Clinical and genetic characteristics of Chinese pediatric patients with chronic granulomatous disease.

BACKGROUND: Chronic granulomatous disease (CGD) is a rare disease in China, and very little large-scale studies have been conducted to date. We aimed to investigate the clinical and genetic features of CGD in Chinese pediatric patients. METHODS: Pediatric patients with CGD from Beijing Children's Hospital, Capital Medical University, China, were enrolled from January 2006 to December 2016. RESULTS: A total of 159 pediatric patients with CGD were enrolled. The median age of clinical onset was 1.4 months, and 73% (116/159) had clinical onset symptoms before the 1 year of age. The most common site of invasion was the lungs. The lymph nodes, liver, and skin were more frequently invaded in X-linked (XL) CGD patients than in autosomal recessive (AR) CGD patients (P < 0.05). Approximately 64% (92/144) of the pediatric patients suffered from abnormal response to BCG vaccination. The most frequent pathogens were Aspergillus and Mycobacterium tuberculosis. Gene analysis indicated that 132 cases (89%, 132/147) harbored CYBB pathogenic variants, 7 (5%, 7/147) carried CYBA pathogenic variants, 4 (3%, 4/147) had NCF1 pathogenic variants, and 4 (3%, 4/147) had NCF2 pathogenic variants. The overall mortality rate in this study was 43%, particularly the patients were males, with CYBB mutant and did not receive HSCT treatment. CONCLUSIONS: Chronic granulomatous disease is a rare disease affecting Chinese children; however, it is often diagnosed at a later age, and thus, the mortality rate is relatively high. The prevalence and the severity of disease in XL-CGD are higher than AR-CGD.

SLC11A1 (Formerly NRAMP1) gene polymorphisms associated with pediatric tuberculosis in China.

BACKGROUND: Tuberculosis is an urgent public health problem caused by Mycobacterium tuberculosis infection. Many factors, including host genetic factors, are involved in tuberculosis pathogenesis. The SLC11A1 gene (formerly NRAMP1) is a primary candidate for association with tuberculosis susceptibility. METHODS: We examined the association between SLC11A1 polymorphisms and susceptibility to tuberculosis in Han Chinese children. The study included 136 pediatric patients with tuberculosis and 435 pediatric control subjects. Polymerase chain reaction restriction fragment-length polymorphism analysis was performed with blood samples to distinguish allele genotypes of INT4 (a G-->C single nucleotide change in intron 4, 469 + 14G/C) and the 3'UTR (TGTG deletion in the 3' untranslated region, 1729 + 55del) in the SLC11A1 gene. Differences in genotype frequency were analyzed for patients with tuberculosis and control subjects. RESULTS: We did not identify any statistically significant differences between the tuberculosis and control groups with regard to the frequency of genotype variants G/C and C/C at the INT4 locus. The frequency of genotype variants (TGTG +/delete and TGTG delete/delete) was significantly higher in the tuberculosis group than in the control group at the 3'UTR locus. Differences in genotype distribution at the 3'UTR locus were only identified in female subjects, with a greater number of variant genotypes in the tuberculosis group. CONCLUSIONS: Variant genotypes at the 3'UTR locus in the SLC11A1 gene were associated with pediatric tuberculosis in Chinese patients. Patient sex may affect the outcome of M. tuberculosis infection in children.

[Association of NRAMP1 gene polymorphisms with the susceptibility to tuberculosis in ethnic Han Chinese children].

OBJECTIVE: Tuberculosis is still a public health problem. Host genetic factors, such as polymorphisms in NRAMP1 gene, may play a role in the development of tuberculosis. To clarify the effect of NRAMP1 gene polymorphisms on the development of childhood tuberculosis, the association of NRAMP1 gene polymorphisms with susceptibility to tuberculosis in the ethnic Han Chinese children was investigated. METHODS: From January 2005 to March 2008, 130 ethnic Han children with tuberculosis (TB group) were enrolled. Three hundred and ninety hospitalized ethnic Han children for physical examination in the surgery department were used as the control group. The controls were matched with tuberculosis children by age, sex and area. PCR-RFLP analysis was performed on DNA samples to identify allele genotypes of INT4 and D543N in NRAMP1 gene. Genotype frequency differences between tuberculosis patients and controls were analyzed using x2 test. RESULTS: No statistical difference was found in the genotype frequency of variants G/C and C/C at the INT4 locus between the TB and the control groups. At the D543N locus, the frequency of genotype variants (G/A and A/A) was significantly higher in the TB group (34/130) than that in the control group (66/390) (x2=5.349, P<0.05; OR=1.74, 95%CI=1.08-2.79). When stratified by sex, differences in the genotype distribution were observed only in females at the D543N locus, which the variant genotypes were higher in the TB group (16/52) than in the control group (21/155) (x2=7.866, P<0.05; OR=2.84, 95%CI=1.34-5.99). For males, there was no difference between the TB and the control groups. At the INT4 locus, no difference was observed between the two groups in boys and girls. CONCLUSIONS: Genotypic variation at the D543N locus in NRAMP1 gene may be associated with susceptibility to tuberculosis in ethnic Han Chinese children. Variant genotypes in NRAMP1 gene (G/A and A/A) may be susceptible genotypes to tuberculosis in ethnic Han Chinese children. Girls with variant genotypes were more susceptible to tuberculosis.

