Objective:To investigate the clinical manifestations of medically refractory hereditary movement disorders in children and the efficacy of deep brain stimulation (DBS).Methods:A case series study.The clinical and follow-up data of 20 children with medically refractory hereditary movement disorders who underwent DBS treatment at the Neurology and Functional Neurosurgery Departments of Beijing Children′s Hospital, Capital Medical University, from July 2018 to April 2024, were retrospectively analyzed.The severity of movement disorder symptoms and surgical effects were evaluated using the Burke-Fahn-Marsden Dystonia Rating Scale Movement(BFMDRS-M) or the Unified Parkinson′s Disease Rating Scale Ⅲ(UPDRS Ⅲ).Results:There were 12 males and 8 females among the 20 children, with an onset age ranging from 4 months to 12 years and 5 months.Fourteen patients had hereditary dystonia, which is related to KMT2B in 11 patients, TOR1A in 2 patients and SGCE in 1 patient.Two patients had choreoathetosis, which is related to ADCY5-related familial movement disorders.Two patients had early-onset Parkinson′s disease, which is related to ATP6AP2 in 1 patient and VPS13C in 1 patient.Two patients had neurodevelopmental disorders with involuntary movements, which is related to GNAO1 in 1 patient, and the other patient was idiopathic.All the children were given oral Levodopa, Benzhexol, Baclofen, Tiapride Hydrochloride, Clonazepam alone or in combination.Three children showed obvious dyskinesia after Levodopa treatment.The symptoms of movement disorders in all children exhibited little to no improvement.Levetiracetam and Zonisamide had unstable effects in the treatment of myoclonia.DBS surgery was performed on all the patients aged from 3 to 16 years.Electrodes were successfully inserted into bilateral globus pallidus internus in 14 cases and bilateral subthalamic nuclei in 4 cases.The target was unknown in 2 cases.No surgery-related complications were observed.The patients were followed up for 3 months to 6 years, and the last follow-up age of the patients ranged from 5 years and 7 months to 22 years and 1 month.The rate of improvement in BFMDRS-M score was 37%-100% in 16 patients and >70% in 7 patients with hereditary dystonia.The rate of improvement in UPDRS Ⅲ score was 23% in 1 patient with VPS13C-related early-onset Parkinson′s disease. Conclusions:Childhood medically refractory hereditary movement disorders are a case series that exhibits significant phenotypic and genotypic heterogeneity.DBS surgery demonstrates significant efficacy for KMT2B-, TOR1A-, and SGCE-related hereditary movement disorders.
Surfactant dysfunction is a genetically heterogeneous pulmonary disease that causes dyspnea. ATP binding cassette protein transporter subunit A3 (ABCA3) is the main pathogenic gene of pulmonary surfactant dysfunction. In this study, we established an induced pluripotent stem cell line (SMCPGHi001-A) from the peripheral blood cells of a 49-day-old male infant, carrying compound heterozygous variations of the ABCA3 gene (c.3997_3998del, p.R1333fs, and c.3137C > T, p.A1046V). This iPSC line would be a useful tool to study the pathogenesis, disease development, and treatment of pulmonary surfactant dysfunction.
Obesity is the main risk factor of cardiovascular diseases.As the indexes of obesity evaluation, body mass index(BMI) and waist circumference are the most common methods to predict metabolic risk of cardiovascular system.Recently, neck circumference, a reliable and low-cost parameter of anthropometry, could be considered as a new indicator to evaluate childhood obesity since it is closed correlation with children′s BMI and waist circumference.Moreover, studies had shown that the increase of neck circumference had strong correlation with cardiometabolic risk, such as high blood pressure, hyperlipidemia, hyperglycaemia and hyperuricemia.This indicates that it might be the index to predict metabolic risk of cardiovascular system.Understanding and illuminating the changes of neck circumference and its relationship with obesity and metabolic disorders would have significances for preventing and identifying metabolic risks of cardiovascular system in children and adolescents.
