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BackgroundCongenital chloride diarrhea (CCD, OMIM 214700) is a rare autosomal recessive disorder caused by loss-of-function mutations in the SLC26A3 gene (SLC26A3; OMIM* 126650), which encodes a protein that exchanges chloride and bicarbonate on enterocyte apical surface. 1, 2 Patients usually have a history of antenatal polyhydramnios and may experience dilated intestinal loops, watery stool, dehydration, hypochloremic hypokalemic metabolic alkalosis, and high fecal chloride excretion. 3 The presence of abdominal distention, absence of meconium, and enlarged intestinal loops may lead to misdiagnosis of meconium ileus or Hirschsprung’s disease, resulting in unnecessary surgical procedures. 4 However, patients with CCD may also be misdiagnosed as Bartter and Pseudo Bartter syndrome due to the similar laboratory findings.

Objectives: Vitamin D deficiency is common in pediatric chronic liver disease despite oral replacement. We evaluated vitamin D deficiency before and after liver transplant and the relationship between posttransplant and pretransplant vitamin D deficiency and graft rejection.Materials and Methods: Pediatric recipients with chronic liver disease (N= 138) were divided into 4 groups: cholestatic liver diseases, cirrhosis, metabolic disorders, and acute liver failure. Pretransplant and posttransplant vitamin D levels, liver function tests, Pediatric End-Stage Liver Disease scores, rejection activity index scores by graft liver biopsy, and posttransplant patient survival were recorded.Results: There were 62 (45%) female and 76 (55%) male participants (mean transplant age, 6.1±5.6 years). Pretransplant mean available vitamin D of 90 patients was 25.2±20.9 ng/mL, with 36 (40%) within reference range. Posttransplant level for 109 patients was 27.3±18 ng/mL, with 64 (58.7%) within reference range. Pretransplant and posttransplant levels were available for 61 patients, and mean pretransplant levels were lower than posttransplant levels (23.7±19.3 vs 28.3±16.9 ng/mL; P=. 01). Patients with cholestatic liver disease had lower pretransplant vitamin D levels (P=. 04), which disappeared after transplant. Pretransplant vitamin D levels were positively correlated with serum albumin levels (r= 0.20) in all patients and negatively correlated with total/direct bilirubin (r= 0.29 and r=-0.30) in those with liver diseases and cirrhosis. No correlations were found between pretransplant vitamin D levels and Pediatric End-Stage Liver Disease scores, rejection activity index scores …

IntroductionWe aim to evaluate of the relationship between high mobility gene box-1 (HMGB1) levels and clinical, laboratory and histopathological findings at diagnosis and in remission in children with Celiac Disease (CD).Material and MethodsThe study included 36 celiac patients at diagnosis, 36 celiac patients in remission, and 36 healthy controls. Patients with intestinal pathologies other than CD, and accompanying inflammatory and/or autoimmune diseases were excluded. Relationship between HMGB1 levels and clinical, laboratory and histopathological findings were evaluated.ResultsA total of 72 celiac patients [36 (18 girls, 18 boys, mean age 9.41±3.9 years) in group 1 and 36 (18 girls, 18 boys, mean age 9.91±3.36 years) in group 2] and 36 healthy controls in group 3 (19 girls, 17 boys, mean age 9.56±4 years) were included. The HMGB1 level was significantly higher in group 1 compared to group 2 and …

Purpose Anti-TNF drugs increase the risk of tuberculosis. In this study we aimed to investigate the incidence of tuberculosis in patients using anti-TNF drugs. Methods One hundred and fifteen pediatric cases which were received anti-TNF drugs were included in the study. The clinical and epidemiological characteristics of the cases were analyzed retrospectively. Results One hundred and fifteen cases using anti-TNF drugs were included in the study. The diagnoses of the cases were as follows; Juvenile Rheumatoid Arthritis 76 (66%), Ulcerative Colitis 11 (9.6%), Crohn's 7 (6%), Ankylosing Spondylitis 6 (5.2%), FMF 5 (4.3%), Psoriasis 4 (%3.5). The distribution of the agents used by the patients was; etanercept 74 (64.3%), infliximab 17 (14.8%), adalimumab 17 (14.8%), anakinra 5 (4.3%), and canakinumab 2 (%1.7). It was learned that all cases had BCG vaccinations when they were two months old, confirmed by the vaccination cards and the ministry of health's vaccination follow-up system. TST was performed in all of the cases and TST response was measured as <5mm in 89 (77.4%), 5-9 mm in 11 (8.7%), 10-14 mm in 8 (7.4%), >15 mm in 7 (5.6%) cases. Isoniazid (INH) prophylaxis was started for nine months in 17 cases with the diagnosis of latent tuberculosis. Active tuberculosis was not detected in any of the cases. Conclusion All patients receiving anti-TNF need to be evaluated for tuberculosis. Although it is not detected at the beginning of the treatment, regular tuberculosis screening should be continued during the treatment with contact history, symptoms, physical examination, chest X-ray, and TST/IGRA in light of current guidelines.

