Membrane Proteins ,DNA Methylation ,medicine.disease ,Precision medicine ,Antioncogens ,High-Throughput Screening Assays ,030104 developmental biology ,DLK1-DIO3 locus ,CHKA ,Cancer research ,business - Abstract
Background & Aims: Hepatoblastoma (HB) is a rare disease. Nevertheless, it is the predominant pediatric liver cancer, with limited therapeutic options for patients with aggressive tumors. Herein, we aimed to uncover the mechanisms of HB pathobiology and to identify new biomarkers and therapeutic targets in a move towards precision medicine for patients with advanced HB. Methods: We performed a comprehensive genomic, transcriptomic and epigenomic characterization of 159 clinically annotated samples from 113 patients with HB, using high-throughput technologies. Results: We discovered a widespread epigenetic footprint of HB that includes hyperediting of the tumor suppressor BLCAP concomitant with a genome-wide dysregulation of RNA editing and the overexpression of mainly non-coding genes of the oncogenic 14q32 DLK1-DIO3 locus. By unsupervised analysis, we identified 2 epigenomic clusters (Epi-CA, Epi-CB) with distinct degrees of DNA hypomethylation and CpG island hypermethylation that are associated with the C1/C2/C2B transcriptomic subtypes. Based on these findings, we defined the first molecular risk stratification of HB (MRS-HB), which encompasses 3 main prognostic categories and improves the current clinical risk stratification approach. The MRS-3 category (28%), defined by strong 14q32 locus expression and Epi-CB methylation features, was characterized by CTNNB1 and NFE2L2 mutations, a progenitor-like phenotype and clinical aggressiveness. Finally, we identified choline kinase alpha as a promising therapeutic target for intermediate and high-risk HBs, as its inhibition in HB cell lines and patient-derived xenografts strongly abrogated tumor growth. Conclusions: These findings provide a detailed insight into the molecular features of HB and could be used to improve current clinical stratification approaches and to develop treatments for patients with HB. Lay summary: Hepatoblastoma is a rare childhood liver cancer that has been understudied. We have used cutting-edge technologies to expand our molecular knowledge of this cancer. Our biological findings can be used to improve clinical management and pave the way for the development of novel therapies for this cancer. (c) 2020 European Association for the Study of the Liver. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)., This article was possible thanks to the inputs from the Instituto de Salud Carlos III, ISCIII (PI09/00751, PI10/02082, PI13/02340). The project has received funding from the European Union's Horizon 2020 research and innovation programme under grant agreement No 668596 (ChiLTERN) and grant agreement No 826121 (iPC). JCR is supported by the Catalan Agency for Management of University and Research Grants (AGAUR, 2019 FI_B01024). LT is supported by an Accelerator Award (CRUCK, AECC, AIRC) (HUNTER, C9380/A26813). DS is supported by the Gilead Research Scholar in Liver Disease. JML is supported by the European Union's Horizon 2020 research and innovation programme (HEPCAR, 667273-2), Institucio Catalana de Recerca i Estudis Avancats (ICREA), U.S. Department of Defense (CA150272P3), an Accelerator Award (CRUCK, AECC, AIRC) (HUNTER, C9380/A26813), National Cancer Institute, Tisch Cancer Institute (P30-CA196521), Samuel Waxman Cancer Research Foundation, Spanish National Health Institute (SAF2016-76390) and AGAUR (SGR-1358). CA and MRS were supported by Ramon y Cajal (RYC-2010-07249) and Miguel Servet (CPII14/00021) programs of the Ministry of Science and Innovation of Spain and ISCIII, respectively. CA, MRS and MS received funding from CIBERehd (CB06/04/0033) and AGAUR (2017-SGR-490). IGTP is a member of the CERCA network of institutes. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
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- 2020
36. Cultural, geographical and ethical questions when looking to enroll pediatric patients in rare disease clinical trials
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Piotr Czauderna, Stefan Anzelewicz, Hanna Garnier, and Arun Rangaswami
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medicine.medical_specialty ,Pathology ,business.industry ,Health Policy ,Public health ,Alternative medicine ,Context (language use) ,Clinical trial ,Orphan drug ,Patient recruitment ,03 medical and health sciences ,0302 clinical medicine ,Clinical research ,030225 pediatrics ,Family medicine ,medicine ,Pharmacology (medical) ,030212 general & internal medicine ,business ,Pharmacology, Toxicology and Pharmaceutics (miscellaneous) ,Rare disease - Abstract
Introduction: Rare diseases are becoming increasingly recognized worldwide as an important public health challenge and Orphan Drugs begin to play an important role in Research and Development. There are several major issues concerning conducting clinical trials in the field of Rare Diseases, especially in children, as 80% of those diseases are genetic in origin and involve, in most instances, children.Areas covered: This review concentrates on challenges associated with the patient recruitment process. The authors experience and opinion was complemented by a systematic search of relevant literature and includes ethical, cultural and geographical issues with rare diseases. The review presents current opinion and provides valuable support for planning and conducting clinical research in rare diseases.Expert opinion: The expert opinion section concentrates on importance of future rare disease clinical research in the context of understanding common diseases and developing new treatment options.
