Using a family history questionnaire, we investigated the occurrence of dementia among relatives of patients with a clinical diagnosis of Alzheimer's disease (AD) and among the relatives of age-matched control subjects. Cumulative lifetime risk of developing AD-type dementia was greater among relatives of AD probands and was consistent with an autosomal dominant genetic mode of transmission. Although the lifetime risk of AD-type dementia was similar among relatives of early-onset and late-onset AD probands, relatives of early-onset probands tended to have an earlier onset of dementia than did relatives of late-onset AD probands. This result raises the possibility that age at onset of dementia in AD may be genetically determined.
Fournier gangrene (FG) is a rare and life-threatening urosurgical emergency characterized most often by a polymicrobial infection of the perineal, genital, or perianal region. FG has an increased incidence in male patients, patients with alcoholism, and patients with immunocompromise including human immunodeficiency virus (HIV) and uncontrolled diabetes. FG often begins as a simple abscess or cellulitis with progression to necrotizing soft tissue infection (NSTI). Delays in diagnosis and treatment confer high mortality. Early recognition and high clinical suspicion are important in making a timely diagnosis, as early manifestations are often subtle. The most significant modifiable risk factor associated with NSTI mortality is delay to surgical intervention. Coordination of both inpatient medical and surgical teams to implement appropriate therapy is vital to successful outcomes. The emergency medicine clinician must be vigilant for this condition and be aware of risk factors, prognostic indicators, and proper treatment protocols to recognize FG early and initiate appropriate management. The objective of this review is to provide updated and relevant information regarding recognition, diagnosis, and management of FG for the emergency medicine provider.
Solid-organ transplant graft-versus-host disease (SOT-GVHD) is a rare complication of organ transplant that is associated with high mortality. The initial signs and symptoms are vague, so this disease is easily confused with other posttransplant complications. A case of SOT-GVHD occurred after orthotopic liver transplant for liver failure due to hepatitis C in a patient in a Veterans Affairs intensive care unit. The patient had dehydration, acute kidney injuries, rashes, diarrhea, and pancytopenia. Results of skin biopsy, bone marrow biopsy, and cytogenetic studies were consistent with SOT-GVHD. Despite supportive care including antibiotics, antiviral and antifungal therapy, high-dose steroids, antithymoglobulin and neupogen, the patient died of overwhelming sepsis. Owing to the rarity of SOT-GVHD, no evidence-based guidelines or recommendations for treatment exist. Treatment includes high-dose corticosteroids and antibiotic, antifungal, and antiviral prophylaxis. Treatment of liver transplant–related GVHD with anti–tumor necrosis factor a agents has been successful.
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