Background：With an increasing number of very preterm infants (VPI, <32 weeks' gestation) treated in China, central vascular catheterization has become a common technique in Chinese neonatal intensive care units (NICUs). Inappropriate use of central vascular catheters(CVCs) may occur in the treatment, but there is still lack of relative data.
Objective：To retrospectively analyze the firstyear data from the standardized database of the Chinese Neonatal Network (CHNN) and preform hospitallevel questionnaires, to reveal the current problem of central vascular catheterization among VPIs in Chinese NICUs and to provide baseline data for future quality improvement program.
Design：Crosssectional study.
Methods：The study described the current situation of central catheterization across gestational age (GA) weeks, including rate, duration and site variation, using the firstyear data of CHNN (from Jan. 1st 2019 to Dec. 31st 2019) from the participating NICUs. Questionnaires were collected on hospital level in terms of the regulation and management of CVC wards, indications of insertion and removal, and related complications of central catheterization. Infants with GA of 24+0~31+6 weeks, admitted into CHNN database from Jan. 1st 2019 to Dec. 31st 2019 within 24 hours after birth were included. Those infants with major congenital anomalies, transferred to other hospitals or discharge against medical advice were excluded. Incomplete and substandard data were also excluded. Subgroup analysis were done for infants with GA at 2428 weeks and 2931 weeks. Research sites were classified into children's specialized hospitals, maternal and children's healthcare centers and general hospitals. Questionnaires were designed to investigate the regulation, management, indications, maintenance and related complications of central catheterization, which were filled by department directors or senior neonatologists authorized by the director.
Main outcome measures：The type, rate and duration of central catheterization.
Results：A total of 6,532 VPIs from 57 CHNN participating sites were included in the analysis. A total of 69.9% (4,563/6,532) cases received central catheterization. Overall, 38.8% (2,532/6,532), 5.6% (368/6,532), 59.6% (3,895/6,532) and 0.8% (55/6,532)infants received umbilical venous catheters(UVC), umbilical artery catheters(UAC), peripherally inserted central catheters(PICC) and surgical central venous catheters(SCVC). Infants with central catheterization had smaller GA and lower birthweight, and were more likely to be smallforGA, multiple birth, outborn and with 5min Apgar score less than 7, compared to noncatheterized infants (P <0.01). The proportion of pregnancy hypertension, antenatal hormone use, and cesarean section was also higher in the mothers of catheterized infants. The mortality rate did not differ between catheterized and noncatheterized infants but catheterized infants had higher rate of each morbidity and longer hospitalization (P <0.01). Rate of any type of central catheterization generally decreased along with the increasing GA, and the rates were 87.8% and 63.1% for 2428 GA weeks and 2931 GA weeks respectively. Rates of UAC and UVC also decreased along with the increasing GA. Rate of PICC was the highest at 2528 GA weeks, and it was still as high as 52.8% for infants at 2931 GA weeks. The rate of using two or more catheters(UVC/PICC/SCVC) was 29% at 2528 GA weeks, which decreased to 21.8% at 2931 GA weeks. The median duration of UAC, UVC and PICC were 6.0 (IQR: 4.08.0) days, 7.0 (IQR: 4.09.0) days and 22 (IQR: 15.031.0)days, respectively. There were significant variations among NICUs with different hospital types on the catheterization rates and the duration. Response rate of the questionnaire was 91.2% (52/57). Only 50% (26/52) hospitals have hospital guidelines for central vascular catheterization. There were also significant variations on the indications of insertion and removal, and the longest duration as well. A total of 62% (32/52) hospitals had central linerelated leakage and 23% (12/52) had thrombosis within one year.
Conclusion：Central vascular catheterization has been commonly used in Chinese NICUs. However, overuse and insufficiency are both existing. Other major problems are the exceeding length of PICC and significant site variation. Guidelines and regulations are on demand. National quality improvement efforts are needed to promote the rationale and standardized use of central catheters for VPIs in NICUs.
Background: Offlabel drug use is common in neonates. Appropriate and effective administration of antibiotics for Carbapenemresistant Enterobacteriaceae （CRE） infection in neonates is challenging. Sulfamethoxazole compound (SMZco) can be taken orally with high bioavailability, however, it is not recommended to be used in infants under 2 months.
Objective: To summarize the experience and therapeutic effect of oral SMZco for the CRE septicemia in newborns.
Design: Case series report.