[Clinical features of 17 cases of X-linked agammaglobulinemia].

OBJECTIVE: To investigate clinical features of X-linked agammaglobulinemia (XLA) in children. METHODS: The medical records of 17 children with XLA between January 2001 and April 2007 were reviewed. RESULTS: The age at first diagnosis in 88.2% of patients was more than 6 years, with a mean of 7.7 years. Twelve patients (70.6%) presented first symptoms over 2 years old, with a mean of 4.2 years. Respiratory infections as first symptoms and complaints occurred in 64.7% of the patients and 35.3% of the patients presented with polyarthritis. Skin and soft tissue infections were rarely seen in less than 1 year old group children. Abrupt sepsis and abscess in deep tissues were seen in the older children. CD4+ T cells decreased and CD8+ T cells increased in 9 patients and an inversed ratio of CD4+/ CD8+ was observed in 11 patients. CONCLUSIONS: Both the age presenting first symptoms and the age at first diagnosis in children with XLA in this study were later than the reported data. Respiratory infection was the most common manifestation. High prevalence of polyarthritis was observed. Abnormal T cell phenotypes occurred in more than one half of patients.

Efficacy and safety of a live attenuated, cold-adapted influenza vaccine, trivalent against culture-confirmed influenza in young children in Asia.

BACKGROUND: This study was designed to evaluate the efficacy and safety of cold-adapted influenza vaccine, trivalent (CAIV-T) against culture-confirmed influenza in children 12 to <36 months of age during 2 consecutive influenza seasons at multiple sites in Asia. METHODS: In year 1, 3174 children 12 to <36 months of age were randomized to receive 2 doses of CAIV-T (n = 1900) or placebo (n = 1274) intranasally > or =28 days apart. In year 2, 2947 subjects were rerandomized to receive 1 dose of CAIV-T or placebo. RESULTS: Mean age at enrollment was 23.5 +/- 7.4 months. In year 1, efficacy of CAIV-T compared with placebo was 72.9% [95% confidence interval (CI): 62.8-80.5%] against antigenically similar influenza subtypes, and 70.1% (95% CI: 60.9-77.3%) against any strain. In year 2, revaccination with CAIV-T demonstrated significant efficacy against antigenically similar (84.3%; 95% CI: 70.1-92.4%) and any (64.2%; 95% CI: 44.2-77.3%) influenza strains. In year 1, fever, runny nose/nasal congestion, decreased activity and appetite, and use of fever medication were more frequent with CAIV-T after dose 1. Runny nose/nasal congestion after dose 2 (year 1) and dose 3 (year 2) and use of fever medication after dose 3 (year 2) were the only other events reported significantly more frequently in CAIV-T recipients. CONCLUSIONS: CAIV-T was well tolerated and effective in preventing culture-confirmed influenza illness over multiple and complex influenza seasons in young children in Asia.

Consecutive allergen challenges resulted in decreased bronchial responsiveness.

OBJECTIVE: To investigate bronchial responsiveness to acetylcholine in allergic airway inflammation of SD rats. METHODS: SD rats were immunized and challenged by chicken ovalbumin (OVA). Airway responsiveness, acetylcholine (Ach) provocation concentration needed to increase baseline airway resistance by 200% (PC(200)) were measured. RESULTS: The value of baseline airway resistance in asthma group was significantly higher than that in control group (2.282 +/- 0.128 vs 3.193 +/- 0.239; P < 0.01). After multiple ovalbumin exposures, airway responsiveness to intravenous injection of acetylcholine decreased significantly (-LogPC(200): 4.006 +/- 0.554 vs 2.059 +/- 0.262; P < 0.01). Bronchial alveolar lavage fluid (BALF) and lung tissue specimen analysis indicated that airway allergic inflammation was present. CONCLUSIONS: The study demonstrates a dissociation between the bronchoconstrictor response and bronchial hyper-responsiveness and indicates that multiple ovalbumin exposures induces persistent bronchoconstriction with airway hypo-responsiveness despite airway allergic inflammation.

