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Background: Definitive chemoradiation is the primary treatment for locally advanced head and neck carcinoma (LAHNSCC). Optimising outcome predictions requires validated biomarkers, since TNM8 and HPV could have limitations. Radiomics may enhance risk stratification., Methods: This single-centre observational study collected clinical data and baseline CT scans from 171 LAHNSCC patients treated with chemoradiation. The dataset was divided into training (80%) and test (20%) sets, with a 5-fold cross-validation on the training set. Researchers extracted 108 radiomics features from each primary tumour and applied survival analysis and classification models to predict progression-free survival (PFS) and 5-year progression, respectively. Performance was evaluated using inverse probability of censoring weights and c-index for the PFS model and AUC, sensitivity, specificity, and accuracy for the 5-year progression model. Feature importance was measured by the SHapley Additive exPlanations (SHAP) method and patient stratification was assessed through Kaplan-Meier curves., Results: The final dataset included 171 LAHNSCC patients, with 53% experiencing disease progression at 5 years. The random survival forest model best predicted PFS, with an AUC of 0.64 and CI of 0.66 on the test set, highlighting 4 radiomics features and TNM8 as significant contributors. It successfully stratified patients into low and high-risk groups (log-rank p < 0.005). The extreme gradient boosting model most effectively predicted a 5-year progression, incorporating 12 radiomics features and four clinical variables, achieving an AUC of 0.74, sensitivity of 0.53, specificity of 0.81, and accuracy of 0.66 on the test set., Conclusion: The combined clinical-radiomics model improved the standard TNM8 and clinical variables in predicting 5-year progression though further validation is necessary., Key Points: Question There is an unmet need for non-invasive biomarkers to guide treatment in locally advanced head and neck cancer. Findings Clinical data (TNM8 staging, primary tumour site, age, and smoking) plus radiomics improved 5-year progression prediction compared with the clinical comprehensive model or TNM staging alone. Clinical relevance SHAP simplifies complex machine learning radiomics models for clinicians by using easy-to-understand graphical representations, promoting explainability., Competing Interests: Compliance with ethical standards. Guarantor: The scientific guarantor of this publication is Professor Andrés Cervantes. INCLIVA Biomedical Research Institute. Conflict of interest: Anna Nogué, Ana Jiménez-Pastor, Fuensanta Bellvís, Almudena Fuster-Matanzo, and Ángel Alberich-Bayarri declare relationships (full or part-time employment) with the following companies: QUIBIM SL. All other authors have declared no relationships with any companies, whose products or services may be related to the subject matter of the article. Statistics and biometry: One of the authors (Ana Jiménez-Pastor) has significant statistical expertise. Informed consent: Written informed consent was obtained from all patients in this study. Ethical approval: IRB approval was obtained (Ethics Committée of Clinical Research of the University Clinical Hospital of Valencia). Methodology: Prospective and retrospective Observational and prognostic study Performed at one institution, (© 2024. The Author(s).)

This report examines the case of a 3-year-old child presenting with a 1-month history of swelling in the right groin. The boy had no associated nausea or vomiting, was afebrile and had had normal bowel movements. Attempts to reduce the swelling were only partially successful. Ultrasonography indicated the presence of turbid hydrocele and a hernia sac containing an intestinal loop. Accordingly, the patient underwent an urgent herniotomy. Exposing the hernia sac revealed 5 cm Meckel's diverticulum, and the base of the diverticulum was resected from the inside of the hernia sac. The boy was discharged 4 days after the operation in good clinical condition. The presented case highlights the need to consider Littre's hernia when dealing with partially reduced inguinal hernias in children with no general signs or evidence of intestinal obstruction., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2021.)

Background: Allergic bronchopulmonary aspergillosis (ABPA) is a condition characterized by an exaggerated immune response (a hypersensitivity response) to the fungus Aspergillus (most commonly Aspergillus fumigatus ).ABPA causes airway inflammation that if left untreated can lead to bronchiectasis (an abnormal dilation of the airways) due to the immune system and fungal spores damaging sensitive lung tissues and ultimately leading to scarring., Case Presentation: We present a case of a 32-year-old female patient who was misdiagnosed with persistent asthma and treated accordingly for several months until a reexamination was done and a diagnosis of ABPA was concluded. Treatment was altered which led to the successful recovery of the patient., Conclusion: A reevaluation of the patients' condition was needed to arrive to the correct diagnosis and to put her on the correct treatment as an ABPA patient instead of persistent asthma, concluding that the medical history and physical examination are both of vital significance to stipulate a correct diagnosis., Competing Interests: There is no conflict of interest., (© 2021 The Authors.)

