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Desquamative interstitial pneumonia in sibs
M Tsukahara1, H Yoshii, T Imamura
1Department of Pediatrics, Yamaguchi University School of Medicine, Ube, Japan.
Insights
Two siblings diagnosed with desquamative interstitial pneumonia suggest an autosomal-recessive inheritance pattern. This rare genetic condition impacts lung function, with one sibling succumbing despite treatment and the other requiring oxygen support.
Area of Science:
- Pediatric pulmonology
- Medical genetics
Background:
- Desquamative interstitial pneumonia (DIP) is a rare lung disease.
- Its etiology in pediatric cases remains largely unknown.
Abstract:
We report on 2 sibs with desquamative interstitial pneumonia. The female died at age 1 7/12 years despite use of prednisolone and methylprednisolone, while the male, now age 3 years. is alive with oxygen support. The occurrence of desquamative interstitial pneumonia in sibs born to normal parents suggests that in some cases the disease is an autosomal-recessive trait.