Gastric (foveolar) metaplasia (GM) in the duodenum occurs in many cases where duodenitis develops histologically. However, few studies have investigated the presence of GM in celiac patients. This study aimed to determine the prevalence of GM and its relationship with clinicopathological parameters in untreated pediatric celiac patients. Duodenal biopsy specimens of 153 pediatric cases were analyzed. MUC5AC immunohistochemical staining was applied to all cases to detect GM. The patient group was evaluated in terms of the disease activity score (DAS), neutrophilic activity score (NAS), eosinophilic infiltration, and presence of intramucosal Brunner glands. The rate of GM was 53.4% in the control group and 98.8% in the patient group. GM was observed as diffuse morphology at a rate of 4.1% (n:3) in the control group and 55% (n:44) in the patient group (p<0.001). Diffuse type of GM was more frequent in girls in the patient group (p=0.03). When GM (focal/diffuse) age, DAS, NAS, and all histopathological parameters were compared, a statistically significant difference was found between diffuse GM and DAS, and NAS. (p=0.023 and p=0.039, respectively). The present study considered that duodenal neutrophilia and increased disease activity might play a role in the development of GM in celiac patients. We think that it would be appropriate to include a diffuse type of GM, which can be easily detected by immunohistochemical or histochemical methods, in pathology reports as a finding of histologically active celiac disease.

Wilson disease (WD) is an autosomal recessive disorder of copper metabolism. Affected children may be asymptomatic, makes the diagnosis more difficult. In this study, we aimed to evaluate the clinical, laboratory, histopathological and genetic characteristics, and outcomes of the patients with WD. Our study includes patients who were diagnosed with WD between January 2010 and December 2020. The presenting complaints, physical examination findings, consanguinity and family history, laboratory, genetic, histopathological evaluation results, treatment and outcomes were all recorded. A total of 21 patients from 18 families [median age 9.5 (1-14) years, 10 girls] were included. Kayser-Fleischer ring was detected in 11 (52.4%) patients. Serum ceruloplasmin (<20 mg/dl) was low in 15 patients. Urinary copper excretion was >100 µg/day in 17 patients. Copper was positively stained with rhodanine in 9 of the18 liver biopsies. Liver copper content was >50 µg/g dry weight in all patients, 50-250 µg/g in 3 patients and >250 µg/g in 15 patients. Genetic evaluation was available in 18 patients and revealed heterozygous mutations in the ATP7B gene in 4 patients, combined heterozygous mutations in 6, and homozygous mutations in 8. Except for two patients with neurological findings and three asymptomatic patients who were diagnosed by family screening, all were presented with liver findings. Neurological involvement was also detected in 2 patients during follow up. D-Penicillamine and zinc sulfate combined treatments were used in 16 patients, zinc sulfate monotherapy was given to a presymptomatic patient diagnosed with family screening, and …

Introduction: Treatment with biological agents, which are classified as monoclonal antibodies, cytokines and fusion proteins, increases patient risk for developing tuberculosis (TB) and non-tuberculous mycobacterial infections. In this study, we aimed to investigate the risk of tuberculosis development in pediatric patients using anti-TNF drugs.Method: One hundred and fifteen pediatric cases who were followed up in Eskişehir Osmangazi University Faculty of Medicine, Department of Pediatrics between January 2011 and December 2021 and received anti-TNF and biological agent treatment were included in the study. The clinical and epidemiological characteristics of the cases were analyzed retrospectively.Results: One hundred and fifteen cases using anti-TNF drugs were included in the study. The mean age of the cases was 13 (2-18) years. Of the cases, 66 (57%) were female and 49 (43%) were male. Of the cases, 76 (66%) had Juvenile Rheumatoid Arthritis, 11 (9.6%) Ulcerative Colitis, 7 (6%) Crohn's, 6 (5.2%) Ankylosing Spondylitis, 5 (% 4, 3) FMF and 4 of them were followed up due to Psoriasis (3.5%). Etanercept in 74 (64.3%) cases, infliximab in 17 (14.8%) cases, adalimumab in 17 (14.8%) cases, anakinra in 5 (4.3%) cases, and canakinumab was using in 2 (% 1.7) cases. PPD was performed in all of the cases and PPD response was measured as< 5mm in 89 (77.4%), 5-9 mm in 11 (8.7%), 10-14 mm in 8 (7.4%),> 15 mm in 7 (5.6%) cases. Isoniazid (INH) prophylaxis was started for 9 months in 17 cases with the diagnosis of latent tuberculosis. Active tuberculosis was not detected in any of the cases.Conclusion: All patients who are …

