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Background: The published experience concerning autologous peripheral blood stem cell collection in children is very limited. Methods: The data of pediatric patients who underwent autologous stem cell mobilization and apheresis between January 2011 and April 2020 were analyzed retrospectively. Results: We studied retrospectively 64 mobilization and apheresis procedures in 48 pediatric patients (34 males, 14 females), mean age of 7.31 ± 5.38 (range, 1.5–19.7) years, the underlying disease was mostly neuroblastoma (NBL). The body weight of 21 patients (43.75%) was 15 kg or less. The targeted autologous peripheral stem cell apheresis (APSCA) was successfully achieved in 98% of patients. Neuroblastoma patients were younger than the rest of the patients and underwent apheresis after receiving fewer chemotherapy cycles than others and all of them mobilized within the first session successfully. Plerixafor was added to mobilization in nine heavily pretreated patients (18.7%), median two doses (range, 1–4 doses). 11 patients (22.9%) underwent radiotherapy (RT) before mobilization with doses of median 24 Gy (range, 10.8–54.0 Gy). Patients with RT were older at the time of apheresis and had received more chemotherapy courses than patients without RT. As a result, patients with a history of RT had significantly lower peripheral CD34+ cells and CD34+ yields than those without RT. In 17 patients (35.4%), 22 different complications were noted. The most common complications were catheter‐related infections (n:10, 20.8%), followed by catheter‐related thrombosis in eight patients (16.7%). Conclusions: Patients who had far less therapy before apheresis were more likely to mobilize successfully. Our study provides a detailed practice approach including complications during APSCA aiming to increase the success rates of apheresis in transplantation centers. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Pediatric Disease / Türkiye Çocuk Hastalıkları Dergisi is the property of Turkish Journal of Pediatric Disease and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: We aimed to evaluate our pediatric HSCT recipients routinely monitored for adenoviremia and to determine the adequacy of this monitoring in predicting adenoviral disease (AD). Methods: A retrospective cohort of patients who underwent allogeneic HSCT between January 2021 and August 2022, and routinely monitored for adenoviremia by real‐time PCR was included in our survey. Demographic and clinical data of the patients were recorded. Incidence rates, risk factors, and mortality rates related to adenoviremia, and AD were analyzed. Results: Among 104 HSCTs performed in 94 patients adenovirus (AdV) was revealed in 27 (26%) episodes and adenoviremia in 18 (17.3%) HSCT episodes. AD without adenoviremia developed in nine episodes (8.6%). Disseminated disease was significantly more frequently detected in episodes with adenoviremia (p =.008). GVHD was independent risk factor for AdV detection (OR: 8.6, 95% CI: 2.03–33.7, p =.001). Viremia developed within a shorter time interval after HSCT in isolated episodes of adenoviremia compared to those with concomitant AD (p =.006). Initial and peak viral loads were significantly higher in adenoviremia with AD (p <.001). Mortality was higher in the AdV‐detected episodes (p <.001) than in the AdV‐undetected episodes. AdV‐related mortality was found to be 22.2%. Adenoviremia increased the risk of mortality (OR: 1.2, 95% CI: 0.22–1.33, p =.01). Conclusions: Adenoviremia monitoring is an important process in the detection of AD. Since some patients may develop AD without accompanying by adenoviremia, monitoring for AdV in blood samples should be supported with other monitoring methods in order to evaluate the probable involvement of different organs or systems. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Pediatric Disease / Türkiye Çocuk Hastalıkları Dergisi is the property of Turkish Journal of Pediatric Disease and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Infections are a significant cause of morbidity and mortality of chemotherapy-induced neutropenia in children with acute leukemia. The aim of this study was to evaluate microbiologically documented infections (MDIs) during febrile neutropenia (FN) episodes and their relation to mortality. Four hundred eighty-seven FN episodes of 140 children were enrolled in this single-center study, and MDI in those FN episodes was retrospectively examined. Eighty-four patients (60%) had at least one positive peripheral blood, central line, or urine culture. MDIs were detected in 163 of 487 (33.4%) FN episodes of 84 children with leukemia. Gram-negative bacteria, Gram-positive bacteria, and fungal agents were isolated in 52.7%, 40.4%, and 6.9% of whole episodes. Coagulase-negative Staphylococci and Enterococci were the most detected Gram-positive bacteria. Klebsiella spp. and Escherichia coli were the most common Gram-negative bacteria isolated in the entire cohort. A central line was present in 145 MDI episodes, and catheter removal was required in 35 cases (17.7%) due to infection with Gram-negative bacteria, Gram-positive bacteria 43%, 28.5%, and fungus 28.5%, respectively. MDI-related mortality was 9.8%. The highest mortality rate (16.7%) was observed in Gram-negative bacteria and patients with relapsed and resistant leukemia. The most common infectious agent related to mortality was Klebsiella (31%). Resistance to third-or fourth-generation cephalosporins in Gram-negative bacteria was found to be over 50% of our cohort uri. Empirical antibiotic therapy at the onset of FN in neutropenic patients is crucial; therefore, the institution's predominant pathogens and resistance patterns should guide the choice of empirical antimicrobials. To reduce mortality and morbidity, each center should know its local epidemiological data and antibiotic susceptibility. [ABSTRACT FROM AUTHOR]

