Abstract: Objective　To explore the pathology, clinical manifestation, diagnosis, treatment and prognosis of desquamative interstitial pneumonia (DIP) in children. Methods　A child with DIP admitted to Children’s Hospital Affiliated to Nanjing Medical University was analyzed retrospectively. The final diagnosis was made based on the principle of clinicalradiologic-pathologic diagnosis. Related literatures were reviewed. Take “desquamative interstitial pneumonia” and “child” as the search word, 42 cases were retrieved in PubMed until July 2019. Eleven (11) cases in China were retrieved in Wanfang Med Online. 30 cases with complete clinical data and a case in this study were included. The pathological, clinical, and radiological features, lung function, response to treatment and prognosis were summarized. Results　A 4-year-old boy presented with chronic cough for 1 month and tachypnea, moist rales and clubbed-fingers. The lung CT showed ground-glass and reticular opacities. After treatment with antibiotics, the condition was not improved. A diagnosis of DIP was confirmed by a thoracoscopic right lung biopsy. The symptoms disappeared after the steroid therapy, and the abnormal findings of chest radiography mostly resolved after 44 months follow-up. A total of 31 cases (14 males and 13 females, gender for 4 cases were not mentioned) were selected into the study with age ranged from newborn to 13 years old. DIP in children is mostly related to an inborn error of surfactant metabolism, and can be idiopathic. Other factors such as secondhand cigarette exposure, drugs as well as systemic disease may be associated with DIP. The main symptoms included dyspnea and dry cough, the chest imaging mostly shows ground glass changes, and pulmonary function tests mainly show a restrictive defect. Main treatments included corticosteroids and immunosuppressive therapy. Conclusions　DIP is less frequent with different etiology in children than in adults. Lung biopsy should be performed as early as possible for early diagnosis. DIP is curable to steroid and/or other immunosuppressive drugs.

Key words: 　desquamative interstitial pneumonia;　risk factors;　treatment;　child