Patients with Addison's disease are at greater risk of having reduced bone mineral density and hip fractures and are thus more likely to receive a bisphosphonate than their peers. Potent intravenous bisphosphonates could provoke an acute phase reaction. An 80-year-old female with Addison's disease received her first infusion of zoledronic acid for osteoporosis at our outpatient clinic around noon. Despite doubling her usual afternoon hydrocortisone dose, she became feverish, nauseous, extremely weak, and hypotensive over the night. When transported to the nearest general hospital the next morning, the patient was found to have signs of hypovolemic shock and she was admitted to the ICU. Crystalloid infusion, followed by dobutamine and norepinephrine drip, had no effect. Only after her European emergency card for glucocorticoid cover was found, adrenal crisis was recognized, and she was immediately given an intravenous bolus of hydrocortisone followed by continuous hydrocortisone infusion. The patient rapidly improved and was transferred to a regular ward the next day, where hydrocortisone dose was gradually tapered. Our experience might suggest that patients with Addison's disease should probably start their treatment with zoledronic acid in a hospital setting. Their usual oral dose of hydrocortisone should be doubled or even tripled. Careful monitoring of these patients seems to be warranted, and intravenous hydrocortisone should be given if any symptoms or signs of the imminent adrenal crisis are noted.
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http://dx.doi.org/10.1007/s00198-018-4508-7 | DOI Listing |
BMC Med Genomics
January 2025
Center for Medical Genomics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, 10400, Thailand.
Background: Congenital Adrenal Hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD CAH) is an autosomal recessive disorder resulting from pathogenic variants in the CYP21A2 gene. The disorder exhibits variable clinical severity, with the classical form manifesting as salt-wasting crisis in neonates, while inducing ambiguous genitalia in females and precocious puberty in males through simple virilization. Identifying at-risk couples during the preconception stage holds significance for optimizing reproductive choices.
View Article and Find Full Text PDFVet Sci
January 2025
Department of Clinics, Faculty of Veterinary Medicine, "Ion Ionescu de la Brad" Iasi University of Life Sciences, 700490 Iasi, Romania.
A 3-year-old spayed male mixed-breed Labrador presented to the Emergency and Critical Care Unit with lethargy, loss of appetite, vomiting, a recent history of presyncopal episodes, and severe exercise intolerance. On admission, the patient had bradycardia, low blood pressure, and mild abdominal pain. Serum biochemistry information revealed severe hyperkalemia, hyponatremia, hypoglycemia, and mildly increased liver and kidney parameters.
View Article and Find Full Text PDFJ Surg Case Rep
January 2025
Department of Endocrine Surgery, Royal Victoria Infirmary, Newcastle Upon Tyne NE1 4LP, United Kingdom.
Paragangliomas, a type of extra-adrenal tumour, albeit rare, are dangerous due to their high metastatic potential and risk of hypertensive crisis from massive catecholamine release. It typically presents with sympathetic overdrive symptoms such as diaphoresis, headache, and palpitation, accompanied by substantially high plasma metanephrines level and mass on contrasted computed tomography abdomen and pelvis, whilst some are found incidentally. In this report, we discuss a case of an extra-adrenal lesion located near susceptible major structures with extensive vascularisation, in a patient with near-death experience.
View Article and Find Full Text PDFJ Clin Endocrinol Metab
January 2025
Department of Pediatrics, University of Washington School of Medicine, Seattle Children's Hospital, Seattle, WA 98105, USA.
Classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is a rare genetic condition that requires lifelong management from birth. Individuals with CAH and their families often face structural barriers to obtaining comprehensive care and treatment, including limited access to appropriate newborn screening, comprehensive care centers, and medications. Social and cultural barriers to care may include stigmatization, discrimination, and adverse medical experiences.
View Article and Find Full Text PDFJ Clin Endocrinol Metab
January 2025
Department of Women's and Children's Health, Karolinska Institutet, SE-171 76 Stockholm, Sweden.
Classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH) is a rare genetic condition that results in cortisol deficiency and excess production of adrenal androgens. While the introduction of newborn screening for CAH has reduced morbidity and mortality, management of CAH remains challenging. Lifelong treatment with glucocorticoids is required to replace the endogenous cortisol deficiency and reduce excess adrenal androgens.
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