Objective: Patients with inherited bleeding disorders faced problems in accessing healthcare during coronavirus disease 2019 pandemic. This study aimed to investigate the health problems of patients with inherited bleeding disorders during the coronavirus disease 2019 pandemic. Material and Methods: Children and adult patients with inherited bleeding disorders who had a coronavirus disease 2019 infection between March 2020 and November 2021 were retrospectively evaluated. Results: Seven hundred seventy-two patients were reviewed, and 65 patients who had a coronavirus disease 2019 infection (Male/Female: 58/7, mean age 28.2 ±14.1 years) were analyzed. Sixty patients (92%) had hemophilia A or B or von Willebrand's disease and 5 (8%) had rare bleeding disorders. Sixteen (24.6%) patients had a comorbid disease and 6 (9.2%) needed hospitalization due to severe coronavirus disease 2019 infection. Seven patients (10.7%) experienced a bleeding episode and were treated with factor concentrates. Totally, 64 (98.46%) patients recovered from the coronavirus disease 2019 infection and 1 died. Conclusion: Patients with inherited bleeding disorders and coronavirus disease 2019 infection mostly had a mild/moderate course of the disease. [ABSTRACT FROM AUTHOR]

Cancer care is progressively became as a significant worldwide challenge. Wars can cause destructions and delays in cancer diagnosis and treatment of displaced people. Cancer cure rates need to be improved in indefensible populations such as refugees. In this study, we purposed to highlight the clinical peculiarities and outcomes of refugee children with cancer in our hospital. Our purpose was to present our findings and contribute to improve the health care for these children. Seventy one refugee pediatric patients admitted to the oncology and hematology units of our hospital between April 2011 and January 2019 were included in this study. The demographic characteristics of the patients at the initial diagnosis, their countries of origin, living conditions, histopathological diagnoses, treatments, relapse, and mortality data were analyzed retrospectively from the patient files. The median age of patients was 6.5±4.5 years, and the male-to-female ratio was 39/32. While 44 patients (61.9%) presented with complaints and had primary diagnoses in our hospital, the remaining 27 patients (38.1%) were diagnosed in their country and applied to our hospital for treatment. Our mean follow-up period was 18.2±18.8 months (1-90 months). As a result, 44 patients (62%) were alive and 22 (31%) were dead. The survival rate without relapse in the second year was 83.6%. Two and fiveyear survival rates were 77.5% vs. 58.1% respectively. Compared to Turkish children, lower survival rates were found in refugee children. In addition to cancer-specific factors such as tumor type and stage, some problems such as shelter, communication, adherence to treatment, and difficulties supplying medicine may be responsible for lower survival rates in refugee children. Further studies are needed to improve the survival rates of patients. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Behcet Uz Children's Hospital is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: The aim of this study is to develop new perspectives to prevent or reduce potential organ damage due to iron-mediated oxidation in thalassemia major patients. Methods: Seventy patients were included in this study. Blood samples were taken from the patients before and after transfusion. Total thiol, native thiol, disulfide, disulfide/native thiol percentage ratio, ischemia modified albumin (IMA), total antioxidant status (TAS), total oxidant status (TOS), and ferroxidase levels were determined. Additionally, undepleted thiol level (UTL) was determined as a new parameter associated with organ damage. Results: After transfusion, the levels of native thiol, total thiol, disulfide, TAS, ferroxidase, and TOS were higher, while the IMA levels and disulfide/native thiol percent ratio were lower. Significant correlations were found between antioxidant and oxidant tests before and after transfusion. Additionally, a negative correlation was found between the TOS and UTL levels of the patients measured before the transfusion. Conclusion: In the present study, transfusion therapy increased both oxidation and the antioxidant levels. In addition, the term UTL has been introduced as a parameter that enables the determination of the oxidation level that may cause potential organ damage in transfusion-dependent thalassemia patients. