Familial opsonization defect associated with fatal infantile dermatitis, infections, and histiocytosis.
نویسندگان
چکیده
منابع مشابه
Familial opsonization defect associated with fatal infantile dermatitis, infections, and histiocytosis.
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 th...
متن کاملYeast opsonization defect and immunoglobulin deficiency in severe infantile dermatitis (Leiner's disease).
The defect in Leiner's disease, which presents in early infancy with extensive dermatitis, diarrhoea, and failure to thrive, has been attributed to a defect of the fifth component of complement (C5). We report 2 brothers with extensive dermatitis and dysgammaglobulinaemia. Both died. The older showed symptoms of Leiner's disease: C5 tests were not performed. The younger had extensive dermatitis...
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For many physicians, retinal haemorrhages (RHs) in infants and young children remain highly diagnostic of non-accidental (abusive) head trauma. Because clinicians have applied indirect ophthalmoscopy selectively to cases of suspected child abuse, the association between RH and other conditions such as infection, coagulopathy and accidental trauma has encountered habitual bias, creating the pote...
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Familial exudative vitreoretinopathy (FEVR) is an inherited blinding condition characterized by abnormal development of the retinal vasculature. The authors describe a rare case of the disease associated with severe infantile osteoporosis in a young female patient. The patient was submitted to multiple vitreoretinal procedures in both eyes due to tractional macular detachments. The case was com...
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ژورنال
عنوان ژورنال: Archives of Disease in Childhood
سال: 1975
ISSN: 0003-9888,1468-2044
DOI: 10.1136/adc.50.4.311