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Baby girl from Nigeria gets treated for rare disorder.

CHISOM Oranye, a resident of Nigeria, was overwhelmed with joy when she was blessed with a baby girl last year. The joy, however, turned to shock as the girl's clitoris began to grow abnormally and in 15 months she had a penile- like growth comparable to a male.

Her appetite had also decreased alarmingly and her weight had dropped to 6 kg -- the weight of a 5 month- old baby. Little Oranye Chimweokwu Sayne was brought to Max Super Speciality Hospital, Shalimar Bagh recently where she was diagnosed with a rare genetic disorder called Congenital Adrenal Hyperplasia whereby the body does not produce the vital 21- hydroxylase enzyme leading to enlargement of the clitoris. She was operated upon on August 26, given hormonal medication and normal female genital features.

She has now returned to her native country a healthy and cheerful girl.

Dr Vaishakhi Rustagi, Consultant, Paediatric Endocrinology, Max Super Speciality Hospital, says, "Deficiency of 21- hydroxylase enzyme affects one in 4,000 to 6,000 people in India.

It is mostly seen in offsprings of consanguineous marriages, that is, alliances between blood relations." In this condition, the body does not synthesize the crucial 21- hydroxylase enzyme. Consequently, the adrenal glands located above the kidneys start producing lesser cortisol (which regulates glucose level) and aldosterone (which regulates blood pressure), and testosterone production increases manifold, Rustagi said.

The parents are carriers in this disease. Only when a child inherits affected genes from both the parents, does the disorder manifest itself.

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Publication:Mail Today (New Delhi, India)
Date:Sep 16, 2014
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