Adrenal rest tissue in gonads of patients with classical congenital adrenal hyperplasia: multicenter study of 45 French male patients.

Peggy Pierre; François Despert; François Tranquart; Régis Coutant; Véronique Tardy; Véronique Kerlan; Emmanuel Sonnet; Sabine Baron; Yannick Lorcy; Philippe Emy; Dominique Delavierre; Françoise Monceaux; Yves Morel; Pierre Lecomte

doi:10.1016/j.ando.2012.09.005

Adrenal rest tissue in gonads of patients with classical congenital adrenal hyperplasia: multicenter study of 45 French male patients.

Peggy Pierre ¹ François Despert ² François Tranquart ³ Régis Coutant ⁴ Véronique Tardy ⁵ Véronique Kerlan ^{6, 7} Emmanuel Sonnet ⁷ Sabine Baron Yannick Lorcy Philippe Emy Dominique Delavierre Françoise Monceaux ⁸ Yves Morel ⁵ Pierre Lecomte ¹

1 TOURS - Endocrino - Service d'Endocrinologie

2 TOURS - Endocrino Pédia - Unité d'Endocrinologie Pédiatrique

3 TOURS - Radio - Service de Radiologie

4 Endocrinologie pédiatrique[CHU Angers]

5 CBPE - Département de Biochimie et Biologie moléculaire

6 GETBO - Groupe d'Etude de la Thrombose de Bretagne Occidentale

7 CHRU - Endocrino - Service d'Endocrinologie

8 ORLEANS - Endocrino Pédiatrique - Endocrinologie Pédiatrique

Abstract : OBJECTIVES: Several cases of testicular adrenal rest tumours have been reported in men with congenital adrenal hyperplasia (CAH) due to the classical form of 21-hydroxylase deficiency but the prevalence has not been established. The aims of this report were to evaluate the frequency of testicular adrenal rest tissue in this population in a retrospective multicentre study involving eight endocrinology centres, and to determine whether treatment or genetic background had an impact on the occurrence of adrenal rest tissue. MATERIAL AND METHODS: Testicular adrenal rest tissue (TART) was sought clinically and with ultrasound examination in forty-five males with CAH due to the classical form of 21-hydroxylase deficiency. When the diagnosis of testicular adrenal rest tumours was sought, good observance of treatment was judged on biological concentrations of 17-hydroxyprogesterone (17OHP), delta4-androstenedione, active renin and testosterone. The results of affected and non-affected subjects were compared. RESULTS: TART was detected in none of the 18 subjects aged 1 to 15years but was detected in 14 of the 27 subjects aged more than 15years. Five patients with an abnormal echography result had no clinical signs. Therapeutic control evaluated at diagnosis of TART seemed less effective when diagnosis was made in patients with adrenal rest tissue compared to TART-free subjects. Various genotypes were observed in patients with or without TART. CONCLUSION: Due to the high prevalence of TART in classical CAH and the delayed clinical diagnosis, testicular ultrasonography must be performed before puberty and thereafter regularly during adulthood even if the clinical examination is normal.

Type de document :

Article dans une revue

Annales d'Endocrinologie, Elsevier Masson, 2012, 73 (6), pp.515-22. 〈10.1016/j.ando.2012.09.005〉

Domaine :

Sciences du Vivant [q-bio]

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http://hal.univ-brest.fr/hal-00932227
Contributeur : Ghislaine Calvez <>
Soumis le : jeudi 16 janvier 2014 - 15:16:00
Dernière modification le : mardi 6 février 2018 - 12:02:03

Adrenal rest tissue in gonads of patients with classical congenital adrenal hyperplasia: multicenter study of 45 French male patients.

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Adrenal rest tissue in gonads of patients with classical congenital adrenal hyperplasia: multicenter study of 45 French male patients.

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