Methods: Through the hospital drug management system, SMZco medical records that meet the following criteria were collected: a. Patients were hospitalized in NICU of Beijing Children's Hospital, Capital Medical University from January 2018 to June 2021; b. The age on admission was ≤ 28 d or the corrected age was ≤ 44 weeks; c. The CRE septicemia was confirmed by positive blood culture results and clinical manifestations. The oral SMZco as a combined use of medications was administered when the pros and cons were thoroughly discussed by neonatal physicians and clinical pharmacists, and formal written consents were signed by parents. The dose of SMZco was referred to that for children above 2 months old. Signs of allergy and manifestations of bilirubin encephalopathy had been closely watched during the treatment. Complete blood counting and renohepatofunction were monitored weekly. The following information was collected: demographic characteristics, history of surgical intervention or long line catheterization, ventilation time, time interval between blood drawing and culture positive, species of the positive culture results together with the drug sensitivity test, age of starting SMZco administration, dosage of SMZco, WBC, PLT and CRP before and after SMZco treatment, adverse reactions and clinical outcomes.
Main outcome measures: Discharge after improvement.
Results: A total of 9 newborns with CRE septicemia were enrolled, among which 5 were males, 6 received surgery intervention before CRE septicemia was confirmed, and 8 had history of long line catheterization. The average gestational age was (31.0 ± 4.4) weeks, ventilation time was (793±381）h . Specimens for the positive CRE cultures were blood (2 cases), tracheal bronchial secretion (3 cases), tip of PICC (1 case), surgical wound swab (1 case), blood and tracheal bronchial secretion (1 case) and blood+cerebrospinal fluid (1 case). Before the administration of SMZco, all cases were treated with broadspectrum antibiotics for more than 2 weeks. There were 2 cases of CRE meningitis. The medium time of positive CRE culture was 39 (23.5,49) d of hospitalization before or after being transferred to our center. Among the 9 cases, 8 were Klebsiella pneumoniae and 1 was Escherichia coli, all of which produced extendedspectrum βlactamases. Analysis of antimicrobial susceptibility assay revealed resistance to Amikacin (3 cases), Gentamicin (6 cases), Ciprofloxacin (7 cases), and Tetracycline (3 cases). All were sensitive to SMZco and Tegacyclin. SMZco treatment was initiated at 49(38,70.5) days of life for patients, and the dose of SMZ was 4060 mg·kg-1·d-1. There were 6 cases treated with oral SMZco in combination of intravenous carbapenems or thirdgeneration cephalosporins, while 3 cases were with oral SMZco only. Six out of nine patients completed the SMZco course of (24.3 ± 11.6) days, while three out of nine patients did not complete the required SMZco course due to their discharge against medical advice. Among the 6 patients with complete SMZco course, 1 was treated for 10 d (tracheal bronchial secretion positive), and the other 5 were treated for more than 2 weeks. All patients presented improved clinical symptoms and inflammatory markers after the starting of SMZco. All patients had normalization of WBC and PLT, 7 patients had normalization of CRP and other 2 patients had dramatically improved CRP before discharge against medical advice. No allergy was found, nor signs of bilirubin encephalopathy or hemolytic anemia or thrombocytopenia. There were 3 patients (33.3%) with an ALT elevation 2.23.5 times of normal value, which was normalized after symptomatic intervention. All patients had normal renal function during SMZco treatment.
Conclusion: For neonates who had culture confirmed CRE septicemia, under the instruction of drug sensitivity results, a combined use of oral SMZco is a choice to treat the CRE infection when the treatment response of strong broad spectrum antibiotics is negative. However, it is necessary to strictly follow the procedures of offlabel drug use, parent consenting, and close monitoring of side effects.
Background: Given the poor prognosis of severe hereditary protein C deficiency (PCD), most of the children with severe PCD reported in China gave up treatment and died.
Objective: We aimed to explore the longterm therapeutic effect of oral anticoagulants on children with PCD.
Design: Case report.
Methods: We reported 2 cases with severe hereditary PCD, both having compound heterozygous protein C gene (PROC) mutations. PubMed, CNKI and Wanfang databases were consulted from 1981 to 2021 to find out other cases reported in China. And all the cases were analyzed together on diagnosis, treatment and prognosis.
Main outcome measures: Thrombotic or bleeding relief.
