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Despite evidence for the role of genetic factors in stroke, only a small proportion of strokes have been clearly attributed to monogenic factors, due to phenotypic heterogeneity. The goal of this study was to determine whether a significant relationship exists between human galectin-7 geneThis two-stage genetic association study included an initial exploratory stage followed by a discovery stage. During the exploratory stage, transgenic galectin-7 mice or transgenic mice with the scrambled sequence of the hairpin structure -silenced down geneDuring the exploratory phase,A and T alleles of two novel SNP loci of 19q13.2, rs567785577 and rs138945880, respectively, were evaluated for associations with susceptibility to ICH. Further studies with expanded case numbers that include subjects of other ethnic populations are needed to elucidate mechanisms underlying associations between these SNPs and ICH risk.

In this study, we report on three immunocompetent children with pulmonary cryptococcosis presenting mediastinal lymphadenopathy as the prominent manifestation. All three children were otherwise healthy previously. Two children had a history of exposure to pigeons and poultry. All three presented persistent fever accompanied by mild cough. There were no obvious positive signs in the lungs. One patient had enlarged cervical lymph nodes. All three had elevated levels of white blood cells, neutrophil count, and C-reactive protein (CRP). The levels of IgG, IgM, IgA, IgE and T cell subsets were normal in all cases, and they were all tested negative for HIV antibody. Two children were tested positive for serum cryptococcal antigen (sCRAG). The chest X-ray and pulmonary CT findings of the three patients all demonstrated marked enlargement of mediastinal lymph nodes, and one patient had nodules in the parenchyma. Surgical biopsies of mediastinal lymph nodes were performed in two children and large numbers of capsule spores were found in the histological examination. In the three cases, definitive diagnosis of pulmonary cryptococcosis were made in two patients, and clinical diagnosis was made in the third patient. Two patients were treated with fluconazole alone. The other patient whose condition was complicated with spleen infection was treated with fluconazole combined with amphotericin B for the first month, and was then given fluconazole for maintenance treatment. The overall treatment course lasted 5-9 months and all three were cured eventually. In conclusion, immunocompetent children with pulmonary cryptococcosis may present mediastinal lymphadenopathy as a prominent or isolated manifestation, which should be considered in differential diagnosis. Treatment with fluconazole alone or in combination with amphotericin B when it was necessary showed good therapeutic outcomes.

OBJECTIVE: To study the efficacy of Huaiqihuang granules as adjuvant therapy for bronchial asthma in children. METHODS: A multicenter, prospective, and registered real-world study was performed for the children, aged 2-5 years, who had a confirmed diagnosis of bronchial asthma in the outpatient service of 21 hospitals in China. Among these children, the children treated with medications for long-term asthma control (inhaled corticosteroid and/or leukotriene receptor antagonist) without Huaiqihuang granules were enrolled as the control treatment group, and those treated with medications for long-term asthma control combined with Huaiqihuang granules were enrolled as the combined treatment group. The medical data of all children were collected. Outpatient or telephone follow-up was performed at weeks 4, 8, 12, 20, 28, and 36 after treatment, including asthma attacks and rhinitis symptoms. A statistical analysis was performed for the changes in these indices. RESULTS: There was no significant difference in the frequency of asthma attacks or rhinitis attacks between the two groups before treatment (P>0.05). After treatment, the combined treatment group had significantly lower frequencies of asthma attacks, severe asthma attacks, and rhinitis attacks compared with the control treatment group (P

Background Disseminated cryptococcosis is a rare and fatal disease, and limited data exist regarding it in children. This study aimed to investigate the clinical characteristics of disseminated cryptococcosis in previously healthy children in China. Methods Hospitalized patients with disseminated cryptococcosis were enrolled during January 1996 to December 2015 in Beijing Children’s Hospital, Capital Medical University, China. Data on clinical manifestations, laboratory tests, treatment, and prognosis were evaluated. Results A total of 52 pediatric patients with no underlying disease were enrolled, including 38 boys and 14 girls. Only 10 cases had a history of exposure to pigeon droppings. Fever, cough, and hepatomegaly were 3 main manifestations of disseminated cryptococcosis. However, headache was more common in patients with central nervous system (CNS) invasion than in patients with non-CNS invasion (P

