Research progress of respiratory virus infection associated acute necrotizing encephalopathy / 中华实用儿科临床杂志

Acute necrotizing encephalopathy (ANE) is a subtype of acute encephalopathy presented with disturbance of consciousness and symmetric bilateral thalamic necrosis in neuroradiology.Patients with ANE had a high mortality or severe neurological sequela.ANE usually secondary to virus infectious disease, in which influenza is a common etiology.During the 3 years of the worldwide pandemic of severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)infection, ANE has become a severe complication and cause of death in children, which has aroused much concern.Here is a review of the research progress of epidemiology, pathogenesis, diagnosis, treatments and prognosis of ANE, in order to improve the knowledge of clinicians on this disease.

Risk factors for postinfectious bronchiolitis obliterans after severe adenovirus pneumonia / 中国小儿急救医学

Objective:To investigate the clinical characteristics and risk factors of postinfectious bronchiolitis obliterans(PIBO)after severe adenovirus pneumonia(SAP).Methods:We retrospectivly analyzed 78 children who were hospitalized for SAP at Shenzhen Children′s Hospital from April 2015 to April 2020.The cases were divided into PIBO group( n=26) and non-PIBO group( n=52) based on the diagnosis results.The general conditions, clinical characteristics, and laboratory data from two groups were analyzed, and the risk factors for PIBO were explored. Results:A total of 78 children were included in this study.There were 18 (69.2%) males and eight (30.8%) females in PIBO group; the average age of onset in PIBO group was younger than that in non-PIBO group[(11.77±3.24)months vs.(15.08±6.48)months, P=0.027]. The cough duration[(11.35±7.35)days vs.(7.15±5.67)days, P=0.010], and heat duration[(13.12±6.78)days vs.(8.62±4.76)days, P=0.007] were longer in PIBO group than those in non-PIBO group.The white blood cell count[(12.46±7.23)×10 9/L vs.(9.17±3.66)×10 9/L), P<0.05], platelet count[(390.12±209.03)×10 9/L vs.(284.69±83.33)10 9/L, P<0.05], C-reactive protein[(37.04±32.16)mg/L vs.(18.14±18.33)mg/L, P<0.05], procalcitonin[(3.51±3.33)μg/L vs.(1.09±1.37)μg/L, P<0.05], lactate dehydrogenase(LDH)[(1 155.88±842.94)IU/L vs.(414.00±218.94)IU/L, P<0.01] were all higher in PIBO group than those in non-PIBO group; The roportion of patients with mycoplasma pneumoniae infection[5(19.2%) cases vs.4(7.7%) cases, P<0.05], admitted to PICU[18(69.2%) cases vs.8(15.4%) cases, P<0.01] , using invasive mechanical ventilation[10(38.5%) cases vs.5(9.6%) cases, P<0.01], using hormones[23(88.5%) cases vs.21(40.4%) cases, P<0.01], and using human immunoglobulin[20 (76.9%) cases vs.10(19.2%) cases, P<0.01] were higher in PIBO group than those in non-PIBO group.The multivariate Logistic regression using stepwise method showed that older age ( OR=0.942, 95% CI 0.890-0.997) was a protective factor for PIBO, while higher LDH levels ( OR=1.005, 95% CI 1.002-1.008), using intravenous corticosteroids ( OR=6.622, 95% CI 0.924-47.436), and using human immunoglobulin ( OR=9.681, 95% CI 1.742-53.802) were the risk factors for PIBO in SAP ( P<0.05). The receiver operating characteristic curve was constructed through the combination of age of onset, LDH level, using intravenous hormone, and using human immunoglobulin.The area under the curve reached 0.954.The overall best cut-off value of the prediction model was 0.272, the sensitivity was 92.3%, and the specificity was 86.5%.When LDH=462 IU/L, the area under the curve reached the maximum value of 0.882, the sensitivity was 100.0%, and the specificity was 61.5%. Conclusion:SAP children with characteristics such as younger age, long cough and fever duration, high inflammatory index, LDH level higher than 462 IU/L, admitted to PICU, mechanical ventilation and need hormones and human immunoglobulin, should be alert to the risk of PIBO.

