skip banner - Return to original view
site viewing options
 
Parents|Medical Information|Professionals|In your area|Campaigns

Congenital Adrenal Hyperplasia

How is it treated?

Treatment consists of surgery in girls to reduce the size of the clitoris and open up the lower end of the vagina. Both sexes require hydrocortisone to suppress ACTH secretion and thus switch off androgen production, fludrocortisone to replace aldosterone and (during the first year of life) salt supplements.

The dose of hydrocortisone has to be carefully adjusted since under- treatment will result in over-production of androgen with excessive growth and virilisation, while over-treatment will cause slow growth and obesity. By adjusting the dose of hydrocortisone against the child's size, growth rate, skeletal maturity ('bone age') and adrenal steroid levels the treatment can be optimised and the general health of these children is good. It is important for parents to learn how to increase the hydrocortisone dose during acute illness, so as to mimic the normal cortisol response to stress. In particular, there is a need for an emergency regimen of hydrocortisone injection and salt replacement in severe illness.

Adults
The management of Congenital Adrenal Hyperplasia is very similar in adults. Treatment needs to be continued in men, particularly for them to maintain fertility. Women may have problems with excessive hair growth which requires specific treatment. It is important to have an assessment of their genitalia and vagina and additional surgery or non-surgical dilatation treatment may be need for comfortable sexual intercourse. Polycystic ovarian syndrome has an increased incidence and probably accounts for the increased risk of infertility. However, the infertility can usually be treated. There are several UK centres offering specialist Congenital Adrenal Hyperplasia clinics for adults.

View What are the symptoms? What are the symptoms?  |  Inheritance patterns and prenatal diagnosis View Inheritance patterns and prenatal diagnosis

Medical text written August 1996 by Dr M Donaldson, Senior Lecturer in Child Health, Royal Hospital for Sick Children, Glasgow, UK and Dr R Stanhope. Material on Adults written October 2002 by Dr R Stanhope. Last updated August 2006 by Dr R Stanhope, Consultant Paediatric Endocrinologist, Great Ormond Street Hospital, London, UK.

 

Tell us what you think of this information...

Print whole article Print whole article

 

This Web Site © Copyright, Contact a Family 2008
Contact a Family, 209-211 City Road, London EC1V 1JN
Tel: (020) 7608 8700

Registered Charity No. 284912. Charity registered in Scotland No. SC039169
Company limited by guarantee, registered in England and Wales No. 1633333.
HM Revenue & Customs charity tax reference No. XN54769. VAT Reg. No. GB 749 3846 82