To analyze the clinical features and genetic variants of a child with type 4 familial partial lipodystrophy (FPLD4) and the initial manifestation of diabetes.The male patient with the age of 13 years and 5 months, and the diabetes course was about 3 years, the patient was admitted to Children′s Hospital of Soochow University on November 10, 2021(4 th hospitalization at the hospital), in the course of diabetes, the children repeatedly suffered from diabetes ketoacidosis, and lipid metabolism complications gradually emerged.The gene sequencing showed that the proband and his mother carried dual gene mutations of PLIN1 c. 1325delG(p.G442Afs*99) and SPINK1 c. 194+ 2T>C(p.? ). The PLIN1 gene was the causal gene of FPLD4.The mutations of c. 1325delG in the PLIN1 gene had not been previously reported.Based on the clinical phenotype, family history and genetic testing findings, the patient was diagnosed as FPLD4.In addition, the mutation of SPINK1 c. 194+ 2T>C(p.? ) might increase the risk of chronic pancreatitis.This case report enriched the clinical characteristics and genotype data of FPLD4.Gene sequencing assisted the accurate diagnosis of the type of diabetes.The effects of dual gene mutations on disease progression should be concerned, which were of great significance to develop treatment regimen and disease management.
The clinical features, examination findings and genetic testing results of a newborn with neurobehavioral developmental abnormality caused by the PAK3 gene mutation in the Department of Neonatology, Anhui Provincial Children′s Hospital were retrospectively analyzed in November 11, 2021.The male 9-day-old newborn presented with the difficult-to-wean for 9 days after birth.The child had repeated startle reflexes, decreased muscle tension in the extremities, and partial primitive reflexes.Amplitude-integrated electroencephalogram (aEEG) showed the lower and upper boundary voltage of 10 μV and 40 μV, respectively.Obvious mature sleep-wake cycles were not found, and 2 electric seizures were recorded.The aEEG suggested the moderate-to-severe abnormal aEEG.Magnetic resonance imaging showed that the corpus callosum was slightly thinner.The family-centered diagnostic exosome sequencing showed a missense mutation of the PAK3 gene[c.1327 (exon18) G>A, p.G443R], which has not been previously reported at home and abroad.This case enriched the clinical phenotype of the PAK3 gene mutation and suggested the potential value of whole genome sequencing in clinical diagnosis and genetic guidance.
Sj?gren′s syndrome(SS) is a chronic inflammatory autoimmune disease, including primary SS (pSS) and secondary SS (sSS). Pediatric sSS has the similar clinical characters with pSS, which can be classified based on the same classification criteria.Compared with those of adult SS, pediatric SS is rare, insidious and lacks the typical manifestations of dry eyes and dry mouth.Therefore, the classification criteria for adult SS is not suitable for pediatric SS.So far, there are no classification criteria for pediatric SS that have been widely applied and validated, which needs to be further explored.The present review described the clinical characteristics and progress of classification criteria for pediatric SS, and compared pSS and sSS in affected children.
Objective:To evaluate the efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) on the childhood Epstein-Barr virus(EBV)-positive lymphoproliferative diseases(EBV + LPD). Methods:The clinical features, treatment course, and prognosis of 9 children with EBV + LPD who underwent allo-HSCT in Children′s Hospital Affiliated to Zhengzhou University from July 2019 to July 2022 were analyzed retrospectively. Results:All the 9 children underwent histopathological examination, including 6 patients with EBV-positive T-cell lymphoproliferative disease (EBV + T-LPD), 1 with pulmonary lymphomatoid granuloma, and 2 with systemic EBV-positive T-cell lymphoma.There were 6 males and 3 females, with the median age of 5.8 (1.5-13.0) years.At the initial diagnosis, plasma and peripheral EBV-DNA copy at the initial diagnosis was (5.67-865.00)×10 2/mL, and (5.13-1 250.00)×10 2/mL, respectively.The EBV-DNA load of cerebrospinal fluid increased to (5.18-291.00)×10 2/mL in 3 cases.The