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Congenital adrenal hyperplasia 11beta-hydroxylase deficiency: two cases managed with bilateral adrenalectomy
1Department of Endocrinology, Seth Gordhandas Sunderdas Medical College & King Edward Memorial Hospital, Parel, Mumbai 400012, India. drmathewjohn@yahoo.com
Abstract:
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.
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