Hypogonadotropic hypogonadism (HH) represents a relatively rare cause of nonobstructive azoospermia (NOA), but its knowledge is crucial for the clinical andrologists, as it represents a condition that can be corrected with medical therapy in 3 quarters of cases. There are forms of congenital HH, whether or not associated with an absent sense of smell (anosmic HH or Kallmann syndrome, and normosmic HH, respectively), and forms of acquired HH. In congenital HH, complete absence of pubertal development is characteristic. On the other hand, if the deficit occurs after the time of pubertal development, as in acquired HH patients, infertility and typical symptoms of late-onset hypogonadism are the main reasons for seeking medical assistance. Gonadotropin-releasing hormone (GnRH) or gonadotropin replacement therapy is the mainstay of drug therapy and offers excellent results, although a small but significant proportion of patients do not achieve sufficient responses.
Humans ; Hypogonadism/drug therapy* ; Male ; Azoospermia/drug therapy* ; Gonadotropin-Releasing Hormone/therapeutic use* ; Kallmann Syndrome/drug therapy* ; Hormone Replacement Therapy

OBJECTIVETo investigate the diagnosis and treatment of male Kallmann syndrome.

METHODSWe retrospectively analyzed the clinical data of 12 cases of male Kallmann syndrome, 3 treated for male sterility and the other 9 for secondary sex characteristics dysplasia and external genitalia developmental anomalies, all by combined replacement therapy with human chorionic gonadotropin (hCG), human menopause gonadotropin (hMG) and testosterone undecanoate for 6 months to 3 years. We compared the secondary sexual development and serum sex hormone levels of the patients before and after treatment.

RESULTSAfter 9 months of treatment, all the 12 patients showed significant improvement in the penile length, testicular volume and sex hormone levels (P < 0.01), with different degrees of promotion of the secondary sexual development. Three married cases could have normal sexual intercourse, and one of them achieved normal pregnancy.

CONCLUSIONThe clinical characteristics of Kallmann syndrome include lack of gonadotropins, lower gonad function and loss or reduction of olfactory sensation. Replacement therapy with hCG, hMG and androgens is an effective treatment method. However, no effective therapy is now available for olfactory dysfunction. Early diagnosis and hormone replacement therapy can best alleviate its clinical symptoms and eventually achieve fertility.

Adolescent ; Adult ; Hormone Replacement Therapy ; Humans ; Kallmann Syndrome ; diagnosis ; drug therapy ; Male ; Retrospective Studies ; Young Adult

AIMIn 11 congenital hypogonadal men, the bone mineral density (BMD) values were determined to assess the effect of long-term androgen replacement therapy (ART) on skeletal integrity.

METHODSEleven congenital hypogonadal men, including 8 isolated gonadotropin deficiency patients, 2 Kallmann's syndrome and 1 vanishing testes syndrome were recruited and treated with 250 mg of testosterone enanthate intramuscularly every 4 weeks for 7-43 years (mean+/-SD: 21.5 +/-13 years). In these patients and a group of 10 healthy young men (controls), the whole and trabecular BMDs were examined at the distal end of radius by means of a peripheral quantitative computerized tomography device.

RESULTSThe whole radial BMD in hypogonadal men was significantly less in the patients than in the healthy men (498+/-115 and 725+/-134 mg/cm(3), respectively; P<0.01); the trabecular BMD was also lower in the hypogonadal men (199+/-80 and 375+/-89 mg/cm(3); P< 0.01). The whole radial BMD values in 10 of 11 hypogonadal men were at least 1 SD below the mean value for healthy young men; 2 hypogonadal men had BMD values more than 2.5 SD lower than the healthy mean. Additionally, the whole radial BMD showed a significant negative correlation with the patient's age at the initiation of ART (r = 0.748, P<0.01). The serum level of bone-specific alkaline phosphatase and the urinary level of deoxypyridinoline were not significantly different between the two groups.

CONCLUSIONOsteopenia persists in the hypogonadal men after long-term ART, suggesting that such patients have a persistent defect in bone development not alleviated by androgen replacement.

Adult ; Age Factors ; Bone Density ; drug effects ; Cryptorchidism ; drug therapy ; Hormone Replacement Therapy ; Humans ; Hypogonadism ; congenital ; drug therapy ; physiopathology ; Injections, Intramuscular ; Kallmann Syndrome ; drug therapy ; Male ; Middle Aged ; Reference Values ; Testosterone ; administration & dosage ; analogs & derivatives ; therapeutic use