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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.
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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.
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Objective:To analyze the clinical characteristics of lower respiratory tract infection of influenza virus-related critical illness in children, and provide reference for early diagnosis and treatment of the disease.Methods:Thirty influenza virus-related critical cases of lower respiratory tract infection admitted to Shenzhen Children′s Hospital from October 2018 to October 2019 were enrolled in our study, and the clinical data were compared with influenza virus-related common severe cases with lower respiratory tract infection.Results:We reported that 63.33% of children with critical illness were less than five years old, and most of them were male(25 cases, 83.33%). The incidence of critical cases in the summer was significantly higher compared with common severe cases( χ2=7.050, P=0.008). In addition, pulmonary rales were the most common lung signs in critical cases( χ2=5.222, P=0.022), and the incidence of severe pneumonia increased significantly( χ2=32.651, P<0.001). Besides, we noticed that 60.00% of critical cases had respiratory failure, and the prevalence rates of plastic bronchitis among critical cases significantly increased( χ2=7.797, P=0.005). The absolute counts of neutrophil and the proportion of C-reactive protein in critical cases were higher than those in common severe cases ( Z=-3.805, P<0.001; χ2=5.833, P=0.016). We also found that 30.00% of critically-ill cases had Streptococcus Pneumoniae in lavage, of whom Epstein-Barr(EB) virus detection rate was higher than that of common severe cases ( χ2=6.909, P=0.009). Conclusion:Critically ill cases of lower respiratory tract with influenza virus infection are more common in children under five years old.These cases mainly occur in summer.When patients with lung rales, the absolute counts of neutrophil and the C-reactive protein increased significantly, and specifically with streptococcus pneumoniae and EB virus infection, which are more likely to progress to severe cases.Severe pneumonia, plastic bronchitis and respiratory failure need to be close monitored as the serious complications of critical illness.Early anti-influenza viral and anti-bacterial treatment, as well as active symptomatic life support can improve the prognosis of the disease.
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Objective@#To investigate the clinical efficacy of recombinant human tumor necrosis factor-α receptor II: IgG Fc fusion protein combined with sodium hyaluronate in the treatment of rheumatoid arthritis (RA).@*Methods@#Forty adult RA patients (12 males, 28 females) aged from 36 to 79 were selected in Department of Rheumatology of the Second People′s hospital of Datong from February 2016 to February 2018.The patients were randomly divided into the observation group and the control group according to the digital table, with 20 cases in each group.The observation group was treated by intra-articular injection of recombinant human tumor necrosis factor-α receptor II: IgG Fc fusion protein and sodium hyaluronate, and the control group was only treated with recombinant human tumor necrosis factor-α receptor II: IgG Fc fusion protein.The joint swelling, joint pain, joint mobility, morning stiffness, X-ray grade and total effective rate of the two groups were statistically analyzed before and after treatment.@*Results@#Before treatment, there were no statistically significant differences in joint swelling, joint pain, joint mobility, morning stiffness, clinical score and X-ray grade between the two groups (all P>0.05). After treatment, the symptoms and signs of the two groups were obviously improved.The joint pain, joint pressure pain, joint activity, morning stiffness and clinical score in the observation group were (1.2±1.2)points, (0.8±0.7)points, (0.5±0.4)points, (0.7±0.7)points and (4.5±2.6)points, respetively, which were significantly better than those in the control group [(2.2±1.4)points, (1.5±0.9)points, (1.5±0.9)points, (1.5±0.6)points and (8.6±4.6)points, U=125, 111, 68, 89 and 97, all P<0.05]. The total effective rate of the observation group was 100% (20/20), while that of the control group was 75% (15/20), there was statistically significant difference between the two groups (χ2=14.10, P<0.01).@*Conclusion@#The injection of recombinant human tumor necrosis factor-α receptor II: IgG Fc fusion protein combined with sodium hyaluronate in the treatment of RA can effectively improve the clinical symptoms of RA patients, and it is more effective than single use.It is worthy of popularizing in clinic.
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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.