To analyze clinical manifestations, treatment and prognosis of 4 cases with endemic typhus.The clinical data of four endemic typhus patients in prognosis were retrospectively analyzed. These four atypical cases of endemic typhus with pneumonia were treated in our department from October 2011 to March 2012. They were all male, with an age range of 15 months to 7 years. The four patients had long history, mild respiratory symptom and no improvement was found after treatment with cephalosporins. There were no evidences of bacterial, viral, or fungal infections and we thought they might have infection with other pathogen. Three were from rural areas. Routine blood tests, Weil-Felix reaction, blood smear (Giemsa staining) , and indirect immunofluorescence assay were performed.Blood smear and IFA tests showed evidences for endemic typhus. The clinical presentations were atypical, the patients had no headache, but all had fever, rash, and pneumonia of varying severity. None of the patients had a severe cough, but bronchial casts were observed in one case. Recurrent fever was reported in three cases. Physical examinations showed no eschars, but one patient had a subconjunctival hemorrhage, and one had skin scratches, cervical lymphadenopathy, pleural effusion, pericardial effusion, and cardiac dilatation. Two patients had remarkably increased peripheral blood leukocyte counts; both these patients also had high alanine aminotransferase (ALT) levels and one had a high C-reactive protein (CRP) level. Weil-Felix testing was negative or the OX19 titer was low. The peripheral blood smear (Giemsa stain) showed intracellular pathogens in all four cases. After combined therapy with doxycycline and macrolide antibiotics, all four patients recovered well.The endemic typhus children often come from rural areas. The clinical presentations were atypical, they usually have no headache, but have fever (often Periodic fever) , rash, and pneumonia of varying severity in these four cases. Combined therapy with doxycycline and macrolide antibiotics was effective in all four patients.

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.The clinical material of the LAD-I patient who was diagnosed by gene analysis was retrospectively analyzed.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 (1062AT). His parents did not participate in this study. He had no fever but had diarrheal disease after 1 month of follow up.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 (1062AT). He received a diagnosis of LAD-I.

To determine risk factors of invasive fungal infections (IFI) in patients admitted to non-hematological oncology department and pediatric intensive care unit (PICU), in order to improve diagnostic level of invasive fungal infections.We retrospectively assessed 85 hospitalized pediatric patients with invasive fungal infections in Beijing Children's Hospital Affiliated to Capital Medical University from Jan.2007 to Nov.2012. All the cases were either from non-hematological oncology department or the PICU.We reviewed risk factors of invasive fungal infections.Among 85 patients, 42 had invasive candida infection, 20 invasive aspergillus infection, 21 cryptococcus infection, 1 Histoplasma capsulatum infection and 1 Mucor mucedo infection.In the 42 patients with invasive candida infection, 5 were young infants, 3 had combined immunodeficiency, 1 cellular immunodeficiency, 25 secondary infection due to long term use of corticosteroids and/or combined use of more than 2 kinds of antibiotics with primary disease, 5 prior intestinal tract surgery or chronic diarrheal disease, 1 reflux gastritis.In the 20 patients with invasive aspergillosis infection, 10 patients had chronic granulomatous disease, 5 long term use of corticosteroids ≥ 1 month, 3 long term use of corticosteroids and combined use of more than 2 kinds of antibiotics, 2 had no apparent host factors.In the 21 patients with cryptococcus infection, 2 patients had used corticosteroids ≥ 1 month, 2 had immunodeficiency mainly for lack of antibodies, while others had no apparent host factors. The child with Mucor mucedo infection had diabetes mellitus. And the one with Histoplasma capsulatum infection had immunodeficiency.High risk factors for IFI in patients admitted to non-hematological oncology department and PICU are primary immunodeficiency disease and long term use of corticosteroids and/or long term combined use of more than 2 kinds of antibiotics. Besides, young infant is also a high risk factor for invasive candida infection. Most of the cryptococcus infections and certain aspergillosis had no obvious host factors.

To investigate clinical characteristics and predictive factors of refractory Mycoplasma pneumoniae pneumonia (RMPP) in children so as to recognize and treat the disease earlier.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.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.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 value40 HU with or without pleural effusion) for the purpose of treating earlier.

