Predictive factors for sequelae of bronchitis obliterans in refractory Mycoplasma pneumoniae pneumonia / 中华儿科杂志

Objective: To investigate the predictive factors for bronchitis obliterans in refractory Mycoplasma pneumoniae pneumonia (RMPP). Methods: A restrospective case summary was conducted 230 patients with RMPP admitted to the Department of No.2 Respiratory Medicine of Beijing Children's Hospital, Capital Medical University from January 2013 to June 2017 were recruited. Clinical data, laboratory results, imaging results and follow-up data were collected. Based on bronchoscopy and imaging findings 1 year after discharge, all patients were divided into two groups: one group had sequelae of bronchitis obliterans (sequelae group) and the other group had not bronchitis obliterans (control group), independent sample t-test and nonparametric test were used to compare the differences in clinical features between the two groups. Receiver operating characteristic (ROC) curve to explore the predictive value of Bronchitis Obliterans in RMPP. Results: Among 230 RMPP children, there were 115 males and 115 females, 95 cases had sequelae group, the age of disease onset was (7.1±2.8) years;135 cases had control group, the age of disease onset was (6.8±2.7) years. The duration of fever, C-reative protein (CRP) and lactate dehydrogenase (LDH) levels, the proportion of ≥2/3 lobe consolidation, pleural effusion and the proportion of airway mucus plug and mucosal necrosis were longer or higher in the sequelae group than those in the control group ((17±9) vs. (12±3) d, (193±59) vs. (98±42) mg/L,730 (660, 814) vs. 486 (452, 522) U/L, 89 cases (93.7%) vs. 73 cases (54.1%), 73 cases (76.8%) vs.59 cases (43.7%), 81 cases (85.3%) vs. 20 cases (14.8%), 67 cases (70.5%) vs. 9 cases (6.7%), t=5.76, 13.35, Z=-6.41, χ2=14.64, 25.04, 22.85, 102.78, all P<0.001). Multivariate Logistic regression analysis showed that the duration of fever ≥10 days (OR=1.200, 95%CI 1.014-1.419), CRP levels increased (OR=1.033, 95%CI 1.022-1.044) and LDH levels increased (OR=1.001, 95%CI 1.000-1.003) were the risk factors for sequelae of bronchitis obliterans in RMPP. ROC curve analysis showed that CRP 137 mg/L had a sensitivity of 82.1% and a specificity of 80.1%; LDH 471 U/L had a sensitivity of 62.7% and a specificity of 60.3% for predicting the development of bronchitis obliterans. Conclusions: The long duration of fever (≥10 d), CRP increase (≥137 mg/L) may be used to predict the occurrence of sequelae of bronchitis obliterans in RMPP. It is helpful for early recognition of risk children.

Clinical features of children with post-primary tuberculosis / 中华儿科杂志

Objective: The aim of this study was to summarize the clinical and imaging characteristics of post-primary tuberculosis in children, so as to improve the early identification and diagnosis of post-primary tuberculosis. Methods: This was a retrospective study which enrolled children who were admitted to the Department No.2 of Respiratory Medicine, Beijing Children's Hospital Affiliated to Capital Medical University between January 2015 to December 2020 and with a diagnosis of post-primary tuberculosis. Results: A total of 30 patients were enrolled, including 10 males and 20 females. The age on admission were 13.0 (12.0, 13.3) years. Their common symptoms were cough and fever, there were 26 cases (87%) with cough and 23 cases (77%) with fever, but only 4 cases (13%) had other toxic symptoms (night sweat, weakness or weight loss) of tuberculosis other than fever. Blood examination showed that the white blood cell count was (10±3)×109/L, accompanied by elevated proportion of neutrophils (0.69±0.11) and increased level of C-reactive protein (31 (15,81) mg/L). The common radiographic findings of CT were nodular or mass shadow with cavitation (19 cases (63%)), consolidation (13 cases (43%)), bronchogenic spread (12 cases (40%)), hilar or mediastinal lymphadenopathy (5 cases (17%)) in this cohort. The affected locations included the right upper lobe (21 cases (70%)), the left lower lobe (17 cases (57%)) and the right lower lobe (15 cases (50%)). Acid-fast bacillus smears and mycobacterial cultures were attempted for all cases, resulting in 33% (10/30) with smear positivity and 50% (15/30) with culture positivity. Conclusions: Post-primary tuberculosis in children has no specific clinical manifestations. Imaging of chest CT is mainly manifested as nodular shadow with cavitation, consolidation or bronchogenic spread. Accurate identification of post-primary tuberculosis is crucial for preventing the spread and early treatment of tuberculosis.