[Clinical analysis of a Chinese child with leukocyte adhesion deficiency type 1].

OBJECTIVE: Leukocyte adhesion deficiency type 1 (LAD-I) is rare. We present 1 case of LAD-I patient diagnosed by gene analysis. His clinical manifestations and genetic mutation features are analyzed in this article. METHOD: The clinical material of the LAD-I patient who was diagnosed by gene analysis was retrospectively analyzed. RESULT: The patient was a 2-month-old boy. He had a complaint of recurrent fever and cough for 30 days. Pulmonary CT indicated a small to moderate quantity pleural effusion on the right side. His peripheral blood leukocyte and C-reactive protein (CRP) was always significantly higher than normal. After hospitalization he had diarrheal diseases, routine stool test showed 2 RBC cells/high power (HP), WBC 30 cells/HP, stool cultures were negative, digestive tract ultrasonography showed an array of defects, in the sigmoid colon and rectal mucosa suggestive of ulcerative colitis. He was treated with cefoperazone and sulbactam and vancomycin. He had a history of impetigo in his neonatal period and without delayed umbilical cord exfoliation. His family history was normal. ITGB2 genetic mutation analysis revealed a homozygous mutation (1062A > T). His parents did not participate in this study. He had no fever but had diarrheal disease after 1 month of follow up. CONCLUSION: This patient had suffered from impetigo, pleural effusion, diarrheal diseases, markedly increased peripheral white blood cell and ITGB2 genetic mutation analysis showed that homozygous mutation (1062A > T). He received a diagnosis of LAD-I.

[Clinical manifestations and gene analysis of 2 Chinese children with cystic fibrosis].

OBJECTIVE: Cystic fibrosis (CF) is rare in Chinese mainland. We present two cases of CF patients diagnosed by gene analysis. Their clinical manifestations and genetic mutation features are analyzed in this article. It will be of special interest to pediatricians in recognition of CF. METHOD: The clinical material of two CF patients who were diagnosed by gene analysis was retrospectively analyzed. RESULT: The first patient is a 13-year-old girl. She had a complaint of recurrent fever and cough for 6 months, expectoration for 2 months and hemoptysis for 20 days. After 3 months of her birth, she was operated on for bullae of lung. She was susceptible to upper respiratory tract infection. There was no family history of recurrent wheeze and other special diseases. Aspergillus fumigatus specific IgE was at grade 3 and aspergillus fumigatus IgG was high. Pseudomonas aeruginosa was positive in sputum culture. Sweat testing was performed and Na+ was higher. Pulmonary CT indicated bronchiectasis. Nasal sinus CT showed optical density of soft tissue within maxillary sinus and chronic bilateral sinusitis. The electron microscopy of cilia suggested immobile cilia syndrome. A heterozygotic mutation (263T > G, 2909G > A) was found after CFTR genetic mutation analysis. Both her parents were carriers. She was treated with inhalation of nebulized hypertonic saline and postural drainage for a long time. And she got better during a follow up period of 1 year. The second patient was a 10-year-old girl who complained of recurrent expectoration for 3 years and shortness of breath for half a year. She had a history of sinusitis and steatorrhea. The family history was normal. Both the lipase and insulin level in blood serum was lower.Pseudomonas aerugino and Aspergillus fumigatus were both positive in sputum culture. Aspergillus fumigatus IgE was normal. Pulmonary CT indicated bronchiolitis and bronchiectasis. Nasal sinus CT showed bilateral maxillary sinusitis. CFTR genetic mutation analysis revealed a homozygous mutation (3196C > T). Her parents and relatives did not participate in this study. Unfortunately, this child died of respiratory failure 3 months after discharge. CONCLUSION: CFTR gene mutation was a main cause of CF. Common symptoms are those of bronchiectasis, pancreatitis and sinusitis. The two Chinese patients were diagnosed by gene analysis. One had a heterozygous mutation (263T > G, 2909G > A) and the other had a homozygous mutation (3196C > T), not ΔF508 which is common in western countries.

