There are no consensus guidelines on the optimum long-term care of patients with primary adrenal failure. Published data suggest increased morbidity and mortality in patients treated with current therapy. Investigations of bone mineral density (BMD) in adults with adrenal failure have reported conflicting results. The objectives of this study were to determine the prevalence of auto-immune and other co-morbidities, describe the treatment regimens and to assess the BMD of adults with auto-immune Addison's disease (AAD). A retrospective, cohort study of adults with primary adrenal failure was used. Electronic and paper records were used to collect demographic, biochemical, BMD data and details of other co-morbidities. 48 patients (35% male; 65% female; 50 ± 16, years, mean ± SD) with primary adrenal failure were identified. There was high prevalence of other auto-immune co-morbidities (hypothyroidism 58%, vitamin B(12) deficiency 29%, type 1 diabetes 10%). The presence of cardiovascular risk factors including dyslipidaemia (65% had total cholesterol >5 mmol/l) and excess weight (65% had a BMI >25 kg/m(2)) were high. Using WHO criteria, 17.9 and 53.5% of patients had spinal osteoporosis and osteopenia, respectively, at the spine. This did not relate to the duration or dose of glucocorticoid replacement. Our data shows a high prevalence of both auto-immune and non-autoimmune co-morbidities in patients with AAD. In addition to common auto-immune diseases, patients should be screened for other cardiovascular risk factors. Further studies are needed to assess the cause of the observed increased prevalence of reduced BMD at the lumbar spine. There is a need for internationally agreed long-term management guidelines.
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http://dx.doi.org/10.1007/s12020-010-9359-8 | DOI Listing |
Am J Med Genet A
January 2025
M Health Fairview Masonic Children's Hospital, Minneapolis, Minnesota, USA.
Our objectives are to report on the outcomes of adrenal insufficiency (AI) and cerebral ALD (cALD) in children diagnosed with X-linked adrenoleukodystrophy (ALD) identified by newborn screening (NBS) in Minnesota in the first 5 years following initiation of NBS in 02/2017. A retrospective chart review was conducted for children diagnosed with ALD via Minnesota NBS from 02/06/2017 through 02/06/2022. Data reviewed included newborn screening data, diagnostic very long chain fatty acid levels, ABCD1 molecular testing results, serial measurements of ACTH and cortisol, and serial brain MRI results.
View Article and Find Full Text PDFCureus
December 2024
Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, PRT.
Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal hyperinflammatory syndrome characterized by dysregulated immune activation and systemic inflammation. Secondary HLH is often triggered by infections, with being an infrequently reported cause. Peripheral axonal neuropathy is a rare and poorly understood complication of HLH.
View Article and Find Full Text PDFJ Clin Res Pediatr Endocrinol
January 2025
Ankara University Faculty of Medicine, Department of Pediatric Endocrinology, Ankara, Turkey.
Nutr Metab Cardiovasc Dis
December 2024
Diabetes and Endocrinology, Department of Internal Medicine, Hennepin Healthcare, Minneapolis, MN, USA.
Background And Aims: Patients with adrenal insufficiency (AI) face elevated risks during various hospitalizations including cardiovascular related admissions. Despite this, limited data exist specifically regarding congestive heart failure (CHF) in the context of AI. This investigation leveraged a comprehensive national database to examine the association between AI and cardiovascular outcomes among patients admitted with CHF.
View Article and Find Full Text PDFPediatr Nephrol
January 2025
Department of Pediatrics, Chacha Nehru Bal Chikitsalaya, Delhi, 110031, India.
Background: Hypothalamic-pituitary-adrenal (HPA) axis recovery after cessation of steroid therapy in children with nephrotic syndrome (NS) has hardly been studied in the literature.
Methods: This 22-month cross-sectional study recruited children (2-14 years) with NS, having received a minimum 3 months of prednisolone, now in remission, and off steroids for 1, 3, or 6 months. Serum cortisol-basal and stimulated (with long-acting intramuscular adrenocorticotropic hormone), and factors affecting them, were assessed.
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