Ceyda Tuna Kırsaçlıoğlu

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.

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...

Objective: This study aimed to evaluate early pulmonary complications (EPCs) and factors affecting mechanical ventilation duration in paediatric patients following liver transplantation (LT). Methods: Between June 2014 and June 2024, 56 paediatric patients who underwent LT at our centre were retrospectively included. Pre‐ and post‐transplant clinical features, nutritional status, surgical details, and respiratory outcomes were analysed. Patients were compared according to the presence or absence of EPCs. Results: Among the 56 patients (53.6% male, median age 26 months [IQR: 11.25–120]), EPCs 9 in 71.4% of patients, with atelectasis being the most frequent (58.9%), followed by pleural effusion (37.5%) and pneumonia (33.9%). The median postoperative PICU stay was 10.5 days (IQR: 5.25–20.75), and 17.9% of patients died within 3 months, none due to acute pulmonary complications. Patients with EPCs had significantly longer PICU stays (13.5 [7–25] vs. 5.5 [3.5–11.8] days, p = 0.007), higher reintubation rates (40% vs. 6.3%, p = 0.022), and longer durations of invasive mechanical ventilation (IMV) (5.5 [1–14.5] vs. 2 [1–4.5] days, p = 0.044). Underweight status ( p = 0.038), history of lower respiratory tract infection (LRTI) before LT ( p = 0.047), and postoperative surgery within 1 month ( p = 0.002) were associated with prolonged IMV. Use of noninvasive ventilation (NIV) post‐LT was associated with a shorter IMV duration ( p = 0.011). Conclusions: Early pulmonary complications are common following paediatric LT and are associated with increased respiratory morbidity and longer PICU stays. Undernutrition, prior LRTI, and early postoperative surgery are risk factors for prolonged IMV. Early recognition and management strategies may improve respiratory outcomes in this population.

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.

Introduction: Rheumatological diseases (RD) in childhood are systemic diseases that occur on the basis of auto-immunity or inflammation, and they can be accompanied by inflammatory bowel disease (IBD). When there is no knowledge of this association, the treatments applied may not be sufficient and/or treatments given for RD may even lead to aggrevation of IBD findings. Thus, early identification of an association is crucial for the correct management of the diseases. Objectives: The aim of this study is to show the frequency of IBD in patients with RDs. We also aimed to investigate in which cases IBD should be suspected in children with RDs. Methods: Electronic medical records of the patients who were followed up between 2012 and 2024 with a diagnosis of RD in our Paediatric Rheumatology Unit and diagnosed with IBD were reviewed retrospectively. Results: Between 2012 and 2024, 20 (3%) of 650 familial Mediterranean fever (FMF) patients, 3 (7.5%) of 40 chronic nonbacterial osteomyelitis (CNO) patients (one of them also had FMF) and 2 (1.2%) of 170 juvenile idiopathic arthritis (JIA) patients were diagnosed with IBD. While 15 (62.5%) of the patients received a RD as the initial diagnosis, 9 (37.5%) of them were initially diagnosed with IBD and then referred to rheumatology for their symptoms. The median age at the diagnosis of RD was 9 years (inter quartile range (IQR), 14.5). The median age at the diagnosis of IBD was 12 years (IQR, 13), and 12 patients (50%) had Crohn’s disease (CD), 10 patients (41.6%) ulcerative colitis (UC) and 2 patients (8.4%) undeterminated disease. Although majority of the patients had classical findings of IBD, 4 patients presented with more vague symptoms including treatment-resistant iron deficiency anaemia, perianal abscess, weight loss and growth retardation. Conclusions: RD and IBD share similar pathological pathways and clinical findings, and IBD can accompany to various RD. The diagnosis of IBD should be considered in the presence of rare and atypical symptoms. Furthermore, RD should also be considered in children with IBD who have complex extraintestinal symptoms. Key Points• RD, especially FMF, JIA and CNO, may be associated with IBD.• Paediatric rheumatologists should consider IBD as a potential diagnosis in the presence of atypical findings that may develop during the course of RD.• The co-existence of RD and IBD is important both in terms of disease progression and treatment decisions.

