Endocrine Abstracts

Congenital adrenal hyperplasia (CAH) refers to an autosomal recessive condition due to 21 hydroxylase (21OHD) deficiency, is characterized by impaired biosynthesis of glucocorticoid and mineralocorticoid. The consequent increase in the ACTH leads to bilateral adrenocortical hyperplasia and hyperandrogenism. Nonclassic CAH (NCCAH) manifests with a less severe clinic that presents later in life with the sympthoms of excessive androgen production. Clinical features include premature pubarche,acne, accelaration in bone age. Although this group often does not require continuous glucocorticoid (GC) replacement, most of the patients with NCCAH receive GCs for different range of durations. Overtreatment with GCs might lead to increases in cardiovascular risk factors. In contrast, insufficient GC therapy can lead to androgen excess,infertility,development of adrenal crest tumors.

Our case, a thirty-seven year old male, presented with acute chest pain in the emergency room. Electrocardiographic evidence of ST-segment elevationin leads II, III and elevation in cardiac biomarkers correlated with acute inferior ST-elevated miyordial infarction. Coronary reperfusion was performedwith primary percutaneous coronary intervention to the infarct-related circumflex artery. İn his medical history,he was diagnosed CAH at the age of ten but he gave up the glucocorticoid therapy four years ago. He was married with two children, both daughters (eight years old and five yerars old). His weight was 92 kg, height was 150 cm. Waist circumferencewas 112 cm. His body mass index was 40.8 kg/m² and his waist to height ratio was 0.74 kg/cm which were shown to be associated with an increase in insuline resistance. Theresults of the biochemical evaluations of the patient are presented in Table-1 which were more consistent with NCCAH according to moderate elevation in 17-OH progesterone levels. We planned genetic consultation. Low dose GC treatment and metformin was started along with antithrombotic therapy, regular endocrinologic follow-up was arranged. The increased cardiovascular risk in CAH patients mainly attributed to GC overtreatment in studies. In our case cardiovascular morbidity may be consistent with adrenal hypofunction and androgen excess, which may both lead to insulin resistance. It is essential to follow-up patients with CAH in terms of cardiovascular morbidity whether on GC treatment or not.