Cases reported "Addison Disease"

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1/150. 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.
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2/150. 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.
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3/150. 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.
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keywords = cortex
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4/150. cardiac tamponade preceding adrenal insufficiency--an unusual presentation of Addison's disease: a report of two cases.

    Two cases of young healthy males presenting with cardiac tamponade and developing clinical adrenal insufficiency within a few weeks are described. On presentation they had a brisk inflammatory response with complement activation. Both had signs of subclinical hepatitis, and both have later shown evidence of thyroid involvement. The possibility of a connection between pericarditis and adrenal insufficiency is discussed.
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5/150. adrenoleukodystrophy associated with vitiligo and ulcerative colitis.

    adrenoleukodystrophy (ALD) is an X-linked inherited disorder of lipid metabolism usually presenting in childhood or early adolescence. It is a progressive disease with symptoms of adrenal insufficiency and central nervous system demyelination. The pathology results from the accumulation of very long-chain fatty acids and an inflammatory reaction in the brain white matter. We report a case of ALD associated with adrenal insufficiency and two autoimmune diseases: vitiligo and ulcerative colitis.
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6/150. Brittle Addison's disease: a new variation on a familiar theme.

    Unstable and unpredictable disease control in diabetes or asthma, with frequent hospitalisations, is frequently referred to as 'brittle'. We describe two cases of Addison's disease with recurrent hospitalisations in hypo-adrenal crises. Both patients had significant psychosocial disruption, and failure to take hydrocortisone replacement therapy was admitted in one and biochemically proven in the other. We propose that 'brittle' Addison's disease in these cases was due to poor treatment compliance related to psychosocial factors. These features have particular similarities with the syndrome of brittle diabetes.
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7/150. female genital coccidioidomycosis (FGC), Addison's disease and sigmoid loop abscess due to coccidioides immites; case report and review of literature on FGC.

    We describe a woman with unusual complications of infection with coccidioides immitis--infection of the genital tract and adrenal insufficiency. The patient also had intestinal coccidioidomycosis (cocci) in conjunction with presumed pulmonary, and asymptomatic central nervous system cocci. To our knowledge, concurrent FGC, intestinal and adrenal cocci have not been reported previously. A medline review from 1966-1997 revealed only 1 case of adrenal insufficiency due to cocci. FGC is rare; we identified 12 reported cases since 1929. No combination of investigations or clinical features is sensitive enough to predict FGC. diagnosis is usually made after microscopy of surgical specimens. FGC presents either as tubo-ovarian disease or endometritis. Treatment generally involves surgical excision and antifungal agents. We hypothesize that an initial trial of antifungals may obviate the need for surgery.
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8/150. Acute adrenal insufficiency after cardiac surgery.

    adrenal insufficiency after cardiac surgery can easily be confused during the course of an immediate unstable postoperative period. If unrecognized, this condition may cause serious morbidity and can be fatal. We report on a 43-yr-old female patient with chronic known adrenal insufficiency, who, despite her adequate preoperative replacement therapy, presented with one episode of acute hypoadrenal crisis after elective open heart surgery, which could serve as a model to illustrate the salient clinical features and possible problems in this setting for diagnosing this problem to patients in whom chronic adrenal insufficiency remains unknown.
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9/150. Addison's disease in type 1 diabetes presenting with recurrent hypoglycaemia.

    Primary adrenal insufficiency (Addison's disease) often develops insidiously. Although a rare disorder, it is more common in type 1 diabetes mellitus. A 19 year old male with type 1 diabetes and autoimmune hypothyroidism experienced recurrent severe hypoglycaemia over several months, despite a reduction in insulin dose, culminating in an adrenal crisis. Recurrent severe hypoglycaemia resolved after identification and treatment of the adrenocortical insufficiency. In type 1 diabetes, undiagnosed Addison's disease can influence glycaemic control and induce severe hypoglycaemia.
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10/150. addison disease after appendicitis.

    A 14-year-old boy manifested acute abdominal pain, vomiting, high temperature and diarrhea. He also underwent increasing hyponatremia and hyperkalemia after appendectomy. Further testing confirmed addison disease. The serum adrenal antibody test was positive, and other autoimmune diseases were excluded.
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