Biosynthesis of steroids in various tissues related to feminizing syndromes.
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Biomedical subjects
Publications and source records attributed to D C Sharma.
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Familial male pseudohermaphroditism (MPH) due to 17,20-desmolase deficiency is rare. Here we present two siblings with MPH possibly due to 17,20-desmolase deficiency. The first patient presented with unambiguous female external genitalia and hypergonadotrophic hypogonadism. Chromosomal analysis revealed 46 XY. Ultrasound evaluation of pelvis revealed gonads in the inguinal canal, and no uterus. These findings were confirmed on laparotomy. Histology revealed the gonads to be testes. The second patient had ambiguous genitalia (perineoscrotal hypospadias, bifid scrotum with palpable gonads) with a 46 XY chromosomal pattern. Both patients had high plasma 17-hydroxy progestrone (17 OHP), low normal dehydro epiandrosterone sulphate (DHEAS) and low plasma testosterone. Plasma testosterone and DHEAS showed no response to ACTH or HCG. These features are compatible with the diagnosis of 17,20-desmolase deficiency.
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Anorectics are clinically used in the management of obesity to accept dietary restriction through decreased desire of food intake. The present study, indicates that the drug SK & F 38393 a dopamine agonist given to albino rats at the doses of 1 mg/kg and 5 mg/kg caused decreased food intake. Central side effects observed with amphetamine and other related drugs were not observed with this drug. The drug thus may be used as an anorectic agent without central side effects.
Isolated splenic abscess is a rarity and remains a diagnostic dilemma. The presentation is nonspecific and diagnosis is often delayed. We present a case which had roentgenographic signs suggestive of splenic suppuration at admission. Of particular interest was the isolation of Salmonella paratyphi in this patient. Antibiotic therapy alone is insufficient and splenectomy remains the treatment of choice. The literature on splenic abscess is briefly reviewed.