Long-Term Gynecological Outcomes in Women with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency | Zendy

T. H. Johannsen | Zendy; C. P. L. Ripa | Zendy; Elisabeth Carlsen | Zendy; J Starup | Zendy; Ole Haagen Nielsen | Zendy; M. Schwartz | Zendy; Drzewiecki | Zendy; Erik Lykke Mortensen | Zendy; Katharina M. Main | Zendy

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Long-Term Gynecological Outcomes in Women with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Author(s) -

T. H. Johannsen,

C. P. L. Ripa,

Elisabeth Carlsen,

J Starup,

Ole Haagen Nielsen,

M. Schwartz,

Drzewiecki,

Erik Lykke Mortensen,

Katharina M. Main

Publication year - 2010

Publication title -

international journal of pediatric endocrinology

Language(s) - English

Resource type - Journals

eISSN - 1687-9856

pISSN - 1687-9848

DOI - 10.1186/1687-9856-2010-784297

Subject(s) - medicine , congenital adrenal hyperplasia , 21 hydroxylase , term (time) , pediatrics , gynecology , endocrinology , physics , quantum mechanics

Background. Our knowledge on long-term outcome in CAH remains incomplete. Methods. In a prospective study (33 CAH patients, 33 age-matched controls), reproductive outcomes, self-rating of genital appearance and function, and sexuality were correlated to degree of initial virilisation, genotype, and surgery. Results. Patients had larger median clitoral lengths (10.0 mm [range 2–30] versus 3.5 [2–8], ), shorter vaginal length (121 mm [100–155] versus 128 [112–153], ), lower uterine volumes (29.1 ml [7.5–56.7] versus 47.4 [15.9–177.5], ), and higher ovarian volumes (4.4 ml [1.3–10.8] versus 2.8 [0.6–10.8], ) than controls. Satisfaction with genital appearance was lower and negatively correlated to degree of initial virilisation (). More patients had never had intercourse (), and age at 1st intercourse was higher (18 yrs versus 16 yrs, ). Conclusion. Despite overall acceptable cosmetic results, reproductive outcomes were suboptimal, supporting that multidisciplinary teams should be involved in adult follow up of CAH patients.