Most of Endobronchial tuberculosis (EBTB) of children is caused by the bronchial erosion from the scrofula close to the bronchus. Due to its complicated mechanism, pediatricians have limited knowledge on the endobronchial tuberculosis, which makes the misdiagnosis rate high. This work explored the clinical features and diagnostic methods of endobronchial tuberculosis (EBTB), to improve the understanding of this disease.A total of 102 cases with endobronchial tuberculosis (EBTB) were investigated by analyzing clinical and bronchoscopic features, imaging manifestation, PPD results and etiological examination; 71 cases were male, 33 were female, the youngest was 4 months old, and the oldest was 10 years old. The patients were treated in the fourth ward of the Department of Internal Medicine, Beijing Children's Hospital, Capital Medical University.Of the 102 cases, 71 (69.6 percent) were infants and young children. (1) Age and clinical presentation: Infants and young children were more common, the main clinical manifestations were fever and cough, physical examination may find decreased breath sounds, wet and dry rale and wheezing. (2) In 81 cases who received CT there was bronchial obstruction, in 43 cases there were simultaneous manifestations of primary pulmonary tuberculosis (mediastinal and hilar lymph node enlargement and/or pulmonary consolidation), 21 cases had only manifestations of primary tuberculo. (3) The results of PPD: the positive rate was 95%. (4) Bronchoscopic feature: presence of mucosal swelling, caseous necrosis, bronchial stenosis/extrabronchial compression, granulomatous lesions and lymph node bronchial fistula. (5) Bacteriologic results: 21 patients were bacteriologically positive.Bronchial tuberculosis was more common in infants and young children, their major clinical manifestations were fever, cough, common findings on lung imaging was mediastinal and hilar lymph node enlargement and/or pulmonary consolidation, bronchial obstruction should be considered manifestations of bronchial tuberculosis, positive PPD should support the clinical diagnosis. Diagnosis can be confirmed by positive bacteriological tests.

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.The clinical material of two CF patients who were diagnosed by gene analysis was retrospectively analyzed.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 (263TG, 2909GA) 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 (3196CT). Her parents and relatives did not participate in this study. Unfortunately, this child died of respiratory failure 3 months after discharge.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 (263TG, 2909GA) and the other had a homozygous mutation (3196CT), not ΔF508 which is common in western countries.

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.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.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.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.

To summarize clinical and molecular features of two children with autosomal recessive chronic granulomatous disease caused by CYBA mutations.The clinical records and CYBA mutations were reviewed for analysis of infections and inflammatory complications.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 35CT, Q3X and IVS-2AG. 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 35CT, Q3X and his parents were all carriers. All of them had BCG related axillary lymphnode calcification.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.

The pediatric interstitial lung disease is a group of poorly understood disease entities. This study aimed to better understand the clinical features, radiological manifestations and pathological patterns of pediatric interstitial lung disease.Patients with diffuse lung disease seen in the year 2009 in 7 hospitals were studied by the Pediatric Interstitial Lung Disease Cooperative Group. Nineteen patients underwent lung biopsy, 11 cases were male, 8 were female and their age ranged from 1 year and 4 months to 13 years. 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. The CT or HRCT of the lung and blood-gas analysis and lung biopsy were performed for all the patients. One case underwent open lung biopsy, two cases received percutaneous biopsy, and other 16 cases were experienced video-assisted thoracoscopic biopsy.Five cases had been excluded, for one case had fungal infection, one had abnormal pneumoangiogram, one had sclerosing hemangioma, and two had no sufficient data. The remaining 14 cases were included into the analysis. All the 14 cases had cough, 12 of them also had tachypnoea, four cases had rales and five had clubbing. High resolution CT showed that 12 cases had ground-glass opacification, 1 had diffuse micronodular opacities, the pathological pattern of this case was pulmonary alveolar microlithiasis, and in the case of diffuse reticulonodular opacities and cysts, the pathology of the lung was NSIP. All the 14 cases had the proof of the diagnosis or the type of the pathology. Four cases were diagnosed by pathology of the lung, including 1 case of pulmonary alveolar microlithiasis, 2 cases of pulmonary alveolar proteinosis, 1 case of lipoid pneumonia. Clinical-radiologic-pathologic (C-R-P) diagnosis of the other 10 cases were as follows: 4 cases had secondary interstitial lung disease, one secondary to LES, one to human immunodeficiency virus (HIV) infection and 2 had hypersensitivity pneumonitis. The other 6 cases had idiopathic interstitial pneumonia with unknown causes, of whom 4 cases had non-specific interstitial pneumonia, 1 case had acute interstitial pneumonia and 1 case had lymphocytic interstitial pneumonia.Pediatric interstitial lung disease is a heterogeneous group of disorders. The clinical manifestation of these disorders was not specific, and the main symptoms were cough and tachypnoea. The ground-glass opacification was the main change in the HRCT of the lung. The different interstitial lung diseases have different pathological features. The C-R-P diagnostic analysis may enhance the rate of specific diagnosis and the awareness level of the pediatrician about the interstitial lung disease in childhood.

