ABSTRACT
BACKGROUND: The prevalence of primary sclerosing cholangitis, a rare progressive liver disorder, is increasing with the advent of endoscopic retrograde cholangiography in the investigation of children with obscure liver disease. The etiology of primary sclerosing cholangitis is not known, clinical presentation is variable, treatment is only of limited success and long-term studies on prognosis in children are incomplete. Primary sclerosing cholangitis has not been described in Arab children. METHODS: To describe detailed clinical, laboratory, histologic and radiological features in 4 children with primary sclerosing cholangitis identified over a 2-year period at a tertiary referral center in Riyadh Saudi Arabia. RESULTS: Four children, all females, between the ages of 4 and 11 years with primary sclerosing cholangitis are identified. The diagnosis was suggested by the histology and confirmed by the characteristic cholangiographic findings. Clinical findings were itching in all 4 children, hepatomegaly in 3 and splenomegaly in 4 patients. Three of 4 patients had portal hypertension and 2 of these three variceal hemorrhage. Antinuclear and smooth muscle antibodies were negative in all four patients. None had clinical manifestations of chronic inflammatory bowel disease but microscopic colitis was documented in all 4 patients. CONCLUSIONS: Primary sclerosing cholangitis is present in Arab children with a prevalence rate of 5% in our study population.
Subject(s)
Bile Ducts, Intrahepatic/diagnostic imaging , Cholangitis, Sclerosing/diagnosis , Liver/pathology , Biopsy , Child , Child, Preschool , Cholangiopancreatography, Endoscopic Retrograde , Cholangitis, Sclerosing/diagnostic imaging , Cholangitis, Sclerosing/pathology , Female , Humans , Liver/diagnostic imaging , Necrosis , Retrospective Studies , Saudi ArabiaABSTRACT
The clinical, biochemical and histological characteristics in six Arab children with progressive familial intrahepatic cholestasis (PFIC) (Byler's disease) are described. The autosomal recessive mode of inheritance is established. Jaundice and pruritus were early symptoms, with onset in the 1st 3 months in all patients. Other features included growth failure, developmental delay, ataxia, areflexia, gall-stones and epistaxis. Gamma-glutamyl-transpeptidase and cholesterol were normal, but total bile acid levels were uniformly elevated in all patients. Histology showed features of hepato-canalicular cholestasis, lack of bile duct proliferation and fibrosis or cirrhosis in all patients. Five patients who were followed up were alive at a mean age of 75.8 months.
Subject(s)
Cholestasis, Intrahepatic/diagnosis , Cholestasis, Intrahepatic/genetics , Biopsy , Child , Child, Preschool , Cholestasis, Intrahepatic/metabolism , Cholestasis, Intrahepatic/pathology , Consanguinity , Female , Humans , Infant , Infant, Newborn , Liver/metabolism , Liver/pathology , Liver Function Tests , Male , Retrospective Studies , Saudi ArabiaABSTRACT
We describe an Arab girl with complete absence of phosphorylase b kinase activity in the liver, symptomatic hypoglycemia, and persistently elevated serum aminotransferase values whose symptoms did not lessen with age; sequential liver biopsies showed progression to cirrhosis. Cirrhosis could not be ascribed to any other known cause. We conclude that type IX glycogenosis is not always associated with a benign outcome.
Subject(s)
Glycogen Storage Disease/complications , Liver Cirrhosis/complications , Liver/enzymology , Phosphorylase Kinase/deficiency , Phosphorylase b/deficiency , Child, Preschool , Consanguinity , Culture Techniques , Female , Glycogen Storage Disease/diagnosis , Humans , Liver/physiopathology , Liver Cirrhosis/physiopathology , Liver GlycogenABSTRACT
Extrahepatic manifestations have been reported in a majority of patients with ACAH. We describe an 11-year-old girl with CAH who developed life-threatening AH and was successfully managed with mechanical ventilation and high-dose steroids. Although a number of pulmonary manifestations have been described in association with AICAH, this article is the first showing its association with AH.
Subject(s)
Autoimmune Diseases/complications , Hemorrhage/complications , Hepatitis/complications , Lung Diseases/complications , Child , Chronic Disease , Female , Hemorrhage/diagnostic imaging , Hemorrhage/pathology , Humans , Lung Diseases/diagnostic imaging , Lung Diseases/pathology , RadiographyABSTRACT
In this study 234 children with shigellosis were evaluated during a 6-year period. The ages ranged from 2 days to 13 years (mean, 3.4 years). Sixty percent of the children were in the 1- to 4-year age group. One hundred four children were hospitalized and 130 were outpatients. Most cases of shigellosis presented during the months of April-May and September-November. Shigella flexneri accounted for 44% and Shigella sonnei for 43% of the isolates. Susceptibility testing showed that 54% were resistant to ampicillin, 72% to trimethoprim-sulfamethoxazole and 77% to tetracycline. Eighty percent were resistant to two or more antimicrobial agents. Morbidity and mortality was higher in children who were initially treated with antimicrobials to which the organism was resistant than in those treated with antimicrobial agent to which the organism was susceptible.
