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单纯男性化型(simplevirilizingSV)

名词解释题
2021-12-30 01:49
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属先天性肾上腺皮质增生症的一种类型。系21-羟化酶不完全缺乏所致,酶缺乏呈中等程度,11-脱氧皮质醇和皮质醇、11-脱氧皮质酮等不能正常合成,其前体物质17-羟孕酮、孕酮、脱氢异雄酮增多,但仍可合成少量皮质醇和醛固酮,故临床无失盐症状,主要表现为雄激素增高的症状和体征。


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