Clinical analysis of a Chinese child with leukocyte adhesion deficiency type 1 / 中华儿科杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHOD</b>The clinical material of the LAD-I patient who was diagnosed by gene analysis was retrospectively analyzed.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>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.</p>

Clinical features of four atypical pediatric cases of endemic typhus with pneumonia / 中华儿科杂志

<p><b>OBJECTIVE</b>To analyze clinical manifestations, treatment and prognosis of 4 cases with endemic typhus.</p><p><b>METHOD</b>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.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>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.</p>

Risk factors of invasive fungal infections in patients admitted to non- hematological oncology department and pediatric intensive care unit / 中华儿科杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHOD</b>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.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>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.</p>

Analysis of clinical manifestations and diagnosis of 102 children with bronchial tuberculosis / 中华儿科杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHOD</b>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.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>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.</p>

Clinical manifestations and gene analysis of 2 Chinese children with cystic fibrosis / 中华儿科杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHOD</b>The clinical material of two CF patients who were diagnosed by gene analysis was retrospectively analyzed.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>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.</p>

Clinical characteristics and predictive factors of refractory Mycoplasma pneumoniae pneumonia / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate clinical characteristics and predictive factors of refractory Mycoplasma pneumoniae pneumonia (RMPP) in children so as to recognize and treat the disease earlier.</p><p><b>METHOD</b>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.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>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.</p>

Clinical analysis of 20 cases with Streptococcus pneumoniae necrotizing pneumonia in China / 中华儿科杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHOD</b>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.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>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.</p>

Clinical features and molecular analysis of 2 Chinese children with autosomal recessive chronic granulomatous disease caused by CYBA mutations / 中华儿科杂志

<p><b>OBJECTIVE</b>To summarize clinical and molecular features of two children with autosomal recessive chronic granulomatous disease caused by CYBA mutations.</p><p><b>METHOD</b>The clinical records and CYBA mutations were reviewed for analysis of infections and inflammatory complications.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>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.</p>

Bronchitis obliterans in children: report of two cases and literature review / 中华儿科杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHOD</b>The clinical manifestations, characteristic imaging of two cases with bronchitis obliterans were summarized.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>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.</p>

Diagnosis of abdominal tuberculosis in children / 中华儿科杂志

<p><b>OBJECTIVE</b>To improve the recognition and diagnosis of abdominal tuberculosis (TB) in children.</p><p><b>METHOD</b>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.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>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.</p>

Clinical analysis of 18 children with disseminated Bacille Calmette-Guérin infection / 中华儿科杂志

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

Idiopathic interstitial pneumonias in 7 children / 中华儿科杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHOD</b>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.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>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.</p>

Value of serum galactomannan assay in the diagnosis of pediatric invasive pulmonary aspergillosis / 中华儿科杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHOD</b>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 s>1/3 x(-), measurement data were expressed as M [minimum, maximum], and the differences among groups were tested by a rank sum test. P < 0.05 was considered to be statistically significant.</p><p><b>RESULT</b>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)] (P < 0.05). 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.</p><p><b>CONCLUSION</b>Serum GM detection was an effective method for the diagnosis of pediatric IPA.</p>

