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Adrenal morphology and associated comorbidities in congenital adrenal hyperplasia
Author(s) -
ElMaouche Diala,
HannahShmouni Fady,
Mallappa Ashwini,
Hargreaves Courtney J.,
Avila Nilo A.,
Merke Deborah P.
Publication year - 2019
Publication title -
clinical endocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.055
H-Index - 147
eISSN - 1365-2265
pISSN - 0300-0664
DOI - 10.1111/cen.13996
Subject(s) - congenital adrenal hyperplasia , medicine , aldosterone , endocrinology , hyperplasia , androstenedione , cohort , hormone , androgen
Objective Adrenonodular hyperplasia and tumour formation are potential long‐term complications of congenital adrenal hyperplasia (CAH) with little known regarding the clinical implications. Our aim was to describe volumetric adrenal morphology and determine the association between radiological findings and comorbidities in adults with classic CAH. Design This was a cross‐sectional study of 88 patients (mean age 29.2 ± 13 years, 47 females) with classic CAH seen in a tertiary referral centre. Methods CT imaging, performed at study entry or when reaching adulthood, was used to create 3‐dimensional volumetric models. Clinical, genetic and hormonal evaluations were collected and correlated with adrenal morphology and tumour formation. Results Over one‐third of the cohort was obese. 53% had elevated 17‐OH‐progesterone or androstenedione; and 60% had adrenal hyperplasia. Tumours included 11 myelolipomas, 8 benign adrenocortical adenomas, 1 pheochromocytoma and 50% of men had testicular adrenal rest tissue. CAH patients with adrenal hyperplasia had significantly higher number of comorbidities than those with morphologically normal adrenals ( P = 0.03). Variables that positively correlated with adrenal volume included hypogonadal/oligomenorrhoeic status, hypertension, androstenedione, aldosterone, and triglyceride levels, and in women, low HDL and insulin resistance. Elevated aldosterone was observed in a subset of patients with simple virilizing CAH. Conclusions Adrenocortical hyperplasia is associated with a number of comorbidities, especially hypogonadism. Aldosterone production associated with adrenal enlargement may play a role in the development of metabolic risk factors. Further studies are needed to assess the long‐term impact of the excess adrenal steroid milieu associated with adrenal enlargement to develop improved management strategies for CAH.