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

Johannsen TH, Ripa CP, Carlsen E, Starup J, Nielsen OH, Schwartz M, Drzewiecki KT, Mortensen EL, Main KM - Int J Pediatr Endocrinol (2010)

Bottom Line: Methods.Conclusion.Despite overall acceptable cosmetic results, reproductive outcomes were suboptimal, supporting that multidisciplinary teams should be involved in adult follow up of CAH patients.

View Article: PubMed Central - PubMed

Affiliation: University Department of Growth and Reproduction, GR-5064, Rigshospitalet, Blegdamsvej 9, DK-2100 Copenhagen, Denmark.

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 [112-153], 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.

No MeSH data available.


Related in: MedlinePlus

Gynecological outcomes in patients with congenital adrenal hyperplasia (CAH) and in controls: clitoral length (mm), vaginal length (mm), uterine volume (ml) separated by primipara/multipara (+) or ipara (–), and ovarian volume (ml) separated by current (+) or no (–) use of hormonal contraception. Lines indicate median values.
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fig1: Gynecological outcomes in patients with congenital adrenal hyperplasia (CAH) and in controls: clitoral length (mm), vaginal length (mm), uterine volume (ml) separated by primipara/multipara (+) or ipara (–), and ovarian volume (ml) separated by current (+) or no (–) use of hormonal contraception. Lines indicate median values.

Mentions: Clitoral operations had been performed in 27 CAH females, including reduction with preservation of the glans (SW: n = 16, SV: n = 5, LO: n = 1) and amputation (SW: n = 4, SV: n = 1). Five SW-patients and 1 SV-patient had clitoral operations performed twice. Age at first clitoral operation was 4.4 years (range 0.8–25 yrs). Clitoral length was larger in CAH patients (10 mm [2–30 mm]) than in controls (3.5 mm [2–8 mm]), P < .001; see Figure 1. In patients, no associations were observed between clitoral length or clitoral surgery procedures and androgen levels, respectively, (data not shown). Serum-antimüllerian hormone (AMH) correlated inversely with clitoral length (rs = −0.59, P = .01). No significant correlation to surgery or number of surgical procedures at clitoris was found (P = .22 and  .13, resp.), but age at clitoral surgery was inversely related to serum-AMH levels (rs = −0.57, P = .01).


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

Johannsen TH, Ripa CP, Carlsen E, Starup J, Nielsen OH, Schwartz M, Drzewiecki KT, Mortensen EL, Main KM - Int J Pediatr Endocrinol (2010)

Gynecological outcomes in patients with congenital adrenal hyperplasia (CAH) and in controls: clitoral length (mm), vaginal length (mm), uterine volume (ml) separated by primipara/multipara (+) or ipara (–), and ovarian volume (ml) separated by current (+) or no (–) use of hormonal contraception. Lines indicate median values.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2963122&req=5

fig1: Gynecological outcomes in patients with congenital adrenal hyperplasia (CAH) and in controls: clitoral length (mm), vaginal length (mm), uterine volume (ml) separated by primipara/multipara (+) or ipara (–), and ovarian volume (ml) separated by current (+) or no (–) use of hormonal contraception. Lines indicate median values.
Mentions: Clitoral operations had been performed in 27 CAH females, including reduction with preservation of the glans (SW: n = 16, SV: n = 5, LO: n = 1) and amputation (SW: n = 4, SV: n = 1). Five SW-patients and 1 SV-patient had clitoral operations performed twice. Age at first clitoral operation was 4.4 years (range 0.8–25 yrs). Clitoral length was larger in CAH patients (10 mm [2–30 mm]) than in controls (3.5 mm [2–8 mm]), P < .001; see Figure 1. In patients, no associations were observed between clitoral length or clitoral surgery procedures and androgen levels, respectively, (data not shown). Serum-antimüllerian hormone (AMH) correlated inversely with clitoral length (rs = −0.59, P = .01). No significant correlation to surgery or number of surgical procedures at clitoris was found (P = .22 and  .13, resp.), but age at clitoral surgery was inversely related to serum-AMH levels (rs = −0.57, P = .01).

Bottom Line: Methods.Conclusion.Despite overall acceptable cosmetic results, reproductive outcomes were suboptimal, supporting that multidisciplinary teams should be involved in adult follow up of CAH patients.

View Article: PubMed Central - PubMed

Affiliation: University Department of Growth and Reproduction, GR-5064, Rigshospitalet, Blegdamsvej 9, DK-2100 Copenhagen, Denmark.

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 [112-153], 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.

No MeSH data available.


Related in: MedlinePlus