[Clinical characteristics and predictive factors of refractory Mycoplasma pneumoniae pneumonia].

OBJECTIVE: To investigate clinical characteristics and predictive factors of refractory Mycoplasma pneumoniae pneumonia (RMPP) in children so as to recognize and treat the disease earlier. METHOD: The data including febrile time, inflammatory markers (WBC, neutrophil, CRP) and radiological features of 213 children hospitalized with Mycoplasma pneumoniae pneumonia (MPP) (72 with refractory MPP and 141 with mild MPP were retrospectively analyzed). The primary diagnostic criteria of refractory MPP: the patient's condition still deteriorates after treatment with macrolides for more than 5 days. The independent variables which had significant difference in univariate analysis was analyzed by multivariate logistic regression analysis. The predictive criteria of RMPP were further applied in 100 other patients prospectively. Kappa test was used to evaluate the accuracy rate. RESULT: Refractory MPP patients: febrile time was more than 10 days, white blood cell (WBC) count was (3.8 - 18.5)×10(9)/L in peripheral blood routine test, CRP was 38 mg/L - > 160 mg/L, large lobar consolidation with high density (> 2/3 pulmonary lobe, CT value 40 - 50 HU, without air bronchogram). Mild MPP patients: febrile time was less than 10 days, CRP was often less than 40 mg/L. Independent risk factors for RMPP were febrile time, CRP, large consolidation area with high density in lungs with or without pleural effusion (OR = 1.586, P = 0.017; OR = 4.344, P = 0.001; OR = 2.660, P = 0.012), CT value 40 - 50 HU which were demonstrated by logistic regression analysis. The specificity, sensitivity and Youden index for this diagnostic test were respectively 0.96, 0.94 and 0.90 at a CRP cut off of 40 mg/L. The sensitivity, specificity, and Kappa value for the above criteria to diagnose RMPP were respectively 0.96, 0.94 and 0.9. CONCLUSION: The predictive factors for RMPP are febrile time (> 10 days), CRP (> 40 mg/L), large lobar consolidation with high density (> 2/3 pulmonary lobe, CT value > 40 HU with or without pleural effusion) for the purpose of treating earlier.

[Clinical analysis of 20 cases with Streptococcus pneumoniae necrotizing pneumonia in China].

OBJECTIVE: Streptococcus pneumoniae necrotizing pneumonia (SPNP) was reported elsewhere but not in China yet. Inappropriate treatment due to poor recognition of this disease could influence its prognosis. This paper presents the clinical characteristics, diagnosis and treatment of SPNP hoping to elevate pediatrician's recognition level for this disease. METHOD: Clinical manifestations, radiological findings, treatment and prognosis of 20 patients (9 boys, 11 girls) who had been hospitalized with SPNP in Beijing Children's Hospital from 2004-2011 were retrospectively analyzed. RESULT: The patients included in this study aged from 9 months to 6 years [(27.9 ± 15.8) m] and were healthy before admission. They were febrile for 8 to 50 days [(27.7 ± 13.5) d] and hospital day was 24 - 55 days [(36.5 ± 8.3) d]. The general condition of all subjects was relatively poor and they all had fever and cough. One child had moderate fever and nineteen children had high fever. Dyspnea was found in sixteen children. Fine rales were found on auscultation in 18 children, among whom diffuse wheeze appeared in 4 children, and wheezy phlegm was found in two children. Signs of pleural effusion were discovered in all cases by physical examination and chest X-ray. White blood cell (WBC) count was 16.2 - 60.95×10(9)/L and neutrophil was 70.5% - 80.2% in peripheral blood routine test. Erythrocyte sedimentation rate (ESR) was 44 - 109 mm/h [(69.6 ± 16) mm/h]and C-reactive protein (CRP) was 80 - > 160 mg/L. The pleural effusion biochemistry and routine test revealed a WBC count of 6400×10(6)/L-too much to count, polykaryocyte of 51% - 90%, glucose of 0.02 - 1.8 mmol/L, protein of 32 - 51 g/L and LDH of 5475 IU/L-or higher. Pleural effusion culture in all cases and blood culture in 2 cases was positive for Streptococcus pneumoniae. Chest X-ray or CT revealed high density and well-distributed lobar consolidation in one lung or two lungs initially. Single or multiple low density lesions in the area of lobar consolidation were found a week later, accompanied by multiple cystic shadow or cavity at the same time or afterwards. Bulla of lung appeared later. Pleural effusions were found in all patients. Seven cases complicated with hydropneumothorax, two with otitis media, one with heart failure, one with cardiac insufficiency. Seventeen patients were treated with vancomycin or teicoplanin or linezolid two with amoxicillin and clavulanate potassium. Other two patients had been treated with meropenem and cephalosporin antibiotics respectively before admission, and they had been at recovery stage when they were hospitalized. Thoracic close drainage and thoracoscopy were performed respectively in 18 cases and 3 cases, respectively. After a follow up of more than 6 months, chest CT showed that almost all lesions in lungs recovered during 4-6 months. No one received pneumonectomy. CONCLUSION: SPNP has special manifestations. The incidence in infants is higher. Patients' general condition is poor and febrile course is relatively long. All patients manifested fever and cough, with a presence of dyspnea in most of them. WBC, neutrophil and CRP elevated apparently. The characteristic of pleural effusion indicates empyema. In early stage, the chest X-ray and CT showed high-density lobar lesions, followed by low-density lesions and cyst gradually. Bulla of lung and/or hydropneumothorax may appear at the late stage. But if diagnosed and treated promptly, the prognosis of SPNP was relatively good.