Nebulization of intravenous Tobramycin for treatment of Pseudomonas aeruginosa in children with cystic fibrosis / 中华实用儿科临床杂志

Objective:To investigate the feasibility of nebulizing injectable Tobramycin for the treatment of Pseudomonas aeruginosa (Pa) in pediatric cystic fibrosis (CF) patients. Methods:The clinical data of 3 CF children with Pa infection who were treated by nebulizing injectable Tobramycin in Shenzhen Children′s Hospital from September 2017 to March 2021 were retrospectively analyzed.The efficacy and safety were explored.The nebulized injectable Tobramycin (160 mg/dose) was given twice daily after airway clearance.After one-month treatment course, oral Ciprofloxacin would be given [30 mg/(kg·d)] to patients for another 1 month if Pa was still positive in repeat sputum cultures.Results:There were 2 males and 1 female in 3 cases recruited.The youngest patient was 1-year-old when receiving Tobramycin treatment.After airway clearance and inhalation of injectable Tobramycin, all had improvements in respiratory symptoms and chest CT scan.Two cases took additional oral Ciprofloxacin as Pa was still positive after the 1-month treatment course of Tobramycin.Pa turned to negative in all 3 cases after treatment for 3 months to 1.5 years.Besides, after treatment all the 3 patients had normal liver and renal functions, and normal hearing in multiple follow ups.One patient had a normal brainstem auditory evoked potential in the reexamination.Conclusions:Nebulizing injectable Tobramycin would be a reasonable alternative to inhaled Tobramycin solution for treating pediatric CF patients with Pa in view of the present condition in mainland China.However, it is still worth further study and discussion.

Application of antibiotics for children with cystic fibrosis: interpretation of clinical guidelines for care of children with cystic fibrosis in Royal Brompton Hospital (2020) / 中华实用儿科临床杂志

Cystic fibrosis (CF) is a multisystem disease caused by CFTR gene mutations, affecting the lungs, digestive system, and reproductive tract.Infectious lung disease is the major cause of exacerbation and mortality in most patients.There are specific pathogen spectrums in infectious lung disease and antibiotics application principles in CF patients.In this article, the application of antibiotics in clinical guidelines for care of children with CF in Royal Brompton Hospital (2020) was introduced.

Etiological characteristics and changes of plastic bronchitis in children in single center / 中国小儿急救医学

Objective:To investigate the etiological characteristics and changes of plastic bronchitis(PB)in children from 2010 to 2019 at Shenzhen Children′s Hospital, and provide reference basis for improving the understanding of PB etiology.Methods:The clinical data of children diagnosed with infectious-associated PB at Shenzhen Children′s Hospital from Jan 2010 to Dec 2019 were retrospectively analyzed, and the etiological characteristics and changes were summarized.Results:There were 94 cases of mycoplasma pneumoniae, 38 cases of influenza virus, 41 cases of adenovirus, 16 cases of mixed infection, 11 cases of bacteria, and 57 cases of unclear etiology in 266 infectious-associated PB children.The distribution of PB in each age group: 15 cases were infants, 63 cases were toddlers, 112 cases were preschoolers, and 76 cases were school-age children.Adenovirus was the main pathogen of PB in infants and toddlers(60.0%, 28.6%), and mycoplasma pneumoniae(34.8%, 60.5%) as well as influenza virus(13.4%, 22.4%) were the main pathogen in preschool and school-age children, with statistically significant difference( P<0.001). From 2010 to 2019, the annual positive rates of pathogens were 62.5%, 60.0%, 66.7%, 74.1%, 64.0%, 50.0%, 93.3%, 57.1%, 75.0%, and 84.7%, respectively.PB was caused by mycoplasma pneumoniae infection every year.From 2016 to 2019, PB caused by mycoplasma pneumoniae infection increased year by year, while PB caused by adenovirus infection increased every other year. Conclusion:Mycoplasma pneumoniae was the most common pathogen of PB, followed by adenoviruses and influenza viruses, while bacteria, fungi and other viruses were relatively rare.In the infant group, adenovirus infection was predominant, while in preschool and school-age children group, mycoplasma pneumoniae and influenza virus infection were predominant.

