Chest
Volume 77, Issue 2, February 1980, Pages 165-171
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Clinical Investigations
Desquamative Interstitial Pneumonitis in Children

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Interstitial pneumonitis in children is a rare and poorly understood disease. Controversy exists as to whether the various histologic changes encountered represent different diseases 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.

Section snippets

Materials and Methods

Desquamative interstitial pneumonitis was identified in two ways. A review of approximately 2,500 cases of pneumonia in children from birth to 16 years of age between 1953 and 1971 revealed 38 cases of biopsy-proven interstitial pneumonitis or pulmonary fibrosis, or both. On microscopic examination of pulmonary tissue from these 38 cases, 12 cases of desquamative interstitial pneumonitis were discovered. Two additional cases were found in a review of the pathologic reports of pulmonary tissue

Results

The age at onset in 28 patients with desquamative interstitial pneumonitis is shown in Figure 2. It is a skewed distribution, with the age at onset being less than 1 year in 14 cases and between ages 2½ and 16 years in 14 cases. Five patients were boys and nine were girls in the group from the Mayo Clinic; seven were boys and seven were girls in the group from the literature.

Table 1 shows the frequency of initial symptoms and signs in the 14 patients from the Mayo Clinic, the 14 cases from the

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  • Cited by (58)

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    Associate Professor of Pediatrics.

    Associate Professor of Medicine.

    Assistant Professor of Pathology.

    Deceased.

    Manuscript received May 11; revision accepted April 26

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