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- 2017
37. A unique defect - persistent posterior cloaca: An example of staged genito-urinary and digestive tract reconstruction with an alternative vaginal creation using the urinary bladder wall
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Andrzej Gołębiewski, Anna Bryks-Laszkowska, Piotr Czauderna, Mariusz Sroka, and Leszek Komasara
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medicine.medical_specialty ,Megaureter ,Urology ,medicine.medical_treatment ,Urinary Bladder ,030232 urology & nephrology ,Pubic symphysis ,Anastomosis ,03 medical and health sciences ,0302 clinical medicine ,Cloaca ,Colon, Sigmoid ,Ileum ,medicine ,Humans ,Abnormalities, Multiple ,urogenital system ,business.industry ,Infant, Newborn ,Colostomy ,Sigmoid colon ,Anatomy ,Plastic Surgery Procedures ,medicine.disease ,Surgery ,Neck of urinary bladder ,Treatment Outcome ,medicine.anatomical_structure ,Cloaca (embryology) ,Urogenital Abnormalities ,030220 oncology & carcinogenesis ,Vagina ,Female ,Ureter ,business - Abstract
We present a case of a girl with an extremely rare, posterior type of persistent cloaca, which was associated with other abnormalities, including an undeveloped vulva and vagina, agenesis of the right kidney, secondary obstructive megaureter, unicornate uterus, persisted tailgut, sacral bone hypoplasia, and pubic symphysis hypertrophy. An operative approach was as follows: (i) colostomy and ureterocutaneostomy; (ii) creation of an ileal conduit with antirefluxing uretero-ileal anastomosis, and then creation of a continent catheterizable ileal reservoir; (iii) anastomosis of sigmoid colon to rectal stump; and (iv) vaginal and external genital reconstruction. Because of abnormal anatomical conditions where the uterus was situated adjacent to the open, incompetent bladder neck, we decided to create a vagina using the bladder wall instead of the bowel segment.
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- 2017
38. Risk-stratified staging in paediatric hepatoblastoma: a unified analysis from the Children's Hepatic tumors International Collaboration
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Yukichi Tanaka, Rudolf Maibach, Mark Krailo, Giorgio Perilongo, Piotr Czauderna, Rebecka L. Meyers, Marisa Derosa, Dolores Lopez-Terrada, Ivo Leuschner, Tomoro Hishiki, Yurong Feng, Arun Rangaswami, Daniel C. Aronson, Marc Ansari, Irene Schmid, Kenichi Yoshimura, Dietrich von Schweinitz, Eiso Hiyama, Rita Alaggio, Beate Häberle, Davide Saraceno, Marcio H. Malogolowkin, Kenichiro Watanabe, Eugenia Rinaldi, University of Zurich, and Meyers, Rebecka L
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Hepatoblastoma ,Male ,0301 basic medicine ,Pediatrics ,Databases, Factual ,Disease ,law.invention ,0302 clinical medicine ,Japan ,Randomized controlled trial ,Risk Factors ,law ,Prospective Studies ,Cooperative Behavior ,Child ,Prospective cohort study ,Cancer ,Pediatric ,Liver Disease ,Liver Neoplasms ,Prognosis ,Combined Modality Therapy ,Survival Rate ,Oncology ,Child, Preschool ,Lymphatic Metastasis ,030220 oncology & carcinogenesis ,Pretext ,2730 Oncology ,Female ,alpha-Fetoproteins ,Risk ,medicine.medical_specialty ,Adolescent ,Oncology and Carcinogenesis ,MEDLINE ,610 Medicine & health ,Article ,Databases ,03 medical and health sciences ,medicine ,Humans ,Oncology & Carcinogenesis ,10220 Clinic for Surgery ,Preschool ,Survival rate ,Factual ,Neoplasm Staging ,business.industry ,Infant, Newborn ,Infant ,International Agencies ,Newborn ,medicine.disease ,Clinical trial ,030104 developmental biology ,Digestive Diseases ,business ,Follow-Up Studies - Abstract
Summary Background Comparative assessment of treatment results in paediatric hepatoblastoma trials has been hampered by small patient numbers and the use of multiple disparate staging systems by the four major trial groups. To address this challenge, we formed a global coalition, the Children's Hepatic tumors International Collaboration (CHIC), with the aim of creating a common approach to staging and risk stratification in this rare cancer. Methods The CHIC steering committee—consisting of leadership from the four major cooperative trial groups (the International Childhood Liver Tumours Strategy Group, Children's Oncology Group, the German Society for Paediatric Oncology and Haematology, and the Japanese Study Group for Paediatric Liver Tumours)—created a shared international database that includes comprehensive data from 1605 children treated in eight multicentre hepatoblastoma trials over 25 years. Diagnostic factors found to be most prognostic on initial analysis were PRETreatment EXTent of disease (PRETEXT) group; age younger than 3 years, 3–7 years, and 8 years or older; α fetoprotein (AFP) concentration of 100 ng/mL or lower and 101–1000 ng/mL; and the PRETEXT annotation factors metastatic disease (M), macrovascular involvement of all hepatic veins (V) or portal bifurcation (P), contiguous extrahepatic tumour (E), multifocal tumour (F), and spontaneous rupture (R). We defined five clinically relevant backbone groups on the basis of established prognostic factors: PRETEXT I/II, PRETEXT III, PRETEXT IV, metastatic disease, and AFP concentration of 100 ng/mL or lower at diagnosis. We then carried the additional factors into a hierarchical backwards elimination multivariable analysis and used the results to create a new international staging system. Results Within each backbone group, we identified constellations of factors that were most predictive of outcome in that group. The robustness of candidate models was then interrogated using the bootstrapping procedure. Using the clinically established PRETEXT groups I, II, III, and IV as our stems, we created risk stratification trees based on 5 year event-free survival and clinical applicability. We defined and adopted four risk groups: very low, low, intermediate, and high. Interpretation We have created a unified global approach to risk stratification in children with hepatoblastoma on the basis of rigorous statistical interrogation of what is, to the best of our knowledge, the largest dataset ever assembled for this rare paediatric tumour. This achievement provides the structural framework for further collaboration and prospective international cooperative study, such as the Paediatric Hepatic International Tumour Trial (PHITT). Funding European Network for Cancer Research in Children and Adolescents, funded through the Framework Program 7 of the European Commission (grant number 261474); Children's Oncology Group CureSearch grant contributed by the Hepatoblastoma Foundation; Practical Research for Innovative Cancer Control and Project Promoting Clinical Trials for Development of New Drugs and Medical Devices, Japan Agency for Medical Research; and Swiss Cancer Research grant.
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- 2017
39. Another point of view on 2017 PRETEXT: reply to Pariente et al
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Alexander J. Towbin, Eiso Hiyama, Osamu Miyazaki, Rebecka L. Meyers, Bruce Morland, Christopher B. Weldon, Derek J. Roebuck, Piotr Czauderna, Helen Woodley, and Greg Tiao
- Subjects
Hepatoblastoma ,Psychoanalysis ,Point (typography) ,business.industry ,Liver Neoplasms ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,0302 clinical medicine ,030220 oncology & carcinogenesis ,Pediatrics, Perinatology and Child Health ,Pretext ,Humans ,Medicine ,Radiology, Nuclear Medicine and imaging ,Child ,business - Published
- 2018
40. The importance of age as prognostic factor for the outcome of patients with hepatoblastoma: Analysis from the Children's Hepatic tumors International Collaboration (CHIC) database
- Author
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Yurong Feng, Piotr Czauderna, Dolores Lopez-Terrada, Sarangarajan Ranganathan, Rudolf Maibach, Arun Rangaswami, Marc Ansari, Marisa Derosa, Mark Krailo, Marcio H. Malogolowkin, Irene Schmid, Giorgio Perilongo, Jin Piao, Yukichi Tanaka, Daniel C. Aronson, Kenichiro Watanabe, Dietrich von Schweinitz, Rita Alaggio, Allison F. O'Neill, Eiso Hiyama, Davide Saraceno, Beate Haeberle, Eugenia Rinaldi, Angela D. Trobaugh-Lotrario, Tomoro Hishiki, Kenichi Yoshimura, and Rebecka L. Meyers
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Hepatoblastoma ,Male ,Pediatric liver tumor ,pediatric liver tumor ,Databases, Factual ,medicine.medical_treatment ,Disease ,Liver Neoplasms / mortality ,computer.software_genre ,0302 clinical medicine ,Risk Factors ,Prospective Studies ,Age of Onset ,Neoplasm Metastasis ,Prospective cohort study ,prognostic factor ,Child ,Prognostic factor ,ddc:618 ,Database ,Liver Neoplasms / diagnosis ,Incidence (epidemiology) ,Incidence ,Liver Neoplasms ,Hematology ,Liver Neoplasms / therapy ,3. Good health ,Survival Rate ,Oncology ,Hepatoblastoma / diagnosis ,030220 oncology & carcinogenesis ,Child, Preschool ,CHIC ,Female ,Adolescent ,Liver Neoplasms / pathology ,Disease-Free Survival ,03 medical and health sciences ,Age ,medicine ,Humans ,Survival rate ,Chemotherapy ,Hepatoblastoma / therapy ,business.industry ,age ,hepatoblastoma ,Hepatoblastoma / pathology ,Infant, Newborn ,Infant ,medicine.disease ,Pediatrics, Perinatology and Child Health ,Age of onset ,Hepatoblastoma / mortality ,business ,computer ,030215 immunology - Abstract
