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We aimed at determining the pattern of liver disease in the Iranian children referred to the Medical Center of Children affiliated with the Tehran University of Medical Sciences. Materials and Methods: In a cross-sectional study conducted over 2 years, 425 liver needle biopsies were sent to the pathology laboratory of our center. Slides were prepared from paraffin-embedded blocks, stained by routine H & E and special stains and were then reviewed. The frequency of each disorder, separately and in combination with the age group or gender of the patients was calculated and compared with other similar studies. Results: The male to female ratio was 1.42:1. The age range was between 1 month and 18 years old and 41.4% were less than 2 years old. The most common histological diagnosis was iron overload due to major thalassemia (17.5%) followed by biliary atresia (9.7%), no significant pathologic change (8.7%), neonatal hepatitis (8.7%), chronic hepatitis (8.5%), cirrhosis (6.5%), metabolic disease (5.5%) and progressive familial intrahepatic cholestasis (5%). Results of the hemosiderosis grading in patients with thalassemia revealed no or minimal, mild, medium, or marked increase in 10%, 27.1%, 10%, 21.4% and 31.5% of the cases, respectively and the degree of iron deposition rose in parallel with age and also the stage of fibrosis (p< 0.05). Conclusion: A liver biopsy is a useful and practical tool for the appropriate diagnosis of pediatric liver diseases. Also, we found that in non thalassemic children, biliary atresia, chronic hepatitis and neonatal hepatitis, in the stated order, are the most prevalent histologic diagnoses in Iranian pediatrics.

Mastocytosis is characterized by the accumulation of mast cells in different tissues either in the skin or extracutaneous organs. Herein, a 13-year-old girl is presented who suffered from intermittent abdominal pain, nausea, diarrhea with erythematous, and papulous cutaneous lesion. She had a history of same lesions from the age of four years. Since 8 months ago, the patient presented with digestive complaints. The histopathological examination of the cutaneous lesions approved the diagnosis of mastocytosis. H1 and H2 antagonists were prescribed for her, while a mast cell stabilizer for digestive and cutaneous symptoms was also utilized, which improved the signs and symptoms of the patient. [ABSTRACT FROM AUTHOR]

Cystic fibrosis (CF) is a common autosomal recessive disorder with different clinical manifestations, mainly in the gastrointestinal and respiratory tracts. This study was performed to access the effect of probiotics in the status of intestinal inflammation in a group of children with CF by measuring the calprotectin level in the fecal samples. Forty-seven patients with CF were enrolled in this study. The fecal calprotectin levels were measured by enzyme linked immunosorbent assay. In a randomized systematic method, the children were divided into two groups - one group received probiotic powder and another received placebo for four weeks. After this period, fecal calprotectin was re-measured. Thirty-one of 47 enrolled patients (65.9%) had abnormal fecal calprotectin levels (>50 µg/g). After the intervention, the fecal calprotectin levels decreased in 29 patients (21 patients in the drug group, and only 8 patients in the placebo group; p<0.001). This study showed that about two-thirds of patients with CF had intestinal inflammation based on fecal calprotectin levels. Probiotic administration was shown to decrease calprotectin concentrations and subsequently intestinal inflammation in CF patients. [ABSTRACT FROM AUTHOR]

Objective: Helicobacter pylori (H. Pylori) is now recognized as a major etiological factor in the pathogenesis of gastritis and peptic ulcer disease. There is concrete evidence that eradication of the bacterium reverses histological gastritis, and results in significant reduction of duodenal and gastric ulcer recurrence. Poor compliance and antibiotic resistance are the main causes for failure of anti H. pylori therapy. In this study we determined efficacy of omeprazole based triple therapy with b.i.d. dosing of furazolidone, amoxicillin for 2 weeks and omeprazole in Iranian children. Methods: This prospective study included 37 children, in whom H. Pylori infection was diagnosed endoscopically. H.Pylori positive children were treated with a two weeks course of furazoidone (6 mg/kg/day) and amoxicillin (50 mg/kg/day) plus omeprazole (1-2 mg/kg/day). Eradication was assessed by 13C UBT. Findings: Mean age of patients was 10.2 yr (5-15 yr), 25 (67.5%) patients were boys. H. Pylori was eradicated in 34 children (per patient 91.9%, per protocol 86%). Side effects occurred in 3 (8.1%) patients, but these were mild and it was not necessary to discontinue treatment. Three children (8.1%) remained H. pylori positive. Conclusion: Our study showed that the association of furazolidone plus amoxicillin with a proton-pump inhibitor could be a valuable alternative for eradication of H. Pylori infection in children. It is an effective, affordable treatment that allows good compliance and produces low adverse effect rates. [ABSTRACT FROM AUTHOR]

Background: Cystic fibrosis is a hereditary disease of mucus and sweat glands characterized by respiratory infections and pancreatic insufficiency. Case presentation: We describe a girl infant with cholestasis as of the first clinical presentation at the age of 1 month. She developed severe anemia which required transfusion for several times; High level of direct bilirubin, low level of albumin and positive sweat tests were detected in laboratory tests. Histopathology report of liver biopsy indicated giant cell neonatal hepatitis. Conclusion: Although neonatal hepatitis is common cause of neonatal cholestasis, such condition is quite rare in cystic fibrosis, which easily could be misdiagnosed. Early diagnosis and appropriate treatment could prevent further complications of the disease. [ABSTRACT FROM AUTHOR]

Cystic fibrosis (CF) is a hereditary disease of mucous and sweat glands, which affects the respiratory and gastrointestinal systems. Herein, we describe a 3-month-old girl with a history of recurrent episodes of urinary tract infections that required hospitalization. She was referred to our center at the age of three months, with massive gastroesophageal variceal bleeding. In physical examination, she had clubbing, hepatosplenomegaly, and mild ascites. Laboratory studies revealed high serum levels of liver enzymes and low level of Albumin. As of suspicious to CF, sweat tests were performed twice which confirmed the diagnosis of CF. Gastrointestinal bleeding due to gastroesophageal varices is a rare complication of CF, which could result as a consequence of hepatobiliary involvement of disease. Early diagnosis of CF could prevent severe complications and even death in this group of patients. [ABSTRACT FROM AUTHOR]

Cystic fibrosis (CF) is a hereditary disease, characterized by chronic pulmonary disease, pancreatic insufficiency and abnormal electrolytes in the sweat. In order to evaluate the clinical manifestations and laboratory findings of Iranian children with CF during a 10-year period, 243 CF patients, with a median age of 5 months, were investigated in this study. The most common manifestations were gastrointestinal disorders and respiratory manifestations. Cough was the most common symptom, followed by malnutrition, diarrhea, respiratory distress, and vomiting. The frequency of these findings after treatment was significantly decreased in comparison with the period before diagnosis. During the mean follow-up of 40.9 months, seven cases died due to severe infections. Cystic fibrosis as a common genetic disorder should be considered in any child with recurrent gastrointestinal and respiratory manifestations, since delayed diagnosis could lead to severe complications and even death in this group of patients.