1/45. Unusual association of thyroiditis, Addison's disease, ovarian failure and celiac disease in a young woman.The coexistence of autoimmune endocrine diseases, particularly autoimmune thyroid disease and celiac disease (CD), has recently been reported. We here present a 23-year-old woman with a diagnosis of hypothyroidism due to Hashimoto's thyroiditis, autoimmune Addison's disease, and kariotypically normal spontaneous premature ovarian failure. Considering the close association between autoimmune diseases and CD, we decided to search for IgA anti-endomysium antibodies (EmA) in the serum. The positivity of EmA and the presence of total villous atrophy at jejunal biopsy allowed the diagnosis of CD. On a gluten-free diet the patient showed a marked clinical improvement accompanied, over a 3-month period, by a progressive decrease in the need for thyroid and adrenal replacement therapies. After 6 months, serum EmA became negative and after 12 months a new jejunal biopsy showed complete mucosal recovery. After 18 months on gluten-free diet, the anti-thyroid antibodies titre decreased significantly, and we could discontinue thyroid substitutive therapy. This case emphasizes the association between autoimmune polyglandular disease and CD; the precocious identification of these cases is clinically relevant not only for the high risk of complications (e.g. lymphoma) inherent to untreated CD, but also because CD is one of the causes for the failure of substitute hormonal therapy in patients with autoimmune thyroid disease.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/45. White Addison's disease: what is the possible cause?A case of chronic primary adrenal insufficiency without hyperpigmentation in a 64-year-old woman is reported. Due to the absence of hyperpigmentation the diagnosis was delayed and she became critically ill. During endocrine evaluation, in order to investigate the mechanism responsible for the absence of hyperpigmentation, skin biopsy was done and hormones responsible for the skin pigmentation were measured. Absence of hyperpigmentation is explained by high degree of melanosome degradation in secondary lysosomes called "compound melanosomes", which overwhelmed increased stimulation of the skin pigmentation. melanocyte-stimulating hormones were elevated with a strikingly high beta-LPH/ACTH ratio. To our knowledge, this is the first study of pathogenic mechanisms responsible for the absence of hyperpigmentation in white Addison's disease.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
3/45. X-linked adrenoleukodystrophy: spinocerebellar variant.The phenotypic variability in X-linked adrenoleukodystrophy (X-ALD) can be wide and varied. Rarely, it can present with clinical signs of spinocerebellar degeneration. There are very few reported cases of selective predominant white matter disease of the cerebellum in these patients. We report a patient with a rare variant of adult onset ALD who was previously diagnosed as spinocerebellar ataxia. He was a 24-year-old male who had delayed developmental milestones, developed signs of spinocerebellar degeneration (SCD) after 10 years of Addison's disease. Serial magnetic resonance imaging (MRI), revealed cerebellar and pontine white matter disease but sparing the cerebral cortex and supratentorial white matter. His diagnosis of X-ALD was subsequently confirmed by the elevated serum very long chain fatty acids. This patient illustrates the unusual clinical presentation and imaging features of X-ALD and the importance of considering X-ALD in the clinical context of spinocerebellar degeneration. Early recognition of this rare variant would allow proper genetic counselling and institution of dietary therapy and/or bone marrow transplantation.- - - - - - - - - - ranking = 0.0037303439846998keywords = bone (Clic here for more details about this article) |
4/45. Successful treatment of high turnover osteoporosis in a patient with adrenocortical insufficiency.We present a case of high-turnover osteoporosis associated with substituted adrenocortical insufficiency (Addison's disease) and its successful treatment with calcitonin and calcitriol. A 43-year-old man presented with markedly reduced bone mineral density (BMD) (lumbar spine BMD -3.46 SD below healthy young adults) after thirteen years of glucocorticoid substitution. Interestingly, his osteocalcin levels indicated an unusually high bone turnover and his alkaline phosphatase levels were also increased. Combination treatment with calcitriol and calcitonin was started. Re-evaluation after twelve months revealed a substantial increase in BMD ( 6.8% for the lumbar spine, 15% for the left hip). alkaline phosphatase levels had normalised and osteocalcin was nearly down to normal. In spite of a thorough evaluation, no other cause of osteoporosis was detected. We discuss these findings in view of the existing literature.- - - - - - - - - - ranking = 0.0074606879693995keywords = bone (Clic here for more details about this article) |
