Evidence for the Bauman variant in Kallmann's syndrome.
OBJECTIVE
Idiopathic hypogonadotrophic hypogonadism (IHH) is a condition of gonadotrophin releasing hormone (GnRH) deficiency. IHH associated with anosmia is Kallmann's syndrome. A variant has been described by Bauman where a patient with Kallmann's syndrome apparently regained normal hypothalamo-pituitary function 2 years after the initial diagnosis. GnRH secretory activity can be assessed by measuring LH pulsatility. Our objective was to define the pattern of LH pulsatility in men with IHH and Kallmann's syndrome compared with those of normal controls, and to determine whether there is evidence for a Bauman variant of Kallmann's syndrome.


METHODS
Patients with IHH and Kallmann's syndrome were recruited from the endocrine clinic. Long-term hormone replacement therapy was discontinued. LH pulsatility was determined.


METHODS
Three men with IHH, 3 men with classical Kallmann's syndrome and 5 normal male volunteers.


METHODS
Baseline serum FSH, LH and testosterone. Intensive blood sampling every 10 minutes for serum LH from 1000 to 1600 h during the day and 2200 to 0400 h during the night to measure LH pulsatility.


RESULTS
The volunteer group showed normal LH pulsatility. In the patient group, LH secretion was apulsatile in one, showed significantly diminished amplitude in four, and there was normal pulsatility in one patient which remained normal 5 months later.


CONCLUSIONS
Three patients with idiopathic hypogonadotrophic hypogonadism and 2 with Kallmann's syndrome had variable degrees of GnRH deficiency. One patient with Kallmann's syndrome had apparently normal GnRH activity, which remained normal 5 months later. This patient appears to have the Bauman variant of Kallmann's syndrome.
Kadva
A
A
Academic Unit of Obstetrics, Gynaecology and Reproductive Physiology, London Hospital Medical College, UK.
Di
W L
WL
Djahanbakhch
O
O
Monson
J
J
Silman
R
R
D000328:Adult; D005640:Follicle Stimulating Hormone / Q000097:blood; D007987:Gonadotropin-Releasing Hormone / Q000097:blood / Q000378:metabolism*; D006801:Humans; D007006:Hypogonadism / Q000097:blood / Q000378:metabolism; D017436:Kallmann Syndrome / Q000097:blood / Q000378:metabolism*; D007986:Luteinizing Hormone / Q000097:blood / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D012636:Secretory Rate; D013739:Testosterone / Q000097:blood
D007987:Gonadotropin-Releasing Hormone; D013739:Testosterone; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone