The American journal of the medical sciences
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Review Case Reports
Metabolic acidosis with extreme elevation of anion gap: case report and literature review.
A patient with severe metabolic acidosis and an extremely elevated (57 mEq/L) serum anion gap (AG) is described, and the multiple factors that produced the patient's complex abnormalities are discussed in detail. These include renal failure, rhabdomyolysis, marked hyperphosphatemia, hemoconcentration, and an unidentified organic metabolic acidosis. A review of the literature indicates that the common thread observed in almost each instance of profoundly elevated AG values is a multifactorial pathogenesis that usually includes renal insufficiency, associated with a proven or likely cause of organic metabolic acidosis, or with exogenous phosphate intoxication.
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This review focuses on new developments in the pathophysiology and treatment of von Willebrand disease (vWd). New aspects of the cell biology, gene control, and structure-function correlates of von Willebrand factor (vWf) are reviewed. vWd is more prevalent than previously recognized, affecting up to 1% of the population; this is particularly evident in women's health. ⋯ Treatment of classical vWd with desmopressin acetate and plasma derivatives is discussed, as is the potential for intravenous immunoglobulin and corticosteroids in acquired vWd. Special situations, such as the management of vWd in pregnancy, are also discussed.
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Fibromyalgia (FM) patients report early morning awakenings, awakening feeling tired or unrefreshed, insomnia, as well as mood and cognitive disturbances; they may also experience primary sleep disorders including sleep apnea. Longitudinal studies have demonstrated the chronic nature of these disturbances in patients with FM. A distinct relationship exists between poor sleep quality and pain intensity. ⋯ Sleep architecture is altered in FM patients showing an increase in stage 1, a reduction in delta sleep, and an increased number of arousals. Before prescribing pharmacologic compounds aimed at modifying sleep, adequate pain control and sleep habits should be achieved; tricyclic antidepressants, trazadone, zopiclone, and selective serotonin reuptake inhibitors, however, may be required. More research is needed to elucidate the cellular and molecular mechanisms involved in the sleep disturbances occurring in patients with FM.
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Increasing early success-post lung transplant has been tempered by the long-term development of histologic bronchiolitis obliterans (OB) or of the progressive airway obstruction which is called bronchiolitis obliterans syndrome (BOS). Multiple lines of evidence suggest that OB/BOS is due to an injury directed against the epithelial cells in the airways of the donor lung by the immune system of the recipient. ⋯ Results of treatment with augmented immunosuppression have been disappointing but the treatment of complicating infections in the allograft can be beneficial. Multicenter studies are needed to assess the efficacy of new immunosuppressive agents in preventing or treating OB/BOS.
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A case of bilateral adrenal myelolipoma in association with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency is presented. The clinical, radiologic, and endocrinologic features of this case are correlated with a review of the literature.