Endocrine Abstracts (2019) 65 P26 | DOI: 10.1530/endoabs.65.P26

A cross-sectional audit reviewing the management of adults with congenital adrenal hyperplasia in Leicester

Lauren Quinn, Andrew Baldwin, Miles Levy, Narendra Reddy & Ragini Bhake


University Hospitals of Leicester, Leicester, UK


Introduction: Following the recent publication of The Endocrine Society Clinical Practice guideline for Congenital Adrenal Hyperplasia (CAH), we performed an evaluation of the service provided for adults with CAH to assess how well we are meeting the guideline standard and to make recommendations for service improvement.

Methods: We performed an audit of adults with CAH under the care of the University Hospitals of Leicester. We undertook a cross-sectional analysis from 1 January 2018 to 31 December 2018. We collected demographic data, treatment regimen, previous surgery for CAH and laboratory biochemical and steroid profiles. Relevant imaging were obtained from PACS. Excel 2013 was used for statistical analysis and graphs.

Results: 80 adult patients with CAH were included for analysis, of which 61% (n=49) were female and 61% (n=49) were Caucasian. 60% (n=48), 33% (n=26) and 9% (n=7) of our cohort were taking Hydrocortisone, Prednisolone or Dexamethasone respectively, and 60% (n=48) were taking Fludrocortisone in combination with a glucocorticoid. Two patients had an adrenalectomy. Of the female patients, 21 had undergone vaginoplasty and 15 had undergone clitoral corrective surgery (all prior to coming under our care). 34% (n=n=27) and 33% (n=26) of our cohort were overweight or obese respectively. Despite this high prevalence, annual HBA1C was only performed in 35% (n=44) of cases and annual cholesterol was only tested in 26% (n=33) of cases; raised cholesterol remained untreated. Benign adrenal rest tumours were identified in 3 patients. Osteoporosis/osteopenia was confirmed on DEXA scanning in only 2 of our patients.

Discussion: The management of CAH in adult patients varies, in terms of frequency of assessment of clinical and biochemical parameters. Our recommendations: 1) Cholesterol and HbA1c should be tested and treated in at risk individuals. 2) Osteoporosis or osteopenia should be confirmed on DEXA scanning. 3) Weight must be reviewed annually.

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