期刊
EXPERIMENTAL AND CLINICAL ENDOCRINOLOGY & DIABETES
卷 125, 期 3, 页码 196-201出版社
JOHANN AMBROSIUS BARTH VERLAG MEDIZINVERLAGE HEIDELBERG GMBH
DOI: 10.1055/s-0042-123037
关键词
adrenal gland neoplasm; congenital adrenal hyperplasia; testicular adrenal rest tumor; 21-hydroxlyase deficiency
资金
- National Research Foundation of Korea, Ministry of Education, Science, and Technology [2016M3A9B4915706]
- National Research Foundation of Korea [2016M3A9B4915706] Funding Source: Korea Institute of Science & Technology Information (KISTI), National Science & Technology Information Service (NTIS)
Background The management of congenital adrenal hyperplasia (CAH) from pediatric to adulthood is challenging to achieve optimal growth and puberty. This study characterizes the clinical outcomes of 21-hydroxylase deficiency. Methods 53 CAH patients were included (33 females, 15 and 18 patients with the salt-wasting [SW] and simple-virilizing [SV] forms; and 20 males, 16 and 4 patients with the SW and SV forms). We reviewed growth parameters, pubertal status, and long-term morbidities. Results In females, the age at pubertal onset and pubarche was 9.6 +/- 0.9 and 10.5 +/- 1.9 years, respectively, which was significantly earlier in the SV form (p = 0.005). In males, the ages at pubertal onset and pubarche were 10.1 +/- 2.0 and 10.7 +/- 2.5 years, respectively, which were not significantly different between the groups. Forty patients reached adult height: -2.1 +/- 1.6 SDS in males and -1.5 +/- 1.1 SDS in females. Obesity and overweight was significantly common in adult patients. Testicular adrenal rest tumors were found in 4 SW males. 5 patients had adrenal tumor including adenoma, adenocarcinoma, or myelolipoma. Conclusions Reduced adult height and obesity/overweight are prevalent in adulthood. Adolescents and adults with 21-hydroxylase deficiency should be monitored for long-term consequences.
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