Recurrent massive bloody pleural effusion caused by pancreatic pleural fistula in a case / 中华儿科杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHOD</b>The clinical data of symptoms, signs, laboratory examination, diagnosis and treatment methods of pancreatic pleural fistula in a child are presented.</p><p><b>RESULT</b>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).</p><p><b>CONCLUSION</b>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.</p>

Clinical analysis and follow-up study of chronic active Epstein-Barr virus infection in 53 pediatric cases / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>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.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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 (P<0.05 for all comparisons).</p><p><b>CONCLUSIONS</b>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.</p>

Association of NRAMP1 gene polymorphisms with the susceptibility to tuberculosis in ethnic Han Chinese children / 中国当代儿科杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>

Diagnosis and treatment of invasive pulmonary aspergillosis in 21 children with non-hematologic diseases / 中华儿科杂志

<p><b>OBJECTIVE</b>To explore diagnosis and treatments of invasive pulmonary aspergillosis (IPA) in children with non-hematologic diseases.</p><p><b>METHOD</b>Twenty one patients without hematological malignancy were diagnosed with proven or possible IPA from July 2002 to June 2008. The risk factors, clinical manifestations, chest radiographic findings, microbiological and histopathological evidence, diagnostic procedures, treatment and prognosis were retrospectively reviewed.</p><p><b>RESULT</b>Five children had proven IPA, and 16 patients had possible IPA. Thirteen children were classified as having acute invasive pulmonary aspergillosis (AIPA), eight children as having chronic necrotizing pulmonary aspergillosis (CNPA). Definitive diagnosis of primary immunodeficiency (PID) was made in 6 children (4 with chronic granulomatous disease, 2 with cellular immunodeficiency); three children were suspected of having PID. Corticosteroids and multiple broad-spectrum antibiotics had been administered in 5 patients (3 of these 5 patients also had invasive mechanical ventilation). Two children had underlying pulmonary disease. Three patients had unknown risk factors. Among these three patients, two had history of environmental exposure. Fever and cough were present in all the children. Fine rales were found in nineteen children. Six children had hepatosplenomegaly. The common roentgenographic feature of AIPA in 13 patients was nodular or mass-like consolidation with multiple cavity. "air-crescent" was seen in 10 of patients with AIPA. Lobar consolidation with cavity and adjacent pleural thickening was found in all children with CNPA. The positive rate of sputum and/or BALF culture in AIPA and CNPA were 72.1% and 22.4%, respectively. A large number of septate hyphae on wet smear were found in all of the children whose sputum and/or BALF culture were positive. Lung biopsy was performed in 3 children with CNPA, and necrosis, granulomatous inflammation, as well as septate, branching hyphae were observed on histopathologic examination. Fifteen children were treated with anti-fungal therapy (amphotericin B, voriconazole, itraconazole and caspofungin used alone or in combination), symptoms and lung lesions resolved in 12 children. Three children died. Six children did not receive anti-fungal therapy and died. The side effects of amphotericin B include chill, fever, hypokalemia and transient increase in BUN, none of which needed discontinuation of the antifungal therapy. Children had a good tolerance to fluconazole and caspofungin, there were no apparent side effects.</p><p><b>CONCLUSION</b>Most of the children without hematologic diseases who suffered from invasive pulmonary aspergillosis had risk factors or exposure history. Roentgenographic findings were relatively characteristic for invasive pulmonary aspergillosis. Risk factors and roentgenographic findings were clues to consider clinically invasive pulmonary aspergillosis. Sputum culture was the key point to clinical diagnosis. The patients in whom the antifungal therapy was initiated early had a good outcome.</p>

Clinical features of 17 cases of X-linked agammaglobulinemia / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate clinical features of X-linked agammaglobulinemia (XLA) in children.</p><p><b>METHODS</b>The medical records of 17 children with XLA between January 2001 and April 2007 were reviewed.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>