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Decreased growth hormone responses to growth hormone-releasing hormone challenge

MedGen UID:
1778726
Concept ID:
C5539698
Finding
Synonym: Impaired growth hormone secretory responses after growth hormone-releasing hormone challenge
 
HPO: HP:0033579

Definition

Insufficient growth hormone secretion following administration of growth hormone-releasing hormone. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDecreased growth hormone responses to growth hormone-releasing hormone challenge

Conditions with this feature

Obesity due to pro-opiomelanocortin deficiency
MedGen UID:
341863
Concept ID:
C1857854
Disease or Syndrome
OBAIRH is an autosomal recessive endocrine disorder characterized by early-onset obesity due to severe hyperphagia, pigmentary abnormalities, mainly pale skin and red hair, and secondary hypocortisolism. In the neonatal period, affected individuals are prone to hypoglycemia, hyperbilirubinemia, and cholestasis that may result in death if not treated. The disorder results from mutation in the POMC gene, which encodes a preproprotein that is processed into a range of bioactive peptides, including alpha-melanocyte-stimulating hormone (MSH) and ACTH (summary by Kuhnen et al., 2016 and Clement et al., 2008).
Acrodysostosis 1 with or without hormone resistance
MedGen UID:
477858
Concept ID:
C3276228
Disease or Syndrome
Acrodysostosis-1 (ACRDYS1) is a form of skeletal dysplasia characterized by short stature, severe brachydactyly, facial dysostosis, and nasal hypoplasia. Affected individuals often have advanced bone age and obesity. Laboratory studies show resistance to multiple hormones, including parathyroid, thyrotropin, calcitonin, growth hormone-releasing hormone, and gonadotropin (summary by Linglart et al., 2011). However, not all patients show endocrine abnormalities (Lee et al., 2012). Genetic Heterogeneity of Acrodysostosis See also ACRDYS2 (614613), caused by mutation in the PDE4D gene (600129) on chromosome 5q12.

Professional guidelines

PubMed

Fasouliotis SJ, Simon A, Laufer N
J Assist Reprod Genet 2000 Aug;17(7):357-73. doi: 10.1023/a:1009465324197. PMID: 11077616Free PMC Article

Recent clinical studies

Etiology

Duval F, Mokrani MC, Erb A, Danila V, Lopera FG, Foucher JR, Jeanjean LC
Psychoneuroendocrinology 2021 Jun;128:105219. Epub 2021 Apr 3 doi: 10.1016/j.psyneuen.2021.105219. PMID: 33839430
Dovolou E, Chadio S, Messinis IE, Rekkas CA, Deligiannis C, Kalogiannis D, Amiridis GS
Theriogenology 2013 Aug;80(3):262-8. Epub 2013 May 9 doi: 10.1016/j.theriogenology.2013.04.005. PMID: 23664792
Fasouliotis SJ, Simon A, Laufer N
J Assist Reprod Genet 2000 Aug;17(7):357-73. doi: 10.1023/a:1009465324197. PMID: 11077616Free PMC Article
Maghnie M, Bossi G, Klersy C, Cosi G, Genovese E, Aricò M
J Clin Endocrinol Metab 1998 Sep;83(9):3089-94. doi: 10.1210/jcem.83.9.5108. PMID: 9745408
Geris KL, Hickey GJ, Berghman LR, Visser TJ, Kühn ER, Darras VM
Gen Comp Endocrinol 1998 Aug;111(2):186-96. doi: 10.1006/gcen.1998.7102. PMID: 9679090