[Clinical features of 3 cases with acute interstitial pneumonia in children].

OBJECTIVE: Acute interstitial pneumonia (AIP) is a rare lung interstitial disease in children. This study was conducted to understand the clinical features of the AIP in children. METHOD: The data of the three cases with AIP admitted to our hospital from March 2008 to November 2009 were reviewed. Of the 3 cases, 2 were male, one was female. Their age ranged from 1 year and 4 months to 10 years. The clinical manifestation, pulmonary function test and the high resolution computed tomography (HRCT) and pathology of the lung were studied retrospectively. Tissue specimens of the lung were obtained by video-assisted thoracoscopic biopsy. Viral etiologic examinations for the respiratory syncytial virus, adenovirus, influenza virus, parainfluenza viruses, EB virus, cytomegalovirus, enterovirus and herpes simplex virus were performed. The IgM antibody to Mycoplasma pneumoniae in the serum was also detected. RESULT: All the 3 cases rapidly developed respiratory failure of unknown origin, none of these cases had failure of any other organs. All three cases had cough and dyspnea. No case had the rales and digital clubbing. The examinations for viruses, bacteria and Mycoplasma pneumoniae infection were all negative. No evidence for the diagnosis of connective tissue disease was obtained. The HRCT of the chest showed diffuse alveolar consolidation, air bronchogram and ground glass appearance in the bilateral lungs, and the traction-associated bronchiectasis in areas. All the three cases had the histological proof of diffuse alveolar damage by the biopsy. All the three cases were treated with CPAP and corticosteroid. Two cases were treated with corticosteroid in early stage of the disease, the condition of these cases were improved obviously. The third case was treated with high-dose steroid pulse therapy days, the condition of this case was improved slightly in a month. One year follow-up showed that case 1 and case 2 had no hypoxemia and the HRCT of the chest showed obvious improvement. The pulmonary function of case 2 had restrictive deficiency. CONCLUSION: AIP has a rapidly progressive clinical course leading to respiratory failure. The HRCT of the chest showed alveolar consolidation and ground glass-like change. The pathology of the lung includes diffuse alveolar damage. The prognosis of the AIP in children may be improved by the treatment with respiratory assistance and corticosteroids.

[Clinical features and molecular analysis of 2 Chinese children with autosomal recessive chronic granulomatous disease caused by CYBA mutations].

OBJECTIVE: To summarize clinical and molecular features of two children with autosomal recessive chronic granulomatous disease caused by CYBA mutations. METHOD: The clinical records and CYBA mutations were reviewed for analysis of infections and inflammatory complications. RESULT: The first case was a girl diagnosed with "liver and spleen abscess" in our hospital when she was 2.9 years old, with past history of neonatal impetigo and recurrent purulent lymphadenitis and positive family history. The results of DHR123 flow-cytometry showed that positive phagocytes after phorbol ester (PMA) stimulation was 84.63%. CYBA mutation analysis showed that she had heterozygous 35C > T, Q3X and IVS-2A > G. The second case was a boy diagnosed with "sepsis (salmonella D)" when he was 4 years old with a past history of impetigo, sepsis, perianal abscess, skin infection and positive family history. The results of flow cytometry showed that positive phagocytes after PMA stimulation was 96.13%. CYBA mutation analysis showed that he had homozygous 35C > T, Q3X and his parents were all carriers. All of them had BCG related axillary lymphnode calcification. CONCLUSION: A22CGD cases had recurrent purulent infections (skin, lymphnode, liver and spleen, lung, blood), DHR123 flow cytometric analysis helped the diagnosis of CGD, CYBA mutation analysis ascertained the diagnosis of A22CGD.