whole exon sequencing data showed no abnormality in 3 cases, pulmonary lymphomatoid granuloma with the IL2RG mutation in 1 case and EBV + T-LPD with a hemizygous mutation in the SH2D1A gene as the pathogenic mutation in 1 case.Pathogenic mutations were not detected in the remaining 4 cases.The course of disease before transplantation was 5.4(3.0-10.0) months.Disease status before transplantation was as follows: all 3 cases of lymphomas had partial regression; 2 cases of EBV + T-LPD had active disease; and 4 cases had no active disease.Among the donors, there were 5 cases of half-matched relatives, 2 cases of full-matched siblings and 2 cases of unrelated full-matched donors.The median number of mononuclear cells in peripheral blood and/or bone marrow hematopoietic stem cell was 6.60(3.64-12.18)×10 8/kg, while the median implantation time of neutrophils was 18(9-23) days.One month after the transfusion of hematopoietic stem cells, plasma EBV-DNA copy was negative in all cases, and peripheral EBV-DNA copy was negative in 7 cases.The copy number in the other 2 cases was 10 2/mL.At the 3-month evaluation, plasma and peripheral EBV-DNA copy were negative in all cases.In addition, 3 cases of lymphomas achieved radiographic complete remission, and 6 cases of EBV + T-LPD were inactive.All transplant-related complications were effectively controlled after medication.Following the median follow-up of 24 (11-42) months, all patients had disease-free survival.Serious impact on the quality of life due to graft versus host disease was not reported. Conclusions:allo-HSCT is an effective treatment of childhood EBV + LPD, which is able to control transplant-related complications.Children with EBV + LPD can achieve long-term disease-free survival through transplantation.
Objective:To study the influence of periventricular-intraventricular hemorrhage (PVH-IVH) on cerebral blood flow (CBF) of preterm infants in the late postnatal period using arterial spin labeling (ASL) magnetic resonance imaging (MRI).Methods:From January 2023 to June 2023, 65 preterm infants (gestational age <32 weeks, birth weight <1 500 g) who were born in the Department of Obstetrics, Third Affiliated Hospital of Zhengzhou University and transferred to the Neonatal Intensive Care Unit were included in the prospective study.They were examined by the brain MRI and ASL at the corrected gestational age of 35-40 weeks.According to the results of the brain ultrasound within 1 week after birth, they were divided into the mild IVH group (25 cases) and the non-IVH group (40 cases). The CBF values in regions of interest (frontal lobe, temporal lobe, parietal lobe, occipital lobe, thalamus, and basal ganglia) on ASL images were compared.Multiple linear regression analysis was used to analyze the effect of PVH-IVH on CBF values in different ASL regions of interest, including frontal cortex, temporal cortex, parietal cortex, occipital cortex, thalamus, and basal ganglia.Results:Compared with those of non-IVH group, infants in the mild IVH group presented significantly older gestational age [29.0 (28.5, 30.4) weeks vs.28.2 (27.0, 31.0) weeks, Z=-2.398, P=0.016], higher hematocrit (HCT) in the latest examination prior to the brain MRI [29.6(26.4, 32.3)% vs.27.8 (25.6, 30.5)%, Z=-2.155, P=0.031], and larger body weight at the time of examination [2 015.0 (1 930.0, 2 127.5) g vs.1 950.0 (1 900.0, 1 997.5) g, Z=-3.314, P=0.001]. After adjustment for confounding factors of gestational age at birth, latest HCT and weight at the time of examination, the multivariable linear regression analysis showed that CBF values in the frontal lobe (95% CI: -8.367--4.042; P<0.001), temporal lobe (95% CI: -19.077--2.854; P=0.008), parietal lobe (95% CI: -8.344--3.502; P<0.001), occipital lobe (95% CI: -9.446--3.645; P<0.001), basal ganglia (95% CI: -7.543--1.963; P=0.001) and thalamus (95% CI: -8.051--2.372; P<0.001) were significantly lower in infants of the mild IVH group than those of non-IVH group. Conclusions:At the same corrected gestational age, mild IVH is correlated with low CBF values in local cerebral cortex and subcortical gray matter in premature infants.However, the predictive potential of CBF values in long-term neurological prognosis requires further explorations.
