[Addison's disease with arterial hypertension].
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This is the first reported case of survival of a significant burn in a patient with established Addison's disease. The systemic stress response to thermal injury is well recognised, there is a marked hypermetabolic response with prolonged periods of catabolism. In particular, the elevation of plasma cortisol levels is crucial for this response to severe systemic stress. Cortisol elevation is maintained for the duration of burn wound healing, is proportional to the burned body surface area and the normal circadian rhythm of endogenous cortisol is lost. Acute adrenal insufficiency has been described in patients suffering major burn injuries with generally poor outcomes. We discuss the management and complications of adrenal replacement therapy in a severe burn setting, as illustrated by this case report.
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Controversial data are available on the association between the retrovirus-like long-terminal repeat (LTR) DQ-LTR13 and genetic susceptibility to type 1 diabetes and other autoimmune diseases. We analyzed DNA samples from 315 type 1 diabetic patients, 166 autoimmune Addison's disease (AAD) patients, 1,054 healthy subjects, and 144 families of type 1 diabetic offspring. DQ-LTR13 was more frequent among patients than healthy subjects (P(c) < 0.0006), and a preferential transmission of DQB1*0302-LTR13(+) from parents to type 1 diabetic offspring was observed. DQ-LTR13 was in linkage disequilibrium (LD) with DQB1*0302 but not DQB1*0201. The presence of DQ-LTR13 increased the odds ratio of DQB1*0302 2.9- to 3.2-fold for type 1 diabetes and AAD. DRB1*0403 was absent in all of the 169 DRB1*04-positive patients but present in 27% (34 of 127) DRB1*04-positive healthy subjects (P(c) < 0.001). DQ-LTR13 was detected in 1 of 34 (3%) DRB1*0403-positive healthy subjects and 36 of 93 (39%) individuals carrying another DRB1*04 allele (P(c) = 0.002). Multivariate logistic regression analysis revealed that DQ-LTR13 is not independently associated with type 1 diabetes and AAD after correction for DQB1*0302 and DRB1*0403. Conversely, DQB1*0201, DQB1*0302, DRB1*0401, and DRB1*0403 were all significantly associated with disease risk also after correction for DQ-LTR13. We provide conclusive evidence that the genetic association of DQ-LTR13 with type 1 diabetes and AAD is primarily due to a LD with DQB1*0302 and DRB1*0403.
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A 48-year-old man was admitted with hypotension, hyperpigmentation, neuromuscular dysfunction and psychiatric symptoms. Serum sodium values were low and serum potassium values high. He had marked eosinophilia. A clinical diagnosis of Addison's disease was made. He initially responded satisfactorily to corticosteroid replacement but died suddenly at home. Postmortem examination showed miliary tuberculosis. The need to treat Addison's disease in the third world with antituberculous drugs even in absence of clear evidence of active tuberculosis is stressed.
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