Long-term results of liver transplantation for maple syrup urine disease: A single-center experience in Turkey.

Objectives: Maple syrup urine disease (MSUD) is an autosomal recessive inherited disorder. Despite the advances in medical nutrition therapies, classical phenotype causes severe neurological disorders and sudden death. It is known that MSUD patients do not experience metabolic attacks despite their free diet after liver transplantation (LT).

Hearing evaluation with ABR in pediatric patients with celiac disease.

Background/aims: Celiac disease (CD) is an autoimmune and genetic disease that is triggered by gluten intolerance. We aimed to investigate whether Celiac disease have any effect on Auditory Brainstem Response (ABR) waves compare to a healthy control group, and present its association with sensorineural hearing loss (SNHL).

Materials And Methods: Thirty-eight patients aged 2 to 16 years old were included in the study.

Role of Soluble Adiponectin Receptor 2 in Non-Alcoholic Fatty Liver Disease in Children.

Purpose: The incidence of non-alcoholic fatty liver disease (NAFLD) in children is gradually increasing. The aim of this study was to investigate the use of serum adiponectin and soluble adiponectin receptor 2 (soluble Adipo R2) levels for the diagnosis of fatty liver disease in obese and overweight children.

Methods: The study included 51 obese and overweight children between the ages of 6 and 18 years diagnosed with NAFLD using ultrasonography and 20 children without fatty liver disease.

Investigation of the Efficacy of Lactobacillus rhamnosus GG in Infants With Cow's Milk Protein Allergy: a Randomised Double-Blind Placebo-Controlled Trial.

This investigation has been performed to show the efficacy of Lactobacillus rhamnosus GG (LGG) together with milk-free diet in patients with cow's milk protein allergy (CMPA). This multicentre prospective investigation has been performed in 0-12 months of age children diagnosed as CMPA clinically and biochemically. Patients have been randomly divided in to two groups.

Liver transplantation from a live donor to a patient with maple syrup urine disease: Two case reports.

Liver transplantation were reported in patients with classic maple syrup urine disease in the literature. Branched chain alpha keto acid dehydrogenase activity can be improved in patients after transplant, and a protein-restricted diet is usually not needed. The first patient was a boy aged 2,5 years who presented with frequent ketosis attacks and epileptic seizures, and the second patient was an 11-month-old boy who also presented with frequent ketosis episodes, both despite adherence to diet therapy.

Gastritis Associated with Initially Pediatric Crohn's Disease and Ulcerative Colitis.

Purpose: The aim of this study is to determine the involvement of the upper gastrointestinal system (GIS) in patients diagnosed with Crohn's disease (CD), ulcerative colitis (UC), and non-inflammatory bowel disease (IBD) and to compare their differences.

Methods: This study included patients aged between 2 and 18 years who underwent colonoscopy and esophagogastroduodenoscopy (EGD) for the first time due to the prediagnosis of IBD. In EGD, samples were taken from duodenum, antrum, corpus, and esophagus; and gastritis, duodenitis, and esophagitis were identified through histopathologic examination.

The incidence of HLA-DQ2/DQ8 in Turkish children with celiac disease and a comparison of the geographical distribution of HLA-DQ.

Introduction: Celiac disease (CD) is an auto-immune enteropathy that occurs in genetically pre-disposed people as a result of the consumption of gluten-containing foods.

Aim: To identify the incidence of HLA-DQ2 and HLA-DQ8 observed in children with CD.

Material And Methods: In this study, we focused on children ranging in age from 2 to 18 years and diagnosed with celiac disease.

Investigation of Efficacy of Lidocaine Spray for Sedated Esophagogastroduodenoscopy in Children.

Purpose: Our aim in this study is to investigate efficacy of topical lidocaine spray for sedated esophagogastroduodenoscopy (EGD) in children.

Methods: The endoscopy of children aged between 3-18 years who underwent EGD in our endoscopy unit. Intravenous (IV) midazolam and ketamine were used for sedation.

Investigation of the efficacy of synbiotics in the treatment of functional constipation in children: a randomized double-blind placebo-controlled study.

Background/aims: The aim of this study was to demonstrate the efficacy of synbiotic (Lactobacillus casei, L. rhamnosus, L. plantarum, and Bifidobacterium lactis and prebiotics [fiber, polydextrose, fructo-oligosaccharides, and galacto-oligosaccharides]) treatment in children with functional constipation.

Efficacy and safety of midazolam and ketamine in paediatric upper endoscopy.

Background And Study Aim: Upper endoscopy can be successfully carried out in children under deep sedation and anaesthesia. However, the best method of upper endoscopy for children who require gastrointestinal intervention has yet to be defined. The aim of this study is to investigate the efficacy and safety of the sedation induced by intravenous midazolam and ketamine during upper endoscopy in children.

