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Reduced response to gonadotropin-releasing hormone stimulation test

MedGen UID:
1694726
Concept ID:
C5209252
Finding
Synonym: Reduce response to GnRH stimulation test
 
HPO: HP:0020159

Definition

Failure of the gonadotropin-releasing hormone (GnRH) stimulation test to induce an appropriate increased in luteinizing hormone (LH), follicle-stimulating hormone (FSH) levels. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVReduced response to gonadotropin-releasing hormone stimulation test

Conditions with this feature

Hypogonadotropic hypogonadism 22 with or without anosmia
MedGen UID:
863425
Concept ID:
C4014988
Disease or Syndrome
Isolated gonadotropin-releasing hormone (GnRH) deficiency (IGD) is characterized by inappropriately low serum concentrations of the gonadotropins LH (luteinizing hormone) and FSH (follicle-stimulating hormone) in the presence of low circulating concentrations of sex steroids. IGD is associated with a normal sense of smell (normosmic IGD) in approximately 40% of affected individuals and an impaired sense of smell (Kallmann syndrome) in approximately 60%. IGD can first become apparent in infancy, adolescence, or adulthood. Infant boys with congenital IGD often have micropenis and cryptorchidism. Adolescents and adults with IGD have clinical evidence of hypogonadism and incomplete sexual maturation on physical examination. Adult males with IGD tend to have prepubertal testicular volume (i.e., <4 mL), absence of secondary sexual features (e.g., facial and axillary hair growth, deepening of the voice), decreased muscle mass, diminished libido, erectile dysfunction, and infertility. Adult females have little or no breast development and primary amenorrhea. Although skeletal maturation is delayed, the rate of linear growth is usually normal except for the absence of a distinct pubertal growth spurt.
Hypogonadotropic hypogonadism 27 without anosmia
MedGen UID:
1810165
Concept ID:
C5676921
Disease or Syndrome
Hypogonadotropic hypogonadism-27 without anosmia (HH27) is characterized by lack of pubertal development associated with onset of obesity in early adolescence (Topaloglu et al., 2022). Congenital idiopathic hypogonadotropic hypogonadism (IHH) is a disorder characterized by absent or incomplete sexual maturation by the age of 18 years, in conjunction with low levels of circulating gonadotropins and testosterone and no other abnormalities of the hypothalamic-pituitary axis. Idiopathic hypogonadotropic hypogonadism can be caused by an isolated defect in gonadotropin-releasing hormone (GNRH; 152760) release, action, or both. Other associated nonreproductive phenotypes, such as anosmia, cleft palate, and sensorineural hearing loss, occur with variable frequency. In the presence of anosmia, idiopathic hypogonadotropic hypogonadism has been called 'Kallmann syndrome (KS),' whereas in the presence of a normal sense of smell, it has been termed 'normosmic idiopathic hypogonadotropic hypogonadism (nIHH)' (summary by Raivio et al., 2007). Because families have been found to segregate both KS and nIHH, the disorder is here referred to as 'hypogonadotropic hypogonadism with or without anosmia (HH).'

Professional guidelines

PubMed

Sansone A, Schubert M, Tüttelmann F, Krallmann C, Zitzmann M, Kliesch S, Gromoll J
Hum Reprod 2021 Apr 20;36(5):1376-1382. doi: 10.1093/humrep/deab033. PMID: 33704441
Ubaldi FM, Rienzi L, Ferrero S, Baroni E, Sapienza F, Cobellis L, Greco E
Reprod Biomed Online 2005 Feb;10(2):235-46. doi: 10.1016/s1472-6483(10)60946-7. PMID: 15823231
Tarlatzis BC, Zepiridis L, Grimbizis G, Bontis J
Hum Reprod Update 2003 Jan-Feb;9(1):61-76. doi: 10.1093/humupd/dmg007. PMID: 12638782

Recent clinical studies

Etiology

Oktem O, Esmaeilian Y, İltumur E, Yusufoglu S, Çimen DU, Incir S, Yakin K, Ata B, Urman B
Hum Reprod 2024 Oct 1;39(10):2331-2340. doi: 10.1093/humrep/deae189. PMID: 39180767
Liu C, Tian T, Lou Y, Li J, Liu P, Li R, Qiao J, Wang Y, Yang R
Expert Rev Mol Med 2023 Dec 14;26:e2. doi: 10.1017/erm.2023.25. PMID: 38095077Free PMC Article
Louden ED, Dougherty MP, Chorich LP, Eroglu A, Layman LC
F S Sci 2023 Nov;4(4):286-293. Epub 2023 Jul 27 doi: 10.1016/j.xfss.2023.07.003. PMID: 37516276
Sansone A, Schubert M, Tüttelmann F, Krallmann C, Zitzmann M, Kliesch S, Gromoll J
Hum Reprod 2021 Apr 20;36(5):1376-1382. doi: 10.1093/humrep/deab033. PMID: 33704441
Ubaldi FM, Rienzi L, Ferrero S, Baroni E, Sapienza F, Cobellis L, Greco E
Reprod Biomed Online 2005 Feb;10(2):235-46. doi: 10.1016/s1472-6483(10)60946-7. PMID: 15823231