Results: Case 1 first manifested as fulminant purpura, while case 2 first presented intracranial hemorrhage and pulmonary hemorrhage due to the chronic disseminated intravascular coagulation (DIC). Gene sequencing on both cases showed compound heterozygous mutation on PROC gene. Daily fresh frozen plasma (FFP) infusion and low molecular heparin (LWMH) worked as emergency treatment. Vitamin K antagonist (VKA) warfarin and direct oral anticoagulant (DOAC) rivaroxaban were used sequentially as longterm treatment to prevent thrombotic events, and hemorrhage. Both cases survived through the 36 years of followup without obvious side effects.
Conclusion: Severe hereditary PCD can have neonate fulminant purpura, as well as intracranial hemorrhage and pulmonary hemorrhage as the initial manifestations. Doctors and parents should choose active treatment instead of giving up. Warfarin and rivaroxaban could be considered as safe and effective longterm alternatives for patients with severe PCD in infancy which could also improve prognosis.
Objective: To summarize the clinical data of 2 children with simulated vasculitis like artificial dermatitis (DA) and improve the understanding of the disease.
Methods: The outpatient and inpatient medical history data of 2 patients were collected, including treatment process, clinical characteristics, relevant laboratory examination and pathological data, and the literature was reviewed.
Results: There were 2 patients, 1 male and 1 female. The onset age was 9 and 10 years old respectively. Two patients underwent frequent and repeated examinations at different hospitals. The shape of skin injury was nonspecific, the boundary with normal skin was clear, and it was distributed in the accessible range of both hands and mouth symmetrically.There was no correlation between skin damage and laboratory examination results.Foreign bodies were found in 1 case by skin biopsypathology, and there was no response to drug treatment. Patients denied that skin damage was selfcaused.
Conclusion: Artificial dermatitis is rare in children. Although the manifestations of skin damage are nonspecific, they are clearly separated from normal skin and distributed in the accessible range of hands and mouth. The discovery of foreign bodies by skin biopsy is helpful to the diagnosis of the disease. Attention should be paid to the potential mental and psychological problems of children with DA. Multidisciplinary collaboration contributes to the diagnosis and management
Background: Infusion Therapy Standards of Practice published in 2021 did not provide a clear recommendation on how to choose between normal saline and heparin saline as the locking solution for neonates and children, however, Clinical Practice Guideline on Infusion Therapy in Children recommended using heparin solution.
Objective: To compare the effects of heparin saline (HS) and 0.9% normal saline (NS) solution as the locking solution of a peripheral intravenous catheter (PIVC) on different pediatric populations.
Design: Systematic review and metaanalysis of RCTs.
Methods: Hospitalized pediatric patients who need PIVC were divided into the intervention group using HS and the control group using NS. Literature was searched in English databases of Ovid MEDLINE, PubMed, Cochrane Library, and Chinese database of SinoMed from April 1, 2014 to November 23, 2021 for there have been two articles of systematic review and metaanalysis on this topic published in 2005 and 2013. RoB 2 was used to evaluate the risk of bias in the included studies.
Main outcome measures: PIVC duration and the related complications.
Results: A total of 21 RCTs were included in the analysis. The duration of PIVC in HS group was 8.62 h longer than that in NS group (95%Cl: 2.9814.26, I2=91%, random effects model), and the difference was statistically significant. After removal of four literature about neonates because of greater heterogeneity, the metaanalysis of other eight literature showed that PIVC duration in neonates of HS group was increased by 6.04 h (95%Cl: 4.177.91, I2=56%, random effects model), and the difference was statistically significant. The pooled results of five studies for both neonates and children showed that the PIVC duration of HS group was prolonged by 6.22 h(95%Cl: 2.729.73, I2=20%, random effects model), and the difference was statistically significant. For the population of children, the pooled results of three studies indicated that the PIVC duration was prolonged by 6.94 h(95%Cl: -1.2715.15, I2=27%, fixed effects model ) in HS group, and there was no statistically significant difference. According to the pooled results of 18 studies, the incidence of complications decreased by 17% (RR=0.83, 95%Cl: 0.710.97, I2=19.5%, fixed effects model) in HS group compared with that of NS group, and the difference was statistically significant. The incidence of complications in neonates decreased by 25% in HS group(RR =0.75, 95%Cl: 0.620.91, I2=0%, fixed effects model) according to the pooled analysis of 9 studies, and the difference was statistically significant. There was no significant difference in reducing the incidence of complications between the two groups for children (4 studies) and the population of both newborns and children (5 studies).
Conclusion: Locking PIVC by heparin saline is desirable for the neonatal population in terms of duration and complication incidence. Whether it is beneficial to the whole pediatric population still needs to be further explored.
