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LLOYD AXELROD, ROBERT M NEER, BERNARD KLIMAN, Hypogonadism in a Male with Immunologically Active, Biologically Inactive Luteinizing Hormone: An Exception to a Venerable Rule, The Journal of Clinical Endocrinology & Metabolism, Volume 48, Issue 2, 1 February 1979, Pages 279–287, https://doi.org/10.1210/jcem-48-2-279
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Abstract
A male patient, the product of a consanguineous mating, had normal primary sex characteristics but failed to enter puberty spontaneously. The plasma total testosterone level was low (30-81 ng/100 ml), the plasma immunoreactive LH level was elevated, and the bioassayable urine gonadotropin excretion rate was increased in the rat ovarian weight assay and the mouse uterine weight assay. Although these findings suggested a primary gonadal defect, he responded normally to a physiological dose of human LH, with a rise of the plasma total testosterone level to 430 ng/100 ml. Thus, the patient's Leydig cells responded to exogenous human LH but not to endogenous LH. The Leydig cells also responded normally to graded single doses of exogenous human LH and hCG, providing evidence against either a subtle abnormality in testicular responsiveness or a circulating LH antagonist. In fact, the patient developed secondary sex characteristics in response to chronic treatment with hCG, confirming the normal testicular responsiveness to exogenous gonadotropins. After administration of LRH, the immunoreactive plasma LH level rose markedly, but the immunoreactive plasma FSH level and the plasma testosterone level did not change. The testicular biopsy was consistent with the diagnosis of LH deficiency, and the karyotype was normal. Three normally developed maternal uncles with a history of infertility were also products of a consanguineous mating, and one had a subnormal plasma testosterone level.
These findings suggest that this syndrome is attributable to the presence of an abnormal LH, which is biologically inert in the affected patients but biologically active in rodents. The defect appears to be inherited. (J Clin Endocrinol Metab48: 279, 1979)