Clinical feature analysis of 541 children with adenovirus pneumonia / 中华实用儿科临床杂志

Objective:To summarize the clinical characteristics of adenovirus (HAdVs) pneumonia in children.Methods:The clinical manifestations, laboratory results, imaging features and treatment courses of 541 children with HAdVs pneumonia treated in Shenzhen Children′s Hospital from January 2017 to August 2019 were retrospectively studied.Results:The number of children diagnosed with HAdVs pneumonia increased year by year.A total of 541 patients were included in this study (214 females and 327 males, median age 31 months). The majority of patients (89.3%, 483/541 cases) were less than 6 years of age.Cough (98.3%, 532/541 cases) was the most common symptom, followed by fever (93.7%, 507/541 cases), tachypnea (36.6%, 198/541 cases) and wheeting (32.5%, 176/541 cases). Thirteen point three percent patients (72/541 cases) had existing primary diseases.Severe HAdVs pneumonia was observed in 33.8% of patients (183/541 cases), the most common intrapulmonary complication were pleural effusion(18.6%, 101/541 cases) and respiratory failure (7.0%, 38/541 cases). In co-infection cases, 46.9% of the patients (254/541 cases) suffered from co-infection with mycoplasma pneumoniae, 21.6% of the patients (117/541 cases) suffered from bacterial co-infection.Ninety-eight point five percent of the patients (533/541 cases) were cured, and 1.5% of the patients (8/541 cases) died or discharged voluntarily.Convulsions [ P=0.026, OR=7.312 (95% CI: 1.267-42.191)] and bacterial co-infections [ P=0.030, OR=2.724 (95% CI: 1.101-6.741)] were independently correlated with the occurrence of severe HAdVs pneumonia. Conclusions:Pre-school aged children are at higher risks of developing HAdVs pneumonia.The majority of patients presents with fever and cough as primary manifestations.Tachypnea and wheezing are also common clinical features of HAdVs pneumonia patients.Approximately a third of children with HAdVs pneumonia may develop into severe pneumonia.The presentation of convulsion and the exis-tence of bacterial co-infections may help to predict the development of severe HAdVs pneumonia in pediatric patients.

Clinical analysis of 30 cases of severe pneumonia-associated hemophagocytic lymphohistiocytosis / 中华实用儿科临床杂志

Objective:To investigate the etiology, clinical characteristics and outcome of severe pneumonia-associated hemophagocytic lymphohistiocytosis, and to analyze the risk factors for mortality.Methods:Clinical data of patients with severe pneumonia-associated hemophagocytic lymphohistiocytosis admitted to Shenzhen Children′s Hospital from February 2009 to February 2019 were retrospectively analyzed.The data included clinical characteristics, etiology, clinical manifestations, laboratory data, treatment and outcomes of the patients.The clinical characteristics and laboratory data of the survival group and the death group were compared by independent sample t-test. Results:(1) Clinical characteristics: the patients were aged from 3 months to 8 years and 7 months, including 15 males and 15 females.Severe pneumonia-associated hemophagocytic lymphohistiocytosis accounted for 2.74% (30/1 096 cases) of severe pneumonia in the same period.(2) Etiology: Mycoplasma pneumoniae infection was found in 8 cases (8/30 cases, 26.67%), virus infection in 7 cases (7/30 cases, 23.33%, including 5 cases with adenovirus infection, 1 case with EB virus infection, and 1 case with cytomegalovirus infection), Mycoplasma pneumoniae complicated with adenovirus infection in 4 cases (4/30 cases, 13.33%), bacterial infection in 3 cases (3/30 cases, 10%), and fungal infection in 2 cases, Mycobacterium tuberculosis infection in 1 case.The pathogens were not identified in 5 patients.(3) Clinical manifestations: fever and hepatomegaly were present in all patients.Besides, 86.67% (26/30)patients had fever duration more than 10 days, 83.33% (25/30 cases) patients had cough, 76.66% (23/30 cases) patients had splenomegaly, and 33.33% (10/30 cases) patients had nervous system symptoms.Laboratory data showed varying degrees of reduction of binary and ternary systems in 80.00%(24/30 cases) of the patients.Liver function impairment was found in half of the patients, and serum ferritin and lactate dehydrogenase levels were elevated in all patients.(4) The mortality rate was 30.00% (9/30 cases). The differences in age, hypertriglyceridemia and high serum ferritin levels between the survival and death groups were significant (all P<0.05). Conclusions:Severe pneumonia-associated hemophagocytic lymphohistiocytosis is a disease with a high mortality rate.Patients with Mycoplasma pneumoniae and adenovirus pneumonia are more likely to suffer from secondary hemophagocytic lymphohistiocytosis.Younger age, hypertriglyceridemia and high serum ferritin levels are indicative of poor prognosis.