Background: Therapeutic agents of refractory Kawasaki disease（KD） include IVIG, corticosteroids, and infliximab(IFX). However, the evaluation of therapeutic efficacy of those agents is not unified.
Objective: To observe the therapeutic effects of IVIG, intravenous methylprednisolone（IMP）, and IFX on patients with refractory KD.
Design: Cohort study.
Methods: Patients with refractory KD who received retreatment and rescue therapy in Jiangxi Province Children's Hospital were taken as the cohort population. They were required to receive the echocardiographic followup of coronary arteries at the time of 3 months after the onset. IVIG (2 g·kg-1) plus oral aspirin (50 mg/ kg/day) was performed as initial treatment within 10 days of the onset. Retreatment and rescue therapy included IVIG(2 g·kg-1), IMP(30 mg·kg-1, 3 d), and IFX (5 mg·kg-1). Parents of these patients could choose the therapeutic agent according to their own will. Response was defined as patients with refractory KD had normal body temperature within 36 hours after the end of the retreatment therapy or rescue therapy.
Main outcome measures: The response rate of total treatment, and coronary artery internal diameters (Zscore) at the time of 3 months after the initial treatment.
Results: There were 73 patients with refractory KD from January 2018 to December 2020 in this study, including 30, 25 and 18 cases in IVIG retreatment group, IFX group and IMP group, respectively. There were no significant difference in age, gender, duration of fever before the initial treatment, duration of fever before rescue therapy, and laboratory parameters before the initial treatment (white blood cells, neutrophils, hemoglobin and Creactive protein). The fever duration of IFX group was shorter than that of IVIG group and IMP group (P=0.012, P=0.016, respectively). The difference in fever duration among the three groups was statistically significant(P=0.024). The retreatment response rate of IFX group was higher than that of IVIG group and IMP group (P=0.001, P=0.015, respectively). One patient in IFX group did not respond to the retreatment of IFX and respond to the rescue therapy of IVIG. Thirteen patients in IVIG group did not respond to the retreatment therapy, among which 7 responded to rescue therapy of IMP, and 6 responded to rescue therapy of IFX. Six patients in IMP group did not respond to the retreatment therapy, and 4 of them responded to the rescue therapy of IVIG, 2 of them did not respond until the rescue therapy of IFX. The total response rate of IFX regimen was higher than that of IVIG and IMP(P=0.035, P<0.001, respectively). The fever duration after retreatment in IFX group were shorter than that of IVIG group and IMP group (P=0.001, P=0.026, respectively). The Zscore at the time of 3 months after the initial treatment in IFX group were significantly lower than that of IVIG group and IMP group (P=0.001, P=0.002, respectively).
Conclusion: IFX could be used as the first drug for the treatment of patients with refractory KD followed by IMP and IVIG.
Background: Pediatric antimyelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) has a variety of clinical manifestations, which can be divided into monophasic and polyphasic courses according to the course of disease, of which about 50% children present with a polyphasic course. Recurrent episodes of polyphasic MOGAD lead to varying degrees of neurological impairment. The main prophylactic drugs for relapse of multiphasic MOGAD include mecophenolate mofetil（MMF）, rituximab (RTX), azathioprine (AZA) and intravenous gamma globulin (IVIG) on a monthly basis. However, there is a lack of highquality clinical evidence for the prophylactic treatment.
Objective: To investigate the effectiveness of rituximab in the treatment of MOGAD through a systematic review and metaanalysis.
Design: Systematic review/metaanalysis.
Methods: Literature on rituximab in the treatment of MOGAD was searched in the databases of Pubmed，Embase，Web of Science，Cochrane，Wanfang Data，CKNI and VIP from Jan 1st, 1997 to June 1st, 2021. After two researchers independently screened the literature, extracted the data, and evaluated the risk of bias in the included studies, the metaanalysis was performed using RevMan 5.3.
Main outcome measures: Annualized relapse rate and relapsefree rate.
Results: Thirteen studies (272 patients) were included. Metaanalysis showed that the relapsefree rate of patients of MOGAD who received rituximab treatment was 32.4%（95%CI:2.9%47.9％，I2=52%，P=0.03）, and the annualized relapse rate changes -1.44（95%CI: -1.67-1.21，I2=71%，P=0.004）.
Conclusion: Rituximab reduced the frequency of relapse in some MOGAD patients, but the relapsefree rate was only32.4%. The results of this study need to be verified by more highquality studies.