Akut karaciğer yetmezliği (AKY), yaşa göre değişen nedenlerle oluşan, nadir görülen fakat hayatı tehdit eden bir durumdur. Daha önce bilinen kronik karaciğer hastalığı olmayan hastalarda, karaciğer fonksiyonlarında bozulma ile birlikte, K vitamini ile düzeltilemeyen koagulopati ve ensefalopati varlığında'international normalized ratio'(INR)'nun 1, 5'in üzerinde, ensefalopatisi olmayanlarda INR'nin 2'nin üzerinde saptanması AKY olarak tanımlanır. Yenidoğan ve bebeklerde enfeksiyonlar ve metabolik hastalıklar en sık nedenleri oluşturmakta iken, daha büyük çocuklarda viral hepatitler ve ilaca bağlı karaciğer hasarı daha sık görülmektedir. Hastaların önemli bir kısmında ise neden bulunamamaktadır. Genellikle bulantı, halsizlik, iştahsızlık, ateş gibi özgül olmayan bulgularla başlamakla birlikte, hastalarda sarılık, koagulopati ve ensefalopati gelişmektedir. Hastalık nadir görüldüğü, şiddetli ve hızlı seyrettiği için …

Inlet patch (IP) is an area of heterotopic gastric mucosa located in proximal esophagus. Although the majority of IP are asymptomatic, they may be associated with digestive and respiratory symptoms. We aimed to assess prevalence, endoscopic and histopathological findings, clinical significance and outcome of inlet patch in children.The patients with histopathologically proven IP and aged between 0-18 years old were enrolled. Demographic data, clinical symptoms, endoscopic and histopathological findings, treatment modality, and outcomes were collected from medical records. Retrospective review of 2674 esophagogastroduodenoscopy records revealed 11 (0.41%) children. Eight of our patients had a solitary patch whereas others had two (n=1) or three (n=2). Histopathological evaluation revealed that 9 patients had fundic and 2 patients had antral type gastric mucosa. One patient with hematemesis and other with dysphagia had hyperemic patchy areas of which were colonized by H. pylori. Inlet patch was the only pathological endoscopic finding in 4 patients with a single symptom each: heartburn, dysphagia, hematemesis and hoarseness. Symptoms were completely resolved with PPI treatment in 8 children. Helicobacter pylori eradication was achieved in all infected patients. No respiratory symptom was recorded except hoarseness in one patient. No complications like perforation, stenosis or dysplasia that might be related to IP were recorded at follow-up. We suggest that an IP may accompany or may be responsible for digestive symptoms in children and PPI treatment is effective. Endoscopist should be aware of this condition, especially …

Hastalar tarafından iyi tolere edilebilen, yeterli bir bağırsak temizliğinin uygulanabilmesi, iyi kalitede ve güvenilir bir kolonoskopi yapılabilmesi açısından önem taşımaktadır. Çalışmamızda, çocuk hastalarda polietilen glikol (PEG) gibi ozmotik bir bağırsak temizleyici ile daha az kullanılan sodyum fosfat (SF) gibi uyarıcı bir ajanın etkinliğini ve kullanılabilirliğini karşılaştırmayı amaçladık. Hastanemizde, Ocak 2016 ve Aralık 2019 yılları arasında kolonoskopi yapılmış ve kolonoskopi hazırlık rejimi olarak PEG veya SF kullanılmış olan çocuk hastalarımızın kayıtlarını geriye dönük olarak değerlendirdik. Bağırsak temizliğinin kullanılabilirliği (tolerabilite), ilacın kullanım kolaylığı ve karın ağrısı, bulantı, kusma, baş dönmesi gibi yan etkileri ve bunların şiddetini sorgulayan bir anket ile değerlendirildi. Bağırsak temizliğinin kalitesi, temizliği ve sıvı miktarını değerlendiren Ottawa ölçeği ile belirlendi. Ottawa ölçeği, kolonoskopi video kayıtlarından kör bir şekilde ve geriye dönük olarak ölçüldü. Ortalama yaşları 12, 3±4, 2 yaş olan toplam 145 hasta (65 erkek,% 44, 8) çalışmaya alındı. Doksan üç (% 64, 1) hastada PEG, 52 hastada (% 35, 9) SF kullanılmıştı. Hastaların ortalama yaşları PEG grubunda (11, 2±4, 6 yaş) SF grubuna (14, 2±2, 7 yaş) göre anlamlı olarak düşüktü (p< 0, 0001). Bağırsak temizliğinin kullanılabilirliği değerlendirildiğinde, PEG’nin, SF kullanan hastalara göre anlamlı olarak daha iyi tolere edildiği görüldü (sırasıyla, 49/93 hasta (% 52, 7) ve 14/52 hasta (% 26, 9), p= 0,003). Yan etki oluşan hastalarda semptom şiddeti değerlendirildiğinde, SF grubunda, PEG grubuna kıyasla orta-ağır şiddetteki etkilerin daha sık oluştuğu gözlendi (sırasıyla, 15/52 …

