ObjectiveTo clarify the value of ultrasound, hepatobiliary scintigraphy and magnetic resonance cholangiopancreatography(MRCP) in the diagnosis of biliary atresia. MethodsAccording to search terms of biliary atresia and bile duct atresia, English literature was searched in Pubmed，Embase，Medline from the establishment of databases to April 26, 2020 and Chinese literature was searched in CBM from January 1, 2000 to April 26, 2020. Retrieved articles were screened and selected according to the inclusion and exclusion criteria. The QUADAS-2 was used to evaluate the risk of bias and applicability concerns. Data were extracted and diagnostic parameters were calculated for the combined data. ResultsThirty studies on ultrasound were included. The sensitivity and specificity of triangular cord sign (24 articles involved) were 79% (95%CI: 68%-86%) and 97% (95%CI: 94%-98%) respectively with I2>97%, and the diagnostic odds ratio (DOR) was 103 (95%CI: 52-203). The SROC curve suggested that the AUC was 0.97 (95%CI: 0.95-0.98), and the funnel plot indicated little publication bias (P=0.28). The sensitivity of abnormal gallbladder morphology (9 articles involved), gallbladder size (12 articles involved), absence of common bile duct (5 articles involved) and hepatic artery diameter (6 articles involved) was 73%, 78%, 92% and 83%, respectively and the specificity was 94%, 76%, 76% and 78% respectively with I2>70%, and DOR was 42, 11,12, and 17, respectively. The SROC curve suggested that AUC was 0.94, 0.81, 0.92 and 0.87, respectively. Thirty-two studies on hepatobiliary scintigraphy were included. The sensitivity and specificity were 98%(95%CI: 95%-99%) and 75%（95%CI: 69%-81%）respectively with I2＞75% and DOR=140（95%CI: 50-392）. The SROC curve suggested that the AUC was 0.93 (95%CI: 0.91-0.95), and the funnel plot indicated little publication bias (P=0.05). In subgroup analysis of liver enzyme inducer before examination (18 articles involved), technetium contrast agent (28 articles involved), detection of contract in intestine only (18 articles involved), and detection of contract in intestine or bile duct (11 articles involved), the sensitivity ranged from 97% to 99%, and the specificity ranged from 75% to 78% with I2>75%, and DOR was 239, 251, 246 and 109, respectively. Seven studies on MRCP were included. The sensitivity and specificity were 92% (95%CI: 79%-97%) and 82% (95%CI: 63%-92%) respectively with I2 of 75% and 83%, and the DOR was 52 (95%CI: 7-382). The SROC curve suggested that the AUC was 0.94 (95%CI: 0.91-0.96). ConclusionCompared with other ultrasonic features (abnormal gallbladder morphology, gallbladder size, absence of common bile duct and hepatic artery diameter), the triangular cord sign detected by ultrasound is more accurate in the diagnosis of biliary atresia. The misdiagnosis rate of hepatobiliary scintigraphy is still high by liver enzyme inducer before examination, technetium contrast agent, detection of contract in intestine or bile duct. MRCP is not stable in terms of diagnosis accuracy of biliary atresia.
ObjectiveTo study the spectrum of neonatal neuromuscular diseases (NMDs), to explore the clinical and laboratory characteristics, and to provide a basis for the diagnosis of NMDs. MethodsThe clinical data of children with confirmed NMDs admitted to the Department of Neonatology, Children's Hospital of Fudan University from January 1, 2016 to December 31, 2018 were retrospectively analyzed, including clinical presentations, routine laboratory examinations, muscle biopsy (MB), next-generation sequencing (NGS) and prognostic outcome, etc. ResultsA total of 25 cases were enrolled in the study. There were 14 males and 11 females. Nine cases were premature and 16 cases were full term infants. The gestational age was 27+4 to 41+5 weeks (median: 38+3 weeks), and the birth weight was 980 to 4,050 g (median: 3,000 g). Fifteen cases (60%) died, 8 cases (32%) survived, and 2 cases (8%) were lost to follow-up. The postmenstrual age of MB was 32+1 to 51+4 weeks (median: 41+3 weeks). The most common clinical presentations in the neonatal period were hypotonia 24 cases (96%), requiring nasogastric feeding (20/22 cases, 90.9%) and respiratory support 22 cases (88%) after birth. Most of them were neuromuscular-related diseases (64%). NGS was positive in 23 cases (92%) with 56% of neuromuscular disease-related genes. MB was positive in 19 cases (76%) with 52.6% of congenital myopathy. Among 15 cases of death, 86.7% was positive for NGS with 53.3% of neuromuscular disease related genes and 73.3% was positive for MB cases with 40% of congenital myopathy. ConclusionThe onset of NMDs in neonatal period was not obvious and the clinical manifestations were quite different and lack of specificity. Detailed medical history, physical examination, MB pathology combined with NGS could improve the diagnosis of NMDs, and provide a basis for the early diagnosis and treatment of NMDs.
