Endocrine Abstracts (2017) 49 S18.2 | DOI: 10.1530/endoabs.49.S18.2

Metabolic perspectives for the non-classical CAH (NCAH)

Fahrettin Kelestimur


Turkey.


Congenital adrenal hyperplasia (CAH) is one of the most common autosomal recessive inherited endocrine diseases and is characterized by complete or partial impairment of adrenal steroidogenesis. 21-hydroxylase deficiency and 11β-hydroxylase deficiency account for 90% and 6–8% of cases of CAH, respectively. NCAH is the mild form of CAH and occurs in approximately 0.1–0.2% in the general population. The worldwide prevalence of NCAH among hyperandrogenic women is approximately 4.0%. Adrenal androgen secretion and its response to ACTH is increased in these cases. Patients with NCAH generally present with manifestations due to high androgen levels including hirsutism, acne and alopecia. Ovulatory and menstrual dysfunction are seen in more than 30–50% of patients with NCAH. Polycystic ovarian morphology is recorded in at least 25% of the patients. The data regarding insulin resistance, diabetes, obesity and abdominal visceral adiposity in NCAH patients is scarce. Chronic androgen excess around puberty may favor abdominal visceral adiposity, insulin resistance and its metabolic consequences. Hyperinsulinemia and insulin insensitivity associated with high androgen levels were reported in untreated newly diagnosed patients with NCAH. Chronic glucocorticoid administration in a limited number of patients may also favor metabolic abnormalities. The frequency of type 2 diabetes was reported to be increased in NCAH patients. It seems that NCAH is associated with increased cardiovascular and metabolic morbidity. Therefore, screening metabolic abnormalities may be required in patients with NCAH.

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