[Clinical analysis of 18 children with disseminated Bacille Calmette-Guérin infection].

OBJECTIVE: To explore the clinical manifestation, immune abnormality and outcome of disseminated Bacille Calmette-Guérin (BCG) infection in children. METHOD: The clinical data of 18 children with disseminated BCG infection seen from January 2000 to December 2007 were analyzed retrospectively. RESULT: Thirteen of the children were male among 18 patients. Disseminated infection first appeared in armpit lymph nodes ipsilateral to the vaccination site, then spread to lungs in 15, lymphnodes of mediastinum or abdominal cavity in 18, skin and soft tissues in 5, skeletons in 4, liver in 4, spleen in 8, kidney, adrenal gland or meninges in 3. Twelve children were diagnosed to have primary immunodeficiency; 3 had severe combined immunodeficiency (SCID); 7 had chronic granulomatous disease (CGD), 2 had IL-12/IFN-gamma passageway deficiency. Eleven of the 18 patients died, and the remaining 7 patients were followed up from 1 to 9 years and are alive at present, but presented recurrent skin and bone tuberculosis in 4 and recurrent other infection in 3. CONCLUSION: Most Children with disseminated BCG infection had primary immunodeficiency. CGD and IL-12/IFN-gamma passageway deficiency accounted for considerable proportion, so special immune function should be detected in these patients. The prognosis was poor. The type of the immunodeficiency diseases should be identified in early stage and the specific immune treatment should be given to the patients.

[Idiopathic interstitial pneumonias in 7 children].

OBJECTIVE: Idiopathic interstitial pneumonias (IIPs) have been increasing in children in recent years. The type and prognosis of IIPs in children in China has not been clear. The purpose of this study was to investigate the type and prognosis of IIPs in children. METHOD: Seven children diagnosed with IIPs from December 2003 to March 2007 were retrospectively studied. The clinical, radiologic, and pathologic features, type and prognosis of IIPs, were reviewed. RESULT: Of the seven patients with IIPs, three were diagnosed with cryptogenic organizing pneumonia (COP)/bronchiolitis obliterans organizing pneumonia (BOOP), one was diagnosed with usual interstitial pneumonia (UIP) and the remaining three were diagnosed with nonspecific interstitial pneumonia (NSIP). The chief complaints of these patients were chronic cough. Six patients had shortness of breath. Clubbed fingers were found in one patient with UIP and two patients with NSIP. The characteristic computed tomographic findings of COP/BOOP were multiple patchy peripheral consolidation, associated with centrilobular nodules and bronchiole wall thickening in 2 cases and bronchiolectasis in 1 case. In NSIP, diffuse or basal ground-glass opacities predominated over reticular opacities with traction bronchiectasis only in one case. UIP was manifested by patchy infiltrate with predominant honeycombing, traction bronchiectasis and interlobular septal thickening. Two patients with COP/BOOP had plugs of connective tissue within a bronchiole and the adjacent alveolar ducts and alveolar spaces. Lung biopsies showed predominantly interstitial chronic inflammation with infiltrate of lymphocytes and some plasma cells and alveolar septum thickening in three patients with NSIP. One case with NSIP showed a mixed fibrosing and prominent interstitial chronic inflammation. The key histological findings of the UIP was a heterogeneous appearance, with alternating areas of relatively normal lung, interstitial inflammation and fibrosis. All patients were treated with prednisone. The abnormal findings of chest radiography resolved completely after four to twelve months' therapy in three patients with COP/BOOP. The combination of prednisone and MTX or 6-MP was given to the two patients with NSIP. The symptoms and chest radiographic abnormality disappeared except the presence of a few cyst after treatment. One case with NSIP and the case with UIP had not been improved remarkably. CONCLUSION: COP/BOOP, NSIP and UIP might occur in children in China. The typing of IIPs was based on clinical-radiologic-pathologic features. Steroid was the first choice for IIPs. The prognosis of IIPs varied among different types.