Bronchitis obliterans is a severe and extremely rare complication of respiratory tract infections in children and is characterized by massive atelectasis and collapse of the affected lung.The clinical manifestations, characteristic imaging of two cases with bronchitis obliterans were summarized.These two cases complained of cough and episodes of wheezing with exercise. Chest auscultation revealed bronchial breath sounds on the right side. High-resolution computed tomography (HRCT) scan showed atelectasis of the right lung and dilated central airways. Mycoplasma complement fixation antibody (CF-Ab) titer was 1:320. The diagnosis of bronchitis obliterans was made according to the current diagnostic criteria. They were treated with low-dose erythromycin [5 mg/(kg × d)], ultrasonically nebulized Pulmicort inhalation, and regular bronchoscopic lavage. Follow-up of the two cases showed that case one had a partial lung re-expansion after six months, but case two had no significant improvement.In the process of diagnosis of atelectasis, bronchitis obliterans should be noticed. A conservative treatment is effective in certain cases, but pneumonectomy or lobectomy should only be considered as the last option.

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.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.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.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.

To explore the clinical manifestation, immune abnormality and outcome of disseminated Bacille Calmette-Guérin (BCG) infection in children.The clinical data of 18 children with disseminated BCG infection seen from January 2000 to December 2007 were analyzed retrospectively.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.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.

To improve the recognition and diagnosis of abdominal tuberculosis (TB) in children.The data from 30 cases with abdominal TB hospitalized in Beijing Children's Hospital were analyzed retrospectively. The clinical significance of various tests was discussed respectively.Twenty of the 30 cases were misdiagnosed as having upper respiratory tract infection, gastroenteritis, indigestion, and only 6 cases were diagnosed as pulmonary tuberculosis while the diagnosis of abdominal TB was made in just 4 cases at initial consultation. Twenty-one cases (70%) experienced the symptoms of abdominal pain or distension, diarrhoea, or constipation. The positive abdominal signs existed in all children including doughy sensation (9 cases), tenderness (8 cases), mass (4 cases), and hepatosplenomegaly (3 cases). Extraabdominal TB was found in 24 children, the positive PPD and abnormal ultrasonic image were seen in 93% and 100% of the cases, respectively. The exploratory laparotomy and colonoscopic biopsy confirmed the diagnosis in 3 and 2 cases, respectively. Twenty-four children received systematic treatment with good results.The abdominal TB in children which is easily misdiagnosed in it's early stage usually consisted of gastrointestinal symptoms and signs. The TB beyond the abdomen can be a clue for making correct diagnosis. PPD and ventral ultrasonic examination are important for ultimate clinical diagnosis while colonoscopy, and laparotomy can provide pathological evidence.

Rubidium (Rb) concentration in different samples collected from Keshan disease (KD) area of Sichuan Province has been determined with PIXE and NAA (Neutron Activity Analysis), and compared with that in three non-KD areas. About 200 erythrocyte samples of healthy children aged from 6 to 11 were collected from four different areas with various selenium status. 20 rice samples and 6 soil samples were taken from two of above four areas. The results showed that Rb content of erythrocyte of children in KD area (Dechang county) was three times that in the other three non-KD areas. The Rb contents of rice and soil in KD area were higher than that in non-KD areas, too. The abnormal high Rb concentration in erythrocyte of children depended on the Rb intake and the Rb level of the living environment.

