11β-hydroxylase deficiency natural history, complications and prognosis: Difference between revisions
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{{CMG}}; {{AE}} {{MJ}} | {{CMG}}; {{AE}} {{MJ}} | ||
==Overview== | ==Overview== | ||
If left untreated, patients with | If left untreated, patients with 11β-hydroxylase deficiency may progress to develop [[malignant hypertension]]. Common [[complications]] of 11β-hydroxylase deficiency include [[muscle weakness]], [[metabolic alkalosis]], [[menstrual irregularities]] in women, [[acne]], [[hirsutism]], and [[infertility]]. Prognosis is generally good with treatment. | ||
==Natural History== | ==Natural History== | ||
If left untreated, patients with | If left untreated, patients with 11β-hydroxylase deficiency may progress to develop [[malignant hypertension|malignant hypertension.]]Untreated children progress into isosexual or contra-sexual pseudo-[[precocious puberty]]; early [[puberty]] in boys, [[acne]] and [[hirsutism]] in girls, [[menstrual irregularities]] in women, and [[infertility]] .<ref name="pmid6296182">{{cite journal |vauthors=Zachmann M, Tassinari D, Prader A |title=Clinical and biochemical variability of congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency. A study of 25 patients |journal=J. Clin. Endocrinol. Metab. |volume=56 |issue=2 |pages=222–9 |year=1983 |pmid=6296182 |doi=10.1210/jcem-56-2-222 |url=}}</ref><ref name="pmid28576284">{{cite journal |vauthors=El-Maouche D, Arlt W, Merke DP |title=Congenital adrenal hyperplasia |journal=Lancet |volume= |issue= |pages= |year=2017 |pmid=28576284 |doi=10.1016/S0140-6736(17)31431-9 |url=}}</ref> | ||
Untreated children progress into isosexual or | |||
==Complications== | ==Complications== | ||
===Complications of Hypertension=== | ===Complications of Hypertension=== | ||
* Vascular hemorrhage | * [[Vascular]] [[hemorrhage]] | ||
* [[Renal insufficiency]] | * [[Renal insufficiency]] | ||
* [[Left ventricular hypertrophy]] | * [[Left ventricular hypertrophy]] | ||
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* [[Stroke]] | * [[Stroke]] | ||
===Complications of Hypokalemia=== | ===Complications of Hypokalemia=== | ||
* Muscle weakness | * [[Muscle weakness]] | ||
* [[Metabolic alkalosis]] | * [[Metabolic alkalosis]] | ||
* [[Paralytic ileus]] | * [[Paralytic ileus]] | ||
===Other Complications=== | ===Other Complications=== | ||
* Early [[puberty]] in boys | |||
* [[Acne]] | |||
* [[Hirsutism]] in girls | |||
* [[Menstrual irregularities]] in women | |||
* [[Azoospermia]] | * [[Azoospermia]] | ||
* [[Oligospermia]] | * [[Oligospermia]] | ||
* [[Infertility]] | * [[Infertility]] | ||
==Prognosis== | ==Prognosis== | ||
*The prognosis of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency is generally good with treatment.<ref name="pmid6296182">{{cite journal |vauthors=Zachmann M, Tassinari D, Prader A |title=Clinical and biochemical variability of congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency. A study of 25 patients |journal=J. Clin. Endocrinol. Metab. |volume=56 |issue=2 |pages=222–9 |year=1983 |pmid=6296182 |doi=10.1210/jcem-56-2-222 |url=}}</ref><ref name="pmid28576284">{{cite journal |vauthors=El-Maouche D, Arlt W, Merke DP |title=Congenital adrenal hyperplasia |journal=Lancet |volume= |issue= |pages= |year=2017 |pmid=28576284 |doi=10.1016/S0140-6736(17)31431-9 |url=}}</ref> | *The prognosis of congenital adrenal hyperplasia due to [[11β-hydroxylase]] deficiency is generally good with treatment.<ref name="pmid6296182">{{cite journal |vauthors=Zachmann M, Tassinari D, Prader A |title=Clinical and biochemical variability of congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency. A study of 25 patients |journal=J. Clin. Endocrinol. Metab. |volume=56 |issue=2 |pages=222–9 |year=1983 |pmid=6296182 |doi=10.1210/jcem-56-2-222 |url=}}</ref><ref name="pmid28576284">{{cite journal |vauthors=El-Maouche D, Arlt W, Merke DP |title=Congenital adrenal hyperplasia |journal=Lancet |volume= |issue= |pages= |year=2017 |pmid=28576284 |doi=10.1016/S0140-6736(17)31431-9 |url=}}</ref> | ||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
Latest revision as of 19:37, 18 October 2017
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11β-hydroxylase deficiency Microchapters |
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Differentiating 11β-hydroxylase deficiency from other Diseases |
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Directions to Hospitals Treating Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency |
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 11β-hydroxylase deficiency may progress to develop malignant hypertension. Common complications of 11β-hydroxylase deficiency include muscle weakness, metabolic alkalosis, menstrual irregularities in women, acne, hirsutism, and infertility. Prognosis is generally good with treatment.
Natural History
If left untreated, patients with 11β-hydroxylase deficiency may progress to develop malignant hypertension.Untreated children progress into isosexual or contra-sexual pseudo-precocious puberty; early puberty in boys, acne and hirsutism in girls, menstrual irregularities in women, and infertility .[1][2]
Complications
Complications of Hypertension
- Vascular hemorrhage
- Renal insufficiency
- Left ventricular hypertrophy
- Hypertensive retinopathy
- Stroke
Complications of Hypokalemia
Other Complications
- Early puberty in boys
- Acne
- Hirsutism in girls
- Menstrual irregularities in women
- Azoospermia
- Oligospermia
- Infertility
Prognosis
- The prognosis of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency is generally good with treatment.[1][2]
References
- ↑ 1.0 1.1 Zachmann M, Tassinari D, Prader A (1983). "Clinical and biochemical variability of congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency. A study of 25 patients". J. Clin. Endocrinol. Metab. 56 (2): 222–9. doi:10.1210/jcem-56-2-222. PMID 6296182.
- ↑ 2.0 2.1 El-Maouche D, Arlt W, Merke DP (2017). "Congenital adrenal hyperplasia". Lancet. doi:10.1016/S0140-6736(17)31431-9. PMID 28576284.