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to explore adolescent survivors' views and expectations about long‐term follow‐up care. Methods: Semi‐structured, in‐depth interviews were conducted with 16 adolescent survivors of childhood acute lymphoblastic leukaemia. Survivors who had completed treatment at least 2 years ago were involved in the study. Interviews were audio‐recorded and transcribed verbatim. An inductive thematic approach was used to analyse the data. Results: Four main themes and sub‐themes were identified from the analysis as follows: information needs with three sub‐themes 'long‐term follow‐up, healthy life and social life', support needs with three sub‐themes 'psychosocial (peer relations etc.), school related and social–emotional (fear of relapse, body image, self‐esteem, etc.)', perceived benefits with two sub‐themes 'social–emotional and related to long‐term follow‐up' and perceived barriers 'medical‐hospital related and social life'. Conclusions: Adolescent survivors mainly need support in terms of psychosocial aspects: self‐esteem–body image, school, peer relations and social activities during follow‐up. Identified barriers related to follow‐up were school absence and not able to participate social activities. Adolescents specify health promotion approaches as benefits aspects of follow‐up. The findings of this study will guide nurses in the long‐term follow‐up care of adolescent survivors of acute lymphoblastic leukaemia and provide an opportunity to plan individualised follow‐up care. [ABSTRACT FROM AUTHOR]

Background. Pyruvate kinase (PK) deficiency is the most common enzyme abnormality in the glycolytic pathway. Here, we describe two siblings with PK deficiency that mimicked congenital dyserythropoietic anemia (CDA) type I. Case. The siblings were referred to our hospital for evaluation of anemia when they were newborns. Their PK enzyme activities were normal. Their bone marrow aspirations and electron microscopies showed CDA-like findings. A CDA panel with next-generation sequencing showed no mutation. Though their PK enzyme levels were normal, a molecular study of the PKLR gene showed a homozygous variant c.1623G>C (p.Lys541Asn) in exon 12 of our patients. Conclusions. Although the diagnosis of pyruvate kinase deficiency is difficult, it can be confused with many other diagnoses. Bone marrow findings of these cases are similar to congenital dyserythropoietic anemia. In patients with normal pyruvate kinase enzyme levels, the diagnosis cannot be excluded and genetic analysis is required. [ABSTRACT FROM AUTHOR]

Objective: Anthracyclines are widely used in the treatment of acute lymphoblastic leukemia (ALL). However, cardiotoxicity is the most critical side effect that requires dose limitation. It is thought to occur at first exposure, but the clinical presentation may occur years later. In this study, we aimed to determine the time of initial damage and cardiotoxicity that develops in children with ALL. Methods: In this prospective study, 13 patients with newly diagnosed intermediate-risk precursor B cell ALL treated with the ALL-IC BFM 2009 protocol were included. Conventional echocardiography, tissue Doppler imaging (TDI), and speckle-tracking echocardiography (STE) were performed in all the patients before chemotherapy, after completing the induction phase, and at the end of the reinduction phase. Results: The mean age of the patients was 7.8±4.6 (3.1–16.3) years. Myocardial velocity during systole (Sm) determined by TDI at the interventricular septum significantly decreased during the induction phase. Despite a decrease in STE parameters, a statistically significant reduction was determined in the global longitudinal strain rate at both left and right ventricles at the end of the induction. Nevertheless, a statistically significant increase was observed among the conventional echocardiographic findings in the left ventricular end-diastolic diameter at the end of the reinduction. Conclusion: During the treatment of ALL, subclinical anthracycline-associated cardiotoxicity develops in the early stages of treatment. The findings detected by TDI and STE could be missed by conventional echocardiography. We recommend evaluating patients with these newly developed techniques to detect subclinical cardiotoxicity at an early stage and starting appropriate therapy on time. [ABSTRACT FROM AUTHOR]