Purpose: Treatment outcomes for hepatoblastoma have improved markedly in the contemporary treatment era, principally due to therapy intensification, with overall survival increasing from 35% in the 1970s to 90% at present. Unfortunately, these advancements are accompanied by an increased incidence of toxicities. A detailed analysis of age as a prognostic factor may support individualized risk-based therapy stratification.Methods: We evaluated 1605 patients with hepatoblastoma included in the CHIC database to assess the relationship between event-free survival (EFS) and age at diagnosis. Further analysis included the age distribution of additional risk factors and the interaction of age with other known prognostic factors.Results: Risk for an event increases progressively with increasing age at diagnosis. This pattern could not be attributed to the differential distribution of other known risk factors across age. Newborns and infants are not at increased risk of treatment failure. The interaction between age and other adverse risk factors demonstrates an attenuation of prognostic relevance with increasing age in the following categories: metastatic disease, AFP < 100 ng/mL, and tumor rupture.Conclusion: Risk for an event increased with advancing age at diagnosis. Increased age attenuates the prognostic influence of metastatic disease, low AFP, and tumor rupture. Age could be used to modify recommended chemotherapy intensity.
- Published
- 2019
41. Is it worth completely resecting hepatoblastoma at diagnosis?
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Piotr Czauderna
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Hepatoblastoma ,medicine.medical_specialty ,business.industry ,General surgery ,Liver Neoplasms ,MEDLINE ,medicine.disease ,Article ,Text mining ,Oncology ,Chemotherapy, Adjuvant ,medicine ,Humans ,business ,Child - Abstract
BACKGROUND: Hepatoblastoma treatment with curative intent requires surgical resection, but only about a third of newly diagnosed patients with hepatoblastoma have resectable disease at diagnosis. Patients who have upfront resection typically receive a total of 4–6 cycles of adjuvant chemotherapy post-surgery, with the combination of cisplatin, fluorouracil, and vincristine. We aimed to investigate whether event-free survival in children with hepatoblastoma who had complete resection at diagnosis could be maintained with two cycles of adjuvant chemotherapy. METHODS: In this Children’s Oncology Group, multicentre, phase 3 trial, patients were enrolled in four risk groups on the basis of Evans surgical stage, tumour histology, and levels of α-fetoprotein at diagnosis to receive risk-adapted therapy. Here, we report on the low-risk stratum of the trial. Eligible patients were younger than 21 years and had histologically confirmed, stage I or II hepatoblastoma without 100% pure fetal stage I or small-cell undifferentiated histology; elevated serum α-fetoprotein level (>100 ng/mL); a complete resection at diagnosis; at least 50% Karnofsky (patients >16 years) or Lansky (patients ≤16 years) performance status; and had received no previous chemotherapy or other hepatoblastoma-directed therapy. Patients received two 21-day cycles of cisplatin, fluorouracil, and vincristine within 42 days of resection, consisting of cisplatin (100 mg/m(2) per dose or 3·3 mg/kg per dose for children
- Published
- 2019
42. Malignant gastrointestinal neuroectodermal tumor (clear cell sarcoma-like tumor of the gastrointestinal tract) of the small intestine in a 12-year-old boy
- Author
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Przemysław, Wolak, Andrzej, Wincewicz, Piotr, Czauderna, Michał, Spałek, Anna, Kruczak, Sławka, Urbaniak-Wąsik, Janusz, Ryś, Elżbieta, Michalak, Martyna, Woltanowska, and Stanisław, Sulkowski
- Subjects
Male ,Rare Diseases ,Treatment Outcome ,Biopsy ,Humans ,Neuroectodermal Tumors ,Antineoplastic Agents ,Poland ,Sarcoma, Clear Cell ,Child ,In Situ Hybridization, Fluorescence ,Endometrial Ablation Techniques ,Gastrointestinal Neoplasms - Abstract
The aim of this paper is a clinical and anatomopathological demonstration of a malignant lesion, a gastrointestinal neuroectodermal tumor (GNET), as an exceedingly rare cause of ileus in the pediatric population. Specifically, we present the case of a 12-year-old boy who showed dramatic weight loss, hypochromic anemia, fever, dehydration, exaggerated granulation of the terminal ileum, and mechanical ileus due to the obstruction by an intramural tumor of the small intestine. A 50cm-long part of the small intestine with pathological stricture was surgically removed, sampled and routinely fixed and stained with hematoxylin and eosin. The additional immunostains that were preformed were: PAS, S-100, HMB-45, NSE, LCA, CK AE1 / AE3, desmin, SMA, vimentin, CD99, NSE, synaptophysin, WT-1, calretinin, and DOG-1. Moreover, fluorescent in situ hybridization (FISH) with the EWSR1 Break Apart FISH Probe was applied. The neoplasm was composed of nests and alveolar patterns of frankly malignant clear cells with immunoreactivity to S-100, vimentin, and CD 99. The FISH technique detected chromosomal breaking at 22q12. The tumor metastasized to both the mesenteric lymph nodes and a number of hepatic segments. With several chemotherapy protocols, repeat laparotomies, and liver thermal ablations, the patient had a 1.5-year-long survival from the moment of diagnosis. The diagnosis of this malignancy requires both histopathological evaluation and molecular analysis, and the follow-up is based on careful clinical imaging of the neoplastic spread in order to apply proper surgical and oncological treatments. In conclusion, the clinical course of GNET was highly aggressive.
