Evaluation of nutritional status and related factors in children with cystic fibrosis.

Background: This study aimed to evaluate the nutritional status and body composition in children with cystic fibrosis (CF), in accordance with the new nutritional targets defined by European Society for Clinical Nutrition and Metabolism (ESPEN), the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) and the European Cystic Fibrosis Society (ECFS) 2016.

Methods: In this cross-sectional study, data were collected prospectively in a single centre. A record was made for a total of 95 patients with CF of clinical data.

The Effect of Enteral Nutrition Support Rich in TGF-β in the Treatment of Inflammatory Bowel Disease in Childhood.

Malnutrition is a major complication of inflammatory bowel disease (IBD). Our aim of the study was to examine the effects of Modulen IBD supplementation, which was administered to IBD patients without limiting their daily diet in addition to medical treatment, on the clinical, laboratory, anthropometric values, and disease activities of these patients. Seventy three children with IBD were evaluated retrospectively.

Budd-chiari syndrome and renal arterial neurysms due to Behcet disease: a rare association.

Behcet's disease is a multisystemic vasculitis of unknown etiology with a chronic relapsing course. Vasculitis in Behcet's disease with predominant vascular involvement is the only vasculitis that affects both arteries and veins of any size. Involvement of the renal artery and inferior vena cava is rare among the arteries and veins, respectively.

Inflammatory bowel disease in Turkish children.

Background: This study was undertaken to evaluate demographics, clinical manifestations, laboratory findings and outcomes of children with inflammatory bowel disease (IBD) in Turkey.

Methods: We analyzed the medical records of 127 children diagnosed with IBD (under 18 years old) between January 2004 and January 2012 in 8 pediatric gastroenterology centers.

Results: Of the 127 patients, 90 (70.

Essential thrombocythemia with Mpl W515 K mutation in a child presenting with Budd-Chiari syndrome.

Essential thrombocythemia (ET) is an extremely rare childhood disorder characterised by clonal expansion of megakaryocytic lineage in bone marrow, leading to a persistent increase in the number of circulating thrombocytes and thus increased risk for thrombotic and haemorrhagic events. The molecular mechanisms of ET are not fully understood. Most children with ET have the JAK2 V617F somatic mutation; however, another mutation, involving a W to L or K substitution at Mpl codon 515, was reported in a small proportion of adult ET patients that is extremely rare in children.

Comparison of standard and standard plus vitamin E therapy for Helicobacter pylori eradications in children.

Background/aims: Although various drugs can be used in adults for Helicobacter pylori eradication in adults, treatment options are limited in children. The aim of this study was to compare the effects of the standard lansoprazole, amoxicillin, and clarithromycin (LAC) protocol to those of LAC+vitamin E (LACE) combination for H. pylori eradication.

Peptic ulcer disease in children: an uncommon disorder with subtle symptomatology.

Background/aims: Data concerning peptic and infectious ulcers in children are limited. The aim of the study was to investigate the prevalence, presenting symptoms and significance of symptomatology in ulcer diagnosis in the pediatric age group.

Materials And Methods: Between January 2000 and 2009, upper gastrointestinal endoscopy charts were examined retrospectively.

An unexpected cause of pleural effusion in paediatric emergency medicine.

Pancreaticopleural fistula (PPF) is an uncommon complication of chronic pancreatitis leading to a large and recurrent pleural effusion. Since the patients presented predominantly with respiratory symptoms, diagnosis and treatment were often delayed. We describe a child who was admitted to our paediatric emergency department with an acute onset of dyspnoea and unilateral massive pleural effusion caused by PPF.

Liver involvement in children with Familial Mediterranean fever.

Aim: Familial Mediterranean fever is characterised by recurrent, febrile, inflammatory attacks of the serosal membranes. Prolonged inflammatory response is triggered secondary to cytokine stimulation due to reduced activity of pyrin. Inflammatory cytokines play major role in the pathogenesis of acute liver injury; and chronic, recurrent cytokine production may cause chronic hepatitis/cirrhosis.

Late-onset graft-versus-host disease after pediatric living-related liver transplantation for Langerhans cell histiocytosis.

GVHD is the most common and well-known cause of morbidity and mortality following allogeneic BM transplantation. The GVHD following OLT is an uncommon complication but has a high mortality and poses a major diagnostic and therapeutic challenge. We herein discussed a 12-month-old girl with multi-system LCH, who developed end-stage liver disease despite intensive chemotherapy.

Evaluation of growth after liver transplantation in Turkish children.