5/45. Addison's disease: the potentially life-threatening tan.Addison's disease is a potentially life-threatening endocrine disorder. The classic dermatologic sign of Addison's disease is diffuse hyperpigmentation of the skin with focal hyperpigmentation of scars, nails, and the oral mucosa. Recognition of these dermatologic manifestations in a patient with otherwise vague constitutional symptoms warrants a consideration of Addison's disease.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
6/45. dehydroepiandrosterone replacement in addison's disease.Addison's disease is a rare endocrine disorder which can be life-threatening. It can also interfere with the normal development of adrenarche, resulting in the absence of pubic and axillary hair growth. We report a case of satisfactory restoration of adrenarche through DHEA administered in conjunction with the standard glucocortisone and fluorocortisone replacement.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
7/45. A patient with autoimmune hepatitis type I, Addison's disease, atrophic thyroiditis, atrophic gastritis, exocrine pancreatic insufficiency, and heterozygous alpha1-antitrypsin deficiency.This report describes a 60-yr-old white male presenting with decompensated liver cirrhosis. He had a history of Addison's disease for 36 yr, primary hypothyroidism for 5 yr, and moderate alcohol consumption. His laboratory studies and a liver biopsy supported the diagnosis of autoimmune hepatitis. Furthermore, he was found to be heterozygous for the piZ allele of the alpha1-antitrypsin gene with normal serum alpha1-antitrypsin levels and absence of pulmonary affection. Mucosal biopsies revealed moderately severe atrophic gastritis; however, signs of pernicious anemia were missing. An association of autoimmune hepatitis with endocrine disorders and atrophic gastritis has been described. Long term hydrocortisone therapy for his adrenal insufficiency may have prevented a faster course of the liver disease, whereas the heterozygous alpha1-antitrypsin deficiency and moderate alcohol consumption constituted additional risk factors ultimately leading to the development of cirrhosis.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
8/45. Hazards of steroid therapy in hepatic failure.Three patients with extensive skin disease and liver failure are described. Two were treated with large amounts of topical corticosteroids and one with a small oral dose of betamethasone. The former two patients developed Addisonian crises when they failed to apply the topical steroids and all three patients developed aseptic necrosis of bone. This report emphasizes that the hazards of steroid therapy may be increased in patients with hepatic failure.- - - - - - - - - - ranking = 0.0037303439846998keywords = bone (Clic here for more details about this article) |
9/45. Localized non-Hodgkin's lymphoma of the adrenal and thyroid glands.A case of immunoblastic lymphoma, involving only the thyroid and the adrenal glands, is presented. The patient had clinical symptoms and findings of Addison's disease, and computed tomography (CT) demonstrated bilateral adrenal tumoral enlargement. He also had euthyroid diffuse multinodular goiter. The diagnosis of the patient was based on the cytological examination of the aspiration materials from both endocrine glands. The patient received "m-BNCOD" chemotherapy regimen and replacement therapy for Addison's disease. At the end of three courses, a partial response was obtained.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
10/45. Addisonian crisis precipitated by thyroxine therapy: a complication of type 2 autoimmune polyglandular syndrome.hypothyroidism is a common condition. Rarely, it may occur in combination with autoimmune failure of other endocrine glands (autoimmune polyendocrinopathy syndrome type 2, previously known as Schmidt's syndrome). In such cases, restoring normal thyroid function may precipitate adrenal failure. Clinicians should have a high index of suspicion for this condition in patients with Addison's disease, those with a family history of autoimmune endocrine gland failure, patients with one autoimmune endocrine disease who develop nonspecific or serious illness, and patients with type 1 diabetes mellitus whose insulin requirements drop without obvious explanation.- - - - - - - - - - ranking = 3keywords = endocrine (Clic here for more details about this article) |
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