Benjamin Bleicken

Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies

OBJECTIVE Adrenal crisis (AC) is a life-threatening complication of adrenal insufficiency (AI). Here, we evaluated frequency, causes and risk factors of AC in patients with chronic AI. METHODS In a cross-sectional study, 883 patients with AI were contacted by mail. Five-hundred and twenty-six patients agreed to participate and received a disease-specific questionnaire. RESULTS Four-hundred and forty-four datasets were available for analysis (primary AI (PAI), n=254; secondary AI (SAI), n=190). Forty-two percent (PAI 47% and SAI 35%) reported at least one crisis. Three hundred and eighty-four AC in 6092 patient years were documented (frequency of 6.3 crises/100 patient years). Precipitating causes were mainly gastrointestinal infection and fever (45%) but also other stressful events (e.g. major pain, surgery, psychic distress, heat and pregnancy). Sudden onset of apparently unexplained AC was also reported (PAI 6.6% and SAI 12.7%). Patients with PAI reported more frequent emergency glucocorticoid administration (42.5 vs 28.4%, P=0.003). Crisis incidence was not influenced by educational status, body mass index, glucocorticoid dose, DHEA treatment, age at diagnosis, hypogonadism, hypothyroidism or GH deficiency. In PAI, patients with concomitant non-endocrine disease were at higher risk of crisis (odds ratio (OR)=2.02, 95% confidence interval (CI) 1.05-3.89, P=0.036). In SAI, female sex (OR=2.18, 95% CI 1.06-4.5, P=0.035) and diabetes insipidus (OR=2.71, 95% CI 1.22-5.99, P=0.014) were associated with higher crisis incidence. CONCLUSION AC occurs in a substantial proportion of patients with chronic AI, mainly triggered by infectious disease. Only a limited number of risk factors suitable for targeting prevention of AC were identified. These findings indicate the need for new concepts of crisis prevention in patients with AI.

Influence of hydrocortisone dosage scheme on health‐related quality of life in patients with adrenal insufficiency

Context  Recent studies suggest that current glucocorticoid replacement therapies fail to completely restore well‐being in patients with adrenal insufficiency (AI).

Delayed Diagnosis of Adrenal Insufficiency Is Common: A Cross-Sectional Study in 216 Patients

Introduction:Little information is available on patients with adrenal insufficiency (AI) in regard to complaints before diagnosis, time until correct diagnosis, false diagnosis, and professional changes due to the diagnosis. Objective:We retrospectively evaluated circumstances before and at diagnosis of AI in patients with primary and secondary AI by using established Hospital Anxiety and Depression Scale, Short Form-36 and Giessen Complaint List (GBB-24) questionnaires, and a self-established general registration form. Methods:In this cross-sectional study, questionnaire sets were available from 216 patients (primary AI, n = 99; secondary AI, n = 117). Time duration before treatment, underlying diagnoses, and disease symptoms were verified by questionnaires and review of medical records. Results regarding subjective health status (SHS) were compared with sex- and age-matched controls drawn from questionnaire-specific reference cohorts. Results:Less than 30% of woman and 50% of men with AI were diagnosed within the first 6 months after onset of symptoms. Twenty percent of patients suffered >5 years before being diagnosed. More than 67% of patients consulted at least 3 physicians, and 68% were primarily false diagnosed. The most common false diagnoses were of psychiatric and gastrointestinal origin. Overall, patients with AI showed an impaired SHS compared with controls, and patients who were diagnosed correctly within 3 months showed a significantly better SHS. Conclusions:Because of the unspecific symptoms, diagnosis is often delayed, not recognized by physicians or diagnosed falsely. An early diagnosis is necessary and might positively influence SHS in patients with AI.

Impaired subjective health status in chronic adrenal insufficiency: impact of different glucocorticoid replacement regimens.

CONTEXT Recent studies have suggested that current glucocorticoid replacement therapies fail to fully restore well-being in patients with adrenal insufficiency (AI). OBJECTIVE To investigate the effect of different glucocorticoid preparations used for replacement therapy on subjective health status (SHS) in AI. DESIGN AND PATIENTS In a cross-sectional study, primary and secondary AI patients were contacted by mail. Individual glucocorticoid replacement regimens, underlying diagnoses and comorbidities were verified by questionnaires and review of medical records. Patients were asked to complete three validated self-assessment questionnaires (Short Form 36 (SF-36), Giessen Complaint List (GBB-24), and Hospital Anxiety and Depression Scale). Results were compared with sex- and age-matched controls drawn from the questionnaire-specific reference cohort. RESULTS Of the 883 patients identified, 526 agreed to participate in the study. Completed questionnaire sets were available from 427 patients (primary AI n=232; secondary AI n=195). AI patients showed significantly impaired SHS compared with controls irrespective of the glucocorticoid used for replacement. The only difference in SHS between patients on prednisolone (PR) and hydrocortisone (all patients and sub-analysis for primary AI) was significant higher bodily pain (lower Z-score in SF-36) in patients on PR (P<0.05, P<0.01 respectively). In patients with secondary AI, the PR group showed significantly (P<0.05) less heart complaints (lower Z-score) in the GBB questionnaire compared with the cortisone acetate group. CONCLUSIONS Glucocorticoid replacement therapy with PR seems to be equivalent to hydrocortisone regarding SHS in patients with AI. However, SHS remains impaired in all patient groups suggesting a need for further improved glucocorticoid replacement strategies.

Quality of life is less impaired in adults with congenital adrenal hyperplasia because of 21-hydroxylase deficiency than in patients with primary adrenal insufficiency

Objective  Current replacement regimens fail to restore well‐being in patients with primary adrenal insufficiency (PAI). Data on health‐related quality of life (HRQoL) in patients with congenital adrenal hyperplasia (CAH) are scarce, inconsistent and largely restricted to women. The objective of the study therefore was to study HRQoL in CAH because of 21‐hydroxylase deficiency in comparison with PAI and healthy controls.