Immunodeficiency diseases with interstitial lung disease as major clinical manifestations: report of six cases / 中华儿科杂志

Objective@#To summarize the clinical features of immunodeficiency diseases with interstitial lung disease (ILD) as major clinical manifestations and to improve understanding etiology of ILD.@*Methods@#The clinical features and clinical clues for diagnosis of six cases with immunodeficiency presented with ILD in Shenzhen Children′s Hospital from January 2014 to December 2016 were retrospectively analyzed.@*Results@#The patients′ age ranged from 3 months to 5 years and 9 months, 5 cases were male. All cases had cough and tachypnea, 3 cases had lung infection and respiratory failure, 2 cases had chronic hypoxia and one had clubbing. Three cases had skin rashes; 5 cases had failure to thrive. Chest CT scan showed diffuse ground glass opacity in all the 6 cases, and 2 cases had cystic changes and one had "crazy-paving" pattern. Five patients were suspected to have surfactant dysfunction and genetic testing was performed before diagnosis of immunodeficiency, of which the results were negative. With human immunodeficiency virus antibody test or immunologic laboratory testing and/or immune genetic panel, acquired immune deficiency syndrome was confirmed in one case, hyper-IgM syndrome was confirmed in two cases and hyper-IgE syndrome in one case, Wiskott-Aldrich syndrome in one and STAT3 gain of function genetic mutation in another. All cases had clinical clues indicative of underlying immunocompromise.@*Conclusions@#The clinical features of immunodeficiency diseases with ILD are cough, tachypnea or hypoxia, respiratory failure with infection, diffuse ground glass opacity in Chest CT imaging. With thorough medical history and immunology screening, there would be clinical clues indicative of underlying immunocompromise. Screening for immunodeficiency disease should be emphasized in the differential diagnosis of ILD, otherwise it may lead to misdiagnosis or unnecessary testing.

The characteristics of respiratory tract microbiota and its clinical significance in children with protracted bacterial bronchitis / 中华实用儿科临床杂志

Objective To explore the characteristics of respiratory tract microbiota and its clinical significance in children with protracted bacterial bronchitis (PBB).Methods Twelve children aged from 5 months to 2 years old with PBB (PBB group) and 12 age-matched tracheomalacia(TM) children (TM group) were included in this study,who were admitted into the Respiratory Department of Shenzhen Children's Hospital.Their bronchoalveolar lavage fluid (BALF) samples were collected.Bacterial DNA was extracted from their BALF samples and the 16S rRNA V3-V4 region was sequenced by using Illumina MiSeq TMII system,and the findings were analyzed by bioinformatics methods.Results Principal component analysis revealed the difference in microbiota composition between 2 groups.Compared with TM group,PBB group exhibited lower microbial diversity:the Shannon indices were also 1.683 ± 0.703 and 2.324 ± 0.142 for PBB group and TM group respectively,and the differences were also significant(all P ＜ 0.05),and the Simpson indices were 0.416 ± 0.216 and 0.191 ± 0.025 for PBB group and TM group,respectively,and the differences were also significant (all P ＜ 0.05).The relative abundance of Actinobacteria was significantly lower in PBB group [(0.215 ± 0.228) ％] than that in TM group [(3.028 ± 0.592) ％] (P ＜ 0.01).The proportions of beneficial genera obviously decreased in PBB group,including Lactococcus [(13.464±7.319)％ in PBB group,and (44.784 ± 5.020)％ in TM group,P ＜0.01],Lactobacillus [(0.153 ±0.076)％ in PBB group,and (0.313 ±0.060)％ in TM group,P＜0.01],andArthrobacter [(0.024 ±0.018)％ in PBB group,and (2.970 ±0.584)％ in TM group,P＜0.01].On the other hand,the relative abundances of opportunistic pathogenic genera increased in PBB group significantly,including Haemophilus [(14.319 ± 29.532) ％ in PBB group,and (0.047 ± 0.127) ％ in TM group,P ＜ 0.Ol],Pseudomonas [(10.406 ± 25.439) ％ in PBB group,and (7.228 ± 0.948) ％ in TM group,P ＜ 0.01],and Escherichia [(0.432 ±0.441)％ in PBB group,and (0.055 ±0.035)％ in TM group,P ＜0.01].Conclusion These findings confirmed the existence of respiratory tract microbiotia dysbiosis in PBB,which probably was one of the pathogenetic mechanisms for PBB.

