Congenital adrenal hyperplasia 11beta-hydroxylase deficiency: two cases managed with bilateral adrenalectomy.
This series describes two patients with congenital adrenal hyperplasia due to 11beta-hydroxylase deficiency. The first patient, a ten-year-old with XX genotype, reared as a male, presented with resistant hypokalaemia and hypertension. The second patient, a 23-year-old with XY genotype, presented with bilateral adrenal masses and resistant hypertension. Both the patients were offered bilateral adrenalectomy. These two patients are described with a discussion on the role of bilateral adrenalectomy in the management of difficult cases of congenital adrenal hyperplasia. The association of myelolipoma and testicular rests with this condition is also discussed.
John
M
M
Department of Endocrinology, Seth Gordhandas Sunderdas Medical College & King Edward Memorial Hospital, Parel, Mumbai 400012, India. drmathewjohn@yahoo.com
Menon
S K
SK
Shah
N S
NS
Menon
P S
PS
D000293:Adolescent; D000311:Adrenal Glands / Q000601:surgery; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood* / Q000235:genetics / Q000601:surgery*; D000315:Adrenalectomy / Q000379:methods*; D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D005838:Genotype; D006801:Humans; D006973:Hypertension / Q000150:complications; D007621:Karyotyping; D008297:Male; D013252:Steroid 11-beta-Hydroxylase / Q000097:blood*; D013733:Testicular Diseases / Q000150:complications / Q000601:surgery; D016896:Treatment Outcome
D013252:Steroid 11-beta-Hydroxylase