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Objective To investigate the effect of recombinant human interleukin 11(rhlL-11)in the treatment of idiopathic thrombocytopenic purpura(ITP)on the levels of Th1,Th2 and the expression of their transcription factors T-bet mRNA,GATA-3 mRNA.Methods Fifty-six cases adult ITP patients hospitalized in the department of hematology of the Second People's Hospital of Datong from May 2015 to December 2016 were collected,including 21 males and 35 females,aged 29~73 years; 10 healthy people in the same period were enrolled as control group,4 males and 6 females,aged 20~52 years.Th1 and Th2 cell ratio and Th1/Th2 ratio of ITP patients were detected by flow cytometry before and after treatment.The expression levels of transcription factor T-bet and GATA-3 were measured using real-time fluorescence quantitative reverse transcription polymerase chain reaction(RT-PCR)before and after treatment.Results The effective rate of rhlL-11 in ITP treatment was 76.8%(43/56).For the effective patients,the median PLT after treatment increased(25.0(15. 0,36.0)×109/L vs.68.0(49.0,108.0)×109/L,Z=-5.712,P<0.001); Th1 cells decreased,compared with that before the treatment(14.8 %(12.6%,17.6%)vs.10.6 %(9.8%,12.6%),Z=-4.825,P<0.001);Th2 cell increased,compared with that before the treatment(0.4%(0.3%,0.5%)vs.1.2%(0.9%,1.4%), Z=-5.720,P<0.001); Th1/Th2 decreased,compared with that before the treatment(40(30,49)vs.10.6(7.8,12.0),Z=-5.711,P<0.001];the expression level of T-bet mRNA decreased(0.36(0.18,0.51)vs 0.09(0.05,0.13),Z=-2.668,P=0.008);the expression level of GATA-3 mRNA increased,compared with that before treatment(0.04(0.03,0.05)vs.0.12(0.09,0.15),Z=-2.366,P=0.018).For ineffective patients,the median PLT before treatment was(11.0(8.0,15.5)×109/L),and the median PLT after treatment was(15.0(10.0,19.5)×109/L)(Z=-3.027,P=0.002); there was no significant difference in Th1,Th2, ratio of Th1/Th2 and T-bet and GATA-3 mRNA expression level before and after treatment in patients with ITP (P>0.05).Conclusion rhIL-11 can effectively correct the imbalance in Th1 and Th2 cells and the imbalance of T-bet and GATA-3 in ITP patients,but it has no obvious therapeutic effect on a small number of patients
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X-linked adrenoleukodystrophy is an X-linked recessive genetic disease that mainly affects adrenal and white matter,mostly onset in childhood or adolescence,mainly manifested as progressive psychomotor disorder,visual and hearing loss and/or adrenocortical dysfunction,etc.A case of adult X-linked adrenoleukodystrophy in our hospital is reported and analyzed.The patient's early performances were unclear speech and low emotions,and no obvious positive signs,repeatedly diagnosed as "neurosis".Then gradually emerged drinking cough and unsteady walking,and physical examination showed brainstem and cerebella positive signs.Brain MRI was characterized by symmetrical lesions of bilateral cerebellar hemisphere,bilateral pontine arm,brainstem and pyramidal tract of internal capsule posterior limb,which was reconsidered as " spinal cerebellar degeneration" Finally,the disease was confirmed by gene examination as adrenoleukodystrophy.By summarizing the characteristics of the patient,we hope to provide diagnostic reference for patients with atypical adrenoleukodystrophy.
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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.