Objective:To analyze the efficacy and safety of Omalizumab (OMA) combined with allergen immunotherapy (AIT) on children with allergic asthma.Methods:Clinical data of 43 children with allergic asthma from the Second Hospital of Tianjin Medical University between August 2018 and October 2022, who were managed by OMA combined with double mite subcutaneous immunotherapy (SCIT) were retrospectively analyzed, including 30 males and 13 females with the age of 5-15 years.Twenty children with allergic asthma who were managed by the monotherapy for SCIT during the same period, including 16 males and 4 females with the age of 4-13 years were included in the control group(group1: conventional immunotherapy; group2: cluster immunotherapy). Among the 43 cases managed by OMA combined with SCIT, 20 were treated with OMA, followed by AIT (OMA-AIT group), and 23 were treated with AIT, followed by OMA (AIT-OMA group). Notably, 6 cases in AIT-OMA group who were additionally given OMA due to the difficulty in increasing doses were subgrouped in AO1 group, and 17 who were additionally given OMA due to poor control of asthma or comorbidities during the course of AIT and frequent adverse events were subgrouped in AO2 group.The number of asthma exacerbations within 1 year and during the combination therapy, the Childhood Asthma Control Test/Asthma Control Test (C-ACT/ACT) findings, the Visual Analogue Scale (VAS) for grading rhinitis, inhaled corticosteroid (ICS) dosage converted to budesonide equivalent, the Total Medication Score (TMS), comorbidities, lung function [percent-predicted forced expiratory volume in 1 second(FEV 1% pred), percent-predicted peak expiratory flow(PEF%pred), percent-predicted maximal mild-expiratory flow(MMEF%pred)], exhaled nitric oxide (FeNO), completion of the initial SCIT and adverse effects [local adverse reactions (LRs) and systemic adverse reactions (SRs)] were analyzed for assessing the efficacy and safety of OMA combined with AIT on children with allergic asthma.The t-test of two independent samples was used for comparison of measurement data that followed normal distribution.Wilcoxon′s test was used for non-normally distributed between-group comparisons.The χ2 test was used for the between-group comparison of counting data. Results:(1)Baseline comparison showed that the male ratio (17/20 cases vs.13/23 cases) and the proportion of moderate-to-severe persistent asthma (18/20 cases vs.18/23 cases) in the OMA-AIT group were significantly higher than those of the AIT-OMA group (all P<0.05). (2)Efficacy: ①In OMA-AIT group, all children reached the AIT maintenance treatment stage successfully after combination therapy.At the maintenance treatment stage, the C-ACT/ACT, VAS and TMS scores(26.0±1.25 vs.24.55±2.28, 1.50±1.24 vs.2.55±1.70, 3.60±1.47 vs.5.45±1.19)were significantly improved from baseline(all P<0.05). There were no significant differences in lung function indexes FEV 1%pred, PEF%pred, and MMEF%pred ( P>0.05), and FeNO level did not change significantly than baseline.After the combination treatment, ICS dosage significantly decreased from 240.00 (160.00, 380.00) μg/d at baseline to 140.00 (80.00, 300.00) μg/d ( P<0.05). Comorbidities, including allergic rhinitis, food allergy, atopic dermatitis and angioedema were improved.Five cases (25.00%) had once asthma exacerbation during the treatment.The duration of maintenance dose of conventional (22.70±7.10 vs.15.20±1.32) and cluster immunotherapy (13.00±4.97 vs.7.30±1.06) were longer than those of the corresponding control group(all P<0.05). ②AIT-OMA group: In AO1 group, the C-ACT/ACT score were improved from baseline( P<0.05), and VAS score, TMS score decreased from 3.00(1.75, 3.00), (4.67±1.97) points at baseline to 1.00(0, 1.00), (2.83±1.60) points by the maintenance dose in AO1 group (all P<0.05). There were no significant differences in the FEV 1%pred, PEF%pred, MMEF%pred and FeNO compared with baseline in AO1 group.ICS dosage in AO1 group significantly decreased from (180.00±78.99) μg/d at baseline to (88.88±26.23) μg/d ( P<0.05). In AO2 group, the C-ACT/ACT, VAS and TMS scores at the completion of OMA treatment[26.53±0.94 vs.25.06±2.05, 1.00 (0, 2.00) vs.2.00(2.00, 3.50), 3.41±0.94 vs.5.53±1.23]were significantly improved from baseline (all P<0.05). PEF%pred[(106.47±22.37)% vs.(94.47±26.39)%] significantly increased than baseline ( P<0.05), and the remaining lung function indexes and FeNO were not significantly improved.ICS dosage significantly decreased from 240.00(160.00, 400.00) μg/d at baseline to 80.00 (20.00, 160.00) μg/d ( P<0.05). During the combination treatment, 1 case (5.88%) had once asthma exacerbation, and all 8 cases with food allergy or atopic dermatitis or conjunctivitis had improved comorbidities.