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Impaired cortisol response to corticotropin releasing hormone stimulation test

MedGen UID:
1378460
Concept ID:
C4476957
Finding
HPO: HP:0031078

Definition

Failure of cortisol levels to respond adequately (by increasing) to the corticotropin releasing hormone stimulation test. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVImpaired cortisol response to corticotropin releasing hormone stimulation test

Conditions with this feature

3 beta-Hydroxysteroid dehydrogenase deficiency
MedGen UID:
452446
Concept ID:
C0342471
Disease or Syndrome
Classic 3-beta-hydroxysteroid dehydrogenase deficiency is an autosomal recessive form of CAH characterized by a severe impairment of steroid biosynthesis in both the adrenals and the gonads, resulting in decreased excretion of cortisol and aldosterone and of progesterone, androgens, and estrogens by these tissues. Affected newborns exhibit signs and symptoms of glucocorticoid and mineralocorticoid deficiencies, which may be fatal if not diagnosed and treated early, especially in the severe salt-wasting form. Moreover, male newborns exhibit pseudohermaphroditism with incomplete masculinization of the external genitalia due to an impairment of androgen biosynthesis in the testis. In contrast, affected females exhibit normal sexual differentiation or partial virilization (summary by Rheaume et al., 1992).

Recent clinical studies

Etiology

van Rossum EF, Binder EB, Majer M, Koper JW, Ising M, Modell S, Salyakina D, Lamberts SW, Holsboer F
Biol Psychiatry 2006 Apr 15;59(8):681-8. doi: 10.1016/j.biopsych.2006.02.007. PMID: 16580345
Schroeder S, Wichers M, Klingmüller D, Höfer M, Lehmann LE, von Spiegel T, Hering R, Putensen C, Hoeft A, Stüber F
Crit Care Med 2001 Feb;29(2):310-6. doi: 10.1097/00003246-200102000-00017. PMID: 11246311
Costa A, Bono G, Martignoni E, Merlo P, Sances G, Nappi G
Psychoneuroendocrinology 1996 Apr;21(3):263-75. doi: 10.1016/0306-4530(96)00001-7. PMID: 8817725
Brambilla F, Ferrari E, Brunetta M, Peirone A, Draisci A, Sacerdote P, Panerai A
Psychiatry Res 1996 Apr 16;62(1):97-104. doi: 10.1016/0165-1781(96)02992-7. PMID: 8739119
Wand GS, Dobs AS
J Clin Endocrinol Metab 1991 Jun;72(6):1290-5. doi: 10.1210/jcem-72-6-1290. PMID: 2026749

Diagnosis

Schroeder S, Wichers M, Klingmüller D, Höfer M, Lehmann LE, von Spiegel T, Hering R, Putensen C, Hoeft A, Stüber F
Crit Care Med 2001 Feb;29(2):310-6. doi: 10.1097/00003246-200102000-00017. PMID: 11246311
Meczekalski B, Tonetti A, Monteleone P, Bernardi F, Luisi S, Stomati M, Luisi M, Petraglia F, Genazzani AR
Eur J Endocrinol 2000 Mar;142(3):280-5. doi: 10.1530/eje.0.1420280. PMID: 10700723
Clark DJ, Lipworth BJ
Chest 1997 Nov 5;112(5):1248-52. doi: 10.1378/chest.112.5.1248. PMID: 9367464
von Bardeleben U, Holsboer F
Biol Psychiatry 1991 May 15;29(10):1042-50. doi: 10.1016/0006-3223(91)90360-x. PMID: 2065137
Gold PW, Loriaux DL, Roy A, Kling MA, Calabrese JR, Kellner CH, Nieman LK, Post RM, Pickar D, Gallucci W
N Engl J Med 1986 May 22;314(21):1329-35. doi: 10.1056/NEJM198605223142101. PMID: 3010108

Therapy

Ising M, Zimmermann US, Künzel HE, Uhr M, Foster AC, Learned-Coughlin SM, Holsboer F, Grigoriadis DE
Neuropsychopharmacology 2007 Sep;32(9):1941-9. Epub 2007 Feb 7 doi: 10.1038/sj.npp.1301328. PMID: 17287823
van Rossum EF, Binder EB, Majer M, Koper JW, Ising M, Modell S, Salyakina D, Lamberts SW, Holsboer F
Biol Psychiatry 2006 Apr 15;59(8):681-8. doi: 10.1016/j.biopsych.2006.02.007. PMID: 16580345
Clark DJ, Lipworth BJ
Chest 1997 Nov 5;112(5):1248-52. doi: 10.1378/chest.112.5.1248. PMID: 9367464
Gudbjörnsson B, Skogseid B, Oberg K, Wide L, Hällgren R
J Rheumatol 1996 Apr;23(4):596-602. PMID: 8730111
Fukata J, Usui T, Tsukada T, Nakai Y, Koh T, Ishihara T, Tanaka I, Uchida K, Yoshimasa T, Nakano R
J Clin Endocrinol Metab 1990 Dec;71(6):1624-31. doi: 10.1210/jcem-71-6-1624. PMID: 2172283

Prognosis

Iwayama H, Hirase S, Nomura Y, Ito T, Morita H, Otake K, Okumura A, Takagi J
BMC Endocr Disord 2018 Mar 27;18(1):19. doi: 10.1186/s12902-018-0246-2. PMID: 29587720Free PMC Article
Perras B, Berkemeier E, Rasch B, Fehm HL, Born J
Neuropeptides 2007 Aug;41(4):233-8. Epub 2007 May 7 doi: 10.1016/j.npep.2007.03.002. PMID: 17482675
Heesen C, Gold SM, Raji A, Wiedemann K, Schulz KH
Psychoneuroendocrinology 2002 May;27(4):505-17. doi: 10.1016/s0306-4530(01)00071-3. PMID: 11912002
Clark DJ, Lipworth BJ
Chest 1997 Nov 5;112(5):1248-52. doi: 10.1378/chest.112.5.1248. PMID: 9367464
von Bardeleben U, Holsboer F
Biol Psychiatry 1991 May 15;29(10):1042-50. doi: 10.1016/0006-3223(91)90360-x. PMID: 2065137

Clinical prediction guides

Iwayama H, Hirase S, Nomura Y, Ito T, Morita H, Otake K, Okumura A, Takagi J
BMC Endocr Disord 2018 Mar 27;18(1):19. doi: 10.1186/s12902-018-0246-2. PMID: 29587720Free PMC Article
Zietz B, Lock G, Plach B, Drobnik W, Grossmann J, Schölmerich J, Straub RH
Eur J Gastroenterol Hepatol 2003 May;15(5):495-501. doi: 10.1097/01.meg.0000059115.41030.e0. PMID: 12702906
Schroeder S, Wichers M, Klingmüller D, Höfer M, Lehmann LE, von Spiegel T, Hering R, Putensen C, Hoeft A, Stüber F
Crit Care Med 2001 Feb;29(2):310-6. doi: 10.1097/00003246-200102000-00017. PMID: 11246311
Clark DJ, Lipworth BJ
Chest 1997 Nov 5;112(5):1248-52. doi: 10.1378/chest.112.5.1248. PMID: 9367464
Fukata J, Usui T, Tsukada T, Nakai Y, Koh T, Ishihara T, Tanaka I, Uchida K, Yoshimasa T, Nakano R
J Clin Endocrinol Metab 1990 Dec;71(6):1624-31. doi: 10.1210/jcem-71-6-1624. PMID: 2172283

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