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Isolated gonadotropin deficiency

MedGen UID:
543431
•Concept ID:
C0271577
•
Disease or Syndrome
Synonym: Isolated gonadotrophin deficiency
SNOMED CT: Isolated gonadotropin deficiency (10340008); Isolated gonadotrophin deficiency (10340008)

Professional guidelines

PubMed

Treatment of hypogonadotropic hypogonadal male patients with the luteinizing hormone-relasing hormone (LH-RH) anolog D-Ser(TBU)6 EA10 LH-RH: transient disappearance of gonadotropin stimulation.
Morel Y, Fournier M, Mazenod B, Tourniaire J, Mornex R
Fertil Steril 1982 Jul;38(1):85-91. doi: 10.1016/s0015-0282(16)46400-9. PMID: 6807722
Treatment of isolated gonadotropin deficiency in men with synthetic LR-RH and a more potent analogue of LH-RH.
Tharandt L, Schulte H, Benker G, Hackenberg K, Reinwein D
Neuroendocrinology 1977;24(3-4):195-207. doi: 10.1159/000122708. PMID: 345145

Recent clinical studies

Etiology

Pituitary gonadotropins and autoimmunity.
Cocco C, Brancia C, D'Amato F, Noli B
Mol Cell Endocrinol 2014 Mar 25;385(1-2):97-104. Epub 2013 Oct 20 doi: 10.1016/j.mce.2013.10.009. PMID: 24153235
Effect of testosterone on bone density and bone metabolism in adolescent male hypogonadism.
Arisaka O, Arisaka M, Nakayama Y, Fujiwara S, Yabuta K
Metabolism 1995 Apr;44(4):419-23. doi: 10.1016/0026-0495(95)90046-2. PMID: 7723663
Isolated gonadotropin deficiency in boys: clinical characteristics and growth.
Van Dop C, Burstein S, Conte FA, Grumbach MM
J Pediatr 1987 Nov;111(5):684-92. doi: 10.1016/s0022-3476(87)80243-3. PMID: 2889818
Diagnosis of gonadotropin deficiency in adolescents: limited usefulness of a standard gonadotropin-releasing hormone test in obese boys.
Kelch RP, Hopwood NJ, Marshall JC
J Pediatr 1980 Nov;97(5):820-4. doi: 10.1016/s0022-3476(80)80279-4. PMID: 6776256
Normal adults and subjects with hypogonadotropic hypogonadism respond differently to D-Ser(TBU)6-LH-RH-EA10.
Smith R, Donald RA, Espiner EA, Stronach SG, Edwards IA
J Clin Endocrinol Metab 1979 Jan;48(1):167-70. doi: 10.1210/jcem-48-1-167. PMID: 106067

Diagnosis

Puberty and its disorders in boys.
Styne DM
Endocrinol Metab Clin North Am 1991 Mar;20(1):43-69. PMID: 2029888
Isolated gonadotropin deficiency in boys: clinical characteristics and growth.
Van Dop C, Burstein S, Conte FA, Grumbach MM
J Pediatr 1987 Nov;111(5):684-92. doi: 10.1016/s0022-3476(87)80243-3. PMID: 2889818
The use of a low-dose LHRH test for the distinction of delayed adolescence and isolated gonadotropin deficiency.
Vourtsis K, Illig R
Helv Paediatr Acta 1982;37(5):437-47. PMID: 6819257
Diagnosis of gonadotropin deficiency in adolescents: limited usefulness of a standard gonadotropin-releasing hormone test in obese boys.
Kelch RP, Hopwood NJ, Marshall JC
J Pediatr 1980 Nov;97(5):820-4. doi: 10.1016/s0022-3476(80)80279-4. PMID: 6776256
Isolated gonadotropin deficiency and related disorders.
Rabinowitz D, Spitz IM
Isr J Med Sci 1975 Oct;11(10):1011-78. PMID: 1104523