(3)Safety: adverse events during OMA injection were not reported.①In OMA-AIT group, a total of 165 OMA injections were performed in the initial treatment stage of the conventional immunotherapy group, with 13 (7.88%) reported LRs and 2 (1.21%) grade-1 SRs.A total of 143 OMA injections were performed in the initial treatment stage of the cluster immunotherapy group, with 19(13.29%) reported LRs and none of SRs.②In AIT-OMA group, there were 6 cases of adverse events in the initial treatment stage of AIT who were successfully reached the maintenance treatment stage after the addition of OMA in AO1 group.In AO2 group, children who were additionally given OMA due to adverse events in the maintenance treatment phase did not report adverse events during the combination therapy. Conclusions:OMA combined with AIT not only expands the scope of AIT, improves allergic and asthma symptoms in children, reduces the use of drugs, but also enhance the safety of AIT and compliance, reduces adverse events during AIT treatment, and even shortens the time of initial treatment.
Fever, especially acute fever of unknown cause, is the most common clinical symptom when children visit the hospital.The principle of ensuring maximum smoothness of the diagnosis process and reducing the risk of cross-infection should be followed.In China, there is uneven development in aspects such as the clarity of the febrile pediatric diagnosis process, the rationality of the configuration of febrile outpatient departments, the early screening ability for infectious diseases, the treatment of acute and severe diseases, and the prevention and control of infectious diseases.This consensus aims to standardize the basic requirements of the febrile pediatric diagnosis process and provide guidance and constructive suggestions for continuous improvement.
Objective:To investigate the value of pulse oxygen saturation (SpO 2) monitoring in predicting children with moderate-to-severe obstructive sleep apnea (OSA). Methods:It was a retrospective study involving 341 children with snoring during nighttime sleep who had visited the Children′s Hospital of Soochow University from June 2017 to November 2020 and monitored for polysomnography (PSG) and SpO 2.The SpO 2 parameters mainly included oxygen desaturation index (ODI), oxygen desaturation index ≥3% (ODI3), oxygen desaturation index ≥4% (ODI4), mean pulse blood oxygen saturation (MSpO 2), lowest pulse blood oxygen saturation (LSpO 2), cumulative time spent with blood oxygen saturation below 95%, 92% and 90%(T95, T92 and T90). According to obstructive sleep apnea hypopnea index (OAHI), patients were divided into the snoring and mild OSA group (OAHI≤5 times/h) and moderate-to-severe OSA group (OAHI>5 times/h). Differences in SpO 2 parameters were compared between groups using the Chi- square test and Mann- Whitney U test. Spearman correlation analysis was used to analyze the correlation between SpO 2 parameters and OAHI in all children.The SpO 2 parameters were included in the Logistic regression model.Receiver operating characteristic (ROC) curve was used to analyze the diagnostic efficiency of SpO 2 parameters on moderate-to-severe OSA. Results:A total of 341 patients were recruited, including 206 male and 135 female patients with the mean age, body mass index (BMI) and OAHI of 6.0 (4.0, 7.5) years, 16.2 (15.1, 18.0) kg/m 2 and 0.6 (0.1, 3.0) times /h, respectively.There were 283(83.0%) and 58 (17.0%) patients in the snoring and mild OSA group and moderate-to-severe OSA group.The ODI3[0.7 (0.3, 1.4) times/h vs.7.7 (4.4, 12.8) times/h], ODI4[0.4 (0.1, 0.8) times/h vs.5.3 (2.7, 9.1) times/h], T95[1.4 (0.3, 5.3) min vs.13.7 (7.0, 33.5) min], T92[0.1 (0, 0.5) min vs.1.8 (0.9, 6.0) min] and T90[0 (0, 0.1) min vs.0.6 (0.2, 2.2) min] were significantly lower in the snoring and mild OSA group than those of moderate-to-severe group, while LSpO 2[91.0 (89.0, 93.0)% vs.86.5 (82.0, 88.0)%] and MSpO 2[ 97.0 (97.0, 98.0)% vs.96.0 (96.0, 97.0)%] were significantly higher(all P<0.001). All SpO 2 parameters were significantly correlated with OAHI (all P<0.001), and the correlation coefficient between ODI3 and OAHI was 0.660.ODI3 was an independent predictor of moderate-to-severe OSA ( OR=3.117, 95% CI: 1.635-5.945, P=0.001). The area under the ROC curve of ODI3 in predicting the moderate-to-severe OSA was 0.957, and the cut-off value of 3.45 times/h and specificity of 95.4%.MSpO 2 was an independent predictor of moderate-to-severe OSA ( OR=2.917, 95% CI: 1.589-5.354, P=0.001). Conclusions:ODI3 can be used to predict the moderate-to-severe OSA in children.