- Published
- 2019
43. Laparoscopic Adrenalectomy in Children
- Author
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Andrzej Gołębiewski, Piotr Czauderna, and Marcin Losin
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medicine.medical_specialty ,education.field_of_study ,medicine.anatomical_structure ,Laparoscopic adrenalectomy ,business.industry ,Adrenal gland ,General surgery ,Population ,Gold standard ,Adult population ,Medicine ,business ,education - Abstract
Laparoscopic approach to the adrenal gland was introduced in 1992; since then laparoscopic adrenalectomy (LA) becomes a gold standard in adult population, and many studies evaluating safety and results have been published [1–3]. In contradistinction to adult population, LA remains still challenging in children population despite the fact that it was first performed in a child over 20 years ago [4, 5].
- Published
- 2019
44. O.031 HEPATOCELLULAR CARCINOMA IN CHILDREN: WHERE WE ARE AND WHERE WE GO—FIRST EXPERIENCE FROM THE SIOPEL 3 STUDY
- Author
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Piotr, Czauderna, Maibach, Rudolf, Morland, Bruce, Roebuck, Derek, Zimmermann, Arthur, Casanova, Michela, Childs, Margaret, and Perilongo, Giorgio
- Published
- 2006
- Full Text
- View/download PDF
45. Reconstructive option after radical mutilating surgery in children with genitourinary rhabdomyosarcoma: When sparing the bladder is not an option
- Author
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Leszek Komasara, Piotr Czauderna, Andrzej Gołębiewski, Joanna Stefanowicz, and Anna Bryks-Laszkowska
- Subjects
Male ,medicine.medical_specialty ,Urology ,medicine.medical_treatment ,030232 urology & nephrology ,Urinary Diversion ,Cystectomy ,Stoma ,03 medical and health sciences ,0302 clinical medicine ,Rhabdomyosarcoma ,medicine ,Humans ,Radical surgery ,Child ,Prostatectomy ,Genitourinary system ,business.industry ,Urinary Reservoirs, Continent ,Urinary diversion ,Prostatic Neoplasms ,Vaginectomy ,Plastic Surgery Procedures ,medicine.disease ,Surgery ,Urinary Bladder Neoplasms ,030220 oncology & carcinogenesis ,business - Abstract
Objectives To present versatile surgical reconstructive techniques and their outcomes in pediatric patients with genitourinary rhabdomyosarcoma. Methods We retrospectively analyzed the oncological and urological outcomes of seven patients treated between 1992 and 2014 according to the Cooperative Weichteilsarkom Studiengruppe protocols. Intergroup Rhabdomyosarcoma Study staging: local, six patients; and IV, one patient. Histology: embryonal, five patients; unclassified, one patient; triton tumor one patient. Surgical treatment included: cystectomy, uterectomy and partial vaginectomy, one patient; radical cystectomy, two patients; cystectomy, one patient; cystectomy with partial prostatectomy, one patient; partial cystectomy, one patient; and partial prostatectomy, one patient. Results All patients were alive in complete remission at last follow up. In four cases, ileal conduit with ureteral reimplantation with serous-lined extramural tunnel (Abol-Enein technique) was carried out, which was followed by conversion into ileal continent bladder with continent appendiceal stoma for clean intermittent catheterization in three patients. In one boy, partial cystectomy and continent reconstruction was carried out during a single surgical procedure. One child with incontinent urinary diversion is still awaiting a continence solution. One child after partial prostatectomy is continent without any voiding disturbances. Conclusions The timing and extent of radical surgery for treatment of genitourinary rhabdomyosarcoma depend on the local anatomical conditions, and the response to previous chemo- and radiotherapy. Cystectomy followed by various reconstructive techniques still remains an important option in the local treatment.
