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1/136. The efficacy of therapeutic plasmapheresis for the treatment of fatal hemophagocytic syndrome: two case reports.

    A potentially fatal hemophagocytic syndrome (HPS) has been noted in patients with reactive HPS. We describe 2 patients with reactive HPS treated with a regimen of therapeutic plasmapheresis and evaluate the efficacy of plasmapheresis for fatal HPS. Case 1 was a 31 year-old woman who had been treated for systemic lupus erythematosus (SLE) with corticosteroid hormones and immunosuppressants. She presented with persistent leukopenia and thrombocytopenia with spiking fever. She had an elevated level of serum ferritin, liver dysfunction, coagulopathy, and plasma inflammatory cytokines. Her bone marrow smear disclosed numerous hemophagocytosis of histiocytes. She was administered therapeutic plasmapheresis with total plasma exchange by fresh frozen plasma. There was an immediate and prominent decrease of cytokines, and she completely recovered. Case 2 was a 34 year-old woman who had been receiving high doses of corticosteroids and plasmapheresis for severe Stevens-Johnson's syndrome. After 18 months, she presented with physical and laboratory findings resembling lupus-like conditions and was administered high doses of corticosteroids and immunosuppressants. Human parvovirus B19 infection was detected by IgM and IgG antibodies and viral dna from a bone marrow sample; moreover, a bone marrow smear disclosed findings of HPS. Repeated therapeutic plasmapheresis was effective for improving her symptoms and laboratory abnormalities; however, she suffered from septic methicilline resistant staphylococcus aureus infection and finally died of a brain hemorrhage resulting from disseminated intravascular coagulation (DIC).
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ranking = 1
keywords = infection
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2/136. Stealth virus epidemic in the Mohave Valley: severe vacuolating encephalopathy in a child presenting with a behavioral disorder.

    An infectious illness, attributed to atypically structured cytopathic "stealth" viruses, occurred in 1996 in the Mohave Valley region of the united states. A stealth virus-infected child from this region has developed a severe noninflammatory, vacuolating (spongiform) en cephalopathy. The illness initially presented as a behavioral problem without overt neurological signs. Extensive investigations, including repeated magnetic resonance imaging, two brain biopsies, and stealth virus cultures, have helped define the disease process occurring in this child. Significant clinical benefit with apparent retardation of disease progression occurred during a 6-week course of ganciclovir therapy. The potential contributing role of stealth virus infections in children presenting with behavioral problems needs to be addressed.
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ranking = 0.5
keywords = infection
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3/136. fatigue that doesn't go away.

    The classic profile of the chronic fatigue syndrome (CFS) patient is a white, middle-age female. Characterized by profound fatigue, CFS often starts with an acute viral infection. While today's medicine provides symptomatic relief, research is offering innovative insights. With this research, is a cure for these patients just around the corner?
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ranking = 0.5
keywords = infection
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4/136. Viral infections in interferon-gamma receptor deficiency.

    interferon-gamma receptor deficiency is a recently described immunodeficiency that is associated with onset of severe mycobacterial infections in childhood. We describe the occurrence of symptomatic and often severe viral infections in 4 patients with interferon-gamma receptor deficiency and mycobacterial disease. The viral pathogens included herpes viruses, parainfluenza virus type 3, and respiratory syncytial virus. We conclude that patients with interferon-gamma receptor deficiency and mycobacterial disease have increased susceptibility to some viral pathogens.
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ranking = 5.1949160046616
keywords = bacterial infection, infection
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5/136. Recurrent viral associated hemophagocytic syndrome in a child with Langerhans cell histiocytosis.