ObjectiveTo explore the clinical characteristics of growth and development, and the factors influencing the catch-up growth of pediatric recipients after kidney transplantation. MethodsFrom July 2017 to November 2019, Guangzhou Women and Children's Medical Center and the First Affiliated Hospital of Sun Yat-sen University cooperated to carry out children's kidney transplantation. A retrospective analysis of the children's follow-up medical records who had received the transplantation operation included growth indexes of height growth rate (Δ HtSDS) and last height standard deviation (HtSDS), survival indicators of serum creatinine, serum albumin, Hemoglobin, serium calcium, serium phosphorus, urine protein/creatinine, urine RBC and blood pressure, complications and medication conditions such as glucocorticoids, immunosuppressants, antihypertensive drugs and calcitriol, etc. ResultsEighteen cases were involved in the study with the median follow-up time of 17.5（11, 24.5） months. Data showed that Δ HtSDS was 0.8 (0.1, 1.2) with the catch-up rate of 61% during the 1st post-transplant year, and at the 2nd year Δ HtSDS was -0.1 (0.3, 0.4) and the last HtSDS during the follow-up was -1.94±0.99 with 50% of control rate. The baseline HtSDS correlated positively with the terminal HtSDS during the follow-up （r=0.64,P=0.005） and correlated negatively with Δ HtSDS at the 12th month of follow-up (r=-0.61，P=0.008). The patient and allograft survival rates were 100% during the follow-up, and there were 8 cases of complications, including 3 cases of severe infection, 4 cases of antibody mediated rejection and 1 case of primary nephropathy recurrence. The cumulative amount of glucocorticoid (GC) was (143.6±86.6) mg·kg-1, and the daily dose was (0.30±0.15) mg·kg-1. Ten cases were treated with antihypertensive drugs and 9 cases were treated with calcitriol. Multiple factor analysis showed that for catch-up growth, HtSDS of baseline, transplantation age and blood pressure were risk factors, while the use of antihypertensive drugs was an independent protective factor. ConclusionThe height at the end of follow-up after kidney transplantation was significantly improved compared with that before transplantation. The lower HtSDS of the pre-transplantation，the younger transplantation age and the lower normal blood pressure were the favorable factors for promoting Δ HtSDS of children after transplantation. The use of antihypertensive drugs was an independent protective factor for post-transplantation catch-up growth .
ObjectiveTo investigate whether children with a risk of bleeding are prone to hematoma at the puncture site during lumbar puncture. MethodsThe medical records of hospitalized children who had undergone lumbar puncture and had abnormal coagulation function (mainly referring to the risk of bleeding) were retrospectively collected from Beijing Children's Hospital of Capital Medical University, and the medical records with hospitalization time ≤24 h were excluded. We investigated whether the hematoma at the puncture site was prone to occur within 3 days after lumbar puncture when there was a risk of bleeding. The indicators of bleeding risk included: a. there were signs or symptoms of bleeding; b. within 3 days before lumbar puncture, anticoagulation treatment, platelet transfusion or plasma transfusion had been used; c. platelet count (PLT) and fibrinogen (FIB) is lower than the lowest limit of normal value, and prothrombin time (PT), partial thromboplastin time (APTT), antithrombin Ⅲ (AT-Ⅲ) activity and international standardized ratio (INR) were above the highest limit. Risk of bleeding was defined as a/b and c. ResultsAccording to the first page of our hospital discharge medical records with lumbar punctures and discharge after 24 hours from January 1st, 2014 to July 30th, 2019, a total of 232 children with 370 lumbar punctures were included. Among the 232 children, the age ranged from 4 hours to 17 years and 8 months, with a median age of 3 years. Among the 370 cases of lumbar puncture, the main indications were suspected various infectious diseases of the central nervous system (209 cases, 56.5%) and the need for intrathecal injection (142 cases, 38.4%). There were no cases with absolute contraindication of lumbar puncture. None of children with abnormal bleeding risk indicators had hematoma at the puncture site within 3 days after lumbar puncture even if they had signs of bleeding / history of anticoagulation drugs and PLT transfusion, and any of the following 6 laboratory indicators of bleeding risk——PLT 1-97(53±30)×109·L-1、PT 13-24.8(15.7±3.4)s、APPT 40.4-157.9(60.2±20.2)s、INR 1.21-2.11(1.53±0.34)、FIB 0.68-1.99(1.34±0.40) g·L-1、AT-Ⅲ 130-158.6(144.3±11.7) %. ConclusionThe bleeding risk at the puncture site was extremely low in children with hemorrhagic manifestations, anticoagulation or blood transfusion therapy before lumbar puncture, and abnormal laboratory bleeding indicators.