- Published
- 2016
46. The Children's Hepatic tumors International Collaboration (CHIC): Novel global rare tumor database yields new prognostic factors in hepatoblastoma and becomes a research model
- Author
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Marisa Derosa, Rebecka L. Meyers, Piotr Czauderna, Marcio H. Malogolowkin, Giorgio Perilongo, Rudolf Maibach, Yurong Feng, Kenichi Yoshimura, Mark Krailo, Tomoro Hishiki, Eiso Hiyama, Beate Haeberle, Daniel C. Aronson, Kenichiro Watanabe, Irene Schmid, Dolores Lopez-Terrada, Dietrich von Schweinitz, Arun Rangaswami, Ivo Leuschner, and Eugenia Rinaldi
- Subjects
Hepatoblastoma ,Male ,0301 basic medicine ,Oncology ,Cancer Research ,Pediatrics ,Time Factors ,Databases, Factual ,International Cooperation ,Research model ,0302 clinical medicine ,Risk Factors ,Pathology ,Cooperative Behavior ,Child ,Liver Neoplasms ,Age Factors ,Rare tumor ,Treatment Outcome ,Child, Preschool ,030220 oncology & carcinogenesis ,Female ,Risk assessment ,medicine.medical_specialty ,Adolescent ,education ,Individualized treatment ,Risk Assessment ,Article ,Disease-Free Survival ,Young Adult ,Databases ,03 medical and health sciences ,Internal medicine ,medicine ,Chemotherapy ,Humans ,Preschool ,Risk stratification ,Factual ,Survival analysis ,Biologic marker ,business.industry ,Infant, Newborn ,Infant ,Survival Analysis ,Cancer ,Newborn ,medicine.disease ,030104 developmental biology ,business - Abstract
Contemporary state-of-the-art management of cancer is increasingly defined by individualized treatment strategies. For very rare tumors, like hepatoblastoma, the development of biologic markers, and the identification of reliable prognostic risk factors for tailoring treatment, remains very challenging. The Children's Hepatic tumors International Collaboration (CHIC) is a novel international response to this challenge.Four multicenter trial groups in the world, who have performed prospective controlled studies of hepatoblastoma over the past two decades (COG; SIOPEL; GPOH; and JPLT), joined forces to form the CHIC consortium. With the support of the data management group CINECA, CHIC developed a centralized online platform where data from eight completed hepatoblastoma trials were merged to form a database of 1605 hepatoblastoma cases treated between 1988 and 2008. The resulting dataset is described and the relationships between selected patient and tumor characteristics, and risk for adverse disease outcome (event-free survival; EFS) are examined.Significantly increased risk for EFS-event was noted for advanced PRETEXT group, macrovascular venous or portal involvement, contiguous extrahepatic disease, primary tumor multifocality and tumor rupture at enrollment. Higher age (≥ 8 years), low AFP (100 ng/ml) and metastatic disease were associated with the worst outcome.We have identified novel prognostic factors for hepatoblastoma, as well as confirmed established factors, that will be used to develop a future common global risk stratification system. The mechanics of developing the globally accessible web-based portal, building and refining the database, and performing this first statistical analysis has laid the foundation for future collaborative efforts. This is an important step for refining of the risk based grouping and approach to future treatment stratification, thus we think our collaboration offers a template for others to follow in the study of rare tumors and diseases.
- Published
- 2016
47. Malformacja naczyniowa jelita czczego przyczyną krwotoku z przewodu pokarmowego – opis przypadku
- Author
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Piotr Czauderna, Karolina Gizelbach-Żochowska, Pawel Plosaj, Hanna Garnier, Maciej Murawski, Michał Szewczuk, and Piotr Landowski
- Subjects
medicine.medical_specialty ,Gastrointestinal tract ,Lower Gastrointestinal Tract ,medicine.diagnostic_test ,Exploratory laparotomy ,Anemia ,business.industry ,medicine.medical_treatment ,Vascular malformation ,Colonoscopy ,medicine.disease ,Surgery ,medicine.anatomical_structure ,Pediatrics, Perinatology and Child Health ,medicine ,Abdomen ,Radiology ,business ,Computed tomography angiography - Abstract
Vascular malformation is a rare cause of bleeding from the lower gastrointestinal tract. We present a case of 16-year-old patient with a hemorrhage from the gastrointestinal tract, which led to the life-threatening anemia. Gastroduodenoscopy and colonoscopy were performed and did not locate a bleeding site. Consecutively, an exploratory laparotomy did not locate the pathology either. A bleeding site was visualized by computed tomography angiography (CTA) of the abdomen. Relaparotomy was performed and the 4-centimeter part of the intestine and a small jejunal vascular malformation were histopathologically revealed.
