Ataxia-telangiectasia in children.
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Biomedical subjects
Publications and source records attributed to S Maqbool.
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Of the 4070 children admitted in the department of paediatrics, 830 (24%) presented with diarrhoea. Eleven of these had haemolytic ureamic syndrome (HUS) characterised by microangiopathic haemolytic anaemia, thrombocytopenia and acute renal failure. Only 3 had positive stool cultures (E. Coli 2, shigella dysenteriae 1). Two children expired while the rest recovered with conservative management and peritoneal dialysis. Thus HUS should be remembered as a complication of diarrhoea and a cause of acute renal failure in children.
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The development of hematologic abnormalities was prospectively evaluated in 50 children treated for ten days each with oral trimethoprim-sulfamethoxazole and compared with a control group of 20 children with similar infections treated with amoxicillin trihydrate. Neutropenia (polymorphonuclear neutrophilic leukocyte counts, less than or equal to 1,500/cu mm) developed in 17 (34%) of the 50 children treated with trimethoprim-sulfamethoxazole compared with one (5%) in the control group of 20 children (P less than 0.001). Thrombocytopenia (platelet count, less than 150,000/cu mm) developed in six (12%) of the children treated with trimethoprim-sulfamethoxazole, but it did not develop in any of the amoxicillin-treated children (P less than .01). Neutropenia occurred mostly during the first week of treatment and lasted a mean of 8.9 days. Thrombocytopenia was noted between the seventh and 16th day (mean, 10.3 days) and lasted a mean of 12.7 days. Both side effects resolved spontaneously without ill effects. Children treated with oral trimethoprim-sulfamethoxazole should be followed up with biweekly leukocyte and platelet counts, and treatment should be discontinued if severe neutropenia or thrombocytopenia develops.
Effectiveness of mebendazole, a new anthelminthic drug recommended for treatment of Trichuris trichiura infection, was evaluated in 50 children attending a pediatric outpatient clinic in New York City. Mebendazole, 100 mg. administered orally twice daily for three days resulted in an apparent cure of 48 children. Posttreatment stools of the remaining two children had a substantial reduction in the number of eggs. A second course of treatment, identical to the first, led to the elimination of eggs in the stool specimens of these two children. Mebendazole appears to be an effective drug against Trichuris trichiura.
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Parental compliance in keeping a return appointment for the purpose of having their child's Tuberculin Tine test read was studied in the setting of a large urban pediatric clinic. This compliance rate was found to be 72%. Various factors which could potentially affect compliance with this procedure were examined, and the only factor which correlated significantly was family size. A modified system whereby parents read their child's Tine test in the home setting and reported the test results to the clinic by mail (a method used today in many pediatric outpatient departments) was implemented and evaluated. With this new system, the compliance rate increased to 90%.
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