Objective:To investigate the growth status and influencing factors of growth retardation in children with juvenile idiopathic arthritis (JIA). Methods：The JIA patients with height-for-age Z-score (HAZ) <-2 SD during the follow up were enrolled as the case group, and patients with normal height (HAZ≥-2 SD) were enrolled as the control group. The factors affecting the growth of patients were investigated by a self-made questionnaire with 21 questions in 5 aspects including basic information, disease history, diet, lifestyle habits and family conditions. As rheumatologists performed diagnosis and follow-up treatment for children with JIA in the outpatient clinic, the child healthcare doctors carried out a face-to-face questionnaire survey and physical measurements during follow-up visits. Univariate and multivariate logistic regression models were used to analyze the risk factors of growth retardation in JIA patients after integrating the results of questionnaire in terms of age of onset, symptoms and physical signs, medications, diagnostic intervals, disease course, parents' education level and job. Results：From September 2018 to April 2019, 221 children with JIA who met the eligibility criteria were included in this analysis, including 103 males (46.6%) with the mean age of (7.8 ±3.7) years. JIA subtypes included oligoarthtiris (48.9%, n=108), systemic onset (26.2%, n=58), rheumatoid factor-negative polyarthritis (7.2%, n=16), rheumatoid factor-positive polyarthritis (16.3%, n=36), enthesitis-raleted arthritis (1.0%, n=2) and psoriatic arthritis (0.5%, n=1). Eighty-eight patients were treated by glucocorticoid (GCs), 72 were treated by biological preparations, and 61 were treated by other medicine. Thirty-three children (14.9%) were combined with growth retardation, and the HAZ among the subtype of oligoarthtiris, systemic-onset and polyarthritis was (-0.34 ±1.09) vs (-1.53 ±1.50) vs (-0.80 ±1.19), respectively. Univariate logistic regression showed clinical course, disease activity, glucocorticoid hormone therapy, pickiness, less outdoor activity time were factors influencing the linear growth of JIA. Multivariate logistic regression analysis showed that the GCs therapy(OR=7.227, 95%CI:1.877-27.817), the course more than 3 years(OR=4.278, 95%CI: 1.322-13.843) and outdoor activities less than 1 hour(OR=4.078, 95%CI: 1.252-13.288) were independent predictors of growth retardation in children with JIA (P＜0.05). Conclusion：Growth retardation was ubiquitous in JIA patients. The long disease course, GCs therapy and less outdoor activity time were main risk factors affecting the linear growth of JIA patients.
Objective:To summarize the echocardiographic features and clinical treatment experience of cardiac tumors in children and adolescents. Methods:The hospitalized cases of cardiac tumors between January 2013 and January 2020 were selected from Pediatric Cardiovascular Center of Beijing Anzhen Hospital Affiliated to Capital Medical University. The echocardiographic features and clinical data were retrospectively analyzed and summarized. Results:A total of 24 patients were included into the analysis. Complete tumor resection was performed in 20 patients and partial resection was performed in 4 patients. Postoperative pathology showed benign lesions accounted for 79.2% (19/24), malignant lesions for 4.2% (1/24) and tumors of uncertain biological behavior for 16.7% (4/24). Among them, there were 7 cases of myxoma, 6 cases of rhabdomyomas, 4 cases of inflammatory myofibroblastoma, 3 cases of fibroma, 1 case of fibrosarcoma，teratoma，hemangioma and thrombus for each. The right ventricle and left atrium were main sites of cardiac tumors. Diagnosis accuracy of echocardiography for cardiac tumors was 29.2% (7/24), and it was 85.7% (6/7) for myxoma due to the high specificity of echocardiography. The echocardiographic features of myxoma included low to moderate echoes, lobulation, irregular shapes, loose structures, high mobility, and attached pedicles.Shapes of myxoma changed with the contraction and relaxation of the heart, and the tumor was embedded in the atrioventricular valve opening to different degrees during diastole, causing secondary stenosis of the atrioventricular valve. Conclusion:Benign tumors are more common in children and adolescents. The common cardiac tumors are rhabdomyomas and myxoma. Diagnosis accuracy of echocardiography is high for myxoma.