Diagnosis

Kageyama K, Sakihara S, Kameda W, Sugiyama A, Takayasu S, Terui K, Daimon M
Endocr J 2018 Oct 29;65(10):1049-1054. Epub 2018 Jul 5 doi: 10.1507/endocrj.EJ17-0527. PMID: 29973439
Wong-Riley MT, Liu Q
Respir Physiol Neurobiol 2005 Nov 15;149(1-3):83-98. doi: 10.1016/j.resp.2005.01.011. PMID: 16203213
Fasouliotis SJ, Simon A, Laufer N
J Assist Reprod Genet 2000 Aug;17(7):357-73. doi: 10.1023/a:1009465324197. PMID: 11077616Free PMC Article
Vasankari TJ, Kujala UM, Taimela S, Huhtaniemi IT
Acta Endocrinol (Copenh) 1993 Jul;129(1):9-14. doi: 10.1530/acta.0.1290009. PMID: 8351961
Blackwell RE, Boots LR, Goldenberg RL, Younger JB
Fertil Steril 1979 Aug;32(2):177-82. PMID: 572785

Therapy

Fasouliotis SJ, Simon A, Laufer N
J Assist Reprod Genet 2000 Aug;17(7):357-73. doi: 10.1023/a:1009465324197. PMID: 11077616Free PMC Article
Lands WE
Alcohol 1999 Jun-Jul;18(2-3):109-22. doi: 10.1016/s0741-8329(98)00073-1. PMID: 10456561
Juul A, Andersson AM, Pedersen SA, Jørgensen JO, Christiansen JS, Groome NP, Skakkebaek NE
Horm Res 1998;49(6):269-78. doi: 10.1159/000023186. PMID: 9623518
Motta M, Dondi D, Moretti RM, Montagnani Marelli M, Pimpinelli F, Maggi R, Limonta P
J Steroid Biochem Mol Biol 1996 Jan;56(1-6 Spec No):107-11. doi: 10.1016/0960-0760(95)00240-5. PMID: 8603030
Vance ML, Kaiser DL, Rivier J, Vale W, Thorner MO
J Clin Endocrinol Metab 1986 Mar;62(3):591-4. doi: 10.1210/jcem-62-3-591. PMID: 3080467

Prognosis

Fasouliotis SJ, Simon A, Laufer N
J Assist Reprod Genet 2000 Aug;17(7):357-73. doi: 10.1023/a:1009465324197. PMID: 11077616Free PMC Article
Maghnie M, Bossi G, Klersy C, Cosi G, Genovese E, Aricò M
J Clin Endocrinol Metab 1998 Sep;83(9):3089-94. doi: 10.1210/jcem.83.9.5108. PMID: 9745408
Fernández-Vázquez G, Kaiser UB, Albarracin CT, Chin WW
Mol Endocrinol 1996 Apr;10(4):356-66. doi: 10.1210/mend.10.4.8721981. PMID: 8721981
Huseman CA, Moriarty CM, Angle CR
Environ Res 1987 Apr;42(2):524-33. doi: 10.1016/s0013-9351(87)80219-0. PMID: 3106028
Blackwell RE, Boots LR, Goldenberg RL, Younger JB
Fertil Steril 1979 Aug;32(2):177-82. PMID: 572785

Clinical prediction guides

Duval F, Mokrani MC, Erb A, Danila V, Lopera FG, Foucher JR, Jeanjean LC
Psychoneuroendocrinology 2021 Jun;128:105219. Epub 2021 Apr 3 doi: 10.1016/j.psyneuen.2021.105219. PMID: 33839430
Kageyama K, Sakihara S, Kameda W, Sugiyama A, Takayasu S, Terui K, Daimon M
Endocr J 2018 Oct 29;65(10):1049-1054. Epub 2018 Jul 5 doi: 10.1507/endocrj.EJ17-0527. PMID: 29973439
Maghnie M, Bossi G, Klersy C, Cosi G, Genovese E, Aricò M
J Clin Endocrinol Metab 1998 Sep;83(9):3089-94. doi: 10.1210/jcem.83.9.5108. PMID: 9745408
Geris KL, Hickey GJ, Berghman LR, Visser TJ, Kühn ER, Darras VM
Gen Comp Endocrinol 1998 Aug;111(2):186-96. doi: 10.1006/gcen.1998.7102. PMID: 9679090
Blackwell RE, Boots LR, Goldenberg RL, Younger JB
Fertil Steril 1979 Aug;32(2):177-82. PMID: 572785

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