Therapy

Isolated gonadotropin deficiency in boys: clinical characteristics and growth.
Van Dop C, Burstein S, Conte FA, Grumbach MM
J Pediatr 1987 Nov;111(5):684-92. doi: 10.1016/s0022-3476(87)80243-3. PMID: 2889818
The thyrotropin (TSH) profile in isolated gonadotropin deficiency: a model to evaluate the effect of sex steroids on TSH secretion.
Spitz IM, Zylber-Haran EA, Trestian S
J Clin Endocrinol Metab 1983 Aug;57(2):415-20. doi: 10.1210/jcem-57-2-415. PMID: 6408116
Normal adults and subjects with hypogonadotropic hypogonadism respond differently to D-Ser(TBU)6-LH-RH-EA10.
Smith R, Donald RA, Espiner EA, Stronach SG, Edwards IA
J Clin Endocrinol Metab 1979 Jan;48(1):167-70. doi: 10.1210/jcem-48-1-167. PMID: 106067
Treatment of isolated gonadotropin deficiency in men with synthetic LR-RH and a more potent analogue of LH-RH.
Tharandt L, Schulte H, Benker G, Hackenberg K, Reinwein D
Neuroendocrinology 1977;24(3-4):195-207. doi: 10.1159/000122708. PMID: 345145
Isolated gonadotropin deficiency. A heterogenous syndrome.
Spitz IM, Diamant Y, Rosen E, Bell J, David MB, Polishuk W, Rabinowitz D
N Engl J Med 1974 Jan 3;290(1):10-5. doi: 10.1056/NEJM197401032900103. PMID: 4586101

Prognosis

Partial hypopituitarism with ACTH deficiency as the main manifestation as a complication of hemorrhagic fever with renal syndrome.
Shi S, Zhang A, Zhang J, Xu S
BMC Endocr Disord 2024 May 7;24(1):61. doi: 10.1186/s12902-024-01587-4. PMID: 38715016Free PMC Article
Adult-onset idiopathic hypogonadotropic hypogonadism due to isolated pituitary gonadotropin deficiency.
Suzuki F, Shimizu C, Umetsu M, Nagai S, Takeuchi J, Endo M, Miyoshi H, Yoshioka N, Kubo M, Koike T
Intern Med 2004 Jul;43(7):571-4. doi: 10.2169/internalmedicine.43.571. PMID: 15335182
Delayed puberty in obese boys: comparison with constitutional delayed puberty and response to testosterone therapy.
Kaplowitz P
J Pediatr 1998 Dec;133(6):745-9. doi: 10.1016/s0022-3476(98)70144-1. PMID: 9842037
Post-traumatic selective hypogonadotropic hypogonadism.
Benvenga S, Lo Giudice F, Campenni A, Longo M, Trimarchi F
J Endocrinol Invest 1997 Dec;20(11):675-80. doi: 10.1007/BF03348031. PMID: 9492108
Induction of ovulation and pregnancy with pulsatile luteinizing hormone releasing factor: dosage and mode of delivery.
Reid RL, Leopold GR, Yen SS
Fertil Steril 1981 Nov;36(5):553-9. doi: 10.1016/s0015-0282(16)45850-4. PMID: 6796436

Clinical prediction guides

Effect of testosterone on bone density and bone metabolism in adolescent male hypogonadism.
Arisaka O, Arisaka M, Nakayama Y, Fujiwara S, Yabuta K
Metabolism 1995 Apr;44(4):419-23. doi: 10.1016/0026-0495(95)90046-2. PMID: 7723663
Gonadotropin-releasing activity of alpha-melanocyte-stimulating hormone in normal subjects and in subjects with hypothalamic-pituitary dysfunction.
Reid RL, Ling N, Yen SS
J Clin Endocrinol Metab 1984 May;58(5):773-7. doi: 10.1210/jcem-58-5-773. PMID: 6423658
Induction of ovulation and pregnancy with pulsatile luteinizing hormone releasing factor: dosage and mode of delivery.
Reid RL, Leopold GR, Yen SS
Fertil Steril 1981 Nov;36(5):553-9. doi: 10.1016/s0015-0282(16)45850-4. PMID: 6796436
Lack of a biphasic steroid response to single human chorionic gonadotropin administration in patients with isolated gonadotropin deficiency.
Smals AG, Pieters GF, Kloppenborg PW, Lozekoot DC, Benraad TJ
J Clin Endocrinol Metab 1980 May;50(5):879-81. doi: 10.1210/jcem-50-5-879. PMID: 6768759
Dissociation of prolactin responsiveness to TRH and chlorpromazine in women with isolated gonadotropin deficiency.
Spitz IM, Almaliach U, Rosen E, Polishuk W, Rabinowitz D
J Clin Endocrinol Metab 1977 Dec;45(6):1173-8. doi: 10.1210/jcem-45-6-1173. PMID: 412859

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