Background: Myasthenia gravis is an organ specific autoimmune disorder that is potentially serious but treatable. It is characterized by fatigability of the voluntary muscles and weakness caused by antibodies against the nicotinic acetylcholine receptor (AChR) on the postsynaptic membrane at the neuromuscular junction.Sometimes, and in very rare cases, it can be associated with other autoimmune conditions in a so called autoimmune polyglandular syndrome type 2, which consists mainly of autoimmune adrenal insufficiency (Addison's disease) with autoimmune thyroid disease and/or type 1 diabetes mellitus., Case Presentation: We describe a case of a 47-year-old male patient presenting with weakness, difficulty swallowing (mainly liquids) and dysarthria. He was discovered to have low cortisol and TSH levels with high T4 and T3. These findings lead to the suspicion of a more complex disease process and through a thorough research of literature we discovered an association between myasthenia gravis and autoimmune polyglandular syndrome specifically type 2 which fits with our patients' presentation., Conclusion: In any autoimmune disease, it is important to keep in mind associations and susceptibilities to other autoimmune processes and syndromes in order to reach a correct diagnosis and treatment preventing life threating events., Competing Interests: There is no conflict of interest., (© 2021 The Authors.)

Foreign bodies' (FBs) ingestion is a common problem among children and the psychologically deranged. Ingested FBs usually pass through the alimentary tract without any incident. They can rarely be placed in the appendix and may cause problems. Here we report a case of an appendectomy with no signs of inflammation indicated for a sharp metallic foreign body trapped in the appendix of a 13-year-old Syrian girl. The diagnosis was made through serially abdominal X-rays and abdominal ultrasound., (© The Author(s) 2020. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

The management of bladder exstrophy (BE) remains one of the most significant challenges encountered by pediatric urologists despite improvements in the operative techniques worldwide. Regardless of surgical technique, timing of primary closure remains a matter of debate. The initial closure may be performed within the first 48-72 hours of life or at ~6-12 weeks of age. Delayed presentation until adolescence is extremely rare. However, due to lack of awareness, and access to healthcare along with poor socioeconomic conditions in developing countries, some patients may not receive treatment during infancy. We present a case of a 10-year-old Syrian male with BE associated with complete rectal prolapse who did not undergo any previous surgical interventions., (Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2020.)

Intussusception (IS) is defined as invagination of part of the bowel into an adjacent section of the intestines and is the second most common cause of small bowel obstruction in children. Postoperative ileoileal intussusception after surgical reduction is a special type of ileoileal intussusception and is considered idiopathic. This research paper examines the case of a female Syrian toddler who had been diagnosed with ileoileal intussusception after surgical reduction. Diagnosis of celiac disease was later histopathologically confirmed. Celiac disease (CD) may be the underlying cause for special types of intussusception that may be considered idiopathic, and further investigations, such as endoscopy, should be considered., (Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2020.)

Omphalopagus twins are one of many forms of conjoined twins sharing part of the gastrointestinal system and abdominal wall. This type of twins has the best chance of survival if successfully separated. Surgical approaches in these cases are generally preferably elective, but sometimes separation may be urgently needed due to life-threatening complications, such as hemodynamic instability, death of either twin, necrotizing enterocolitis, among many others. We report a case of successfully separated omphalopagus twins at day two of life., (Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2020.)

(1) Background: Malnutrition is prevalent in hemodialysis (HD) patients and is associated with an increased risk of morbidity and mortality. The aim of this study was to explore the prevalence of malnutrition using the malnutrition-inflammation score (MIS) and phase angle (PhA) and compare their concordance with the new Global Leadership Initiative on Malnutrition (GLIM) criteria for the diagnosis of malnutrition. (2) Methods: Seventy HD patients were assessed. Malnutrition was diagnosed based on the GLIM criteria and MIS questionnaire. The agreement between the diagnostic tools (MIS, PhA derived from the bioelectrical impedance analysis (BIA), and GLIM criteria) was assessed. The optimal gender-specific cutoff points were identified for the PhA according to the GLIM criteria. (3) Results: Almost half of the sample was diagnosed as malnourished according to the MIS (48.57%) and GLIM criteria (54.29%). A fair agreement was observed between the GLIM criteria, MIS (k = 0.202), and PhA (k = 0.279) among the malnourished patients. The PhA had better sensitivity but worse specificity compared to the MIS. The optimum cutoff points of PhA to detect malnutrition according to the GLIM criteria were a PhA value of ≤5.7° for males and ≤3.8° for females. (4) Conclusion: The MIS performed slightly better than PhA in the diagnosis of malnutrition among HD patients within the spectrum of the GLIM criteria.

