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Familial Opsonization Defect Associated with Fatal Infantile Dermatitis, Infections, and Histiocytosis

Overview
Journal Arch Dis Child
Specialty Pediatrics
Date 1975 Apr 1
PMID 1096830
Citations 8
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Abstract

Members of four generations of a family had a defect of serum opsonization for yeast phagocytosis consistent with dominant inheritance. 2 were healthy, one had chronic osteomyelitis, and the fourth developed a fatal illness in infancy characterized by exfoliative dermatitis, diarrhoea, multiple bacterial infections, and failure to thrive, which resembled the two prevously reported cases with this opsonization defect. At necropsy the infant also had lymphoid depletion, which was possibly secondary, and massive histiocytic infiltration.

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