ABSTRACT
Twelve patients with congenital hepatic fibrosis have been retrospectively studied and followed for 1 to 14 years. Clinical features, hepatic function tests and biopsy have been analyzed. Presence of portal hypertension and congenital malformation have been investigated. Clinical presentations varies from newborn to nine years of age without male or female predominance. Most frequent clinical form has been hypertensive type. Cholangitic type has worse prognosis. Familiar recurrence rate is 20%. Congenital malformations are associated in 92% most frequently infantile polycystic kidney disease. Hepatic biopsy has confirmed diagnosis in all patients.
Subject(s)
Liver Cirrhosis/congenital , Child , Child, Preschool , Cholangitis/etiology , Female , Humans , Hypertension/etiology , Infant , Infant, Newborn , Kidney Diseases/etiology , Liver Cirrhosis/complications , Liver Cirrhosis/genetics , Liver Cirrhosis/pathology , Male , Retrospective StudiesABSTRACT
Plasma vitamin E levels were measured by high performance liquid chromatography in 42 children with cystic fibrosis and were correlated with the following parameters: sex, age, time of follow-up, clinical evolution (Schwachman score), vitamin E/cholesterol and faecal fat excretion. All children in this study received oral alfa-tocoferol (50-100 mg daily) from the diagnosis. According to the vitamin E level patients were distributed in two groups. Group I: 27 patients (64.3%) with normal concentrations. Group II: 15 patients (35.7%) with decreases plasma levels but without clinical manifestations. Steatorrhea was present in all children except 4 patients from group I and one patient from group II. On the other hand, vitamin E/cholesterol was normal in 80% of patients with vitamin E deficiency (group II). We did not find any correlation between plasma vitamin E levels and the different clinical and biological parameters studied. Further studies should be carried out to throw more light on the mechanism underlying the pathogenesis of vitamin E deficiency in patients with cystic fibrosis.
Subject(s)
Cystic Fibrosis/blood , Vitamin E Deficiency/etiology , Vitamin E/blood , Adolescent , Child , Child, Preschool , Cholesterol/blood , Chromatography, High Pressure Liquid , Cystic Fibrosis/complications , Feces/analysis , Female , Humans , Infant , Lipids/analysis , Male , Vitamin E/analysisABSTRACT
Thirteen cases of abdominal tuberculosis are presented; some of them in active or symptomatic stage and others in latent or abdominal calcification form. Many of these patients were diagnosed through the finding of a source of infected cattle after diagnosis of one of symptomatic patients. Pathogenesis, clinical findings and diagnostic difficulty are commented, as well a sanitary importance of the problem, since M. bovis is still a currently found organism in daily practice.