Background: Early screening for autism spectrum disorder (ASD) has become a consensus, but it is unclear whether the children's health physical examination system can be used for screening, and the screening tools and paths need to be explored further.
Objective: To explore the path and tools of ASD early screening suitable for children's health physical examination system.
Design: Diagnostic accuracy study.
Methods: From August 1, 2018 to October 31, 2020,the ASD screening clinical pathway was embedded in the child health physical examination system of Shanghai Changning Maternal and Child Health Hospital for children aged 1836 months. The gold standard was the Autism Diagnostic Observation Scale version 2 (ADOS2) combined with Diagnostic and Statistical Manual of Mental Disorders version 5(DSM5), and the modified checklist for autism in toddlers (MCHATR) + MCHATR/F or two behavioral observations (customized) were the test criteria. After primary MCHATR screening, positive children were rescreened by MCHATR/F, and negative children were rescreened by two rounds of behavior observation. Children with positive rescreening were diagnosed as ASD or not. Children with negative rescreening were inquried in routine physical examination, and after the diagnosis of ASD was confirmed, that would be recorded in the children health physical examination system. Those who were positive in the primary screening but were not rescreened and those who were positive in the rescreening but were not recorded in the children's health examination system were lost to followup and were not included in the analysis.
Main outcome measures: The Youden's index of primary screening and secondary screening of ASD.
Results: In the primary screening，there were 10,635 children, 5,579 boys with an average age of (22.3±2.9) months and 5,056 girls with an average age of (22.4±2.9) months. A total of 252 cases were positive and 38 cases were lost to followup (loss rate 15.1%). There were 53 cases positive in MCHATR/F rescreening, 13 cases were lost to followup (loss rate 24.5%), and 26 cases were diagnosed as ASD. A total of 161 cases were negative in MCHATR/F rescreening, and 4 cases were inquiny ASD by routine physical examination. Among 10,383 cases negative in the primary screening, there were 8 ASD in the 2018 subgroup, and 3 ASD in the 2019 subgroup by inquiry in the routine physical examination. In rescreening positive patients of the first behavior observation (n= 54),33 cases were positive, 18 cases were lost to followup (loss rate 54.5%), and 13 cases were diagnosed as ASD. There were 54 case of ASD dignosed by the overall screening, 41 boys and 13 girls with an average age of (26.2±5.9) and (23.8±5.3)months, respectively. The Youden's index of MCHATR was 0.539, the specificity was 98.4%, and the sensitivity was 55.6%. On the basis of positive MCHATR primary screening, the rescreening Youden's index of MCHATR/F was 0.78, the specificity was 91.3%, and the sensitivity was 86.7%. On the basis of negative MCHATR primary screening, the rescreening Youden's index of two rounds behavior observation was 0.81, the specificity was 99.96%, and the sensitivity was 81.2%. Eight children in the 2018 subgroup with negative results in MCHATR screening were diagnosed as ASD through inquiry in the following routine physical examination, and the age of diagnosis was (34.8±2.7) months. In 2019 subgroup of negative results in MCHATR screening, 3 cases were diagnosed as ASD during one round of behavior observation, and for negative subjects the age of diagnosis was(32.7±3.8) months. In the second round of behavior observation, 13 cases were diagnosed as ASD, and the age of diagnosis was (24.2±4.0) months. The difference between groups was statistically significant (F=22.809, P＜0.001), and there was also statistically significant difference in further pairwise comparison (P＜0.001, P=0.004).
Conclusion: It is feasible for children aged 1836 months to embed ASD screening path in children's health physical examination system, and the screening efficiency is desirable. Behavior observation rescreening in children negative in the MCHATR screening can diagnose ASD 10 months earlier.
Background: Kidney injury is one of the risk factors for poor prognosis of HenochSchnlein purpura nephritis(HSPN) , however, there were few researches about the clinical and pathological features of HSPN with acute kidney injury (AKI).
Objective: To investigate the pathological features and risk factors for HSPN complicated with AKI in children.
Design: Casecontrol study.
Methods: Consecutive cases of HSPN diagnosed by kidney biopsy from January 2016 to December 2020 in Guiyang Maternity and Child Care Hospital were included. According to whether AKI was combined or not, the children were divided into the AKI group and the nonAKI group. Demographic data, clinical symptoms, clinical classification, laboratory examination on the day of admission, 24hour blood pressure, pathological grade of kidney biopsy (including classification of ISKDC pathology and Oxford classification) and treatment were extracted from the medical records.