Pediatric Liver Transplantation: Our Experiences.

Objective: The aim of our study was to evaluate our liver transplant pediatric patients and to report our experience in the complications and the long-term follow-up results.

Materials And Methods: Patients between the ages of 0 and 18 years, who had liver transplantation in the organ transplantation center of our university hospital between 1997 and 2016, were included in the study. The age, sex, indications for the liver transplantation, complications after the transplantation, and long-term follow-up findings were retrospectively evaluated.

Efficacy of synbiotic, probiotic, and prebiotic treatments for irritable bowel syndrome in children: A randomized controlled trial.

Background/aims: Irritable bowel syndrome (IBS) is an important health problem that presents serious social burdens and high costs. Our study investigated the efficacy of synbiotic (Bifidobacterium lactis B94 with inulin), probiotic (B. lactis B94), and prebiotic (inulin) treatment for IBS in a pediatric age group.

Acute vanishing bile duct syndrome after the use of ibuprofen.

We present a case report of a 7-year-old patient who developed toxic epidermal necrolysis (TEN) and vanishing bile duct syndrome (VBDS) after oral ibuprofen intake. Acute VBDS is a rare disease with unknown aetiology, often presenting with progressive loss of the intrahepatic biliary tract. TEN is an immune complex-mediated hypersensitivity reaction involving the skin and mucosa, which is induced by drugs or infectious diseases, sometimes leading to systemic symptoms.

Childhood chronic gastritis and duodenitis: Role of altered sensory neuromediators.

Aim: To investigate the roles of the neuropeptides vasoactive intestinal peptide (VIP), substance P (SP), and calcitonin gene-related peptide (CGRP) in chronic gastritis and duodenitis in children.

Methods: Biopsy samples from the gastric and duodenal mucosa of 52 patients and 30 control subjects were obtained. Samples were taken for pathological examination, immunohistochemical staining, enzyme activity measurements and quantitative measurements of tissue peptide levels.

Total oxidant status, total antioxidant capacity and ischemia modified albumin levels in children with celiac disease.

In our study, we aimed to investigate ischemia modified albumin (IMA) as an oxidative stress marker, as well as other oxidant and antioxidant markers that have not been evaluated in children with celiac disease. A total of 37 pediatric patients who were diagnosed with celiac disease (CD) and 29 healthy children were enrolled in this prospective study. We evaluated the IMA, total oxidant status, total antioxidant capacity, sulfhydryl, and advanced oxidation protein products in all of the subjects.

A very rare cause of acute pancreatitis: Berardinelli-Seip congenital lipodystrophy.

Pancreatitis is among rare diseases in pediatrics clinics. It is usually presented with a sign of underlying systemic disease. Berardinelli-Seip congenital lipodystrophy (BSCL) is a very rare disease characterized by near absence of adipose tissue resulting in apparent muscle hypertrophy from birth or early infancy associated with severe insulin resistance.

Low-cost home-use light-emitting-diode phototherapy as an alternative to conventional methods.

Background: The aim of our study was to evaluate whether a portable, light-weight, light-emitting-diode phototherapy unit designed for home use is as effective as conventional blue-light fluorescent phototherapy (CFP) for treating hyperbilirubinemia in neonates.

Methods: A total of 50 patients were recruited sequentially for treatment using CFP (n = 25) and the home-type phototherapy unit (n = 25).

Results: The average rate of decrease in bilirubin levels was 0.

The role of Bifidobacterium lactis B94 plus inulin in the treatment of acute infectious diarrhea in children.

Background/aims: In contrast to many other studies of probiotic species, the number of publications evaluating Bifidobacterium lactis and its combinations with prebiotics as treatments for acute infectious diarrhea is limited. We investigated the synbiotic effects of B. lactis B94 plus inulin on acute infectious diarrhea.

Celiac disease in patients with Williams-Beuren syndrome.

Celiac disease is an autoimmune, gastrointestinal disorder characterized by intolerance to the dietary grain protein gluten. An increased prevalence of celiac disease has been reported in Down syndrome and Turner syndrome, but there has been only few previous reports with respect to the association of celiac disease in Williams-Beuren syndrome. The aim of this study was to evaluate the frequency of celiac disease in our 24 Williams-Beuren syndrome patients.

Microvillus inclusion disease associated with necrotizing enterocolitis in a premature infant.

Microvillus inclusion disease is one of the congenital diarrheal disorders characterized by the appearance of inclusion bodies on the intestinal epithelium. To date there are a few cases and also a few other associated finding reports related to this life-threatening disease in literature. In this report, we present a premature infant with microvillus inclusion disease that was associated with necrotizing enterocolitis.