Genetic disorders of surfactant dysfunction are a group of diseases caused by genetic mutations involved in the synthesis and metabolism process of surfactant, which are important etiologies in pediatric interstitial lung diseases.Despite the widespread application of genetic diagnostic technologies, there is still a need to improve the recognition of clinical clues, as well as treatments and management for this disease.In order to enhance the understanding of pediatricians regarding this disease, promote early diagnosis and standardized treatment, and improve the prognosis, this study has convened an experts panel to establish this consensus.
Mannose phosphate isomerase-congenital disorders of glycosylation (MPI-CDG) is a treatable congenital genetic metabolic disease caused by the pathogenic variation of the gene encoding MPI.It is mainly manifested as diarrhea, hepatomegaly, hypoglycemia, and coagulation dysfunction.This review described the pathogenesis, clinical manifestations, genotypes, diagnosis, treatment and management of MPI-CDG, aiming to enhance the understanding of MPI-CDG.
Objective:To analyze the clinical characteristics, treatment course and prognosis of children with intermediate-high risk pulmonary embolism.Methods:The clinical data of 48 children with pulmonary embolism treated in Beijing Children′s Hospital, Capital Medical University from January 2017 to December 2021 were analyzed retrospectively.Including 12 intermediate-high risk cases and 36 low-risk cases.The clinical manifestations, laboratory results, treatment and prognosis were compared between groups by the t-test, rank sum test and Chi- square test with the yates continuity correlation or Fisher′ s exact test. Results:There were no significant differences in the sex and age between the intermediate-high risk group and the low-risk group.The proportions of patients with shortness of breath, dyspnea, cyanosis or hypoxemia were higher in the intermediate-high risk group than those of in low-risk group.Twelve children in the low-risk group did not have specific symptoms of pulmonary embolism.There were no significant differences in the D-dimer level, and the distribution of pulmonary embolism between the two groups (all P>0.05). However, the proportion of children with other thromboembolism in the intermediate-high risk group was significantly higher than that of the low-risk group, among which heart thrombosis was the most common (7 cases). There were no significant differences in the underlying diseases and thrombophilia between the two groups (all P>0.05). The treatment of the intermediate-high risk group was more active: 6/12(50.00%) patients in the intermediate-high risk group received reperfusion treatment, including 3 cases of systemic thrombolysis, 1 case of catheter thrombolysis, and 2 cases of thrombectomy.In the low-risk group, only 1 case was treated with systematic thrombolysis.Unfavorable outcomes were reported in 3/48 (6.25%) patients, including 1 death of massive bleeding after catheter-directed thrombolysis in the acute phase, 1 case of recurrent pulmonary embolism after self-decided withdrawal and 1 case of progression of pulmonary embolism that was managed by surgical thrombectomy, all of whom were in the intermediate-high risk group. Conclusions:Shortness of breath, dyspnea, cyanosis or hypoxemia and co-existed venous thromboembolism were more common in intermediate-high risk cases.The treatment regimen of was more aggressive, but the incidence of unfavorable outcomes was higher in intermediate-high risk group; further research is needed to determine the risk factors for intermediate-high risk pulmonary embolism in children.
Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of lipoproteinaceous material within alveoli due to various reasons.The clinical features are diverse, including progressive dyspnea, cough, cyanosis, clubbing, which seriously affects the quality of life of children, even life-threatening.Based on the related literature at home and abroad, combined with the clinical needs of pediatrics, experts′ advice on the diagnosis and treatment of PAP in children is formulated to improve the understanding of pediatricians on the pathogenesis, clinical manifestations, diagnostic criteria and procedures and treatment principles of pediatric PAP, aiming to improve the prognosis of PAP children.
Objective:To assess the current status of fever outpatient clinics and capacity building in children′s hospitals and maternal and child health institutions of different regions and levels.Methods:A total of 296 children′s hospitals and maternal and child health institutions from 7 regions and 30 provinces/autonomous regions/municipalities were randomly selected in this comprehensive study.The pre-triage, emergency and diagnostic capabilities, personnel allocation, and hospital infection prevention and control of fever outpatient clinics were investigated. Chi square test or Fisher′ s exact probability method was used. Results:The results of investigation and analysis showed that 84.12% (249/296) of the medical institutions were equipped with independent fever clinics (consulting rooms), of which 109 were tertiary medical institutions (109/125, 87.20%) and 72 were secondary medical institutions (72/171, 42.11%). In children′s hospitals, 91.43% (32/35) set up independent fever clinics, and the independent setting rate of maternal and child health care hospital was 46.71% (78/167), which was significantly lower than that of children′s hospitals ( P<0.001); in the medical institutions with dual functions of children′s and maternal and child health care hospitals, 76.56% (49/64) of the pediatric independent fever clinics were set up; the setting rate in western China (41.38% in Southwest China and 47.06% in Northwest China) was relatively low.The 51.41% of fever clinics in medical institutions were not equipped with life support equipment such as invasive or non-invasive ventilators; SARS-CoV-2, influenza A and B virus testing methods(including nucleic acid testing, antigen testing and antibody testing)were over 90% prevalent in pediatric fever clinics.However, the prevalence of adenovirus, respiratory syncytial virus and mycoplasma pneumoniae antigen detection was less than 45%.The average number of doctors and nurses in pediatric fever clinics in tertiary medical institutions was 5 and 9, respectively, and they all had at least 1 technician and pharmacist, while the average number of doctors and nurses in tertiary medical institutions was 3 and 4, respectively, and some of them did not have technicians and pharmacists.Tertiary medical institutions had significantly more staff than secondary medical institutions ( P<0.001), the per capita performance of 87.55% of medical staff in fever clinics/consulting rooms ranked at or above the average level of all clinical departments in the hospital. Conclusions:Among children′s hospitals/maternal and child health institutions in China, the area and personnel allocation of fever clinics/consultation areas in children′s specialized hospitals were relatively standardized.However, some hospitals′ existing fever clinics/consultation areas did not have adequate space, and the reception and emergency care capabilities could not meet the short-term surge in needs of children with fever.The rapid detection ability of common infectious diseases pathogens in children with fever was still obviously insufficient, especially during the regional outbreaks of common infectious diseases in children, which could not meet the needs of early screening.How to formulate standardized treatment procedures for children with fever according to the hardware and software, available medical resources and diagnosis and treatment status of various medical institutions and meet the needs of " anti-epidemic combination" still needs great attention.
Buckwheat is one of the most common source of food allergen in children.The clinical manifestations of buckwheat allergy are varied, which mainly affects the skin, and sometimes the respiratory and digestive system.Buckwheat allergy leads to a high incidence of anaphylaxis, which can even be life-threatening.In recent years, the prevalence of buckwheat allergy in Chinese children and adolescents is on the rise, although it is rarely reported in relevant literatures.This review summarized the prevalence, pathogenesis, clinical manifestations, diagnosis and management of buckwheat allergy in children, aiming to provide some suggestions.