Main outcome measures: The risk factors for HSPN with AKI.
Results: Among 181 cases diagnosed with HSPN, there were 18 cases(10.0%) complicated with AKI, and 163 cases without AKI. The differences in sex, age of onset, albumin levels and PLT counts between the two groups of children were not statistically significant. The incidence of gross hematuria, the duration of gross hematuria, the level of 24hour urine protein, the level of 24hour creatinine and ambulatory blood pressure in the AKI group were higher than those of children in the nonAKI group, and the differences were statistically significant. The clinical classification difference was statistically significant between the two groups(χ2=8.942,P=0.003).The main clinical classification of AKI group was acute nephritis and nephrotic syndrome, while for nonAKI group it was hematuria and proteinuria type and nephrotic syndrome type. ISKDC classification difference was statistically significant between the two groups(χ2=4.586,P=0.032) that the proportion of type Ⅲ and above was higher in AKI group. According to the Oxford typing score,the proportion of tubular/interstital lesions (T1/T2) and crescent (C1/C2) in AKI group was higher than those in the nonAKI group, and the differences were statistically significant(χ2 was 9.625 and 7.961,P Value was 0.002 and 0.005). Logistic regression showed that acute nephritis in clinical classification, and the tubular stromal lesions in the Oxford classification were two risk factors for AKI in children with HSPN. After treatment, 17 cases in AKI group achieved normal renal function, and one case developed chronic kidney disease.
Conclusion: HSPN with AKI is featured by severe clinical and pathologic classifications. Acute nephritis by clinical classification and tubulointerstitial lesions by Oxford typing score were the two risk factors for AKI in children with HSPN.
Background: Juvenile idiopathic arthritis (JIA) associated uveitis is the most common extraarticular manifestation of JIA. Untimely treatment may lead to serious complications and the risk of blindness.
Objective: To investigate the clinical features of JIA patients complicated with uveitis.
Design: Case series report.
Methods: Patients complicated with uveitis admitted to the department of rheumatology and immunology of the Children's Hospital Affiliated to the Capital Institute of Pediatrics from March 2018 to May 2020 were included. Demographic data, laboratory examination, subtype of JIA, ocular manifestations and complications, treatment, and followup were intercepted from the medical records.
Main outcome measures: Uveitis and complications, treatment and outcome.
Results: Of the 278 cases with JIA, fifteen cases were complicated with uveitis, including 4 males and 11 females. The onset age was within 8 years old. Among the 15 patients, there were 13 cases of oligoarticular type, 1 case of polyarticular type and 1 case of enthesitisrelated arthritis type; there were 2 patients with the onset of visual impairment and the other 13 cases with the onset of arthritis; eye lesions were found in 12 cases at the initial diagnosis, and in 3 patients at the course of 12.5 years. All of the 15 patients were diagnosed with anterior uveitis with 8 cases of bilateral lesions and 7 cases of unilateral lesions. Visual impairment was detected in 7 cases. There were 12 patients treated with biological agents, 13 with methotrexate, 1 with sulfasalazine and mycophenolate mofetil, and 3 with glucocorticoids. For local medication, 13 cases were treated with glucocorticoids, 11 cases with mydriasis drugs, 11 cases with nonsteroidal antiinflammatory drugs, and 3 cases with antibiotics. During the followup (1441 months), the arthritis of all patients was improved after treatment. Eye lesions recovered in 7 cases, improved in 1 case, and delayed and repeated in 2 cases, remained no changes in 5 cases.
Conclusion: Uveitis is the main extraarticular manifestation of JIA, which is more common in oligoarticular type, occult onset, and highly disabling. Without treatment in time, serious complications may occur. Regular visual and fundus examination in children with JIA is very important for early detection and treatment of ocular lesions.
Background: The diagnosis of allergic diseases lacks definite test criteria and mainly depends on the clinical history. In the absence of allergen stimulation, there are no clinical symptoms, making the diagnosis more difficult. Therefore, it is very important to find auxiliary diagnostic markers for allergic diseases.
Objective: Detection of lymphocyte subsets in allergic children is expected to provide a new marker for the diagnosis of allergic diseases.
Design: Casecontrol study.
Methods: Children with food allergy and respiratory allergy were selected as the allergic disease group, and healthy children matching with gender and age in the same period were selected as the control group. Lymphocyte subsets was analyzed by flow cytometry.
Main outcome measures: Lymphocyte subsets.
