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;
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