Aims: Currently, the main interest in childhood liver transplantation (LT) is to prevent long-term complications and optimize growth. The aim of this study is to analyze (1) nutritional status in the pretransplantation period, and (2) posttransplantation growth and associated factors in children.

Patients And Methods: Eighty children were included in the study.

Therapeutic management of neonatal hemochromatosis: report of four cases and literature review.

Neonatal hemochromatosis (NH) is a rare disease of iron metabolism that starts at intrauterine period causing liver failure and extrahepatic siderozis. The etiology of NH has not been understood exactly, yet it is accepted that a maternofetal alloimmune disorder that leads to liver failure in fetus causes the illness. The prognosis of NH is generally bad and death is inevitable if left untreated.

Intestinal involvement in metachromatic leukodystrophy.

Metachromatic leukodystrophy is a lysosomal storage disease caused by the deficiency of the enzyme arylsulfatase A. If arylsulfatase A is deficient, sulfatide accumulates. Functionally, this accumulation results in progressive neurological deterioration.

Chronic constipation in Turkish children: clinical findings and applicability of classification criteria.

We aimed to evaluate general features of children with chronic constipation and classified them according to the Iowa criteria and Rome II criteria in order to analyze applicability of these criteria in our population. The medical records of 485 children who were referred for chronic constipation over a six-year period were evaluated retrospectively. We found that 7.

Foreign body ingestion in Turkish children.

Foreign body ingestion (FBI) is a common problem in the pediatric population. Even though morbidity and mortality due to foreign body ingestion are rare in childhood, they may cause serious anxiety in parents. We aimed to analyze the clinical presentation, etiology and management strategy of FBI in children in our country.

Liver transplantation for acute liver failure due to toxic agent ingestion in children.

ALF is characterized by sudden onset, impaired liver function, jaundice and encephalopathy, without previous liver disease. We analyzed the patients who underwent LT due to toxic agent induced ALF to raise community awareness about preventing the toxic agent induced ALF. Five children (three boys, two girls) underwent LT due to toxic agent ingestion.

Long-term effect of gluten-free diet on growth velocity in Turkish children with celiac disease.

We sought to analyze the long-term growth in children with celiac disease (CD) as well as the association of height and weight SD score at the end of 4 years with the demographic and clinical presentation at initial admission. Thirty-four children with CD were enrolled in the study and followed for at least 4 years. Patients were divided into three groups (Group 1: patients 10 years, at the time of diagnosis).

Helicobacter pylori infection in children with celiac disease.

Objective: To analyze the prevalence of Helicobacter pylori (H. pylori) infection in children with celiac disease (CD) and to examine the role of H. pylori infection in clinical, laboratory and histopathological presentations of CD.

A rare complication of central catheter in an infant with intestinal failure.

Objective: To report the successful percutaneous transcatheter removal of a snapped intracardiac fragment of the central vein catheter in an infant.

Clinical Presentation And Intervention: A 3-month-old infant with short bowel syndrome who required long-term parenteral nutrition via portacath central catheter was hospitalized. Two months after the insertion of the second catheter, the distal tip of the catheter snapped and became tangled in the heart.

Childhood cirrhosis, hepatopulmonary syndrome and liver transplantation.

Objectives: The hepatopulmonary syndrome (HPS) is characterized as a triad: liver disease, intrapulmonary vascular dilatation, and arterial hypoxemia. The aim of this study is to analyze outcome of children with HPS in liver transplant era.

Methods: Between September 1996 and November 2006, 172 cirrhotic patients (median age 5 years; range 0.

Association of mast cells and liver allograft rejection.

MCs are important effector cells in a broad range of immune responses. Their role in liver allograft rejection is not clear. Twenty-one liver transplant recipients (mean age +/- s.

Nutritional status of infants with neonatal cholestasis.

Purpose: We constructed a study to determine the association of anthropometric measurements, biochemical parameters and bone mineral content with nutritional status in infants with neonatal cholestasis.

Methods: The study included 38 children with neonatal cholestasis. Nutritional status was assessed by Waterlow criteria, and anthropometric measurements, biochemical parameters and bone mineral content were correlated with the nutritional status at initial admission and at the end of 2 months after nutritional support.

Liver transplantation for progressive familial intrahepatic cholestasis: clinical and histopathological findings, outcome and impact on growth.

In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive familial intrahepatic cholestasis. We also analyze outcome and impact of liver transplantation on growth and bone mineral content. Most of the patients were presented with jaundice mainly beginning within the first six months.