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《ACTA ACADEMIAE MEDICINAE NANJING》 1996-05
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Clinical and Laboratory Study of 5α reductase Deficiency

Cui Yugui Department of Endocrinology, First Affiliated Hospital of Nanjing Medical University, Nanjing 210029 Zhang Guiyuan National Research Institute for Family Planning, Bejing 100081  
Steroid 5α reductase plays a central role in androgen action by catalyzing the conversion of testosterone (T) into the more potent hormone dihydrotestosterone (DHT). Failure to synthesize DHT in androgen target tissues, during male fetal sexual differentiation and development, manifests a rare form of male pseudohermaphroditism, termed 5α reductase deficiency, in which the male (46, XY) external genitalia differentiate as female structures. We studied 2 patients of 13 and 6.5 ys age with 5α reductase deficiency and 12 normal men at same age ranges in clinical and laboratory diagnosis. Comparing with normal men, 2 patients had normal T level, but lower DHT level, in blood and target tiscues, genitalia skins. T level of patients raised rapidly after hCG stimulation, while DHT level keep on low level and T/DHT ratio would more raised. Although normal ligand binding capacity of androgen receptor and normal activity of 5α reductase isozyme Ⅰ, the patients had much lower activity of isozyme Ⅱ. So they may be diagnosed as 5α reductase deficiency on pathophysiology, and diagnosed differentially from other male pseudohermaphroditism, such as androgen insensitive syndrogen due to defect of androgen receptor.
【Key Words】: α reductase deficiency 5α reductase
【CateGory Index】: R588.102
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