Results: The average age of 30 patients in the allergy group was 3.6 (0.710.6) years, and that of 27 healthy controls was 4.1 (0.811) years. There was no significant difference in age and gender between the two groups (P value was 0.616 and 0.574). For T lymphocyte subsets, the ratio of Th2 cells/effectors helper T cells and Th2/Th1 ratio in the allergic disease group were higher than those in the healthy control group ［(31.34±2.52)% vs (20.02±2.05)%, (6.86±1.51) vs (2.73±0.35)］, and the differences were statistically significant. Percentage and absolute count of mature B cells, absolute count of plasmablasts, percentage of IgE+ plasmablasts, percentage of IgE+ memory B cells in allergic children were higher than those of healthy controls ［(11.53 ± 1.22) % vs (6.02±0.52)%, (1,068±107.3) cells per μL vs (578.74±58.49)cells per μL , (40.71±6.44) cells per μL vs (17.08±2.93)cells per μL , (8.21±1.33) % vs (1.64±0.53)%, (4.48±0.81) % vs (0.47±0.18)%］.
Conclusion: The increased percentage of Th2 cells, IgE+ plasmablasts and memory B cells in allergic children may be a marker for the diagnosis of allergy.
BackgroundKidney injury is common in Henoch Schonlein purpura(HSP) children, and it will result in HSP nephritis. The longterm prognosis of HSP is closely related to the degree of renal involvement in children. Therefore, it is of great significance to evaluate the possibility of nephritis and give targeted intervention as soon as possible.
ObjectiveTo investigate the expression level of serum miR374b in children with HSP and its relationship with nephritis.
DesignCasecontrol study.
MethodsThe case group included the firstepisode children with HSP and was divided into the subgroup with nephritis and the subgroup without nephritis. The control group included healthy children in physical examination. The age in both groups were ≤14 years old. Peripheral venous blood(5 mL) was collected in both groups. The level of serum miR374b was detected by realtime fluorescence quantitative PCR. The clinical data of children in the case group were intercepted from the medical records, and the demographic data of children in the control group were obtained from the physical examination records. The influencing factors of nephritis in HSP children were analyzed. The diagnostic value of serum miR374b level in HSP children complicated with nephritis was analyzed by ROC curve.
Main outcome measuresSerum miR374b level.
ResultsThere were 103 cases in the case group, including 58 males, with an average age of (7.2±1.2) years and an average course of disease of (19.0±3.4) d, and there were 68 cases of simple skin purpura, 23 cases with abdominal pain, 17 cases with joint pain and 15 cases with gastrointestinal bleeding. There were 43 cases in the subgroup with nephritis and 60 cases in the subgroup without nephritis. There were 86 cases in the control group, including 46 males, with an average age of (7.0±1.1) years. The level of miR374b in the case group (0.69±0.13) was lower than that in the control group (1.36±0.24), P<0.01. The level of miR374b in the subgroup with nephritis (0.51±0.09) was lower than that in the subgroup without nephritis(0.82±0.15), P<0.01. Multivariate logistic regression analysis showed that age ≥ 7 years old (OR=2.82, 95%CI: 1.943.93), long course of disease (OR=3.50, 95%CI: 2.484.62), abdominal pain (OR=2.62, 95%CI: 1.873.57), joint pain (OR=2.59, 95%CI: 1.753.52), gastrointestinal bleeding (OR=2.68, 95%CI: 1.903.61), PLT high level (OR=3.66, 95%CI: 2.544.77) , WBC high level (OR=3.24, 95%CI: 2.114.42), FIB high level (OR=3.58, 95%CI: 2.514.71), abnormal urinary microglobulin (OR=3.36, 95%CI: 2.284.46), elevated blood IgA (OR=3.50, 95%CI: 2.394.58), elevated blood IgG (OR=3.53, 95%CI: 2.514.71) and elevated blood IgM (OR=3.96, 95%CI: 2.875.12) were the risk factors for nephritis in HSP children, while the ALB high level (OR=0.60, 95%CI: 0.370.83), HDL high level (OR=0.55, 95%CI: 0.310.72) and miR374b high level (OR=0.47, 95%CI: 0.140.65) were the protective factors for it. ROC curve showed that the cutoff value of serum miR374b level in predicting nephritis in HSP children was ≤ 0.65, and the sensitivity was 90.7%, and the specificity was 83.3%, and the AUC was 0.919 (95%CI: 0.8490.964).