Background: Pneumatosis cystoides intestinalis (PCI) is a disorder in which widespread air sacs are present in mucosa, submucosa, subserosa, and intraabdominal area of the intestinal wall. It has a heterogeneous clinical presentation as a rare complication of intestinal graft‐versus‐host disease (GVHD). Computed tomography is the preferred imaging method for the diagnosis. Since the air sacs could be ruptured spontaneously, the presence of free air in the peritoneal cavity does not confirm intestinal perforation. The conservative treatment approach is sufficient in cases that do not require urgent surgical intervention, such as perforation or obstruction. Case: Here, we present a 2.5‐year‐old patient diagnosed with primary hemophagocytic lymphohistiocytosis (pHLH), who underwent allogeneic hematopoietic stem cell transplantation from a matched unrelated donor (MUD) and developed PCI secondary to intestinal GVHD 14th months after HSCT. Conclusions: Pneumatosis cystoides intestinalis, which is a rare complication, should be kept in mind, especially in patients with intestinal GVHD and receiving intensive immunosuppressive, octreotide, and steroid treatment after HSCT. [ABSTRACT FROM AUTHOR]

Background. Febrile neutropenia (FN) is a common and serious complication in patients with leukemia. Hemostasis and inflammation are two interrelated systems in response to infection. We aimed to investigate the course of thrombin formation in febrile neutropenia attack of children with acute lymphoblastic leukemia (ALL). Methods. Thrombin generation was monitored in children treated for ALL at diagnosis of febrile neutropenia (FN) (t0), at 48th hour of FN (t1) and after recovery from neutropenia (t2). Results. Twenty-nine patients and 50 healthy children as control were enrolled into the study. Mean endogenous thrombin potential (ETP) and mean peak value of thrombin results at t1 were significantly higher than at t0, t2 and control groups, respectively. A positive but statistically nonsignificant correlation between ETP values at t1 and duration of neutropenia was observed. Conclusion. Although thrombin generation is enhanced both due to chemotherapy or malignancy itself, our results revealed that thrombin formation also increased in neutropenic infection of children with leukemia. [ABSTRACT FROM AUTHOR]

Background: Autoimmune atrophic gastritis (AIG) is very rare in children. Despite a better understanding of histopathologic changes and serological markers in this disease, underlying etiopathogenic mechanisms and the effect of Helicobacter pylori (H pylori) infection are not well known. We aimed to investigate the relation between AIG and H pylori infection in children. Materials and Methods: We evaluated the presence of AIG and H pylori infection in fifty‐three patients with positive antiparietal cell antibody (APCA). Demographic data, clinical symptoms, laboratory and endoscopic findings, histopathology, and presence of H pylori were recorded. Results: The children were aged between 5 and 18 years, and 28 (52.8%) of them were male. Mean age was 14.7 ± 2.6 years (median: 15.3; min‐max: 5.2‐18), and 10 (18.8%) of them had AIG confirmed by histopathology. In the AIG group, the duration of vitamin B12 deficiency was longer (P =.022), hemoglobin levels were lower (P =.018), and APCA (P =.039) and gastrin (P =.002) levels were higher than those in the non‐AIG group. Endoscopic findings were similar between the two groups. Intestinal metaplasia was higher (P =.018) in the AIG group. None of the patients in the AIG group had H pylori infection (P =.004). One patient in the AIG group had enterochromaffin‐like cell hyperplasia. Conclusions: Our results show that, in children, H pylori infection may not play a role in AIG. AIG could be associated with vitamin B12 deficiency, iron deficiency, and APCA positivity in children. APCA and gastrin levels should be investigated for the early diagnosis of AIG and intestinal metaplasia. [ABSTRACT FROM AUTHOR]

