Document Detail

Bilateral adrenalectomy for severe hypertension in congenital adrenal hyperplasia due to 11beta-hydroxylase deficiency: long term follow-up.
MedLine Citation:
PMID:  19195642     Owner:  NLM     Status:  MEDLINE    
CONTEXT: Bilateral adrenalectomy has been recently proposed as a surgical treatment option for patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. There is however little documented data about the long-term efficiency and potential side effects of this treatment. Patients with 11beta-hydroxylase deficiency (11betaHD) have been also concerned by this new approach.
OBJECTIVE: Our objective was to describe our experience with bilateral adrenalectomy as a treatment of severe hypertension in a patient with 11betaHD deficiency and to report the long term follow-up (72 months) results after surgery.
PATIENT AND INTERVENTION: A 22-year-old genetically female patient with 11betaHD deficiency was raised as a male because of severe pseudohermaphroditism. The patient has been managed by conventional steroid suppressive therapy and antihypertensive drugs with limited success; hypertension remained uncontrolled and led to severe complications. Bilateral adrenalectomy was offered to him.
RESULTS: The intervention was followed by immediate blood pressure normalization and resulted in remarkable clinical improvement. Good compliance with glucocorticoid and androgen substitutive therapies was noted. However, a high 11-deoxycortisol, presumably due to non-ovarian ectopic adrenal rests was noted 24 months after surgery.
CONCLUSION: Bilateral adrenalectomy is a safe and efficient method of managing CAH with selected patients. Long-term clinical and biochemical follow-up of patients with CAH treated by bilateral adrenalectomy is needed to earlier detect ectopic adrenal rests.
M Kacem; A Moussa; I Khochtali; R Nabouli; Y Morel; A Zakhama
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2009-02-04
Journal Detail:
Title:  Annales d'endocrinologie     Volume:  70     ISSN:  0003-4266     ISO Abbreviation:  Ann. Endocrinol. (Paris)     Publication Date:  2009 Apr 
Date Detail:
Created Date:  2009-04-27     Completed Date:  2009-06-12     Revised Date:  2012-09-10    
Medline Journal Info:
Nlm Unique ID:  0116744     Medline TA:  Ann Endocrinol (Paris)     Country:  France    
Other Details:
Languages:  eng     Pagination:  113-8     Citation Subset:  IM    
Endocrinology Unit, Department of Internal Medicine and Endocrinology, Fattouma Bourguiba University Hospital, 5000 Monastir, Tunisia.
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MeSH Terms
Adrenal Glands / pathology,  surgery
Adrenal Hyperplasia, Congenital / complications,  genetics*,  surgery*
Antihypertensive Agents / therapeutic use
Brain / pathology
DNA / biosynthesis,  genetics
Follow-Up Studies
Hormone Antagonists / therapeutic use
Hypertension / etiology,  surgery*
Hypothalamo-Hypophyseal System / pathology
Retinal Diseases / etiology
Reverse Transcriptase Polymerase Chain Reaction
Sexual Maturation
Steroid 11-beta-Hydroxylase / genetics*
Reg. No./Substance:
0/Antihypertensive Agents; 0/Hormone Antagonists; 9007-49-2/DNA; EC 11-beta-Hydroxylase

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