Objective:To analyze the echocardiographic characteristics of anomalous left coronary artery from the pulmonary artery (ALCAPA) with normal left ventricular systolic function. Methods:The echocardiographic characteristics and clinical data of 13 ALCAPA patients with left ventricular ejection fraction (LVEF) ≥55% were retrospectively analyzed. All of the included 13 patients were admitted to Guangzhou Women and Children's Medical Center from January 2010 to December 2019. Results:Right coronary artery dilation was revealed in all of the 13 patients. Other echocardiographic markers included left coronary artery flow reversal (12 cases), visualization of coronary artery origin from the pulmonary trunk (9 cases), abnormal doppler signal in pulmonary trunk (7 cases), collateral vessels in myocardium (8 cases), left ventricular enlargement (8 cases), mitral regurgitation (7 cases) and endocardial fibroelastosis (5 cases). Patients ＜1 year were more likely to be lack of collateral coronary artery circulation，and showed normal or mild enlarged left ventricle with normal mitral function, while patients ≥1 year always showed significant collateral formation, dilated left ventricle and mitral dysfunction. In this study, four misdiagnosed patients showed that the left coronary artery originated from the right-rear side of pulmonary artery. Conclusion：The echocardiography can diagnose ALCAPA with normal left ventricular systolic function accurately. The rate of misdiagnosis and missed diagnosis will be reduced if thorough echocardiographic interrogation of coronary artery origins and flow is carried out.
CAI Jie-hao, XIA Ai-mei, WANG Xiang-shi, ZENG Mei, WANG Jia-li, TIAN He, CHANG Hai-ling, ZHANG Jin-qiang, WEI Zhong-qiu, LI Jing-jing, LU Xiao-xiao, WANG Jian-she, ZHU Qi-rong, GE Yan-ling, ZHAO Jun
Objective:To understand the epidemiological and clinical features of the 38 imported pediatric cases of SARS-CoV-2 infection in Shanghai. Methods:The clinical data were collected from the 38 imported pediatric cases with laboratory-confirmed SARS-CoV-2 infection from March 14 to April 8 2020. The epidemiological history, clinical presentations, laboratory findings, chest radiological findings, treatment and outcomes were analyzed. Results:Of the 38 imported cases, 16 (42.1%) were asymptomatic, 14 (36.8%) had non-severe pneumonia, and 8 (21.1%) had mild illness. There were 37 cases (97.4%) from Europe and North America, 26 cases (68.4%) without a definitive contact history, and 12 cases (31.6%) linked with family cluster infection or exposure to suspected cases in school dormitory. There was no statistical difference between the pneumonia group and the non-pneumonia group in terms of gender and age（P>0.05）. The 14 pneumonia cases showed absorption of lung lesions by follow-up chest radiology, and the 16 asymptomatic cases did not develop any symptoms and radiological abnormalities during hospitalization. Seven cases had fever with a mean duration of 2.5 days, 11 had mild cough, 5 had sore throat, and 4 had runny nose. Hydroxychloroquine was used in the 6 cases for 1-6 days. All cases recovered uneventfully with a mean length of hospitalization of 18.5±3.4 days. Conclusion:Asymptomatic infection was common among the imported pediatric cases of COVID-19 from abroad. There was no obvious difference of the laboratory findings between the pneumonia group and the non-pneumonia group. Routine re-detection of radiography is not recommended for asymptomatic and mild cases.