Childhood origins of adulthood respiratory diseases / 中华实用儿科临床杂志

Asthma and chronic obstructive pulmonary disease (COPD) are main chronic respiratory diseases in adulthood,which bring a heavy burden to society and economy.The adulthood asthma and COPD originate from childhood.Atopy and certain respiratory viral infection are the risk factors of asthma.Some factors in childhood including premature,childhood asthma,serious lung infection,air pollution,active smoking will damage pulmonary function in adulthood,which contribute to COPD onset.Strategies such as preventing premature birth and controlling asthma will reduce the burden of chrouic respiratory diseases in adults.

Clinical characteristics and literature review of five cases with recurrence of congenital tracheoesophageal fistula / 中国小儿急救医学

Objective To retrospectively analyze the clinical characteristics of 5 cases with recurrence of congenital tracheoesophageal fistula (rTEF) and to further understand the diagnosis and treatment of this disease.Methods We totally diagnosed 5 cases of rTEF from September 2015 to July 2016 in the department of respiration of Shenzhen Children′s Hospital.The clinical materials,characteristics,risk factors of recurrence and diagnostic methods were analyzed.Results Two cases were diagnosed of rTEF at 8 years after the first repair,one case was at one year after the initial repair and two cases were at 1 to 2 months after their operations.Three cases manifested mainly in bucking after feeding,recurrent pneumonia and growth retardation.One case was admitted for vomiting and abdominal distension.One case was asymptomatic.Esophageal anastomotic leaks occurred in three cases and esophageal trictures occurred in four cases after their repairs.Three cases accompanied with gastroesophageal reflux and five cases with tracheomalacia.Four cases were performed esophagography,but only two cases were suspected of recurrence.All of five cases were performed bronchoscopy.One case was diagnosed of rTEF directly.Two cases were found suspected fistulas.And another two cases were only found surgical scars.But these four cases were confirmed by Methylene blue test observed by bronchoscopy.Conclusion Although the leading clinical manifestations of rTEF are respiratory symptoms and feeding difficulties,sometimes rTEF is asymptomatic.Because rTEF is usually complicated with the other diseases with similar symptoms,it is possible to miss the diagnosis.Esophageal anastomotic leaks and trictures are the risk factors of recurrence.Bronchoscopy is the first choice for the diagnosis of rTEF,and Methylene blue test is golden standard.Thoracotomy is recommended for the treatment of rTEF.

Impact and changes of maternal hemoglobin on birth weight in pregnant women of Zhuang Nationality, in Guangxi / 中华流行病学杂志

Objective To investigate the hemoglobin (Hb) levels during pregnancy and Hb changes from early pregnancy and association with birth weight on infants.Methods Mothers of Zhuang Nationality who participated in the pregnancy care program and delivered at the Pingguo County Hospital from May 2013 to May 2015 were included in this study.Retrospective analysis was applied to collect data of health care and pregnancy outcomes.Multiple regression analysis and unconditional logistic regression model were used for data analysis.Results The mean birth weight appeared as (313 5.92± 435.84) grams.The Hb levels at early pregnancy showed significantly positive association with birth weight.Results from our study demonstrated that when Hb levels increased +1 g/dl at early pregnancy,birth weight would increase 17.61(95％CI:0.60-34.67) grams,in the adjusted model.The Hb levels at late pregnancy were significantly inversely associated with birth weight.Our findings suggested that when Hb levels increased + 1 g/di at late pregnancy,birth weight would reduce 19.61(95％ CI:-37.53--1.70) grams in the adjusted model.Changes in Hb from early pregnancy stages were significantly inversely associated with birth weight after adjusting for confounders and Hb levels in the early pregnancy stages.The results also indicated that when Hb levels increased a + 1 g/dl from early to late pregnancy,the birth weight would decrease 32.63 g(95％ CI:-48.93--16.32).Compared to the non-anemia group,the anemia group showed significantly increase of small-for-gestational-age (SGA)(OR=1.58,95％CI:1.08-2.32) in early pregnancy.Compared to women under the most reduction status,women with the least reduction had a significantly increase of SGA (OR=1.87,95％CI:1.24-2.81) among their infants.With the magnitude of reduction on Hb concentration during pregnancy,the risk of delivering babies with SGA showed a gradual trends of increase.Conclusion Hb levels at early pregnancy were positively associated with birth weight,but the changes of Hb were inversely associated with birth weight at late pregnancy,in women of Zhuang Nationality.Anemia in early pregnancy and the low amplitude of decreased Hb concentration during pregnancy were both risk factors for newborns under less gestational ages.

Impact and changes of maternal hemoglobin on birth weight in pregnant women of Zhuang Nationality, in Guangxi / 中华流行病学杂志

Objective To investigate the hemoglobin (Hb) levels during pregnancy and Hb changes from early pregnancy and association with birth weight on infants.Methods Mothers of Zhuang Nationality who participated in the pregnancy care program and delivered at the Pingguo County Hospital from May 2013 to May 2015 were included in this study.Retrospective analysis was applied to collect data of health care and pregnancy outcomes.Multiple regression analysis and unconditional logistic regression model were used for data analysis.Results The mean birth weight appeared as (313 5.92± 435.84) grams.The Hb levels at early pregnancy showed significantly positive association with birth weight.Results from our study demonstrated that when Hb levels increased +1 g/dl at early pregnancy,birth weight would increase 17.61(95％CI:0.60-34.67) grams,in the adjusted model.The Hb levels at late pregnancy were significantly inversely associated with birth weight.Our findings suggested that when Hb levels increased + 1 g/di at late pregnancy,birth weight would reduce 19.61(95％ CI:-37.53--1.70) grams in the adjusted model.Changes in Hb from early pregnancy stages were significantly inversely associated with birth weight after adjusting for confounders and Hb levels in the early pregnancy stages.The results also indicated that when Hb levels increased a + 1 g/dl from early to late pregnancy,the birth weight would decrease 32.63 g(95％ CI:-48.93--16.32).Compared to the non-anemia group,the anemia group showed significantly increase of small-for-gestational-age (SGA)(OR=1.58,95％CI:1.08-2.32) in early pregnancy.Compared to women under the most reduction status,women with the least reduction had a significantly increase of SGA (OR=1.87,95％CI:1.24-2.81) among their infants.With the magnitude of reduction on Hb concentration during pregnancy,the risk of delivering babies with SGA showed a gradual trends of increase.Conclusion Hb levels at early pregnancy were positively associated with birth weight,but the changes of Hb were inversely associated with birth weight at late pregnancy,in women of Zhuang Nationality.Anemia in early pregnancy and the low amplitude of decreased Hb concentration during pregnancy were both risk factors for newborns under less gestational ages.

