Endocrine Abstracts (2009) 19 OC8

Quality of life (QoL) in male and female patients with congenital adrenal hyperplasia (CAH) compared to healthy subjects and patients with primary adrenal insufficiency

B Bleicken1, S Hahner2, M Loeffler2, M Ventz1, B Allolio2 & M Quinkler1

1Clinical Endocrinology, Charite Campus Mitte, Berlin, Germany; 2Endocrinology and Diabetes, University of Wuerzburg, Wuerzburg, Germany.

Objective: There is evidence that current replacement regimens fail to restore well-being in patients with primary adrenal insufficiency (PAI). Few data is available on QoL in adult male and female patients with CAH.

Methods: Patients with CAH or PAI were contacted and received a disease specific questionnaire and three standardized questionnaires (SF-36, GBB-24 and HADS). Reference data for SF-36 scores were obtained from the German National Health Survey comprising a representative random sample of 7124 subjects from the German population aged 18–79 years. Reference data for the GBB-24 (n=2076) and HADS (n=2081) were obtained from surveys performed by Brahler and colleagues. Finally, 15 male CAH patients and 58 male patients with PAI, and 22 female CAH patients and 174 female PAI patients were compared to healthy controls.

Results: Male, but not female, CAH patients showed significantly higher anxiety scores (HADS) than healthy subjects or patients with PAI. Scores for exhaustion tendency, and global score of discomfort assessed by GBB-24 were significantly higher in both male and female CAH patients than in healthy controls. Also PAI patients showed higher scores in the GBB-24 questionnaire than healthy controls. The score of physical functioning (pf) as assessed by SF-36 was generally comparable between CAH and PAI patients, and showed lower scores (=worse pf) than healthy controls. However, female CAH patients experienced less physical pain than healthy controls, and had higher scores in mental health than control subjects or PAI patients.

Conclusion: Health related QoL is impaired not only in patients with PAI, but also in patients with CAH. Sex-specific differences in QoL were observed in CAH patients and QoL was different from patients with PAI in several dimensions of the questionnaires suggesting specific effects of CAH unrelated to hormone replacement therapy.

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