Objective: Despite varying presentations of acute pancreatitis (AP) in children, the diagnosis has been increasing in recent years due to increased awareness. We aimed to identify the relationship among etiology, presentation symptoms, treatment response and complications of the children with acute pancreatitis. Material and Methods: Thirty children diagnosed with AP were evaluated for clinical and laboratory findings, treatment approaches, complications in the tertiary children hospital retrospectively. Results: The mean age of the patients was 12.4 ± 4.3 years and 60% were male. The drugs (30%), biliary tract diseases (26.7%), infections (16.7%), hyperlipidemia (10%) were the main causes of AP, in 10% of patients no etiological factor was detected. Abdominal pain (83.3%), nausea (70%), loss of appetite (63.3%), vomiting (56.7%), and fever (20%) were the most common symptoms. Ultrasonography, abdominal tomography and magnetic resonance cholangiopancreatography revealed pancreatitis related changes 63.3%, 85%, 70% of patients, respectively. Oral feeding was started on median 4 days (1-30), with polymeric diet (30%), and medium chain triglyceride rich enteral diet (70%). The median length of hospitalization (LOH) was 16.5 days (4-66). The patients fed with polymeric diet had a shorter hospitalization duration (p<0.036). The delayed initiation of oral feeding caused longer LOH (p<0.001). Conclusion: Consequently, this study underlines the children with acute abdominal pain, especially who use drugs like asparaginase and valproic acid, or that are known to have gallstone/biliary sludge, need to be examined for acute pancreatitis through pancreatic enzymes and ultrasonography. Moreover, the study also highlights that early feeding in acute pancreatitis is related with shorter hospitalization duration.

Aims: Diagnosis of coeliac disease (CD) with mild mucosal changes is difficult for all parties involved. We aimed to determine the power of T cell receptor (TCR)γδ⁺ intra‐epithelial lymphocytes (IELs) in discriminating CD from other causes of intra‐epithelial lymphocytosis using a new monoclonal antibody.Methods: A total of 167 cases categorised as coeliac (117 untreated CD, classified according to Marsh, updated by Ensari, including 29 type 1, 29 type 2, 39 type 3 and 20 treated CD), and non‐coeliac groups (24 controls and 26 non‐coeliac IELosis) based on clinical, serological and histological data were studied for IEL counts enumerated per 100 enterocytes using haematoxylin and eosin, CD3, TCR δ‐stains.Results: TCRγδ⁺ IELs were significantly higher in CD (24.83 ± 16.13) compared to non‐CD (6.72 ± 6.32) and were correlated with the degree of mucosal damage. Both γδ⁺ IEL count and ratio showed higher performance in differentiating untreated coeliacs from controls, with a sensitivity of 83.76; 85.57 and specificity of 95.83; 79.17, respectively. TCRγδ⁺ IEL counts distinguished type 1 CD (20.41 ± 13.57) from non‐coeliac IELosis (9.42 ± 7.28) (p = 0.025). Discriminant analysis revealed that villus/crypt ratio, γδ⁺ and CD3⁺ IEL counts, γδ⁺/CD3⁺IEL ratio, IEL distribution pattern were potent discriminants and correctly classified 82.3% of cases while the algorithm accurately diagnosed 93.4% of cases.Conclusions: The new antibody detecting γδ⁺ IELs in FFPE sections revealed thresholds of 10.5 for γδ⁺ IELs and 14% for γδ⁺/CD3⁺IEL ratio which distinguished coeliacs from non‐coeliacs with high sensitivity and specificity, particularly in cases with normal villus/crypt axis including type 1 CD, non‐CD IELosis and controls. A ‘coeliac algorithm’ based on γδ⁺ IELs is proposed with the hope that it will be used in the histopathological diagnostic approach by the pathology community.

The monogenic causes of very-early-onset inflammatory bowel disease (VEO-IBD) have been defined by genetic studies, which were usually related to primary immunodeficiencies. Receptor-interacting serine/threonine-protein kinase-1 (RIPK1) protein is an important signalling molecule in inflammation and cell death pathways. Its deficiency may lead to various clinical features linked to immunodeficiency and/or inflammation, including IBD. Here, we discuss an infant with malnutrition, VEO-IBD, recurrent infections and polyathritis who has a homozygous partial deletion in RIPK1 gene.