Zarife Kuloğlu

Introduction: Necrotizing enterocolitis (NEC) is predominantly a disease of premature infants and is rarely in older children. In immunocompromised patients, however, infectious enteritis may present with clinical and radiological findings resembling NEC. In this article, we present a pediatric liver transplant recipient who developed astrovirus‐induced gastroenteritis, pneumatous intestinalis, and portal venous gas and was successfully treated without surgery. Case Report: A 24‐month‐old girl presented with hematochezia 4 months after liver transplantation for hepatoblastoma while receiving tacrolimus, mycophenolate mofetil, and corticosteroid therapy. She had a recent history of diarrhea followed by abdominal distention and lower gastrointestinal bleeding. Imaging studies demonstrated pneumatosis intestinalis and portal venous gas. Blood and stool cultures, Clostridium difficile antigen testing, and respiratory pathogen panels were negative, whereas stool PCR identified astrovirus. Based on the clinical presentation and imaging findings, Bell stage II NEC was diagnosed. The patient was managed conservatively with bowel rest, total parenteral nutrition, broad‐spectrum antimicrobial therapy, and temporary adjustment of immunosuppressive treatment. Her bleeding resolved within 48 h, serial imaging showed regression of pneumatosis intestinalis, and she was discharged after 14 days with complete clinical recovery. Conclusion: Astrovirus infection should be considered among the potential causes of NEC‐like gastrointestinal manifestations in pediatric liver transplant recipients. Although pneumatosis intestinalis and portal venous gas often due to intestinal ischemia, clinically stable transplant recipients may recover with careful monitoring and individualized conservative treatment, avoiding unnecessary surgical intervention.

Background: Cytomegalovirus (CMV) remains a frequent infectious complication after pediatric liver transplantation despite antiviral prophylaxis. However, the clinical significance of CMV DNAemia and predictors of treatment-requiring CMV DNAemia remain incompletely defined. We evaluated the epidemiology, timing, clinical characteristics and predictors of CMV DNAemia in pediatric liver transplant recipients managed with a hybrid prevention strategy. Methods: We conducted a retrospective cohort study of consecutive pediatric liver transplant recipients who underwent transplantation between 2014 and 2025 at a tertiary center. CMV DNAemia was identified through routine quantitative polymerase chain reaction surveillance during antiviral prophylaxis and the subsequent preemptive monitoring period. Treatment-requiring CMV DNAemia was defined as CMV DNAemia prompting antiviral therapy based on viral load kinetics, absolute viral load and accompanying clinical findings. Demographic, transplant-related, clinical and virological factors were analyzed. Results: Fifty-one recipients were included. CMV DNAemia developed in 35 recipients (68.6%), accounting for 77 episodes. Breakthrough CMV DNAemia occurred in 22 recipients (62.8%), whereas 13 developed CMV DNAemia only after prophylaxis discontinuation. Twenty-eight episodes occurred during prophylaxis, but only 7 (25.0%) required treatment. Overall, 15 recipients experienced 25 treatment-requiring episodes, representing 32.5% of all CMV DNAemia episodes; 72% occurred after prophylaxis discontinuation. No patient developed tissue-invasive CMV disease. Younger age at transplantation (OR: 0.979; 95% CI: 0.963–0.995; P = 0.013) and pretransplant intensive care unit requirement (OR: 7.75; 95% CI: 1.44–41.84; P = 0.017) independently predicted treatment-requiring CMV DNAemia. An age threshold of ≤26 months showed acceptable discrimination (area under the curve, 0.773) and was associated with lower CMV-free survival ( P < 0.001). Conclusions: CMV DNAemia was common, but only one-third of episodes required antiviral treatment, and no tissue-invasive disease occurred. Younger recipients and those requiring pretransplant intensive care were at increased risk for clinically significant CMV infection. CMV DNAemia alone should not be considered a surrogate for clinically significant infection, supporting individualized, risk-adapted surveillance and prevention in pediatric liver transplant recipients.

Objectives: Strict exclusion of gluten is the cornerstone of celiac disease (CD) management; nevertheless, maintaining dietary adherence remains challenging. The COVID-19 pandemic may have influenced dietary adherence in children with CD. This study aimed to investigate the association between the COVID-19 pandemic and gluten-free diet (GFD) adherence and anthropometric measurements. Methods: This retrospective longitudinal cohort study with a cross-sectional survey component included 81 pediatric patients with CD (67.9% female; mean age 14.9 ± 3.4 years) who had been following a GFD for at least one year before the pandemic. Demographic, clinical, laboratory, and anthropometric data from the pre-pandemic, restriction, and post-pandemic periods were obtained from medical records. Dietary adherence was assessed via a structured telephone questionnaire. Participants reporting either complete avoidance of gluten or fewer than six inadvertent gluten exposures annually were categorized as self-reported adherent. Serological adherence was defined by a negative tTG-IgA result. Results: At the beginning of the pandemic in March 2020, the mean duration of GFD was 3.9 ± 2.4 years. At the time of the survey, self-reported adherence was 67.9% (55/81), whereas serological adherence was 77.1% (54/70). During the pandemic, 49.4% of participants reported no change in dietary adherence, 33.3% reported increased adherence, and 17.3% reported decreased adherence. Serological adherence differed significantly across the three longitudinal assessment periods (Cochran's Q = 31.818, p < 0.001). Compared with the pre-pandemic period (51.9%, 42/81), serological adherence was significantly higher during both the restriction period (82.7%, 67/81) and the post-pandemic period (76.5%, 62/81) (McNemar p < 0.001 for both comparisons), whereas no significant difference was observed between the restriction and post-pandemic periods (McNemar p = 0.227). Serological adherence was associated with home-cooking and selected family and socioeconomic factors. Most participants (87.7%) reported difficulty accessing gluten-free products, mainly due to high cost and limited variety. No significant differences were observed in anthropometric measurements across the study periods. Conclusion: Serological adherence was significantly higher during both the COVID-19 restriction and post-pandemic periods than during the pre-pandemic period, while no significant changes in anthropometric measurements were observed.

