Familial desquamative interstitial pneumonitis occurring in infants.
Desquamative interstitial pneumonitis (DIP) is rare in children. Its cause is unknown. In general, it is of sporadic occurrence. We report 4 infants: 2 sibs in each of 2 separate families, who had DIP. All 4 infants died despite intensive care and immunosuppressive therapy. Our cases, plus one other similar kindred in the literature, confirm the occurrence of familial DIP in infancy. Further, our experience suggests that DIP in these familial cases carries a worse prognosis than that reported in sporadic cases.
D005260:Female; D005808:Genes, Recessive; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000473:pathology; D008297:Male; D011658:Pulmonary Fibrosis / Q000235:genetics* / Q000473:pathology