[Effect on renal hemodynamics and electrolyte transport of (2-(2,6 dichlorophenoxy) ethyl) amino guanidine sulphate in hypertensive patients with uremia].
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Biomedical subjects
Publications and source records attributed to N Koçak.
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Cholesteryl ester storage disease (CESD) is rare and characterized by accumulation of cholesteryl esters and triglycerides in many tissues due to the deficiency of lysosomal acid lipase. We report a 3(1/2)-year-old child with CESD. The diagnosis was indicated by liver biopsy and confirmed by reduced acid lipase activity in leukocytes.
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Interferon is the most promising therapeutic agent for the treatment of chronic viral hepatitis. The results of studies suggest that corticosteroid pretreatment may improve the response rate. Twenty-nine children with chronic hepatitis B (CHB) were randomly assigned to receive recombinant interferon alpha (rIFN-alpha) alone (Group 1.5 million units/m2 body surface, 3 times a week for 24 weeks) or to receive oral prednisone (Group 2.2 mg/kg/day for 3 weeks, discontinued by tapering the dose within 1 week) followed by rIFN-alpha (same dose as above). Tests for liver function and hepatitis B virus (HBV) markers including HBV-DNA were done periodically. Overall, 10 patients (34.5%) cleared hepatitis Be antigen and 13 (44.8%) HBV-DNA. Anti-HBe seroconversion was observed in nine patients (31%). Only three patients (10.3%) cleared hepatitis B surface antigen and seroconverted to anti-HBs. No response was obtained in 11 patients (37.9%). There was no statistically significant difference between the two treatment groups regarding response rate. Baseline transaminases levels and HBV-DNA concentrations were predictive parameters for HBeAg clearance. It is concluded that prednisone pretreatment does not have a beneficial effect in children with CHB.
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Variceal bleeding due to portal hypertension is a frequent and severe complication of cirrhosis in children as in adults. The prophylactic approach is important for these high mortality bleedings, both for the first and for recurrent attacks. Variceal bleeding/rebleeding rates were evaluated in sixty patients with cirrhosis who received 1-2 mg/kg/day propranolol p.o. for 1-14 years. According to Child-Pugh classification, 33 patients were Class A, 22 Class B, and five Class C. Patients were divided into two groups according to whether they had variceal bleeding before starting propranolol treatment (secondary prevention; 15 patients) or not (primary prevention; 45 patients). Seven (15.6%) of 45 patients experienced bleeding on propranolol therapy in the primary prevention group, while eight (53.3%) of 15 patients bled in the secondary prevention group (p < 0.01). Propranolol was found effective in primary and secondary prevention in Class A patients, while it was effective only for primary prevention in Class B and C patients. Propranolol administration is useful for preventing first and recurrent variceal bleeding in Class A cirrhotic patients. In Class B and C cirrhotic patients, it is effective only for preventing the first bleeding episode.