- Published
- 2015
48. Hepatoblastoma: current understanding, recent advances, and controversies
- Author
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Hanna Garnier and Piotr Czauderna
- Subjects
0301 basic medicine ,Oncology ,medicine.medical_specialty ,Hepatoblastoma ,medicine.medical_treatment ,surgical treatment ,Disease ,Review ,Liver transplantation ,chemotherapy ,Primary Malignant Liver Neoplasm ,General Biochemistry, Genetics and Molecular Biology ,03 medical and health sciences ,Therapeutic approach ,0302 clinical medicine ,Internal medicine ,medicine ,General Pharmacology, Toxicology and Pharmaceutics ,Survival rate ,Chemotherapy ,PRETEXT ,General Immunology and Microbiology ,liver transplantation ,business.industry ,biology ,General Medicine ,Articles ,hepatoblastoma ,medicine.disease ,030104 developmental biology ,030220 oncology & carcinogenesis ,Metastasectomy ,business - Abstract
Introduction: Hepatoblastoma (HB) is the most common primary malignant liver neoplasm in children. Its increasing survival rate is related to the progress in modern imaging, surgical techniques, and new chemotherapy regimens. Clinical approach: One of the past achievements was the development of the pretreatment extension of disease (PRETEXT) system. Gradually, the HB therapeutic approach has become more individualized with better stratification of patients. Controversies: These include the need for preoperative chemotherapy and its optimal duration; intensity of preoperative chemotherapy required for locally advanced cases (PRETEXT 4); optimal surgical treatment for locally advanced tumors: aggressive hepatic resections versus liver transplantation; the role of postoperative chemotherapy in the post-transplant setting; the timing and role of metastasectomy in patients with disseminated disease who undergo partial liver resection; and the prognostic significance of several HB pathology variants. Hepatoblastoma biology: Beta-catenin mutations and the beta-catenin/Wnt pathway play an important role in HB development. There have been at least two molecular signatures in HB published. Unluckily, all of these findings are based on relatively small clinical series and require confirmation. Conclusion: The treatment of HB started from one and the same therapy for all patients and aimed at increased treatment individualization, but the future seems to lie in biology-driven patient-tailored therapies.
- Published
- 2018
49. Sodium Thiosulfate for Protection from Cisplatin-Induced Hearing Loss
- Author
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M Elena Mateos, Michael J. Sullivan, Gareth J. Veal, Piotr Czauderna, Eiso Hiyama, Michael Capra, Kaukab Rajput, Catherine Rechnitzer, Edward A. Neuwelt, Arun Rangaswami, Milind Ronghe, Jane Skeen, Laurence Brugières, Rudolf Maibach, Marc Ansari, Anna Covezzoli, Patrizia Dall'Igna, Penelope Brock, Margaret Childs, Benedicte Brichard, Giorgio Perilongo, Bruce Morland, Derek J. Roebuck, Véronique Laithier, UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, and UCL - (SLuc) Service d'hématologie et d'oncologie pédiatrique
- Subjects
0301 basic medicine ,Oncology ,Hepatoblastoma ,Male ,medicine.medical_treatment ,Sodium thiosulfate ,chemistry.chemical_compound ,0302 clinical medicine ,Single-Blind Method ,Child ,ddc:618 ,Medicine (all) ,Incidence ,Liver Neoplasms ,General Medicine ,Cisplatin/administration & dosage/adverse effects ,Hepatoblastoma/drug therapy/mortality ,030220 oncology & carcinogenesis ,Child, Preschool ,Combination ,Drug Therapy, Combination ,Female ,Thiosulfates/administration & dosage/adverse effects/therapeutic use ,medicine.symptom ,Liver Neoplasms/drug therapy/mortality ,medicine.drug ,Glomerular Filtration Rate ,Hearing Loss/chemically induced/prevention & control ,medicine.medical_specialty ,Adolescent ,Hearing loss ,Thiosulfates ,Article ,Cisplatin ,Doxorubicin ,Follow-Up Studies ,Hearing Loss ,Humans ,Infant ,Survival Analysis ,03 medical and health sciences ,Drug Therapy ,Ototoxicity ,Internal medicine ,otorhinolaryngologic diseases ,medicine ,Preschool ,Chemotherapy ,business.industry ,Doxorubicin/administration & dosage ,medicine.disease ,digestive system diseases ,Carboplatin ,030104 developmental biology ,chemistry ,business - Abstract
