21-hydroxylase deficiency natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]

Overview

If left untreated, patients with 21-hydroxylase deficiency may progress to develop complications. Common complications of 21-hydroxylase deficient congenital adrenal hyperplasia include short stature, adrenal crisis, infertility, and precocious puberty. The prognosis of 21-hydroxylase deficiency is generally good with treatment.

Natural History

Complications

Common complications associated with 21-hydroxylase deficiency include:[1][2]

Prognosis

  • The prognosis of 21-hydroxylase deficiency is generally good with treatment.
  • A small percentage of children and adults with infancy or childhood onset 21 hydroxylase deficiency die of adrenal crisis, even after diagnosis and initiation of treatment.
  • There may be no immediate worsening of symptoms if a person is well and has missed a dose or even several doses. However, glucocorticoid needs are increased during illness and stress.
  • Missed doses during time of illness can lead (within hours) to hypotension, shock, and death.[1][2]

References

  1. 1.0 1.1 1.2 Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP, Meyer-Bahlburg HF, Miller WL, Montori VM, Oberfield SE, Ritzen M, White PC (2010). "Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline". J. Clin. Endocrinol. Metab. 95 (9): 4133–60. PMC 2936060Freely accessible. PMID 20823466. doi:10.1210/jc.2009-2631. 
  2. 2.0 2.1 2.2 van der Kamp HJ, Wit JM (2004). "Neonatal screening for congenital adrenal hyperplasia". Eur. J. Endocrinol. 151 Suppl 3: U71–5. PMID 15554889. 

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