ConclusionThe expression level of serum miR374b in HSP children is decreased, and the decrease is more obvious in children with nephritis, which has a certain diagnostic value for children with HSP complicated with nephritis.
Objective: To explore the pulmonary vein spectral characteristics of fetal total anomalous pulmonary venous connection (TAPVC).
Methods: Fetal pulmonary vein spectral characteristics were analyzed retrospectively in cases with the prenatal diagnosis of TAPVC in the Ultrasound Department, Weifang Maternal and Child Health Hospital from February 2017 to June 2021. The fetal pulmonary vein spectral characteristics of TAPVC were summarized.
Results: We enrolled 19 patients diagnosed with TAPVC (11 isolated types and 8 complex types) in the antenatal period, including 11 supracardiac cases, 4 cardiac cases, 4 infracardiac cases, and 8 cases combined with vertical vein obstruction. Whether the vertical vein obstruction was involved or not, the pulmonary vein spectrum of supracardiac and infracardiac types (including both isolated and complex types) was featured by a continuous lowvelocity pattern with aperiodic pulsation. The pulmonary vein spectrum of the intracardiac type (coronary sinus dilation) was consistent with that of normal pulmonary veins.
Conclusion: Fetal pulmonary vein spectral changes have some value in TAPVC classification, but cannot be used to distinguish between supracardiac and infracardiac types. The pulmonary vein spectrum should be routinely scanned for TAPVC fetal diagnosis to further increase diagnostic confidence.
Background: Large Bcell lymphoma in children with interferon regulatory factor 4(IRF4)gene rearrangement is defined as a unique subtype in the 2017 WTO lymphoma classification. Since it is clinically rare with special clinical features, it is necessary to distinguish it from other large Bcell lymphomas.
Objective: To investigate the clinical manifestations, pathological features, treatment and prognosis of large Bcell lymphoma with IRF4 gene rearrangement in children.
Design: Case series report.
Methods: The clinical data of children with large Bcell lymphoma with IRF4 gene rearrangement diagnosed and treated in Beijing Children's Hospital affiliated to Capital Medical University from May 2018 to October 2021 were collected retrospectively. The clinical characteristics were summarized, and PubMed, Wanfang and CNKI were searched for literature review.
Main outcome measures: Complete response rate of large Bcell lymphoma in children with IRF4 gene rearrangement.
Results: Six cases were included in the analysis, accounting for 2.7% of the invasive mature Bcell lymphoma treated in our hospital in the same period. There were 5 males (83.3%) and 1 female. The age of onset was 7 (4 13) years old. The involved sites were the neck and head in 4 cases, the intestine in 1 case and both of the head and neck and intestine in 1 case. No metastasis was found from the onset to followup. Four cases were in stageⅡ and 2 cases were in stage Ⅲ. Among the 62 cases including 56 from literature review, 61.2% were male, the age of onset was 11.15 (3,18) years old, the main site of tumor was 79% in the head and neck, and 21% in the intestine and groin. Most of them were isolated lesions, and no metastasis was found from the onset to followup. Clinical stage ⅠⅡ accounted for 79%. Under light microscope, 2 of 6 cases showed complete nodular follicular structure featured by nodules of different sizes, lack of "starry sky phenomenon" formed by mantle area and phagocytic nuclear fragments and 4 cases showed completely diffuse like structure with medium or large tumor cells, scattered nuclear chromatin and small basophilic nucleoli. Immunohistochemistry showed the expression of CD20 and PAX5, the strong expression of BCL6 and MUM1, and the positive index of 90% to 95% for Ki67. Three cases were positive for CD10 and BCL2, six cases were positive for IRF4 rearrangement by fluorescence in situ hybridization (FISH), and BCL6, BCL2 and CMYC gene rearrangements were not detected. Sixtytwo cases showed complete diffuse like structure (43.5%), complete nodular follicular like structure (37.1%) and mixed like structure (19.4%) and 88.7% could detect rearrangement with IRF4 .
Conclusion: Large Bcell lymphoma with with IRF4 rearrangement is clinically rare. The tumor is mainly involved in the head and neck. The clinical stage is mainly early lesions, which are more isolated and inert, slow progress, weak invasiveness and good prognosis. The pathological morphology showed complete diffuse like, follicular like and mixed like structures. Immunohistochemistry showed the expression of CD20 and PAX5, the strong expression of BCL6 and MUM1, and the high positive index of Ki67. IRF4 rearrangement could be detected by FISH combined with secondgeneration gene sequencing.
