Pediatric Hematology and Oncology · 2003 · 29 citations · 33 references
MedicineInfectious DiseasesPediatricsImmunotherapyPediatric ImmunologyPediatric Infectious DiseasesImmunocompromised Host InfectionsPediatric Antibiotic TherapyPediatric Antimicrobial TherapyPediatric Clinical ImmunologyAntibiotic Regimen OptimizationImmunomodulatory EffectsInnate Immune DefectsPediatric Infection TreatmentPediatric Infectious Diseases ManagementPediatric Viral InfectionsPneumonia Antibiotic TherapyViral Infection Treatment
A 2-year-old boy with severe multiorgan disease (i.e., otitis, enteritis, bilateral pneumonia, encephalopathy, myocarditis, rash) was diagnosed with adenovirus-associated macrophage activation syndrome according to clinical and laboratory parameters (fever, hepatosplenomegaly, bicytopenia, hyperferritinemia, hypertriglyceridemia). He had no unusual history of earlier infections or a family history of hemophagocytic syndrome or immune defects. Intravenous immunoglobulin was administered to prevent exacerbation of the suspected incipient hemophagocytic syndrome. Clarithromycin, a macrolide with immunomodulatory effect, was included in the antibiotic regimen of the progressive pneumonia, followed by rapid amelioration and remission of clinical and laboratory findings. Causal links between treatment and clinical improvement are discussed and a brief review of the recent literature is included.
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Perforin Gene Defects in Familial Hemophagocytic Lymphohistiocytosis
Susan E. Stepp, Rémi Dufourcq-Lagelouse, Françoise Le Deist et al. · Science · 1999 · 1.2K citations
Life Sciences, Genetics, Immunology +15