Intradialytic exercise (IDE) is not routinely prescribed in hemodialysis (HD) units despite its potential benefits on patients' outcomes. This study was the first in the United Arab Emirates to examine the effect of aerobic IDE on hyperphosphatemia, malnutrition, and other health outcomes among HD patients. Participants were chosen from the largest HD unit in Sharjah Emirate for a quasi-experimental intervention with pre and post evaluation. The study lasted for 12 months. Study parameters were collected at baseline, post intervention, and follow-up. The intervention included a moderate-intensity aerobic IDE of 45 min per HD session; intensity was assessed using the Borg Scale. Patients were educated on the importance of exercise. Study outcomes were serum phosphorus (P), malnutrition inflammation score (MIS), quality of life (QOL), and pertinent blood tests. Forty-one eligible consenting HD patients were included in the study. Results at follow-up showed a non-significant reduction in P ( p = 0.06) in patients who were hyperphosphatemic at baseline, but not in the sample as whole. MIS did not deteriorate throughout the study ( p = 0.97). IDE resulted in a non-significant increase in the QOL visual analogue scale ( p = 0.34). To conclude, aerobic IDE for 45 min is safe and could be beneficial, especially for hyperphosphatemic patients.

Objective: Intradialytic exercise (IDE) is not yet a routine practice for hemodialysis patients, the lack of guidelines supporting it being a major reason. This systematic review and meta-analysis of aerobic IDE interventions examined the efficacy of IDE regarding quality of life (QOL), serum phosphorus, dialysis efficiency, inflammatory status, vitamin D3, parathyroid hormone, intake of phosphate binders, mortality and hospitalization rate., Methods: Pubmed, Medline (Ovid), Embase (Ovid), Cochrane, and Cinahl (EBSCO) databases were searched to retrieve studies up to June 12, 2018. A manual reference search was also performed. Studies were included if they evaluated (a) aerobic IDE effect on at least one of our study parameters, (b) adult hemodialysis patients, (c) patients for > 1 month., Results: Twenty-two studies were retrieved (706 participants), of which 12 were eligible for meta-analysis. Aerobic IDE had a significant positive effect on the QOL physical component score (QOL-PSC) and on mental component score (QOL-MCS) of SF36, but not on serum phosphorus or Kt/V., Conclusions: IDE incorporation into clinical practice has a significant positive effect on QOL-PSC and QOL-MCS. In the reviewed studies, IDE did not result in any health hazard in hemodialysis patients. Nevertheless, future research should assess the long-term effectiveness and safety of IDE. The limitations of this review include the lack of quality analysis of the studies, the limited number of studies that could be included in the meta-analysis, the diversity in the exercise intensity, duration and modality, and the limited data for several outcomes., Prospero Registration Id: CRD42016052062.

Background & Aims: Protein-Energy Wasting (PEW) is the depletion of protein/energy stores observed in the most advanced stages of Chronic Kidney Disease (CKD). PEW is highly prevalent among patients on chronic dialysis, and is associated with adverse clinical outcomes, high morbidity/mortality rates and increased healthcare costs. This narrative review was aimed at exploring the pathophysiology of PEW in end-stage renal disease (ESRD) on hemodialysis. The main aspects of nutritional status evaluation, intervention and monitoring in this clinical setting were described, as well as the current approaches for the prevention and treatment of ESRD-related PEW., Methods: An exhaustive literature search was performed, in order to identify the relevant studies describing the epidemiology, pathogenesis, nutritional intervention and outcome of PEW in ESRD on hemodialysis., Results and Conclusion: The pathogenesis of PEW is multifactorial. Loss of appetite, reduced intake of nutrients and altered lean body mass anabolism/catabolism play a key role. Nutritional approach to PEW should be based on a careful and periodic assessment of nutritional status and on timely dietary counseling. When protein and energy intakes are reduced, nutritional supplementation by means of specific oral formulations administered during the hemodialysis session may be the first-step intervention, and represents a valid nutritional approach to PEW prevention and treatment since it is easy, effective and safe. Omega-3 fatty acids and fibers, now included in commercially available preparations for renal patients, could lend relevant added value to macronutrient supplementation. When oral supplementation fails, intradialytic parenteral nutrition can be implemented in selected patients., (Copyright © 2016 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

