OBJECTIVEInterstitial lung disease in children represents a heterogeneous group of disorders of both known and unknown causes. This study aimed to understand better the causes of the disease in children and to provide information on the current approach to diagnosis and management of the disease.

METHODThrough the Pediatric Diffuse Parenchymal Lung Disease/Pediatric Interstitial Lung Disease Cooperative Group of China, data of 93 cases of interstitial lung disease of children from 11 hospitals were collected with the same questionnaire in 2009. Respiratory tract secretions were obtained for bacterial culture. Respiratory virus antigen examination, mycoplasma antibody, EB virus, cytomegalovirus, and herpes simplex viruses antibody detection were performed. Cells in the sputum, gastric juice and bronchoalveolar lavage fluid (BALF) were tested for hemosiderin. The CT or high resolution CT (HRCT) of the lung and blood-gas analysis were also performed. Fourteen cases underwent lung biopsy and 25 cases underwent bronchomicrocopy. Data were then pooled and discussed through a series of meetings.

RESULTFifty-three cases were male, 40 were female and their age ranged from 8 months to 14 years. Thirty-nine cases were diagnosed as bronchiolitis obliterans (BO); 39 as idiopathic pulmonary hemosiderosis (IPH); 7 as idiopathic interstitial pneumonia (IIP) of unknown causes, of whom 4 cases had non specific interstitial pneumonia, 1 case as acute interstitial pneumonia and 1 case as lymphocytic interstitial pneumonia, 1 case as idiopathic pulmonary fibrosis; 2 cases as secondary interstitial lung disease, one was secondary to SLE, one to human immunodeficiency virus (HIV) infection; 2 cases had hypersensitive pneumonitis; 2 cases had pulmonary alveolar proteinosis; 1 case had bronchiolitis obliterans organizing pneumonia; 1 case had lipoid pneumonia;1 case of diffuse panbronchiolitis; 1 case of microlithiasis alveolaris pulmonum. Forty two cases had cough, 24 of them also had tachypnea, 8 cases had clubbing. HRCT showed that 56 cases had groundglass-like opacification; 30 cases had mosaic appearance; 1 case had diffuse micronodular opacities, 1 case had diffuse reticulonodular opacities and cysts.

CONCLUSIONInterstitial lung disease in children is a heterogeneous group of disorders. In this study diagnoses of 11 diseases were made, the top third diagnoses were BO, IPH and IIP, respectively.

Adolescent ; Child ; Child, Preschool ; China ; epidemiology ; Female ; Humans ; Infant ; Lung Diseases, Interstitial ; diagnosis ; epidemiology ; etiology ; Male

BACKGROUND: This study was purposed to find out the differences in the lymphocyte subsets and differential cell counts of the bronchoalveolar lavage (BAL) fluid in patients with interstitial lung disease (ILD) and to analyze the differences according to their ages, gender and smoking habits. METHODS: BAL fluid samples of 141 ILD patients were examined for lymphocyte subsets and differential cell counts, and the differences among the patients were analyzed according to their diseases. Then, within the three most common disease groups, the differences were further analyzed by the age, gender and smoking habit of the patients. RESULTS: There were no statistically significant differences in total cell counts (per millimeters of BAL fluid) among the patient groups with each ILD. However, significant differences were observed in the percentages of neutrophils, lymphocytes, eosinophils, and macrophages of BAL fluid. Also, in lymphocyte subset analyses, the percentages of total T cells, B cells, CD4+ T cells, CD8+ T cells, CD4/CD8 T cell ratios, and NK cells were significantly different among the patients with each ILD. However, within the same disease group, there were no differences in differential cell counts and lymphocyte subset analyses according to the age, smoking habit, and gender of the patients. CONCLUSIONS: Although the age, smoking habit and gender did not have an effect on the BAL fluid analyses among the patients with the same ILD, there were significant differences among the patients with each ILD; therefore, the differential cell counts and lymphocyte subset analyses of BAL fluid can be useful in differential diagnosis for determining the types of ILD.
Adult ; Aged ; Bronchoalveolar Lavage Fluid/*cytology ; Diagnosis, Differential ; Female ; Humans ; Lung Diseases, Interstitial/diagnosis/*epidemiology/etiology ; Lymphocyte Count ; Lymphocyte Subsets/immunology ; Male ; Middle Aged ; Smoking

OBJECTIVETo explore the clinical and laboratory features and the prognosis of juvenile dermatomyositis (JDM) complicated with interstitial lung disease (ILD).

METHODData of 39 cases of JDM complicated with ILD hospitalized in Beijing Children's Hospital from January 2005 to December 2011 were collected. The clinical features, laboratory data and prognosis of these children were analyzed.

RESULTOf the 39 cases studied, 16 were boys, and 23 girls. The average age of onset was 5.6 years, and 61.5% of the patients' age of onset (24 cases) was under 6 years. Rashes (17 cases, 43.6%), simultaneous eruption of rashes and muscle weakness (14 cases, 35.9%), fever (4 cases, 10.1%), or muscle weakness (3 cases, 7.7%) were common initial symptoms of the disease. Only 51.3% of the patients (20 cases) had the symptoms of respiratory system, but (24 cases) 61.5% were complicated with that of the gastrointestinal system; (27 cases) 69.2% had at the same time electrocardiographic and echocardiographic abnormalities. The chest high resolution computed tomography (HRCT) showed cord or band-like shadows in their lungs of more than half of the cases (25 cases, 64.1%), and other changes included ground glass-like shadow (10 cases, 25.6%), net and lineation-like shadow (9 cases, 23.1%), nodular change (5 cases, 12.8%). The patients complicated with lung essential infiltration accounted for as high as 71.8% (28 cases). These imaging changes were largely seen on both dorsal sides of their lungs. Severe patients also had mediastinal emphysema, pneumothorax, pneumorrhagia or aerodermectasia. Twenty-four patients underwent pulmonary function examination, and 62.5% of the patients' pulmonary function (15 cases) was abnormal. The fatality rate of the cases studied was 10.1%.

CONCLUSIONThe imaging changes of patients suffering from JDM with ILD were often more severe as compared to the clinical symptoms, and were often complicated with damages to other systems and organs. The prognosis of those patients was poorer than others. Patients with JDM especially at a younger age of onset and with various organ damages should be examined with chest HRCT examinations as early as possible.

Child ; Child, Preschool ; Dermatomyositis ; complications ; diagnosis ; drug therapy ; Female ; Glucocorticoids ; administration & dosage ; therapeutic use ; Humans ; Immunosuppressive Agents ; administration & dosage ; therapeutic use ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; diagnosis ; drug therapy ; etiology ; Male ; Methotrexate ; administration & dosage ; therapeutic use ; Muscle Weakness ; diagnosis ; epidemiology ; etiology ; Prognosis ; Respiratory Function Tests ; Retrospective Studies ; Tomography, X-Ray Computed