ECE2007 Poster Presentations (1) (659 abstracts)
Adrenal rest tissue in gonads in 70 French patients with classical congenital adrenal hyperplasia (21 hydroxylase deficiency)
Pierre Peggy 1 , Despert Francois 1 , Tranquart Francois 1 , Chabrolle Christine 1 , Kerlan Veronique 2 , Sonnet Emmanuel 2 , Metz Chantal 2 , Baron Sabine 3 , De Kerdanet Marc 6 , Coutant Regis 4 , Emy Philippe 5 , Monceau Francoise 5 , Bekka Said 1 & Lecomte Pierre 1
1University, Tours, France; 2University, Brest, France; 3University, Nantes, France; 4University, Angers, France; 5Hospital, Orleans, France; 6University, Rennes, France; 7Hospital, Chartres, France.
Congenital adrenal hyperplasia (CAH) due to 21 hydroxylase deficiency is one of the most frequent endocrine genetic diseases. Adrenal rests have been described and can decrease fertility in men1. In a retrospective multi-center study we wanted 1.to evaluate the frequency of adrenal rests in classical forms of CAH (21-OH deficiency) by systematic ultrasonography (US); 2.to try to find the cause of this abnormality looking for a relationship between genotype and phenotype or with their therapeutic equilibrium. All patients with classical form of CAH and who have had an US were studied. In 24 women, none had adrenal rest in their ovaries, in accord with a very few cases published in the literature2. On the contrary, adrenal rests were detected in 30,4% of 46 men aged 1 to 38 years. We observed an increased frequency in testicular adrenal rest with age: none in the group less than 10 years, 15% in the group 10–17 years and 66,7% above 18 years. The role of an insufficiency in treatment in the development of adrenal rests has been evaluated. The therapeutic equilibrium is judged upon bone age and growth chart evolution in infants and upon 17 OHP or urinary pregnanetriol in adults. The appropriate equilibrium seems more often observed in patients without testicular adrenal rests: among patients, 55% without testicular adrenal rests were good treated during infancy compared to 14,3% with testicular adrenal rests, 44,4% compared to none during adolescence and 25% compared to 9% during adulthood. No relationship could be figure out between genotype and phenotype but the number of cases was probably too small in this cohort. Among 6 patients with adrenal rests and wishing fatherhood, an azoospermia was observed in 3; 2 had a very low sperm count and only 1 patient was able to procreate without any difficulty (2 children). In conclusion, ultrasonography of the ovaries is not usually necessary. On the opposite, testicular ultrasonography must be done during infancy, puberty and every five years during adulthood. This should reinforce better control of the disease by a more intensive treatment to try to reduce the number and volume of adrenal rests and improve fertility.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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