We evaluated the utilization and potential benefits of recombinant human growth hormone (rhGH) in children with chronic kidney disease (CKD) and following renal transplantation in a large patient cohort. We queried the chronic renal insufficiency (CRI), dialysis, and transplant registries of the North American Pediatric Renal Trials and Collaborative Studies (NAPRTCS) to characterize the frequency of rhGH utilization, factors related to its usage, and the relationship between rhGH usage and catch-up growth. Data from 6,505, 5,122, and 4,478 CRI, dialysis, and transplant patients, respectively, was evaluated. Percentage utilization of rhGH 2 years after registry entry was 22%, 33%, and 3% in children with a height standard deviation score (SDS)<-1 and age<17 years (termed candidate group) in CRI, dialysis, and transplant patients, respectively. Multivariate logistic regression analysis showed that the likelihood of using rhGH was significantly correlated with age, gender, geographical region of residence and height category within the candidate group (p<0.01). The use of rhGH was associated with catch-up growth in 27%, 11%, and 25% of candidate CRI, dialysis, and transplant patients, respectively. In the candidate group, percentage catch-up growth was highest in children who were Tanner stage 1-2, who comprised 19.4%, 7.1%, and 25.5% of the CRI, dialysis, and transplant patients, respectively. Using multiple regression analysis, the estimated impact of rhGH on final adult height (age>19 years) was 0.80, 0.50, and 0.19 SDS, in CRI, dialysis, and transplant patients, respectively. Thus, rhGH can improve height gain in some children with CKD. The use of rhGH appears to be most effective in prepubertal children with CRI.

Despite recent advances in the management of children with chronic kidney disease (CKD), growth remains suboptimal. The purpose of this study was to evaluate factors associated with short stature in children with CKD. We evaluated the chronic renal failure registry of the North American Pediatric Renal Transplant Cooperative Studies (NAPRTCS) to determine the relations among primary diagnosis, age, race, residual renal function, acidosis, anemia, serum phosphorous, calcium, parathyroid hormone (PTH), albumin, and height at entry into the registry in children with CKD. A total of 5,615 patients were entered into the registry between January 1994 and January 2004. We found that older patients, those with glomerular filtration rate (GFR) >50 ml min(-1) 1.73 m(-2), black patients and patients with focal segmental glomerulosclerosis (FSGS) were at lower risk of being short at entry. Anemia (hematocrit below 33%) was an independent risk factor for short stature. Acidosis, serum phosphorous, calcium, albumin and PTH at registration were poor predictors of short stature. Age, race, primary diagnosis, and residual renal function were associated with short stature in children with CKD.

Osteogenesis imperfecta (OI) is a debilitating clinical condition characterized by fragile bone and skeletal deformity. Over the past decade frequent reports have suggested that the cyclical administration of intravenous pamidronate has a positive impact on bone density and skeletal fractures; however, the impact of such therapy on the quality of life (QOL) has rarely been reported. Alendronate, an oral bisphosphonate, is widely used to treat osteoporosis. The purpose of this study was to evaluate the impact of daily alendronate on QOL and bone parameters in children with OI. A prospective double-blind crossover study was designed in which placebo was alternated with daily alendronate. Twenty children with types I, III, and IV OI were recruited. Seventeen patients completed the study. Markers of QOL were measured in children with type III and IV OI (n = 15) using total mobility (PEDI), self-care (WeeFIM), well-being, pain, and use of analgesic scores. After 1 year of alendronate therapy, vertebral bone mineral density (BMD) improved from a change in standard deviation score (z-score) of 0.89 +/- 0.19 to -0.12 +/- 0.14 after 1 year of placebo (P < 0.001). All QOL markers, except for mobility score, improved in response to alendronate therapy. Change in height z-score also improved in response to 1 year of alendronate therapy (0.41 +/- 0.21 vs. -0.09 +/- 0.11, P < 0.05). Alendronate therapy did not alter serum levels of calcium, osteocalcin, parathyroid hormone (PTH), 1, 5 (OH)2 vitamin D, cholesterol, or urinary hydroxyproline or any other biochemical marker evaluated. Alendronate decreased by 56% urinary cross-linked N-telopeptide of type 1 collagen divided by urinary creatinine (uNTX/uCr). Daily alendronate therapy was well tolerated. Only two patients had mild gastrointestinal discomfort, responding to minor adjustments in alendronate intake. Daily alendronate therapy is safe and effective in improving QOL in children with OI.

