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Pathogenesis of primary adrenal insufficiency
Best Practice & Research Clinical Endocrinology & Metabolism, 2009Autoimmune Addison's disease is caused by autoreactivity towards the adrenal cortex involving 21-hydroxylase autoantibodies and autoreactive T cells. Autoimmune destruction of the adrenal cortex is triggered by hitherto unknown environmental factors in individuals with genetic susceptibility.
Eystein, Husebye, Kristian, Løvås
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Clinical Medicine (Russian Journal)
Due to the non-specificity of a number of clinical manifestations of Addison’s disease, the diagnosis of primary adrenal insufficiency in general therapeutic profile hospitals causes certain difficulties. The article presents a clinical case of diagnosing primary adrenal insufficiency in combination with autoimmune thyroiditis and hypogonadism as a ...
T. I. Kalenchits +3 more
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Due to the non-specificity of a number of clinical manifestations of Addison’s disease, the diagnosis of primary adrenal insufficiency in general therapeutic profile hospitals causes certain difficulties. The article presents a clinical case of diagnosing primary adrenal insufficiency in combination with autoimmune thyroiditis and hypogonadism as a ...
T. I. Kalenchits +3 more
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The expanding world of primary adrenal insufficiencies
European Journal of Endocrinology, 1997The past few years have seen a phenomenal increase in our understanding of three genetic causes of primary adrenal and gonadal hormone deficiency. Lin and coworkers reported in 1995 (1) that patients with congenital lipoid adrenal hyperplasia have mutations of the steroidogenic acute regulatory protein.
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Primary Adrenal Insufficiency and Hyperpigmentation
The American Journal of Medicine, 2023Manidipa Majumder +2 more
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Adrenal hypoplasia congenita – an uncommon reason of primary adrenal insufficiency
Annales d'Endocrinologie, 2010Adrenal hypoplasia congenita (AHC) is a rare inherited condition characterised by primary adrenal failure and hypogonadotropic hypogonadism. Most cases arise from mutations in the NR0B1 gene (Xp21.3), which encodes an orphan nuclear receptor DAX-1. A 20-year-old patient was recently diagnosed with AHC.
M, Fichna +4 more
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Primary T-cell lymphoma of the adrenal glands with adrenal insufficiency
Human Pathology, 1986A 74-year-old man with bilateral adrenal gland masses presented with adrenal insufficiency. Biopsies and immunohistochemical staining of both masses revealed a large cell T-cell lymphoma. The lymphoma subsequently involved the central nervous system. A review of the literature disclosed that this is an extremely uncommon presentation of lymphoma.
B, Schnitzer, D, Smid, R V, Lloyd
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The "Primary Change" in Adrenal Insufficiency
Science, 1937R F, Loeb, D W, Atchley
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Rare monogenic causes of primary adrenal insufficiency
Current Opinion in Endocrinology, Diabetes & Obesity, 2018Purpose of review Monogenic disorders play significant roles in the pathogenesis of childhood-onset primary adrenal insufficiency (PAI). The most common form of PAI is congenital adrenal hyperplasia (CAH), which includes the enzymatic defects of the steroidogenic pathway.
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Primary Adrenal Insufficiency in Childhood: Data From a Large Nationwide Cohort
Journal of Clinical Endocrinology and Metabolism, 2021Antonio Balsamo +2 more
exaly
Approach to the Patient: Diagnosis of Primary Adrenal Insufficiency in Adults
Journal of Clinical Endocrinology and Metabolism, 2023Eystein S Husebye, Husebye Eystein S
exaly