1. Characterized by the targeted destruction of pancreatic beta cells, type 1 diabetes mellitus (T1DM) is a chronic autoimmune disease that ultimately leads to permanent insulin deficiency [1–3]. C...

Background: Biliary atresia (BA) is the most common cause of liver transplantation (LT) in children. Despite a successful Kasai procedure, liver cirrhosis develops in half of the patients in the first 2 years of life. Hypersplenism secondary to cirrhosis causes cytopenias, immune dysregulation, and increased intra‐abdominal pressure. LT reduces hypersplenism complications due to normalized portal pressure. In cases with normal liver function but severe hypersplenism, interventions are needed before or simultaneous with LT, despite the possible negative effects of splenic interventions on graft perfusion and, thus, function. Methods: Here, we present a 23‐month‐old boy with BA and hypersplenism symptoms despite a normally functional liver. Using cases from the literature, we discussed the advantages and disadvantages of hypersplenism management in the peritransplantation period with our patient. Results: Interventions like splenectomy and splenic artery ligation (SAL) to reduce hypersplenism‐related complications can be performed in the pre‐ and/or post‐transplantation period. Conclusions: Necessary interventions should be personalized for each patient according to their clinical conditions and possible issues.

To determine the frequency of malnutrition, including undernutrition and overnutrition, in pediatric patients with familial Mediterranean fever (FMF), and to assess the impact of colchicine treatment on nutritional outcomes and factors associated with malnutrition. The medical records of patients with FMF who were followed up in our pediatric rheumatology clinic between 2011 and 2024 were reviewed. Patients were divided into 3 groups according to their nutritional status: normal, undernutrition and overnutrition. Demographic and clinical characteristics and disease severity were compared before and after treatment. The international severity scoring system for FMF (ISSF) was used to assess disease severity. This retrospective study included 532 (50.6% female) pediatric patients with a median (IQR) age at diagnosis of 6.5 (6) years. Malnutrition was detected in 32.7% of patients at the time of diagnosis, after colchicine treatment this rate declined to 20.7%, significant reductions in undernutrition (from 23.5% to 12.8%) and marginal improvements in overnutrition (from 9.2% to 7.9%) (p < 0.001) groups were observed. The ISSF was significantly higher in both the undernutrition and overnutrition groups compared to patients with normal nutritional status before and after colchicine treatment (p < 0.001). A severe disease phenotype and malnutrition at the time of diagnosis were identified as significant risk factors for persistent malnutrition (p < 0.001). Malnutrition, including undernutrition and overnutrition, is a common condition in pediatric FMF patients that can be improved with colchicine. The presence of malnutrition at diagnosis and a more severe disease phenotype seemed to be the most important determinants of persistent malnutrition.

Objectives: Wolcott–Rallison syndrome (WRS) is an autosomal recessive multisystemic genetic disorder caused by homozygous mutation in the eukaryotic translation initiation factor 2 alpha kinase 3 gene. It typically presents with neonatal onset insulin-dependent diabetes. Here, we report a 14-year-old male patient with WRS who presented with late-onset diabetes mellitus. Case presentation: A 14-year-old male patient presented with acute liver failure secondary to acute gastroenteritis. On physical examination, signs of chronic malnutrition and muscle atrophy in the extremities were evident, accompanied by a waddling gait. Infectious, autoimmune, and metabolic diseases were excluded. Liver tests improved within 3 weeks with supportive treatment. Direct radiographs were consistent with multiple epiphyseal dysplasia. High blood glucose levels were observed at follow-up in the intensive care unit. At the 4-month follow-up, HbA1c increased to 55 mmol/mol (7.2 %) and basal insulin treatment was started at a dose of 0.4U/kg/day. Glycemic control was achieved after 6 months. Conclusions: Diabetes typically manifests within the first 6 months of life, with a median age of 2.5 months, and has been observed in all patients with WRS reported in the literature. Our case is an interesting WRS patient whose diabetes started at the age of 14 years with a novel mutation.

Transient infantile hypertriglyceridemia is one of the diseases that should be considered in case of unexplained elevated liver enzymes, hypertriglyceridemia and hepatosteatosis. We report 2 siblings with novel homozygous variants in the GPD1 gene with transient infantile hypertriglyceridemia. Two siblings born from consanguineous marriage were referred due to hepatomegaly, elevated transaminases and fatty liver. After excluding other possible causes of fatty liver and elevated transaminase levels; whole-exome sequencing (WES) was performed on genomic DNA isolated from the peripheral blood samples of both patients. Whole exome sequencing revealed the identification of a novel homozygous variant, c.628 G > C:p.G210R, in GPD1. Our report underscores the importance of genome sequencing in diagnosing unexplained childhood fatty liver disease and/or elevated enzyme levels. In patients with transient infantile hypertriglyceridemia, investigation into novel homozygous variants in the GPD1 gene should be conducted using whole exome sequencing.