Alpha 1-antitrypsin phenotype of children with liver diseases in Thailand.

Alpha1-antitrypsin deficiency (PiZZ) constitutes not only the most common hereditary cause of liver diseases, but also of the most prevalent metabolic diseases in need of liver transplantation. It is a codominantly inherited disorder which predisposes to chronic liver disease, usually beginning in early infancy. The purpose of the present study has been to investigate alpha 1-antitrypsin phenotype in pediatric patients with various liver diseases. Phenotypic identification of alpha 1-antitrypsin variants has been carried out in 69 children with various liver diseases and 100 healthy controls using isoelectric focusing on polyacrylamide gel slabs. PIMM represents the most common phenotype detected in both groups (92% in the group with liver diseases and 88% in normal controls). We could detect PiZZ in only one healthy child but in none of those with liver diseases. Consequently alpha 1-antitrypsin deficiency does not appear to be a common cause for liver disease among children in Thailand. Further studies are necessary to elucidate the frequency of various alpha 1-antitrypsin variants and the clinical relevance with respect to liver diseases in Thailand.

Non-cirrhotic portal fibrosis associated with pulmonary arteriovenous communication and pulmonary arterial hypertension.

A case of non-cirrhotic portal fibrosis associated with pulmonary arteriovenous communication and pulmonary arterial hypertension is reported. The patient was a 7-year old boy who presented with hematemesis, cyanosis, hypoxemia and orthodeoxia. His liver pathology was compatible with non-cirrhotic portal fibrosis. His pulmonary angiography showed arteriovenous shunting and pulmonary arterial hypertension (mean pulmonary artery pressure 34 mmHg). His sister also had non-cirrhotic portal fibrosis with neither hypoxemia nor orthodeoxia. This report raises the possibility of non-cirrhotic portal fibrosis having a genetic etiology.

Control study of oral rehydration solution (ORS)/ORS + dioctahedral smectite in hospitalized Thai infants with acute secretory diarrhea.

Dioctahedral smectite, a non systemic antidiarrheal agent, is mucoprotective and absorbs enterotoxins and rotavirus as demonstrated in animal models. Smectite has been successfully used in various countries in children and adults with acute diarrhea. This study was to assess the efficiency of smectite associated with rehydration in infants with acute secretory diarrhea. Sixty-two hospitalized Thai infants, aged 1-24 months, with acute secretory diarrhea were randomly divided into 2 groups receiving (1) oral rehydration solution (ORS) (30 cases), (2) ORS and Smectite (3.6 g/day) (32 cases). Both groups were comparable for age, weight, nutritional status and duration of symptoms before treatment. All 62 infants received lactose free formula and chicken rice soup as the standard diet. Stool frequency, weight change and duration of diarrhea were recorded. The mean duration of diarrhea was 84.7 +/- 48.5 hours in group 1, and 43.3 +/- 25.1 hours in group 2 (p = 0.005). The number of infants with diarrhea was significantly lower in group 2 on Day 1 (p < 0.01) and Day 3 (p = 0.001); furthermore 27% of infants in group 1 and 3% in group 2 had still diarrhea on Day 5. The stool frequency and weight changes were not statistically different in the two groups. No major side effects were observed except two cases of vomiting and hardened stools. It is concluded that (1) Smectite shortens the course of acute secretory diarrhea in Thai infants; (2) smectite may reduce the occurrence of prolonged diarrhea; furthermore (3) in our study dioctahedral smectite was found to be safe in children aged 1 to 24 months.