" /> Leydig cell hypoplasia - CISMeF





Preferred Label : Leydig cell hypoplasia;

ICD-11 definition : This is a rare autosomal recessive genetic and endocrine syndrome, characterized by an inability of the body to respond to luteinizing hormone (LH), a gonadotropin which is normally responsible for signaling Leydig cells of the testicles to produce testosterone and other androgen sex hormones;

ICD-11 synonym : 46,XY disorder of sex development due to LH defects; 46,XY DSD due to LH defects; LH resistance due to LH receptor inactivation; Male pseudohermaphrodism due to LH defects;

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This is a rare autosomal recessive genetic and endocrine syndrome, characterized by an inability of the body to respond to luteinizing hormone (LH), a gonadotropin which is normally responsible for signaling Leydig cells of the testicles to produce testosterone and other androgen sex hormones

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08/07/2025


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