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

T. H. Johannsen, C. P. L. Ripa, Elisabeth Carlsen, J Starup, M. Schwartz, Drzewiecki

International Journal of Pediatric Endocrinology · 2010 · 17 citations · 20 references

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Abstract

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], P < .001),shorter vaginal length (121 mm [100-155] versus 128 , P = .12),lower uterine volumes (29.1 ml [7.5-56.7]versus 47.4 [15.9-177.5],P = .009),and higher ovarian volumes (4.4 ml [1.3-10.8]versus 2.8 [0.6-10.8],P = .09)than controls.Satisfaction with genital appearance was lower and negatively correlated to degree of initial virilisation (r s =≤ -0.39,P ≤ .05).More patients had never had intercourse (P = .001),and age at 1st intercourse was higher (18 yrs versus 16 yrs, P = .02).Conclusion.Despite overall acceptable cosmetic results, reproductive outcomes were suboptimal, supporting that multidisciplinary teams should be involved in adult follow up of CAH patients.

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