BACKGROUND: Cisplatin chemotherapy and surgery are effective treatments for children with standard-risk hepatoblastoma but may cause considerable and irreversible hearing loss. This trial compared cisplatin with cisplatin plus delayed administration of sodium thiosulfate, aiming to reduce the incidence and severity of cisplatin-related ototoxic effects without jeopardizing overall and event-free survival. METHODS: We randomly assigned children older than 1 month and younger than 18 years of age who had standard-risk hepatoblastoma (≤3 involved liver sectors, no metastatic disease, and an alpha-fetoprotein level of >100 ng per milliliter) to receive cisplatin alone (at a dose of 80 mg per square meter of body-surface area, administered over a period of 6 hours) or cisplatin plus sodium thiosulfate (at a dose of 20 g per square meter, administered intravenously over a 15-minute period, 6 hours after the discontinuation of cisplatin) for four preoperative and two postoperative courses. The primary end point was the absolute hearing threshold, as measured by pure-tone audiometry, at a minimum age of 3.5 years. Hearing loss was assessed according to the Brock grade (on a scale from 0 to 4, with higher grades indicating greater hearing loss). The main secondary end points were overall survival and event-free survival at 3 years. RESULTS: A total of 109 children were randomly assigned to receive cisplatin plus sodium thiosulfate (57 children) or cisplatin alone (52) and could be evaluated. Sodium thiosulfate was associated with few high-grade toxic effects. The absolute hearing threshold was assessed in 101 children. Hearing loss of grade 1 or higher occurred in 18 of 55 children (33%) in the cisplatin-sodium thiosulfate group, as compared with 29 of 46 (63%) in the cisplatin-alone group, indicating a 48% lower incidence of hearing loss in the cisplatin-sodium thiosulfate group (relative risk, 0.52; 95% confidence interval [CI], 0.33 to 0.81; P=0.002). At a median of 52 months of follow-up, the 3-year rates of event-free survival were 82% (95% CI, 69 to 90) in the cisplatin-sodium thiosulfate group and 79% (95% CI, 65 to 88) in the cisplatin-alone group, and the 3-year rates of overall survival were 98% (95% CI, 88 to 100) and 92% (95% CI, 81 to 97), respectively. CONCLUSIONS: The addition of sodium thiosulfate, administered 6 hours after cisplatin chemotherapy, resulted in a lower incidence of cisplatin-induced hearing loss among children with standard-risk hepatoblastoma, without jeopardizing overall or event-free survival. (Funded by Cancer Research UK and others; SIOPEL 6 ClinicalTrials.gov number, NCT00652132 ; EudraCT number, 2007-002402-21 .).
- Published
- 2018
50. Ten years later – scalp still a primary donor site in children
- Author
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Piotr Czauderna, Barbara Chrzanowska, and Dariusz Wyrzykowski
- Subjects
Male ,medicine.medical_specialty ,Time Factors ,Adolescent ,Cicatrix, Hypertrophic ,Scar assessment ,medicine.medical_treatment ,Silicones ,Scars ,Critical Care and Intensive Care Medicine ,Transplant Donor Site ,Hypertrophic scar ,Split thickness skin graft ,medicine ,Humans ,Child ,Scalp ,Emollients ,business.industry ,Infant ,Mean age ,Skin Transplantation ,General Medicine ,medicine.disease ,Surgery ,medicine.anatomical_structure ,Child, Preschool ,Emergency Medicine ,Forehead ,Skin grafting ,Female ,medicine.symptom ,Burns ,business ,Gels - Abstract
Choice of the donor site for a split thickness skin graft depends on skin availability, possible complications and anticipated esthetic results. We selected the scalp to be the primary donor site at our institution. During a period of ten years (1998–2008), a group of 123 pediatric patients aged 4 months to 15 years (65% were below the age of 2; mean age 2.98 years) underwent skin grafting from this particular site. In 2 cases the same area was re-harvested. All donor sites healed by the 10th post-operative day. Donor site complications included: 2 microalopecia regions, 5 pressure sores in a close proximity, 1 hypertrophic scar and 1 visible mark on the forehead due to technical mistake in graft harvesting. All children started scar management of the recipient site with contact therapy using adhesive tape Hypafix (BSN Medical); subsequently moving on to silicone sheets or gel in selected refractory cases. We present results for 68 scars in 41 patients with the longest follow-up period. Scar quality was evaluated after minimum of 10 years and scored according to the Vancouver Scar Assessment Scale. Very good and good results were obtained in 55 scars (80.9%), satisfactory in 11 scars (16.2%) and unsatisfactory in 2 scars (2.9%). Conclusions Our results confirm, that the scalp is a reliable donor site in children and contact therapy is an adequate form of scar prevention/treatment of the recipient site.
- Published
- 2015
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