Objective:To understand the pathogenic variation spectrum and the hotspot variation of COL7A1 gene in epidermolysis bullosa (EB), and to analyze the correlation between different inheritance patterns of COL7A1 gene and the severity of skin lesions in neonatal period. Methods:We collected neonates diagnosed as EB caused by COL7A1 gene pathogenic or likely pathogenic variants detected by high-throughput sequencing from Department of Neonatology of Children's Hospital of Fudan University.The clinical manifestations of them were summarized, and the phenotype-genotype correlation was analyzed. Results:A total of 28 EB newborns were enrolled including 5 cases of autosomal dominant(AD) inheritance and 23 cases of autosomal recessive(AR) inheritance. There was no significant difference between AD and AR mode EB in skin lesions, skin loss and other clinical findings. There was no statistically significant difference between the initial stage and the extreme stage of skin lesions in AD mode EB (χ2=0.668, P=0.414). The skin lesions in AR mode EB at the initial stage were significantly different from those at the extreme stage (χ2=16.896, P<0.01). There was no significant difference in the percentage of skin lesions in AD mode EB and AR mode EB in the initial and extreme stages of disease (P=0.131, P=0.071). Fifty-four COL7A1 pathogenic variants were detected, including 30 (55.6%) known pathogenic variants, 21 novel pathogenic variants and 3 with unclear clinical significance. The hotspot pathogenic variants were c.8569G>T(p.E2857X) in 4 cases, c.3625_3636delinsC(p.S1209Lfs*6) in 3 cases and c.4089_4090delinsC(p.P1364Lfs*35) in 3 cases. Conclusion:It has no characteristic changes in skin lesions, skin loss and other clinical findings between different inheritance (AR or AD) in EB caused by pathogenic variation of COL7A1 gene in neonatal period. The scope of skin findings of AR mode in the extreme stage is more extensive than that in the initial stage.
Objective：To evaluate the efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in the treatment of children with hyper-IgE syndrome （HIES）caused by DOCK8 gene mutation. Methods：Clinical features, laboratory data, treatment and outcome of 2 patients with HIES caused by DOCK8 gene mutation treated by allo-HSCT were retrospectively analyzed. Results：There was a boy and a girl in 2 cases with HIES. The age of allo-HSCT was 7 for the boy and 9 for the girl. The boy received matched sibling donor allo-HSCT, and the girl received matched unrelated umbilical cord blood transplantation. Reduced-intensity conditioning regimens based on Busulfan and Fludarabine were used for the 2 patients. Both patients were successfully engrafted and achieved hematopoietic reconstruction. After allo-HSCT, neutrophil engraftment occurred on day 12 and 24, and platelet engraftment occured on day 13 and 35 for the boy and girl, respectively. Both patients showed stable complete donor chimerism 14 days after allo-HSCT. Pulmonary infection in both patients aggravated during transplantation, and conditions were improved with active therapy. The boy who received matched sibling donor allo-HSCT suffered grade Ⅰ acute skin graft-versus-host disease (GVHD), and his condition was alleviated after the methylprednisone treatment. No other complications were reported. The levels of eosinophils and serum IgE were much lower than those before transplantation. Two children survived disease-free for 18 months and 23 months after transplantation. Conclusion：Children with HIES caused by DOCK8 gene mutation can be cured by allo-HSCT. It is advisable to adopt a reduced-intensity conditioning regimen. Matched sibling donors are the best choice for allo-HSCT, and unrelated umbilical cord blood stem cell transplantation can also achieve good results.
Objective:To investigate the deviation of physical growth status of children with autism spectrum disorder (ASD). Methods:Data were collected from the specialty medical record database of ASD diagnosed by qualified developmental behavior specialists from Children's Hospital of Fudan University from January 2016 to August 2019. The data were collected from routine physical measurement at the time of suspicion for (<2 years old) and diagnosis of ASD. Children with suspected ASD were followed up to be diagnosed at the age of 2. Anthropometric parameters (weight, length/height, BMI) were converted to Z-scores for age using the WHO Anthro. BMI Z-score > +2 SD refers to obesity, and BMI Z-score < -2 SD refers to emaciation. Obesity and emaciation together are defined as deviation. Influencing factors of physical growth deviation were analyzed. Results:A total of 511 consecutive ASD samples from the ASD medical record database were analyzed, including 420 cases (82.2%) aged 1 to 4 and 91 cases (17.8%) aged 5 to 8. There were 431 males and 80 females. The proportion of obesity in all included ASD patients and the group of 1-4 years old was higher than that of emaciation (P<0.05). There was no significant difference between the ratio of obesity and emaciation in the group of 5-8 years old. There was no significant difference in obesity and emaciation ratio between boys and girls with ASD. The incidences of growth deviation and emaciation in the group of 5-8 years old were 3.3 times (95%CI: 1.81-6.00) and 5.64 times (95%CI: 2.22-14.31) higher than that in the group of 1-4 years old. Compared with the group of 1-4 years old , the Z-score distribution curves of weight, length/height and BMI shifted to the right for all included ASD patients against the reference line of Z-score normal distribution, showing a trend of obesity. The distribution curve of BMI Z-score in ASD children aged 5-8 years had a low peak, and the proportion of body symmetry decreased. It extended to over ±2 SD, and deviated to obese and wasted growth status. Conclusion:The deviation of physical growth status of children with ASD included both obesity and emaciation. School-age children with ASD had a higher incidence of physical growth deviation, a lower ratio of fit-shape figure, and deviated to obesity and emaciation.