To introduce the early clinical features and the main points of diagnosis and treatment of children with pancreatic pleural fistula presenting massive bloody pleural effusion.The clinical data of symptoms, signs, laboratory examination, diagnosis and treatment methods of pancreatic pleural fistula in a child are presented.A 4-year-old boy presented with one month history of intermittent fever, cough, chest tightness and chest pain. His pleural effusion collected from three thoracic closed drainage was hemorrhagic. He had no positive abdominal signs. His abdominal B-mode ultrasonography showed rough pancreatic echo. The levels of amylase in blood and pleural fluid were 495 U/L and 35 938 U/L, respectively. There was a fistulous tract from the thoracic cavity leading to the pancreas at thoracic surgical exploration. The child was cured by total parenteral nutrition, intravenous infusion of somatostatin and a stent placed in the pancreatic duct by endoscopic retrograde cholangiopancreatography (ERCP).The child with pancreatic pleural fistula in early stage mainly present respiratory symptoms and lack abdominal symptoms, so the diagnosis and treatment is often delayed. Significant increase of pancreatic amylase level in pleural effusion can be an important clue and basis for the diagnosis. If conservative therapy fails, the examination and treatment with ERCP should be considered.

Galactomannan (GM) is a major aspergilli cell-wall constituent released into circulation during the early stage of invasive disease, and can be detected. Many studies suggest that serum galactomannan assay has an excellent sensitivity and specificity for the early diagnosis of adult invasive aspergillosis (IA). However, there have been few studies on serum galactomannan assay in pediatric patients. Therefore, we evaluate the value of serum galactomannan assay in the diagnosis of pediatric invasive pulmonary aspergillosis in this study.Blood samples were obtained from 88 children, of whom 14 had definitive or possible invasive pulmonary aspergillosis (IPA), 16 had other invasive pulmonary fungal infection and 58 had pulmonary non-fungal infection. Of the 58 patients, 23 had bacterial pneumonia, 20 had mycoplasma pneumonia and 15 had pulmonary tuberculosis. A double-direct sandwich ELISA was employed to detect GM optical density index (ODI) in the serum sample. GM ODI were observed before and after treatment in six children with IPA. Measurement data followed the Gaussian distribution were expressed as x(-) +/- s; differences among groups were tested using a single factor analysis of variance. If the s1/3 x(-), measurement data were expressed as M [minimum, maximum], and the differences among groups were tested by a rank sum test. P0.05 was considered to be statistically significant.The serum GM ODI in IPA group [1.03 (0.16 - 3.73)] was significantly higher than that in the other invasive pulmonary fungal infection group [0.30 (0.04 - 1.28)] and pulmonary non-fungal infection group [0.24 (0.08 - 0.69)] (P0.05). If the cut-off GM ODI was set at 0.5, the sensitivity and specificity of the assay for IPA were 71.4% and 91.9% respectively. The accuracy rate for IPA was 88.6%. If the cut-off GM ODI was set at 0.8, the sensitivity, specificity and accuracy rate for IPA were 64.2%, 98.6% and 93.2% respectively. Of 6 children whose GM were observed serially, GM ODI declined consistently with the clinical remission in 3 children. GM ODI raised in 2 children corresponding to clinical exacerbation. Whereas GM ODI elevated paradoxically regardless of clinical remission in the remaining one patient.Serum GM detection was an effective method for the diagnosis of pediatric IPA.

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.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.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, P0.05; 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, P0.05; 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.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.