A novel compound heterozygous mutation in ABCA3 gene in a child with diffuse parenchymal lung disease / 中华儿科杂志

Objective@#To summarize the clinical characteristics of the diffuse parenchymal lung diseases in a child caused by a novel compound heterozygous ABCA3 mutation and explore the association between the phenotype and ABCA3 mutation.@*Method@#The clinical material of a patient diagnosed with diffuse parenchymal lung disease with ABCA3 mutation in December 2016 in Shenzhen Children's Hospital was analyzed. The information about ABCA3 gene mutation updated before April, 2017 was searched and collected from the gene databases (including 1000Genomes, HGMD, EXAC) and the literatures (including Wanfang Chinese database and Pubmed).@*Result@#The girl was one year and nine months old. She presented with chronic cough, tachypnea, cyanosis and failure to thrive since she was one year and three months old. Her condition gradually deteriorated after she was empirically treated. Physical examination showed malnutrition, tachypnea and clubbed-fingers. Her high resolution computed tomography (HRCT) revealed diffused ground-glass opacities, thickened interlobular septum, and multiple subpleural small air-filled lung cysts. The second generation sequencing study identified a novel compound heterozygous mutation (c.1755delC+c.2890G>A) in her ABCA3 gene, which derived respectively from her parents and has not been reported in the database and the literatures mentioned above.@*Conclusion@#c.1755delC+c.2890G>A is a new kind of compound heterozygous mutation in ABCA3, which can cause children's diffuse parenchymal lung disease. Its phenotype is related to its genotype.

Clinical manifestations of three cases of surfactant protein C p. V39L mutation / 中华儿科杂志

Objective@#To investigate the clinical manifestations of surfactant protein C gene (SFTPC) exon-2 c. 115G>G/T (p.V39L).@*Method@#Patients were screened for the entire coding sequence of SFTPC. Three cases from three children′s hospital with mutation in p. V39L were reported.@*Result@#All the three cases were females. The age of onset ranged from 2 months to 7 years. Two cases had recurrent lower respiratory tract infection and failed to thrive. One had chronic anoxia and clubbing fingers. Chest computed tomography (CT) showed diffused ground glass pattern, localized emphysema and intralobular septal thickening. In one case, early sign of cyst formation was also shown on CT. Two were lost to follow-up after alleviation of acute respiratory infection. One was treated with oral low-dose azithromycin and nebulized budesonide and terbutaline. She had recurrent lower respiratory tract infection in more than one year of follow-up.@*Conclusion@#Mutations in SFTPC p. V39L cause interstitial lung diseases. Clinical manifestations included recurrent respiratory tract infections, chronic lung disease. Chest CT showing diffused ground glass pattern, localized emphysema, intralobular septal thickening and early sign of cyst formation. The treatment and prognosis need further study.

Clinical features and treatment of protracted bacterial bronchitis in children / 临床儿科杂志

Objective To study the diagnosis and treatment of protracted bacterial bronchitis (PBB) in children. Methods Children with PBB conifrmed by bronchoscopy were recruited from May 2013 to April 2015 . The clinical data were retrospectively analyzed. Results All 31 cases include 18 boys and 13 girls were recruited. 28/31 were younger than 6 years old. They all complained of wet cough, some of them were reported with wheeze ( 17/31 ) and with ruttle in the lungs ( 16/31 ). White blood cell were in normal range ( 18/31 ) or slightly elevated ( 13/31 ). The C-reactin protein was in normal range ( 28/31 ). Chest X-ray test of 16 cases were normal. Twenty-four cases taken chest computerized tomograph scan, 5 had a sign of tracheobronchial stenosis. The purulent bronchitis without tracheobronchial stenosis were conifrmed by bronchoscopy. Four cases had tracheomalacia. The medians of proportion of neutrophil were 80% in bronchoalveolar lavage lfuid (BALF). The pathogens were identiifed in BALF in 17 cases, 6 with Streptococcus pneumoniae, 6 with Haemophilus parainfluenzae, 3 with Moraxella catarrhalis, 2 with Staphylococcus aureus and 1 with Haemophilus influenzae. The symptoms were improved in all cases and co-amoxiclav was prescribed to most cases when discharged. The course of antibiotics therapy was 2-4 weeks in 23 cases, and more than 4 weeks in 8 cases. Twenty-three ( 23 ) cases were cured but 8 of them relapsed. Another 8 cases were improved but not completely remitted, 7/8 were cured by further treatment for concomitant diseases such as nasosinusitis and allergic rhinitis. Conclusions Children with PBB are typically younger than six years old, and presented with prolonged wet cough and parent-reported wheeze, normal or with ruttle in the lungs. A conifrmed diagnosis was reached by bronchoscopy. The antibiotics therapy were effective, the course should be more than 2-4 weeks, however, relapse were common. When antibiotics therapy does not lead to complete remission, concomitant diseases should be considered.