Giriş ve Amaç Üst gastrointestinal sistem kanaması çocukluk çağında çoğunlukla hafif olmakla birlikte, hayatı tehdit eden ciddi kanama şeklinde de görülebilmektedir. Bu çalışmada klinik olarak anlamlı üst gastrointestinal sistem kanamasına işaret eden bulguların ve risk faktörlerinin belirlenmesi amaçlandı. Gereç ve Yöntem Çalışmaya üst gastrointestinal sistem kanaması tanısı alan, 0-18 yaş aralığında çocuklar alındı. Tanı anındaki yaşı, cinsiyeti, kanama miktarı, hematemez, melena varlığı, başvuru anındaki yakınmaları, eşlik eden hastalıkları, kanamaya yatkınlık yaratan ilaç kullanımı, vital bulguları, kapiller dolum zamanı ve sistemik fizik muayene bulguları kaydedildi. Laboratuvar tetkiklerinden hemogram, biyokimya, koagülasyon testleri, endoskopik işlem bulguları, eritrosit transfüzyonu sayısı, uygulanan medikal ve/veya endoskopik tedaviler, acilde ya da serviste izlemleri ve kanama açısından konulan son tanısı kaydedildi. Sheffield skorlamasına göre 8 puan ve üzeri alanlar anlamlı üst gastrointestinal sistem kanaması olanlar olarak gruplandı, veriler gruplar arasında karşılaştırıldı. Bulgular Elli beş çocuk [29 (%52.7) kız, 26 (%47.3) erkek; ortalama tanı yaşı 8.4±5.4 yıl] çalışmaya alındı. Başvuru anında 22 hastada anemi, 20 hastada kan üre azotu yüksekliği, 14 hastada eritrosit sayısında düşüklük, 5 hastada hipoalbüminemi vardı. Anlamlı kanaması olan 17 hastada; melena (%76.5 vs. %21.1, p <0.001), solukluk (%52.9 vs %5.3, p <0.001), splenomegali (%23.5 vs %2.6, p <0.001), özofageal varis (%23.5 vs. %2.6, p=0.02), bolus sıvı (%41.2 vs. %5.3, p <0.001) ve transfüzyon gereksinimi (%70.6 vs. %5.3, p <0.001) daha sık, kalp hızı (137.4±22 …

ObjectivesIn this retrospective study, we aimed to determine the most common infectious agents in infants within the first 6 months of liver transplant.Materials and methodsThirty-four infant patients with median age of 8 months (range, 4-12 mo) at the time of liver transplant were retrospectively evaluated. We evaluated causative organisms in bloodstream cultures and in subclavian catheter, urine, and intra-abdominal drainage fluid cultures. We also evaluated Epstein-Barr and cytomegalovirus infections by polymerase chain reaction in all recipients.ResultsThe most common isolated bacteria from the bloodstream were Klebsiella pneumoniae, Staphylococcus epidermidis, and Enterococcus faecium. Staphylococcus epidermidis was the most common isolated bacteria from subclavian catheter cultures. Klebsiella pneumoniae was the most common bacteria isolated from intra-abdominal drainage fluid. Only 1 recipient had cytomegalovirus infection during this period.ConclusionsOur study showed a high incidence of Klebsiella pneumoniae infections in infants after liver transplant. New prophylactic antibiotic strategies can be promoted to prevent Klebsiella pneumoniae infections in infants.