Chronic active Epstein-Barr virus infection (CAEBV) has been previously reported to be sometimes associated with an aggressive clinical course. The characteristics of CAEBV in Mainland Chinese pediatric patients are largely unreported. The main aims of this survey were to recognize the clinical features of CAEBV in children and to explore its diagnostic criteria and risk factors.A retrospective study was performed on 53 pediatric patients (36 boys and 17 girls) with CAEBV who were admitted to Beijing Children's Hospital between 2003 and 2007. All their medical records were reviewed and analyzed. For each patient, demographic, clinical, laboratory data and outcome were collected. Independent-samples t test was used for statistical analysis.The age at onset of CAEBV was from 2 months to 14.6 years (mean (5.3+/-3.3) years). At the time of onset, 43.4% patients had an infectious mononucleosis-like symptom. Most patients exhibited intermittent fever (92.5%, 49/53), hepatomegaly (81.1%, 43/53) and splenomegaly (77.4%, 41/53). Life-threatening complications including hemophagocytic syndrome (24.5%, 13/53), interstitial pneumonia (24.5%, 13/53), hepatic failure (15.1%, 8/53) and malignant lymphoma (11.3%, 6/53) were also observed. The serum EBV DNA level in 23 patients with CAEBV was in the range of 5.05 x 10(2)-4.60 x 10(6) copies/ml with a mean value of 10(3.7) copies/ml. Many patients with CAEBV generally had continuous symptoms during the observational period. Eleven out of 42 patients (26.2%) died 7 months to 3 years after onset. Deceased patients were more likely to have had lower platelet counts and albumin levels than the living patients (P0.05 for all comparisons).The study reveals that CAEBV in Chinese pediatric patients has a severe clinical course and prognosis is poor. Thrombocytopenia and decreases in albumin might potentially be risk factors for a poor prognosis. EBV loads should be measured and tissue should be stained on hybridization probes for EBV-encoded small RNA (EBER) if a patient presents with the known symptoms of CAEBV.

To investigate clinical features of X-linked agammaglobulinemia (XLA) in children.The medical records of 17 children with XLA between January 2001 and April 2007 were reviewed.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.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.

Diffuse panbronchiolitis (DPB) is a chronic progressive disease of the lower respiratory tract, which is prevalent in Asian population. So far, many DPB cases have been found in adults in China. To our knowledge, no pediatric DPB case has ever been reported in China. We describe the first pediatric DPB case in Chinese literature and the second case in the English-language literature.The clinical manifestations, characteristic imaging and histological features of this DPB case were summarized.The patient was a 13-year old girl complained of chronic productive cough with wheezing. Chest auscultation revealed fine moist rales and wheezing over both lung fields. The chest X-ray showed small nodules and reticular opacities in left lower lobe. High-resolution thorax computerized tomography (HRCT) demonstrated bilateral diffuse small centrilobuler nodules and bronchial wall thickening or bronchiectasis in some parts of the lungs. Histopathological examination of transbronchial biopsy specimen revealed lymphocytes and foamy histocytes infiltrated in the walls of bronchi, respiratory bronchioles and adjacent alveoli. Lymphoid follicles were present around some bronchi. Sinus radiographs revealed sinusitis. Lung function studies showed obstruction and restriction. PaO2 was 65 mm Hg. The diagnosis of DPB was made according to the current diagnostic criteria. Low-dose erythromycin [5 - 10 mg/(kg.d)] was effective.DPB could occur in children in China. The major diagnostic clues may include the following: (1) persistent cough, sputum, and dyspnea; (2) coexistent chronic sinusitis; (3) bilateral diffuse small nodular opacities on HRCT. Low-dose erythromycin was effective in treatment of the case with DPB.

It is supposed that bronchial epithelial cells responses to the environmental stimuli are different between asthmatic and non-asthmatic individuals, which contribute to the pathogenesis of asthma. These different responses produce different mediators. If differential gene expressions are found in bronchial epithelial cells of asthmatic and non-asthmatic individuals after the same stimuli in vitro, and these genes are overexpressed in asthmatic children in vivo, then it is concluded that these genes may be associated with asthma. Therefore the authors analyzed the differential gene expressions in the bronchial epithelium cells of asthmatic and non-asthmatic children after RSV infection in vitro. Among these genes, Galectine-7 (lectin, galactoside-binding, soluble, 7, Galectin-7) was 8 times up-regulated in asthmatic children. Galectine-7 was associated with skin keratinocyte apoptosis. The authors hypothesized that Galectin-7 may also be associated with bronchial epithelial cell apoptosis in asthmatic children. The aim of this study was to understand the role of Galectine-7 in bronchial epithelial cell apoptosis in asthma.The bronchial mucosae of one asthmatic child and one non-asthmatic child were obtained by biopsy and cultured in vitro. The bronchial epithelial cells were infected by RSV. The differential gene expressions were analyzed with micro array. Among those differentially expressed genes, Galectin-7 was 8 times up-regulated in asthmatic children. The bronchial mucosae from 10 asthmatic children and 17 non-asthma children were investigated for cell DNA break, Galectine-7 and mRNA expression, Caspase-3 expression by TUNEL, hybridization in situ and immunochemistry. Image analysis was used for quantitative assessment.Galectine-7 gene was 8 times up-regulated in bronchial epithelial cells from asthmatic children after RSV infection in vitro. Galectin-7 and mRNA were overexpressed in bronchial epithelial cells in asthma in vivo. Bronchial epithelial cell apoptosis increased in asthma in vivo.Galectin-7 may be associated with bronchial epithelial cell apoptosis in asthma.

To investigate whether erythromycin exerts anti-inflammatory effect on allergic airway inflammation and whether erythromycin modulate allergic airway inflammation by inhibiting nuclear factor kappa B (NF-kappa B) activation.Ovalbumin (OVA) together with aluminum hydroxide and Bordetella Pertussis was injected intraperitoneally to immunize SD rats and two weeks later 1% OVA was inhaled to challenge them for consecutive 7 days to mimic allergic airway inflammation. In treatment group, erythromycin was given orally (180 mg/kg.d) during the course of allergen exposure. WBC counts in bronchoalveolar lavage fluid (BALF) and lung specimen analysis were used to describe lung tissue inflammation. The expression of NF-kappa B subunit p65 in cell nucleus of lung tissue was measured by immunohistochemistry and NF-kappa B binding activation in lung tissue by electrophoresis mobility shift assay (EMSA).Lung tissue specimen analysis indicated that the severity of allergic inflammation was reduced in treatment group. The number of total WBC in BALF (x 10(8)/L) (31 +/- 22) was lower than that in model group animals (66 +/- 28), P0.01. The number (x 10(3)/mm(2)) of cells with nuclear staining of NF-kappa B per square millimeter of submucosal region around large bronchus (1.4 +/- 0.4) was lower than that in model group animals (2.6 +/- 0.6), P0.01. NF-kappa B binding activity (32 +/- 14) was lower than that of model group (46 +/- 17), P0.05.Erythromycin had an obvious protective role in allergic airway inflammation. Erythromycin inhibited NF-kappa B transcriptional activity to exert anti-inflammatory effect.

Chronic pulmonary aspergillosis is different from acute pulmonary aspergillosis in clinical picture, radiogram, diagnostic procedures and prognosis. Four patients with chronic pulmonary aspergillosis had been misdiagnosed as having pneumonia or pulmonary tuberculosis for a long time before admission to the hospital. The purpose of this report was to summarize the clinical manifestations and laboratory findings for correct diagnosis of chronic pulmonary aspergillosis.Four patients with chronic pulmonary aspergillosis seen between October 2002 and October 2004 were retrospectively studied. Their clinical manifestations, chest radiographic feature, immune status, diagnostic procedure, therapy and prognosis were reviewed.The chief complaints of these patients were chronic cough and fever for 3 to 12 months. Chest wall abscess developed in the late course in case 1 and 4. Fine moist rales were heard and hepatosplenomegaly was found in case 1 and 2. No abnormal sign was found in case 3 and 4. Chest radiographic feature: lobar consolidation with adjacent pleural thickening was present in all cases. In early phase, solitary or multiple small nodules were found in 2 cases. Case 1-3 had normal IgG, IgM, IgA, IgE, T Cell subsets and NBT test. Case 4 had chronic granulomatous disease. Etiologic evidences: culture was positive for Aspergillus (A.) fulmigatus in sputum and in chest wall abscess in case 1 and 4; for A. niger in sputum and spore existing in lung tissue in case 2; for A. fulmigatus in sputum and hypha existing in lung tissue in case 3. All patients were treated with combination of amphotericin B and itraconazole. Their symptoms were controlled 10-30 d after treatment. In case 1 the disease relapsed 6 months later and the patient died at last due to giving up treatment by his parents. Case 2 was free of symptom for 12 months and his chest radiographic lesion disappeared completely 6 months later. Treatment of case 4 was given up. Case 3 continued to receive treatment and observation.Chronic pulmonary aspergillosis should be considered in children with long period fever and cough and lobar consolidation associated with adjacent pleural thickening or with nodular infiltration. The diagnosis of chronic pulmonary aspergillosis depended on identification of aspergillus from sputum or lung tissue. Combined amphotericin B and itraconazole might control the disease.

Diffuse lung disease comprises a large, heterogeneous group of pulmonary interstitial and parenchymal disease. It is therefore difficult to some extent to make etiologic diagnosis. Little information on clinical spectrum and diagnostic evaluation of pediatric diffuse lung disease is available in our country. The purpose of this study was to explore the causes of and diagnostic approach to diffuse lung disease in children.Twenty-eight children with diffuse lung disease aged 2 months to 14 years were studied retrospectively. Their history, physical examination, radiographic findings, final diagnosis and diagnostic processes were reviewed.Confirmed diagnosis was established in 25 cases and suggestive diagnosis in 3 cases. Confirmed diagnoses included: mycoplasma pneumonia in 1 case, Chlamydia trachomatis pneumonia in 2 cases, Epstein-Barr virus pneumonia in 1, CMV pneumonia in 2, hematogenous disseminated pulmonary tuberculosis in 3, pulmonary cryptococcosis in 1, invasive pulmonary aspergillosis in 2, Staphylococcus aureus sepsis in 1, diffuse bronchiectasis in 2, idiopathic pulmonary hemosiderosis in 1, idiopathic pulmonary fibrosis in 1, extrinsic allergic alveolitis in 1, HIV-related lymphocytic interstitial pneumonitis in 1, Wegner's granulomatosis in 1, Langerhan's cell histiocytosis in 2, and lymphoma in 3. Suggestive diagnoses included Nocardia pneumonia in 1, Pneumocystis carinii pneumonia in 1, and juvenile rheumatoid arthritis-associated pulmonary fibrosis in 1. The diagnostic directions of 26 patients were conducted by radiographic features. In 17 of 26 cases, the diagnostic range was confined by history. The diagnosis of 14 cases was made by noninvasive tests including antibody detection, bacterial culture, those of 8 cases by examination of biopsy material, and those of 2 cases by autopsy.The causes of pediatric diffuse lung disease included pulmonary infectious disease, idiopathic pulmonary disease and pulmonary lesion associated with systemic diseases. The diagnosis may be made by radiography, history, physical examination, noninvasive tests in most cases, while in some cases invasive procedures were necessary.

To study clinical characteristics of pediatric SARS cases in Beijing.Eighteen pediatric cases with SARS diagnosed on admission were analyzed. The cases were admitted to Beijing Children's Hospital and Ditan Hospital (pediatric ward) from April 8 to May 12.The 18 children aged 5 months to 15 years (10 male and 8 female) had epidemiologically linked findings. Fourteen cases had close contact with SARS patients. Four cases were living in the community where adult SARS patients were found. All the 18 patients but one presented with fever and cough. Most of them had high fever, 2 cases had bradycardia, 2 had diarrhea, and another 2 had tachypnea. Malaise and headache were noted only in 3 cases respectively which were much less frequently seen than in adult patients. Symptoms and signs of the children were much less severe and aggressive than adults cases. Thirteen children had chest radiographic consolidation. Of them, 9 cases had progressive changes after admission, then improved quickly. We did not find significant lower hemoglobin and platelet levels. Most patients had leukopenia and lymphopenia. Serologic test was performed for 15 cases and 8 were positive for SARS virus-IgG and 6 for IgM antibody. Of the 4 cases who had close contact with SARS adults and without chest radiograph abnormal findings, 3 were negative for SARS virus-antibodies. Part of the patients had temporary abnormality of myocardial enzyme and liver function. All these children finally had rapid improvement on chest radiograph. The patients were treated with antiviral agents and corticosteroid. Only two cases required oxygen therapy. No child needed assisted ventilation and no death, nor lung fibrosis occurred. After hospitalization, all patients were improved and discharged when this paper was being written. The average hospital stay of these patients was 14.6 days (6 - 22 days).Compared with adults, pediatric SARS patients seemed to have their own clinical characteristics. The disease in children had lower severity and infectivity than that in adults. The mechanisms of the disease in children should be studied in well-designed clinical trials. Cases like the 4 children who had close contact with SARS adult patients but without chest radiographic changes deserve further studies with the help of more reliable and sensitive etiologic tests.