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Objective To investigate the distribution of surfactant protein-B(SP-B) gene single nucleotide polymorphisms and to clarify the correlation between SP-B gene polymorphisms and idiopathic interstitial lung disease(ILD) in children.Methods Sixty-seven children with idiopathic ILD(case group) and 102 children without idiopathic ILD(control group)were selected from October 2013 to September 2016 in Shenzhen Children's Hospital and the First Affiliated Hospital of Guangxi Medical University.Total exons and flanking region of SP-B were detected by high-throughput sequencing,genotype and allele distribution of exon 4(T131I)were analyzed.Results SP-B exon 4(T131I) genotypes could check out three genotypes:namely CC,CT and TT.The frequencies of genotype CC,CT and TT of exon 4(T131I) in the case group were 67.16%,25.37%,7.46%,and in the control group were 56.86%,35.29%,7.84%,respectively.There was no significant difference in genotype distribution between the two groups(χ2=1.981,P=0.371).Frequency of allele C was 79.85% in the case group and 74.51% in the control group,no significant difference showed between the two groups(χ2=1.288,P=0.256).In the control group,the mutation frequency of SP-B exon 4(T131I) was 43.14%(44/102),compared to the frequency of mutations in the population data in the thousands of human genome programs was 52.00%,in European was 53.88%,in South Asia was 45.50%,and in American was 41.93%(P>0.05);but the frequency of gene mutations was 26.39% in East Asia and 80.18% in Africa,there were significant differences compared to the control group(P<0.05).Conclusion The genetic polymorphism of SP-B exon 4(T131I)is not correlated with the susceptibility of idiopathic ILD in children.The mutation frequency of SP-B exon 4(T131I)is related to the race and the region.
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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.
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Objective:To explore the clinical characteristics, mechanism and countermeasures of drug-induced second thrombocy-tosis ( ST) in order to ensure safe medication. Methods:Drug-induced ST published during 1996 and 2016 in domestic and overseas medical journals was collected and statistically analyzed in respects of age, gender, kind and distribution of drug-induced ST, treatment and outcome of ST. Results:Totally 198 cases were collected, which involved 30 different drugs of eight types mainly including antitu-mor drugs, blood system drugs and antibiotics. Among the patients, 112 ones (56. 6％) were male and the other 86 ones (43. 4％) were female. Totally 14 patients had embolization with the overall incidence of 7. 0％. Conclusion:Without timely and favorable treat-ment, drug-induced ST may lead to thrombosis. To ensure the safety of medication, platelet level should be monitored regularly during medication.
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Objective To investigate the clinical value of the peripheral blood monocyte human leukocyte antigen-DR (mHLA-DR) for assessment of degree of severity and the diagnosis of acute pancreatitis (AP). Methods A case-control study was conducted. Eighty-six AP patients admitted to Shandong Liaocheng People's Hospital from June 2014 to May 2015 were enrolled. Patients were classified into four groups [mild (n = 33), moderate (n = 25), severe (n = 16), critical (n = 12)] according to the disease classification. Eighty healthy persons subjected to physical examination center of our hospital at the same time were served as controls. Acute physiology and chronic health evaluation Ⅱ (APACHE Ⅱ) scores in patients were estimated. Flow cytometry was used to measure the expression of the peripheral blood mHLA-DR, and the Pearson method was used to analyze the relationship between the level of mHLA-DR and the APACHE Ⅱ score. The receiver-operating characteristic curve (ROC) was plotted, and then the clinical value of the peripheral blood mHLA-DR was analyzed for the diagnostic value in AP patients. Results The expression of the mHLA-DR in patients with AP was significantly lower than that of healthy control group [(63.7±18.6)% vs. (86.4±8.3)%, t = 5.319, P < 0.001]. The expression levels of the mHLA-DR in mild group, moderate group, severe group, and critical group were (79.6±6.5)%, (66.4±9.4)%, (49.9±8.1)%, (32.5±12.0)%, respectively, and the APACHE Ⅱ score were 4.67±1.99, 5.88±2.05, 9.06±2.62, 12.33±3.96, respectively. Pair wise comparisons were statistically significant (all P < 0.05). The HLA-DR expression level in the peripheral blood of patients with AP was negatively correlated with the APACHE Ⅱ score (r = -0.695, P < 0.001). The area under the ROC curve (AUC) of mHLA-DR expression in peripheral blood for AP was 0.894 [95% confidence interval (95%CI) = 0.847-0.941, P < 0.001], and the cut-off point was 84.40%, with the sensitivity of 75.0%, the specificity of 90.7%, and the accuracy rate of 83.1%. The AUC of mHLA-DR expression for mild AP was 0.938 (95%CI = 0.889-0.987, P < 0.001), and the cut-off point was 72.70%, with the sensitivity of 87.9%, the specificity of 88.7%, and the accuracy rate of 88.4%. The AUC of mHLA-DR expression for severe and critical AP was 0.943 (95%CI = 0.881-1.005, P < 0.001), and the cut-off point was 57.85%, with the sensitivity of 84.0%, the specificity of 96.4%, and the accuracy rate of 90.6%. Conclusions The expression levels of the peripheral blood mHLA-DR in AP patients can reflect the degree of disease, and contribute to the diagnosis of AP. The value of mHLA-DR may be used as a new biological indicator in the diagnosis and assessment for the severity of AP.