Diagnosis

Oktem O, Esmaeilian Y, İltumur E, Yusufoglu S, Çimen DU, Incir S, Yakin K, Ata B, Urman B
Hum Reprod 2024 Oct 1;39(10):2331-2340. doi: 10.1093/humrep/deae189. PMID: 39180767
Luu TH, Kuhn K, Bradford AP, Wempe MF, Wittenburg L, Johnson RL, Carlson NE, Kumar TR, Polotsky AJ
Fertil Steril 2023 Oct;120(4):890-898. Epub 2023 Jun 3 doi: 10.1016/j.fertnstert.2023.05.170. PMID: 37276947Free PMC Article
Sansone A, Schubert M, Tüttelmann F, Krallmann C, Zitzmann M, Kliesch S, Gromoll J
Hum Reprod 2021 Apr 20;36(5):1376-1382. doi: 10.1093/humrep/deab033. PMID: 33704441
Al-Safi ZA, Liu H, Carlson NE, Chosich J, Lesh J, Robledo C, Bradford AP, Gee NA, Phang T, Santoro N, Kohrt W, Polotsky AJ
J Clin Endocrinol Metab 2015 Nov;100(11):4372-81. Epub 2015 Oct 1 doi: 10.1210/jc.2015-1946. PMID: 26425884Free PMC Article
Dickerman Z, Bar-Haim Y, Prager-Lewin R, Kaufman H, Laron Z
Acta Endocrinol (Copenh) 1977 Jul;85(3):459-64. PMID: 325981

Therapy

Liu C, Tian T, Lou Y, Li J, Liu P, Li R, Qiao J, Wang Y, Yang R
Expert Rev Mol Med 2023 Dec 14;26:e2. doi: 10.1017/erm.2023.25. PMID: 38095077Free PMC Article
Fraser GL, Obermayer-Pietsch B, Laven J, Griesinger G, Pintiaux A, Timmerman D, Fauser BCJM, Lademacher C, Combalbert J, Hoveyda HR, Ramael S
J Clin Endocrinol Metab 2021 Aug 18;106(9):e3519-e3532. doi: 10.1210/clinem/dgab320. PMID: 34000049Free PMC Article
Santoro N, Schauer IE, Kuhn K, Fought AJ, Babcock-Gilbert S, Bradford AP
Fertil Steril 2021 Aug;116(2):566-574. Epub 2021 Apr 8 doi: 10.1016/j.fertnstert.2021.03.005. PMID: 33838870Free PMC Article
Sansone A, Schubert M, Tüttelmann F, Krallmann C, Zitzmann M, Kliesch S, Gromoll J
Hum Reprod 2021 Apr 20;36(5):1376-1382. doi: 10.1093/humrep/deab033. PMID: 33704441
Tarlatzis BC, Zepiridis L, Grimbizis G, Bontis J
Hum Reprod Update 2003 Jan-Feb;9(1):61-76. doi: 10.1093/humupd/dmg007. PMID: 12638782

Prognosis

Baronio F, Assirelli V, Deiana G, AlQaisi R, Ortolano R, Di Natale V, Candela E, Cassio A
Medicina (Kaunas) 2024 Mar 18;60(3) doi: 10.3390/medicina60030497. PMID: 38541222Free PMC Article
Archer DF, Ng J, Chwalisz K, Chiu YL, Feinberg EC, Miller CE, Feldman RA, Klein CE
J Clin Endocrinol Metab 2020 Mar 1;105(3) doi: 10.1210/clinem/dgz086. PMID: 31650182
Nasioudis D, Minis E, Irani M, Kreines F, Witkin SS, Spandorfer SD
J Assist Reprod Genet 2019 Dec;36(12):2485-2491. Epub 2019 Nov 21 doi: 10.1007/s10815-019-01618-3. PMID: 31755001Free PMC Article
Ubaldi FM, Rienzi L, Ferrero S, Baroni E, Sapienza F, Cobellis L, Greco E
Reprod Biomed Online 2005 Feb;10(2):235-46. doi: 10.1016/s1472-6483(10)60946-7. PMID: 15823231
Tarlatzis BC, Zepiridis L, Grimbizis G, Bontis J
Hum Reprod Update 2003 Jan-Feb;9(1):61-76. doi: 10.1093/humupd/dmg007. PMID: 12638782

Clinical prediction guides

Luu TH, Kuhn K, Bradford AP, Wempe MF, Wittenburg L, Johnson RL, Carlson NE, Kumar TR, Polotsky AJ
Fertil Steril 2023 Oct;120(4):890-898. Epub 2023 Jun 3 doi: 10.1016/j.fertnstert.2023.05.170. PMID: 37276947Free PMC Article
Nasioudis D, Minis E, Irani M, Kreines F, Witkin SS, Spandorfer SD
J Assist Reprod Genet 2019 Dec;36(12):2485-2491. Epub 2019 Nov 21 doi: 10.1007/s10815-019-01618-3. PMID: 31755001Free PMC Article
Kolibianakis EM, Venetis CA, Kalogeropoulou L, Papanikolaou E, Tarlatzis BC
Fertil Steril 2011 Feb;95(2):558-62. Epub 2010 Jul 16 doi: 10.1016/j.fertnstert.2010.05.052. PMID: 20637457
Ubaldi FM, Rienzi L, Ferrero S, Baroni E, Sapienza F, Cobellis L, Greco E
Reprod Biomed Online 2005 Feb;10(2):235-46. doi: 10.1016/s1472-6483(10)60946-7. PMID: 15823231
Tarlatzis BC, Zepiridis L, Grimbizis G, Bontis J
Hum Reprod Update 2003 Jan-Feb;9(1):61-76. doi: 10.1093/humupd/dmg007. PMID: 12638782

Recent systematic reviews

Liu C, Tian T, Lou Y, Li J, Liu P, Li R, Qiao J, Wang Y, Yang R
Expert Rev Mol Med 2023 Dec 14;26:e2. doi: 10.1017/erm.2023.25. PMID: 38095077Free PMC Article
Tarlatzis BC, Zepiridis L, Grimbizis G, Bontis J
Hum Reprod Update 2003 Jan-Feb;9(1):61-76. doi: 10.1093/humupd/dmg007. PMID: 12638782

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