Objective:To record stereoelectroencephalography (SEEG) data and to induce cortical electrical stimulation in children with tuberous sclerosis complex (TSC), thus exploring the epileptogenicity of different types of cortical tubers.Methods:The SEEG recording and cortical electrical stimulation data of 50 children with TSC who underwent preoperative evaluation for drug-resistant epilepsy at Epilepsy Center, Tsinghua University Yuquan Hospital from November 2016 to September 2022 were retrospectively analyzed, involving 27 boys and 23 girls with the age of (5.5±3.4) years.According to the results of 3.0T magnetic resonance imaging (3T-MRI) and computed tomography(CT), cortical tubers were classified.The incidences of electroclinical seizures, electrical seizures and seizures induced by cortical electrical stimulation in different types of tubers recorded by SEEG were analyzed, and the differences in the proportion of the above seizures among different types of tubers were compared using the Fisher′ s exact test. Results:A total of 303 cortical tubers were explored using SEEG in 50 patients.The tubers were divided into 6 types, including Type A, B, C, D and E, and focal cortical dysplasia like (FCD-like) type, among which Type E was for the first time proposed in the world.Among these explored tubers, 7 tubers had electrical seizures, and 57 tubers had electroclinical seizures.A total of 64 tubers (21.1%) were epileptogenic.The incidence of epileptogenic tubers in Type A-E and FCD-like type were 3.6%, 1.4%, 19.0%, 77.8%, 77.5%, and 90.0%, respectively. Fisher′ s exact test and Bonferroni correction were performed for pairwise comparisons( P<0.003). There was no significant difference in the incidence of epileptogenic tubes among Type A, B and C. There was significant difference in the incidence of epileptogenic tubes between Type A-C with Type D, Type E and FCD-like type, respectively.There was no significant difference in the incidence of epileptogenic tubes between Type D, Type E and FCD-like like.Electrical stimulation-induced seizures occurred in 36 cortical tubers (11.9%). The positive rate of electrical stimulation seizures in Type A-E and FCD-like type were 0.7%, 1.4%, 4.8%, 44.4%, 45.0%, and 70.0%, respectively.There was significant difference in the positive rate of electrical stimulation seizures between Type A-B and Type D, Type E and FCD-like type, respectively, so as that between Type C versus Type E and FCD-like type.No significant difference in the positive rate of electrical stimulation seizures was found between other pairwise comparisons. Conclusions:This study proposed a new classification of cortical tubers in TSC patients, and Type E is proposed for the first time in the world.SEEG records confirmed great differences in epileptogenicity indifferent types of cortical tubers.Type D, Type E and FCD-like type have higher epileptogenicity, which is of great value for the preoperative evaluation of TSC epilepsy surgery and the placement strategy of SEEG electrodes.
Allergic rhinitis (AR) in children, as a common chronic inflammatory disease, has significant adverse effects on the physical and mental health, and increases the risk of developing other allergic diseases.Intranasal corticosteroids (INCS), as the most effective drug for treating AR, still have misunderstandings in clinical standardized use.Therefore, Pharmacy Professional Committee, Futang Research Center of Pediatric Development, Pediatric Otorhinolaryngology Head and Neck Surgery Professional Committee, Futang Research Center of Pediatric Development and Clinical Pharmacology Society, Beijing Medical Association organized relevant experts to guide the common clinical usage issues of pediatric INCS, referring to the latest evidence-based medicine research results at home and abroad, and reaching a consensus through discussions among multidisciplinary pharmaceutical and clinical experts, with the aim of providing help for the standardized use of INCS in clinical practice.
The home environment plays a crucial role in ensuring the safe management of respiratory infections in children, yet it is often overlooked.Antipyretic and analgesic medications, cough suppressants, antibiotics, antiviral drugs, expectorants, nebulized inhalation treatments are commonly administered in households when children are affected by respiratory tract infections.Unfortunately, there is still a widespread concerning trend of improper medication usage.It is of paramount importance to grasp the principles of rational drug utilization, clarify usage precautions, accurately interpret medication instructions and prescription information, and understand the key aspects of drug storage when it comes to the safe administration of medications for children with respiratory tract infections.To enhance the management of medication for children with respiratory tract infections within families and to boost the health literacy of family members, a consensus was formulated involving respiratory-related experts, healthcare professionals, and pharmacists, with parents as the primary target audience.
