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Published on: March 6, 2019
Desquamative interstitial pneumonitis in children
Abstract:
Interstitial pneumonitis in children is a rare and poorly understood disease. Controversy exists as to whether the varoius histologic changes encountered represent different disease or a spectrum of the same disease. Fourteen biopsy-confirmed cases of desquamative interstitial pneumonitis in children were seen at the Mayo Clinic between 1953 and 1975. A search of the literature revealed 14 additional cases but no series of exclusively desquamative interstitial pneumonitis. The most frequent symptoms were retardation of growth and dyspnea, often accompanied by cough. Tachypnea was the most common finding on examination; rales, cyanosis, and clubbing were variably present. The chest roentgenogram was distinctly abnormal in all cases; it usually revealed a combined interstitial and alveolar pattern extending bilaterally from the hilus to the base. Results of laboratory studies were nonspecific for desquamative interstitial pneumonitis. All 28 patients in this review were treated with corticosteroids; 17 (61 percent) survived. Desquamative interstitial pneumonitis was found in association with a variety of other major illnesses. The cause remains unknown.
Insights
Desquamative interstitial pneumonitis in children is a rare lung disease. Corticosteroid treatment showed a 61% survival rate in a review of 28 pediatric cases.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Interstitial Lung Diseases
Background:
- Interstitial pneumonitis in children is poorly understood, with ongoing debate about its classification.
- Desquamative interstitial pneumonitis (DIP) is a specific form of this condition.
Purpose of the Study:
- To review biopsy-confirmed cases of desquamative interstitial pneumonitis in children.
- To analyze clinical presentation, treatment outcomes, and associated conditions.
Main Methods:
- Retrospective review of 14 Mayo Clinic cases (1953-1975).
- Literature search identifying an additional 14 cases, totaling 28 patients.
- Analysis of clinical, radiographic, and laboratory findings.
Main Results:
- Common symptoms included growth retardation and dyspnea; tachypnea was the most frequent sign.
- Chest X-rays consistently showed abnormal interstitial and alveolar patterns.
- Corticosteroid treatment resulted in a 61% survival rate (17 of 28 patients).
Conclusions:
- Desquamative interstitial pneumonitis in children presents with specific clinical and radiographic features.
- Corticosteroids appear to be a viable treatment option, though the disease's etiology remains unknown.
- DIP can occur alongside other significant medical conditions.
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