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S13-11
(P)
LONG TERM FOLLOW UP OF FEMALE PATIENTS WITH CONGENITAL ADRENAL
HYPERPLASIA
Sang Hoon SONG
1
, Jaeyoon JUNG
1
, Ja-Hye KIM
2
, Han-Wook YOO
2
, Jae Ho KIM
3
and Kun Suk KIM
1
1) Asan Medical Center, University of Ulsan College of Medicine, Department of Urology, Seoul, KOREA (REPUBLIC OF) -
2) Asan Medical Center, University of Ulsan College of Medicine, Department of Pediatrics, Seoul, KOREA (REPUBLIC OF)
- 3) Soonchunhyang University Gumi Hospital, Department of urology, Soonchunhyang University College of Medicine,
Gumi, KOREA (REPUBLIC OF)
PURPOSE
To evaluate clinical outcomes, including physical growth and metabolic syndrome complications, during long-term
follow-up of female patients with congenital adrenal hyperplasia (CAH) who had reached adolescence.
MATERIAL AND METHODS
This was a retrospective study of 27 patients who were diagnosed with CAH due to 21-hydroxylase deficiency, who had
reached adolescence with a 46XX genotype. We investigated the type of external genital surgery, puberty onset, final
height, medication status, and complications, and compared patients with saltwasting (SW) CAH and those with simple
virilizing (SV) CAH.
RESULTS
The mean age of patients was 17.5 ± 5.1 years and the mean follow-up period after surgery was 141 (47-277) months.
In total, 16 out of 27 patients underwent external genital surgery; 16 underwent clitoris reduction, 11 underwent
clitoris relocation, 10 underwent vaginoplasty, 2 underwent labia minora molding, and 1 underwent labia majora
molding. The mean body weight was 51.6 ± 5.8 kg, the mean final height was 154.8 ± 3.2 cm, and the mean body
mass index (BMI) was 25.4 ± 10.2 kg/m2. The mean age of first menarche was not different between the SV group
(13.5 ± 2.5 years) and in the SW group (12.3 ± 1.9 years). The mean final height of patients was 153.5 ± 2.3 cm (-1.5
± 0.6SD) in the SW group and 151.1 ± 5.9 cm (-2.0 ± 1.3SD) in the SV group, and these were shorter than the
corresponding mid parental heights (5.1 ± 1.9cm and 7.4 ± 8.7cm, respectively). Although 76.2% of patients at
adolescence had normal BMI, 13 patients (48.1%) were obese at adult age. Two out of 6 patients of child-bearing age
tried to become pregnant naturally; however, both failed.
CONCLUSIONS
Adults with CAH tend to be short and are often obese, which might predispose to the metabolic syndrome and adverse
cardiovascular risk.