The American journal of the medical sciences
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Common variable immunodeficiency (CVI) is a heterogeneous immunodeficiency syndrome characterized by hypogammaglobulinemia, recurrent bacterial infections, and a variety of immunological abnormalities. In addition to recurrent infections, patients with this syndrome also suffer from an increased incidence of autoimmune disease and malignancy. Because the spectrum of associated diseases is broad, patients with CVI are seen by a variety of medical specialists. In this review, the pathogenesis, clinical manifestations, diagnosis, and treatment of CVI are discussed.
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Review Case Reports
Management of myxedematous respiratory failure: review of ventilation and weaning principles.
Respiratory failure in myxedema is a complex medical emergency and may require prolonged ventilatory assistance. Appropriate management of this medical problem requires an understanding of its effects on the central nervous system and peripheral neuromusculoskeletal system. ⋯ The first part of this review discusses the mechanisms of respiratory failure in myxedema. The second part explores strategies in mechanical ventilation and weaning of myxedematous patients.
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Review Case Reports
Bone marrow necrosis in sickle cell disease: a description of three cases and a review of the literature.
Bone marrow necrosis (BMN) ranges from a localized to a widespread generalized process. Most often seen in patients with leukemia and other malignant conditions, generalized BMN has also been observed in patients with sickle cell disease (SCD), where it is almost certainly a consequence of blood vessel occlusion. Activation of the clotting system seems to play a role in this clinical setting. ⋯ Despite such suggestive clinical features, diagnosis of BMN still requires a bone marrow biopsy. Particularly in patients with SCD, the early institution of transfusion therapy can be life-saving. The ominous prognosis ascribed to generalized BMN seems to reflect the poor outcome of such underlying conditions as leukemia; however, the prognosis of generalized BMN is not so poor in association with SCD and other nonmalignant states.
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Case Reports
Transfusion-associated falciparum malaria successfully treated with red blood cell exchange transfusion.
Falciparum malaria is frequently associated with significant morbidity and mortality. The use of exchange transfusion as a therapeutic modality for severe cases of malaria has been described previously. ⋯ He was treated with antimalarial chemotherapy and also underwent a 12-unit red blood cell exchange transfusion, decreasing his parasite load to < 1%, as determined on repeat smear. It is prudent to be aware of the efficacy of this adjunctive treatment, especially with ever-increasing travel and a resultant increase in the prevalence of tropical diseases in the United States.
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Review Case Reports
Unilateral internuclear ophthalmoplegia and recovery with thiamine in Wernicke syndrome.
Internuclear ophthalmoplegia is usually caused by multiple sclerosis, tumors, or vascular lesions of the brain stem. We report a patient with Wernicke syndrome who presented with a right-sided internuclear ophthalmoplegia. He recovered completely with intravenous thiamine (vitamin B1). There were no lesions in the magnetic resonance image (MRI) of the brain, suggesting a derangement at the cellular level as the cause.