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Abstract Disclosure: J.G. Kallet: None. W. Hover: None. A. Krein: None. M.F. Slovick: None. S. Majka: None. G.L. Kinney: None. S. Watts: None. M.T. McDermott: None. E.A. Regan: None. Background: A major challenge in the treatment of adrenal insufficiency is determining adequate cortisol replacement dosing. There is currently no standard process for determining dosing and timing of cortisol replacement or determination of adequate replacement based on patients’ reported within-day symptoms, overall quality of life, or risk of adrenal crisis. Methods: A subset of patients recruited from the MyAI Patient Registry were evaluated for symptoms over 6 hours in the clinic. Symptoms and severity were collected prior to taking usual morning dose of hydrocortisone followed by collection each hour for five hours after the morning dose. Symptoms were individually scored mild (1 point), moderate (2 points), or severe (3 points) and summed to create a severity score at each time point. Patients were eligible for the study if they were at least 18 years of age, had documented primary adrenal insufficiency (PAI), central adrenal insufficiency (CAI), or CAH based on medical records and record review, and were on a stable hydrocortisone replacement regimen for at least three months. Exclusionary criteria included drug-induced AI and patients on prednisone or hormone replacement other than hydrocortisone for AI. Patient reports of adrenal crises events and associated records (ER visit, hospitalization) within the past year and AddiQoL assessment of quality of life were captured as part of longitudinal data collection in the MyAI Registry. Results: Of 16 patients, 12 were diagnosed with PAI and 4 were diagnosed with CAI or CAH. Severity scores ranged from 0-17 for individual time points across participants with mean score 3.3 and SD 4.2. Asymptomatic patients (severity score=0 for 3 or more time points; n=7), mildly symptomatic patients (severity score 0, ≤ 7 at 3 or more time points; n=5), and moderately/severely symptomatic patients (2 or more time points 7; n=4) scored a mean 94.3 (12.4), 81.0 (10.2), and 65.3 (18.2) on AddiQoL, respectively. Of the four patients scoring highest symptom severity, three were diagnosed CAI or CAH. All four patients with the highest symptom severity scores reported an adrenal crisis event in the past year. Of the 15 participants with longitudinal data available, 6 reported an adrenal crisis event in the past year with mean AddiQoL 61.6 and SD 18.5, and 9 did not report an adrenal crises event with a mean AddiQoL 89.0 and SD 13.4. Conclusion: There is a large spectrum of disease, symptom severity, and quality of life among patients with adrenal insufficiency. We found that CAI or CAH patients experienced more severe within-day symptoms in this group. We also found patients with greater symptom severity scored lower on quality of life. Increased symptoms and lower quality of life were also associated with a history of adrenal crises in the past year. Presentation: 6/3/2024
Kallet et al. (Tue,) studied this question.
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