    Langerhans cell histiocytosis (LCH) with subsequent viral-associated hemophagocytic syndrome (VAHS) or secondary hemophagocytic lymphohistiocytosis (HLH) is extremely rare. A 15-month-old girl with disseminated LCH experienced three episodes of VAHS during maintenance therapy. Viral infection, with influenza A, herpes simplex, and adenovirus, respectively, was documented at each episode. She recovered each time after interruption of maintenance therapy. The occurrence of fever and pancytopenia in patients with chemotherapy-treated LCH can be associated with VAHS and not with relapsing LCH.
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ranking = 0.5
keywords = infection
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6/136. Non-specific viral infections as possible synchronising events of the manifestation of type 1 diabetes.

    Four cases of simultaneous manifestation of Type 1 diabetes in two members of the same household are reported. In all cases, a flu-like infection preceded diabetes onset. Surprisingly, despite simultaneous development of insulin dependency, insulin requirements were strikingly different at 3 months in all cases. These observations suggest that increased insulin resistance during infection may cause insulin deficiency in individuals with widely varying residual beta cell activity.
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ranking = 3
keywords = infection
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7/136. Papular acrodermatitis of childhood: the Gianotti-Crosti syndrome.

    Papular acrodermatitis of childhood (PAC), also known as Gianotti-Crosti syndrome, is a self-limited disorder with acute onset generalized lymphadenopathy and monomorphic lentil-sized, dense, nonconfluent, symmetric, flat-topped, non-pruritic papules. We describe 2 patients, one with anicteric hepatitis, lymphocytosis, and positive hepatitis b surface antigenemia, and the other with a cytomegalovirus (CMV) infection.
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ranking = 0.5
keywords = infection
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8/136. Anesthetic management of toxic epidermal necrolysis: report of three adult cases.

    Toxic epidermal necrolysis is a rare but acute life-threatening syndrome in which the epidermis blisters and peels in large sheets. In general, patients with this syndrome are managed as severe second-degree burn patients, but special consideration should be given to mucous membrane involvement that reduces fluid intake and worsens the fluid deficit, systemic involvement that makes these patients hemodynamically unstable, and progression of cutaneous lesions that enhances the risk of infection and sepsis.
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ranking = 0.5
keywords = infection
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9/136. Vagal neuropathy after upper respiratory infection: a viral etiology?

    PURPOSE: To describe a condition that occurs following an upper respiratory illness, which represents injury to various branches of the vagus nerve. patients with this condition may present with breathy dysphonia, vocal fatigue, effortful phonation, odynophonia, cough, globus, and/or dysphagia, lasting long after resolution of the acute viral illness. The patterns of symptoms and findings in this condition are consistent with the hypothesis that viral infection causes or triggers vagal dysfunction. This so-called postviral vagal neuropathy (PVVN) appears to have similarities with other postviral neuropathic disorders, such as glossopharyngeal neuralgia and Bell's palsy. MATERIALS AND methods: Five patients were identified with PVVN. Each patient's chart was reviewed, and elements of the history were recorded. RESULTS: Each of the 5 patients showed different features of PVVN. CONCLUSIONS: Respiratory infection can trigger or cause vocal fold paresis, laryngopharyngeal reflux, and neuropathic pain.
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ranking = 3
keywords = infection
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10/136. The role of infections in primary hemophagocytic lymphohistiocytosis: a case series and review of the literature.

    There is a paucity of literature addressing infection-related morbidity and mortality in children with primary hemophagocytic lymphohistiocytosis (HLH), a rare condition characterized by abnormal proliferation of macrophages, hypercytokinemia, and T cell immunosuppression. Therefore, a retrospective chart review was done of patients diagnosed with primary HLH over a 15-year period. Significant infections present at diagnosis, during the course of illness, and just prior to death or at autopsy were noted. Of the 18 children identified with primary HLH, an infectious agent was documented at the initial presentation of HLH in 5. Significant infections occurred during therapy in 10 (56%) of 18. Of the 12 fatal cases, invasive infection was the cause of death in 8 children, and 6 of these deaths were directly attributable to invasive fungal infection. Significant infections were common during therapy in children with primary HLH, and fungal infections were an important cause of mortality in this group.
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ranking = 5.5
keywords = infection
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