Objective:To investigate the effect of delayed infliximab infusion on the disease activity index among children with inflammatory bowel disease during the SARS-CoV-2 epidemic. Methods:This is a multicenter cross-sectional study. Children from 19 hospitals in China who received infliximab infusion for inflammatory bowel disease between January 20th and March 20th were enrolled in the study. Patients whose disease stages were deteriorated according to PCDAI/PUCAI were categorized as case group. Others were in control group. The impact of age, sex, the average length of postpone infusion, WBC count, Hb level, PLT count and CRP level on disease stage were analyzed. Results:Among 232 patients who should receive scheduled infliximab infusion, 67 cases (28.9%) had their infusion delayed due to the epidemic including 2 patients of discontinuing the therapy and losing follow up and 65 patients of delaying the infusion with 9 lost cases. A total of 56 patients (27 males, 29 females, with average age of 11.3±4.1 years) were finally included in the study. The average length of infusion postponement was 17.9±9.9 days. There were 11 patients (3 with induction therapy and 8 with maintenance therapy) in case group and 45 patients (2 with induction therapy and 43 with maintenance therapy) in control group. WBC count, Hb level, PLT count, CRP level and disease activity index were not significantly changed in patients who delayed the infusion (P was 0.44，0.60，0.80，0.34 and 0.33, respectively) compared with that before the epidemic. Disease stages of 11 patients were deteriorated, 6 of which needed hospitalization. There was no case of surgery or death. Conclusion:Postponing infliximab infusion for three weeks during maintenance therapy did not change the disease activity index among children with inflammatory bowel disease.
Objective:To investigate the clinical characteristics, diagnosis, treatment and prognosis of mycoplasma pneumoniae-associated hemophagocytic syndrome (MP-HPS). Methods:The clinical data of MP-HPS children admitted to Shenzhen Children's Hospital between June 1, 2013 and December 31, 2018 were retrospectively analyzed. Results：The median age of 11 patients with MP-HPS was 3 years and 3 months.There were 5 cases aged younger than 3 years, 6 males and 5 females. Clinical manifestations included fever in 11 cases, cough in 8 cases, diarrhea in 3 cases, and poor mental health in 1 case. All patients had large liver, and large spleen was found in 6 cases. Laboratory examination results showed all of 11 patients had different degrees of hemocytopenia, elevated serum ferritin (≥ 500 μg·L-1) and lactate dehydrogenase.Bone marrow images suggested hemophagocytosis in 11 cases. There were 8 cases of abnormal liver function, 9 cases of high triglyceride, 6 cases of low fibrinogenemia, and 8 cases of decreased NK cell activity. MP infection was confirmed by MP-IgM MP-DNA tests in all 11 cases with 2 cases of combined bacterial infections and 2 cases of fungal infections. No other viral infections were detected. Imaging examination (chest X-ray and chest CT) results showed all of 11 cases presented pneumonia, 7 cases were complicated with pleural effusion, and 1 case was complicated with mediastinal emphysema and subcutaneous emphysema of the neck and chest. Seven cases underwent bronchoscopy and 2 cases had plastic bronchitis. Macrolides were combined with other antibiotics. Glucocorticoid and gamma globulin were used in 10 patients, immunosuppressant (cyclosporin) was used in 4 patients, and cyclosporin + etoposide was used in 1 patient. Among the 7 patients admitted to PICU, 5 were intubated. Nine cases were discharged after improvement and 2 cases died. Conclusion:MP-HPS should be diagnosed early and treated actively. Cytokine storm should be blocked in time to improve the prognosis of children.