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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.
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<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>
Subject(s)
Humans , Infant , Male , Cysts , Pathology , Down Syndrome , Lung , Pathology , Lung Diseases, Interstitial , Diagnosis , Postoperative Period , Pulmonary Alveoli , Pathology , Respiratory Insufficiency , Respiratory Tract Infections , Tomography, X-Ray ComputedABSTRACT
<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>
Subject(s)
Child , Child, Preschool , Humans , Infant , Male , Acute Disease , Airway Obstruction , Foreign Bodies , Intensive Care Units , Intubation, Intratracheal , Methods , Laryngismus , Larynx , Lung , Diagnostic Imaging , Pathology , Oxygen Inhalation Therapy , Positive-Pressure Respiration , Methods , Pulmonary Edema , Diagnosis , Therapeutics , Radiography, Thoracic , Retrospective Studies , Tomography, X-Ray ComputedABSTRACT
<p><b>OBJECTIVE</b>To analyze the clinical characteristics of Mycoplasma pneumoniae-associated hemophagocytic syndrome (MP-HLH).</p><p><b>METHOD</b>A retrospective investigation of the clinical manifestation, laboratory test, imagelogy, clinical course and outcome of 3 cases with MP-HLH seen between June 2013 and July 2013 in Shenzhen Children's Hospital, and review of relevant literature were conducted.</p><p><b>RESULT</b>Of the 3 cases of MP-HLH, 2 were males, one was female, the ages were 1 year, 3 years and 6 years, respectively. They had no underlying disease previously. All the 3 cases had onset of fever, cough as main symptoms. Diagnosis of refractory Mycoplasma pneumoniae pneumonia was made, which was accompanied by decreased neutrophils [(0.08-0.68)×10(9)/L], hemoglobin [(79-103) g/L], platelet [(64-157)×10(9)/L], plasma fibrinogen [(1.3-1.5) g/L], lactate dehydrogenase [(1,170-1,285) U/L] and increased serum ferritin [(936.7-39 789.0) µg/L] in the third week of course. In two cases the T lymphocytes decreased, and the NK cell activity decreased significantly in one. Bone marrow cytology showed prompted bone marrow hyperplasia, and the phenomenon of phagocytosed blood cells. CT scan was performed for all the cases and consolidation with pleural effusion were shown. Two cases were admitted to PICU, and required endotracheal intubation and mechanical ventilation. Flexible bronchoscopy and bronchial lavage were performed and bronchial cast was found in two cases. All of them were treated with macrolide combined with other antibiotics, glucocorticoids and gamma globulin combination therapy, including one case given dexamethasone [10 mg/(m2·d)], cyclosporine[6 mg/(kg·d)], etoposide [150 mg/(m2·d)] chemotherapy. Two cases were cured, and 1 case died. The authors summarized the 18 cases reported in domestic and foreign literature. Foreign children were diagnosed and treated with steroids in 1-2 weeks, and 10 cases were cured, and 2 cases died. They died of massive hemorrhage and meningoencephalitis, and domestic children were diagnosed and treated within two to 4 weeks after onset, 5 cases were cured, one case died of severe pneumonia.</p><p><b>CONCLUSION</b>MP-HLH is a rare disease in children, and had acute onset, rapid progression and high mortality. Early treatment with steroids was associated with a good prognosis, the key to successful treatment is early diagnosis and treatment, avoiding the immune cascade. Too late a diagnosis or development of serious complications may lead to death.</p>