Cyclic vomiting syndrome in Thai children.

Cyclic vomiting syndrome (CVS) is a severe childhood vomiting disorder of unknown etiology and pathogenesis. Clinical manifestations and prophylactic therapy of vomiting have been described in the literature. The data were limited in Asian children. The aim of this study was to study the clinical manifestation, to evaluate using antimigraine prophylactic drugs and response in Thai children with CVS. The medical records of children with a diagnosis of CVS in the Department of Pediatrics, Siriraj Hospital, Mahidol University from 1994 to 2001 were retrospectively reviewed. Demographic data, clinical manifestations, investigations, treatment and outcome were collected and analyzed. Twenty five patients were enrolled in this study including 13 females and 12 males. Their ages ranged from 2.3 years to 14 years (7.8 +/- 3.4 years). The age of onset was 5.2 +/- 3.2 years. They had 14.7 +/- 6.5 episodes per year with a duration of each attack 4 +/- 1.8 days. There were 8 mild, 10 moderate and 7 severe cases. There were only 6 patients (24%) who had headache and 50 per cent of these had a family history of migraine. Eight patients received pizotifen which had 3 good, 1 fair, and 4 poor responses. Of this group, in 3 patients pizotifen was changed to amitriptyline. Eighteen patients received amitriptyline and the result of treatments were 11 good, 4 fair, and 3 poor. The other 2 patients were on propranolol with one good and one poor responses. The efficacy of amitriptyline and pizotifen were compared (83.3% vs 50%) which revealed no statistical significance (p = 0.14). There was no side effect from any of the medication in this study. In conclusion, the present report showed similar data of clinical features, prophylactic treatment and outcome as previous reports, except for fewer migraine headaches in patients and their families. Amitriptyline and pizotifen were effective in prophylactic therapy of vomiting episodes.

Clinical trial comparing the efficacy of ursodeoxycholic acid and cholestyramine on idiopathic neonatal hepatitis.

common cause of neonatal cholestasis is idiopathic neonatal hepatitis. Several agents have been proposed to counteract the effects of accumulated toxic bile acid such as phenobarbital and cholestyramine. Ursodexycholic acid ( UDCA) is a choleretic agent used in chronic intrahepatic cholestasis. The aim of this study is to compare the efficacy of ursodeoxycholic acid and cholestyramine on hepatic function in idiopathic neonatal hepatitis. Twenty patients were enrolled in this study. The patients were randomized to receive UDCA or cholestyramine orally. There were ten patients in each group. The doses of UDCA and cholestyramine were 15 mg/ kg / day and 350 mg / kg/ day respectively. The duration of the study was 8 weeks. Conventional liver function tests were done initially and at 1,2,4, and 8 weeks. Any side effects of these medications were noted. The ursodeoxycholic acid group showed a significant improvement in levels of total bilirubin, direct bilirubin, and alkaline phosphatase ( P< 0.05). The cholestyramine group showed a significant improvement in levels of total bilirubin, and direct bilirubin ( P< 0.05). There was no significant difference on liver function tests between the groups at any time ( P> 0.05). No side effects were observed. The results showed that both drugs improved cholestasis resulting from idiopathic neonatal hepatitis. The results of this study suggest that UDCA would be an alternative choleretic agent in treatment of idiopathic neonatal hepatitis.

Long-term treatment of ursodeoxycholic acid in paediatric cholestatic liver diseases.

Children with chronic cholestasis usually have pruritus, jaundice, failure to thrive and later, develop diliary cirrhosis. Ursodeoxycholic acid (UDCA) is a tertiary bile acid which has a positive effect on decreasing cholestasis. The aim of this study is to determine the efficacy of the drug on hepatic functions and symptoms in such patients. Nine children (six boys, three girls) diagnosed with biliary atresia (six cases), idiopathic neonatal hepatitis (two cases) and cryptogenic cirrhosis (one cases), received UDCA in a dose of 15 mg/kg daily for one year. Clinical features, symptoms and liver function tests were evaluated initially and at 1369 and 12 months after starting therapy. Seven patients had a complete 12-month therapy, but two patients were treated for only six months. Decreased cholestatis parameters were found including bilirubin from 11.7+ 4.9 to 7.7+ 3.2 mg/dl, GGT from 637.1+ 146.6 to 328.3+ 59.4 U/L, and AP from 1112.6+ 89.1 787.3+ 103.1 U/L., but there were no statistically significant results. Pruritus was improved in two of four patient with pruritus patients while four patients showed improved appetite.

Congenital hepatic with polycystic kidney: Report of 2 cases.

Two patients with congenital hepatic fibrosis with polycystic kidney are reported. One patient, a known case of polycystic kidney disease, presented with hematemesis and hepatosplenomegaly. The other had a problem of chronic pyronic pyrexia for 5 months. Both cases were diagnosed as congenital hepatic fibrosis by liver biopsy.