Trisomy 21 syndrome associated interstitial lung disease: a case report / 中华儿科杂志

<p><b>OBJECTIVE</b>To study the pathology, imaging and clinical features of a child with trisomy 21 syndrome associated interstitial lung disease.</p><p><b>METHOD</b>Data of a case with trisomy 21 syndrome associated interstitial lung disease confirmed by lung imaging and pathology were collected, analyzed and the related reports in literature were reviewed.</p><p><b>RESULT</b>The patient was a one year and 7 months old boy who suffered from severe pneumonia and recurrent infection during his hospital stay. When his disease was stable, he did not have shortness of breath and cyanosis, but a chest computed tomography (CT) showed ground-glass opacity, regional emphysema, band-like change in lung parenchyma, which indicated interstitial lung diseases. Unequal air inflation in bilateral lungs and diffuse over-distension of peripheral air spaces in lung surface were seen through thoracoscope. Pathological examination indicated that alveolar, alveolar ducts and alveolar sac were enlarged, alveolar septa was expanded. There were two reports in lung pathology of trisomy 21 syndrome, alveolar growth abnormalities was seen in 86%-88% cases. The multiple subpleural cysts in chest CT was characteristic. Clinically, trisomy 21 syndrome had high morbidity of respiratory tract infection and progress to respiratory failure frequently. Prolonged postoperative desaturation was constant which required long duration of respiratory support.</p><p><b>CONCLUSION</b>Trisomy 21 syndrome associated alveolar growth abnormalities were confirmed, which manifest as alveolar simplification in pathology and interstitial lung diseases in imaging. The risk of respiratory failure in these cases caused by infection and surgery should be considered.</p>

Clinical analysis of 4 children with negative pressure pulmonary edema / 中华儿科杂志

<p><b>OBJECTIVE</b>To analyze the clinical characteristics of negative pressure pulmonary edema (NPPE).</p><p><b>METHOD</b>A retrospective investigation of the clinical manifestation, imageology, clinical course and outcome of 4 children with NPPE seen between June 2012 and July 2013 in a children's hospital. The causation of the airway obstruction was also explored.</p><p><b>RESULT</b>All the 4 cases were boys, the range of age was 40 days to 9 years. They had no history of respiratory and circulatory system disease. In 3 cases the disease had a sudden onset after the obstruction of airway, and in one the onset occurred 1.5 hours after removing the airway foreign body. All these cases presented with tachypnea, dyspnea, and cyanosis, none had fever. Three cases had coarse rales. Chest radiography was performed in 3 cases and CT scan was performed in 1 case, in all of them both lungs displayed diffuse ground-glass-like change and patchy consolidative infiltrates. Three cases were admitted to the ICU, duration of mechanical ventilation was less than 24 hours in 2 cases and 39 hours in one. Oxygen was given by mask to the remaining one in emergency department, whose symptoms were obviously improved in 10 hours. None was treated with diuretics, glucocorticoids or inotropic agents. Chest radiographs were taken within 24 hours of treatment in 2 cases and 24-48 hours in the other 2; almost all the pulmonary infiltrates were resolved. All the 4 cases were cured. The causes of airway obstruction were airway foreign bodies in two cases, laryngospasm in one and laryngomalacia in the other.</p><p><b>CONCLUSION</b>NPPE is a life-threatening emergency, which is manifested by rapid onset of respiratory distress rapidly (usually in several minutes, but might be hours later) after relief of the airway obstruction, with findings of pulmonary edema in chest radiograph. The symptoms resolve rapidly by oxygen therapy timely with or without mechanical ventilation. In children with airway obstruction, NPPE should be considered.</p>