TY - JOUR
T1 - Idiopathic hypogonadotropic hypogonadism
T2 - Diagnosis, pathogenesis, genetics, and treatment
AU - Layman, Lawrence C
PY - 1991/1/1
Y1 - 1991/1/1
N2 - Idiopathic hypogonadotropic hypogonadism (IHH) is a heterogeneous disorder displaying a range of phenotypic, genetic, and endocrinologic features. Current evidence suggests a deficiency in the hypothalamic release of pulsatile gonadatropin-releasing hormone (GnRH) in most patients. No genetic defects in the genes for GnRH, LHβ, or FSHβ have been described, although a deletion in the steroid sulfatase gene has been reported in X-linked Kallmann's syndrome with associated ichthyosis. Treatment with GnRH administered in a pulsatile fashion has been successful in inducing steroidogenesis and gametogenesis.
AB - Idiopathic hypogonadotropic hypogonadism (IHH) is a heterogeneous disorder displaying a range of phenotypic, genetic, and endocrinologic features. Current evidence suggests a deficiency in the hypothalamic release of pulsatile gonadatropin-releasing hormone (GnRH) in most patients. No genetic defects in the genes for GnRH, LHβ, or FSHβ have been described, although a deletion in the steroid sulfatase gene has been reported in X-linked Kallmann's syndrome with associated ichthyosis. Treatment with GnRH administered in a pulsatile fashion has been successful in inducing steroidogenesis and gametogenesis.
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U2 - 10.1016/S0932-8610(19)80016-6
DO - 10.1016/S0932-8610(19)80016-6
M3 - Short survey
AN - SCOPUS:0026336979
VL - 4
SP - 111
EP - 118
JO - Journal of Pediatric and Adolescent Gynecology
JF - Journal of Pediatric and Adolescent Gynecology
SN - 1083-3188
IS - 3
ER -