The purpose of this communication is to study the clinical patterns and level of residual renal function at the initiation of dialysis in children in the United States of America (US). Data were reviewed for 7,039 children under the age of 20 years and 647,600 adults extracted from the patient incidence report, obtained from the United States Renal Data Systems (USRDS), who were initiated on dialysis between January 1995 and September 2002. Based on pre-defined exclusion criteria, only 4,808 of the 7,093 (67.8%) pediatric entries were included in the analysis. About 6.9% of the entries were not used because of missing data only, 23.3% because of out of range data only, and 2.0% because of both missing and out of range data, a total of 32.2% exclusions. For adults, 570,808 (88.1%) had acceptable data. The percentage of the 4,808 children who were initiated on dialysis with an estimated GFR greater than 10 mL min(-1) per 1.73 m(2) (early start) was 49.6%. Using logistic regression, the factors affecting the probability of an early start were sex, race, type of insurance, region of the country, age at initiation of dialysis, and the year dialysis was initiated. The highest chance of starting dialysis early (0.77) was for a white male, aged 15-19 years, with insurance and residing in the Northwest part of the US The percentage of 4,808 children who initiated dialysis with an estimated GFR less than 5 mL min(-1) per 1.73 m(2) (late start) was 7.3%. The factors affecting the probability of a late start were sex, race, type of insurance, and the year dialysis was initiated. The greatest chance for a late start of dialysis was for black female patients without insurance (0.21). Mean estimated GFR at the start of dialysis was higher for children than for adults (10.7+/-4.6 vs 8.2+/-4.1 mL min(-1) per 1.73 m(2), respectively, P<0.0001). It was concluded that patterns of management of children with ESRD are not uniform among US children.

Nutritional rickets is uncommon in North America, particularly in regions where sunlight is plentiful. Recent epidemics in North America occurred in dark-skinned toddlers with poor nutrition who had insufficient exposure to sunlight and whose parents were members of ethnic, social, and socioeconomic groups with predisposing practices. Nine children (8 toddlers and 1 infant) were referred to the Bone Metabolic Clinic at Texas Scottish Rite Hospital for suspected rickets between October 1997 and October 1998. The diagnosis of nutritional rickets was based on clinical, biochemical, and radiological evidence. All children were dark-skinned: 8 were African Americans and 1 was of Hispanic parentage. All children were breast-fed with minimal intake of dairy products; none received vitamin supplementation. All children were followed up by health care professionals. Two patients were children of upper-middle class parents. Birth order was not a contributing factor in the development of nutritional rickets. Radiological and biochemical rachitic changes remitted within 3 months of vitamin D therapy combined with dietary modification. Primary care providers should consider vitamin D supplementation in all infants with increased skin pigmentation and especially in those who are primarily breast-fed. Nutritional rickets can develop in dark-skinned infants of any social or ethnic background. Residing in a geographical area with abundant sunlight is not a guarantee against the development of nutritional rickets in dark-skinned children.

The clinicopathologic staging of colorectal cancer is the subject of recent debate. We studied morphologic variables in a series of tumors resected from 284 patients. Half had been prospectively, randomly allocated to receive a 4-day schedule of preoperative pelvic radiotherapy followed by immediate surgery. There was a significant (P less than 0.01) difference in the distribution of tumors of various histopathologic grades between irradiated (XS) and unirradiated (S) patients and borderline differences in the predictive values of venous spread, tumor grading, and local spread. However, these differences were less marked in 180 tumors examined by one review pathologist. They were thought to be due to misinterpretation of changes induced by radiotherapy. No differences were detected in the distribution of tumors of various sizes and Dukes' stage in the XS and S groups. The review pathologist recorded a borderline (P = 0.049) difference in the distribution of tumors of various CEA staining patterns between the XS and S groups. In a Cox regression model. Dukes' staging remained the most important predictive variable for survival and pelvic recurrence in the XS and S groups. Dukes' staging was apparently unchanged by this schedule of preoperative radiotherapy, but Broders' grading may be unreliable. Any new clinicopathologic staging system for colorectal cancer should record when preoperative radiotherapy is delivered. More studies of radiotherapy effects are required.

DNA extracted from 31 primary colorectal carcinomas was analyzed for the presence of ras gene amplification and mutations. Nine carcinomas had Ha-ras amplification and seven Ki-ras amplification. Nine carcinomas had codon 12 Ki-ras mutations. Immunohistochemical staining for ras proteins revealed a normal membrane association in normal mucosa and benign polyps but an abnormal cytoplasmic distribution in carcinomas. Amplification, mutations, and immunohistochemical staining were independent of histologic differentiation, Dukes' stage, or DNA ploidy status. This study demonstrates that abnormalities of ras genes are a common finding in colorectal carcinomas. They are potentially important biologic changes associated with malignancy, although they do not appear to be related to clinical behavior.