Copyright of Turkish Journal of Hematology is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background. Hemophagocytic lymphohistiocytosis (HLH) may precede malignancy, in particular lymphomas and leukemias. However, the causative factors, appropriate treatment and the prognosis of this association is not established. Case. Herein, we present two patients, one with nodular sclerosing Hodgkin lymphoma (HL) and concomitant Epstein-Barr virus (EBV) infection, and the other with anaplastic large cell lymphoma (ALCL), presented as malignancy associated HLH. Conclusion. In our patients, malignancy directed therapy was sufficient to treat HLH symptoms both at presentation and at recurrence in the second patient. [ABSTRACT FROM AUTHOR]

Despite improvements in diagnosis and treatment, invasive fungal infection (IFI) is still a major cause of morbidity and mortality in immunocompromised patients. In patients with hematologic malignancy, the most invasive fungal infections are caused by Candida and Aspergillus fumigatus. This study was designed retrospectively to summarize data in pediatric patients with acute lymphoblastic leukemia (ALL) and acute myeloblastic leukemia (AML) including risk factors for IFI, epidemiological and clinical features and treatment choices involving combination therapy from January 2006 through December 2014. We analyzed the records of 154 pediatric patients (125 ALL and 29 AML) receiving chemotherapy for hematologic malignancy. During follow-up 60 IFI episodes were observed. IFI episodes were observed more common in AML, compared to ALL (p=0.002). Among 60 IFI episodes, eight were proven, seven were probable and 45 were possible IFI episodes. Galactomannan antigen was investigated in 37 IFI episodes and found positive in seven probable IFI episodes. Fungemia was detected in seven patients with proven IFI and the most common microorganism was non-albicans candida spp. The most common antifungal drug was fluconazole (14.8%). A total of 29 patients (48%) had received empirically liposomal amphotericin B and 10 patients (16.6%) had received caspofungin. Crude mortality was 10.3% and attributable mortality was 6.4% during the study period. Invasive fungal infections continue to be a major cause of morbidity and mortality in children with hematologic cancer. The most common isolated agent from hemoculture was non-albicans Candida spp. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Dr. Behcet Uz Children's Hospital is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Turkish Journal of Hematology is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

PH is a rare condition with high mortality rate after pediatric HSCT. As clinical presentation is non-specific and may mimic other conditions, a high degree of suspicion is required for diagnosis. Here, we present a patient with stage- IV neuroblastoma who developed PAH after autologous HSCT. After exclusion of other causes of PH, we regarded that this condition was secondary to HSCT. [ABSTRACT FROM AUTHOR]

Copyright of Turkish Journal of Hematology is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Acquired aplastic anemia (AAA) is a rare and potentially life threatening disorder. We retrospectively compared the outcomes of 29 children with AAA who received immunosuppressive therapy (IST) or underwent hematopoietic stem cell transplantation (HSCT). Median age at diagnosis was 9.0 years (range, 2–18 years) and median follow-up period was 36 months (range, 3–108 months). Viral infection associated/post hepatitis AAA was in 6 patients (20.6%). According to the initial laboratory findings, 8 patients were classified as very severe AA (vSAA), 8 as severe AA (SAA), and 13 patients as transfusion-dependent moderate AA (MAA). Out of 13, 5 transfusion-dependent MAA patients progressed SAA in median one month (range, 1–5 months), another 6 MAA patients developed remission or became transfusion free during follow-up. Eight patients underwent upfront matched family donor (MFD) HSCT at median 6 months (range, 1–9 months) and achieved complete response (100%). Fifteen cycles of IST were given to 10 (34%) patients lacking MFD at median 3 months (range, 2–6 months). Fifty percent of patients had complete/partial response after IST protocol. Three patients who were unresponsive to IST, proceeded to alternative donor HSCT, in 2nd or 3rd year after the diagnosis and only 1 patient was sustained remission. Several drugs such as mycophenolatemofetil, high-dose cyclophosphamide, levamisole and eltrombopag have been investigated in order to improve the outcome of patients with AAA. Early intervention in AAA patients results in significantly better outcomes. [ABSTRACT FROM AUTHOR]