Hepatoportal Sclerosis in Childhood: Descriptive Analysis of 12 Patients

Hepatoportal sclerosis (HPS) is defined as sclerosis of portal areas in the absence of cirrhosis. There is little information about HPS in children in the literature. The aim of this study was to describe the clinical presentation, associated disorders, laboratory characteristics and outcome of children who were diagnosed as HPS. This study included 12 children diagnosed as HPS by the Pathology Department between 2005 and 2011. Data were collected from the gastroenterology clinic charts retrospectively, including demographics, presentation characteristics, laboratory data and recent status of patients. Twelve patients were enrolled (6 girls, 6 boys). The median age of patients was 13.5 yr. Median age at the time of biopsy was 11 yr. Four patients had splenomegaly, 3 had esophageal varices, one had hepatopulmonary syndrome and had been transplanted. Smooth muscle antibody was found positive in 4 patients, without autoimmune hepatitis findings in liver biopsy. One patient had celiac disease and another patient had positive celiac disease serology but pathology findings. Another patient had Turner's syndrome. Mean follow-up time was 39 months (3.3 yr) after biopsy. Hepatoportal sclerosis does not necessarily present with portal hypertension in children.

Effect of melatonin on bone mineral density of irradiated rats

Melatonin is a powerful endogenous antioxidant and it may play a role in prevention of radiation-induced damage. The aim of this study was to investigate the effect of melatonin on bone mineral density in rats receiving radiation. Sprague Dawley rats were divided into four groups. Group 1 [control group] received neither melatonin nor radiation [control group]. Group 2 [Mel group] was administered intraperitoneal injections of 5mg/kg melatonin daily for ten days. Group 3 [RT group] and Group 4 were exposed to total cranium radiation of 5 Gy in a single dose by using a cobalt-60 teletherapy unit. In addition to irradiation, group 4 [RT + Mel group] was administered 5mg/kg of melatonin intraperitoneally. At the end of the 10th day, the rats' cranium and vertebrae bone mineral densities [BMDs] were measured. When cranial BMDs were evaluated, statistically more significant BMD increases were seen in the Mel group and the RT + Mel groups than in the control group. No significant difference was seen in the Mel group versus the RT + Mel group; however, there was a significant difference between RT and RT + Mel groups. When vertebral BMDs were evaluated, the only significant difference was found between the control and Mel groups. We think that melatonin is a radioprotective agent. However, we would like to emphasize that further studies are needed before clinical trials with melatonin are initiated

First Turkish Patient with Floating Harbor Syndrome with Additional Findings: Cryptorchidim and Microcephaly

We report the first Turkish patient with Floating Harbor Syndrome (FHS). The 12-year old male patient exhibited classical dysmorphic features of FHS, mental retardation, celiac disease and additional undescribed findings: microcephaly and cryptorchidism.

Is Rhabdomyolysis a Rare Manifestation in Celiac Disease?

No abstract available.

Rhabdomyolysis in Celiac Disease

A 12-year-old female presented with chronic diarrhea, fatigue, failure to thrive, sudden weakness of her upper and lower extremities and inability to walk. On neurological examination, atrophy was found of the lower extremity muscles, coupled with muscle weakness. Hypokalemia and a high creatine kinase (CK) level were detected. Antigliadin IgA, IgG and antiendomysial antibodies were positive. A duodenal biopsy revealed the classical findings of celiac disease. To our knowledge this is the first childhood case of celiac disease presenting with rhabdomyolysis.

An Unusual Combination of Relapsing and Cholestatic Hepatitis A in Childhood

Clinical variants of hepatitis A include the prolonged, relapsing and cholestatic forms. Here, the first childhood case of hepatitis A, with a combination of the relapsing and cholestatic forms is presented, a 14-year old boy. In the first phase of the illness, while the AST and ALT levels were declined, th total and direct bilirubin and GGT were increased. The patient was thought to have the cholestatic form of hepatitis A. Du to intense pruritus and high bilirunbin levels, ursodeoxycholic acid (UDCA) therapy was started. On the 17th day, the decreased AST and ALT levels began to increase, reaching levels as high as 484 U/L and 862 U/L, respectively. The UDCA treatment was stopped on the 64th day. On the 164th day, all his laboratory parameters were within normal limits, but the anti-HAV IgM was still positive.