Children and adults differ significantly in physiology,biochemistry and immune function,which leads to sig-nificant differences in blood transfusion strategies between children and adults.To guide the clinical transfusion practice of pediatric patients and improve the prognosis of children,the National Health Commission organized the formulation and re-lease of the health industry standard Guideline for Pediatric Transfusion(WS/T 795-2022).This paper will briefly introduce some concepts that help understand of the Standard and the preparation process of the Standard,and explain and interpret the preparation of the"scope","general provisions"and"factors to consider"of the Standard,hoping to contribute to the understanding and implementation of the Standard.

Objective To analyze the epidemiological characteristics and temporal spatial clustering of hand, foot, and mouth disease in Hubei Province from 2010 to 2023, and to provide evidence for formulating prevention and control measures. Methods Descriptive epidemiological method was used to analyze the surveillance data of hand, foot, and mouth disease of Hubei Province from 2010 to 2023, and spatial clustering analysis was conducted at the district/county level using ArcGIS 10.5 software. Results A total of 1 007 600 cases of hand, foot, and mouth disease were reported from 2010 to 2023, and the average annual incidence rate was 123.60/100 000. Overall , it exhibited a cyclical pattern of high incidence every other year. Except for a few years, two peaks of incidence were observed each year, , with the first peak occurring between April to July and the second occurring in October to December, and the popular season was concentrated from April to July. Children aged < 5 years were primarily affected, with a high incidence in male patients (1.53:1). The incidence of hand, foot, and mouth disease showed a positive spatial autocorrelation（Moran's I was between 0.15 to 0.76, P<0.05）at the district/county level. The hot spots were concentrated in the northwest and southeast of Hubei Province, and the cool spots were concentrated in the east of central Hubei Province. Conclusion The incidence of hand, foot, and mouth disease has obvious seasonality and spatial clustering in Hubei Province. The key prevention and control areas are concentrated in the northwest and southeast of Hubei. Enhanced prevention and control measures should be targeted on children under 5 years old and key areas to effectively reduce the occurrence of cases.

[摘 要] 以靶向PD-1/PD-L1和CTLA-4为代表的免疫检查点阻断治疗在实体瘤的治疗中取得了不俗疗效，但仅有不到30%的患者能够从中受益的现实表明，还存在其他免疫检查点分子介导的免疫抑制。B7H3属于免疫球蛋白超家族B7家族成员，与CTLA-4/PD-1主要表达在T细胞并介导后者的免疫抑制或耗竭不同，B7H3蛋白不仅诱导性表达在免疫细胞，还组成性高表达在多种肿瘤细胞和肿瘤相关脉管系统。B7H3不仅能够激活多条信号通路直接促进肿瘤细胞恶性表型，还可以通过重塑肿瘤免疫抑制微环境间接促进肿瘤的进展、转移和耐药。因此，基于B7H3的多项靶向抗肿瘤策略，包括特异性抗体、抗体偶联药物及CAR-T细胞等均已进入临床试验并展示出较好的应用前景。但总体而言，该领域的研究依然处于探索阶段，在相互作用受体的鉴定、降低毒性、打破耐药及联合用药策略优化等方面还面临着许多问题和挑战亟待突破。

Platinum-based chemotherapy resistance is a key factor of poor prognosis and recurrence in hepatocellular carcinoma (HCC). Herein, RNAseq analysis revealed that elevated tubulin folding cofactor E (TBCE) expression is associated with platinum-based chemotherapy resistance. High expression of TBCE contributes to worse prognoses and earlier recurrence among liver cancer patients. Mechanistically, TBCE silencing significantly affects cytoskeleton rearrangement, which in turn increases cisplatin-induced cycle arrest and apoptosis. To develop these findings into potential therapeutic drugs, endosomal pH-responsive nanoparticles (NPs) were developed to simultaneously encapsulate TBCE siRNA and cisplatin (DDP) to reverse this phenomena. NPs (siTBCE + DDP) concurrently silenced TBCE expression, increased cell sensitivity to platinum treatment, and subsequently resulted in superior anti-tumor effects both in vitro and in vivo in orthotopic and patient-derived xenograft (PDX) models. Taken together, NP-mediated delivery and the co-treatment of siTBCE + DDP proved to be effective in reversing chemotherapy resistance of DDP in multiple tumor models.

OBJECTIVE: To explore the coagulation deficit and genetic basis for a Chinese pedigree affected with Congenital dysfibrinogenemia (CD). METHODS: Peripheral venous blood samples of the proband and her family members (including 4 individuals from three generations) were subjected to routine blood test and assays of liver and kidney functions and viral hepatitis to exclude related diseases. Clauss method and DFg-PT method were used to determine the fibrinogen activity (Fg:C), and an immunoturbidimetric assay was used to determine the level of fibrinogen antigen (Fg:Ag). All of the exons (22 in total) and their flanking sequences of the FGA, FGB and FGG genes were amplified by PCR and directly sequenced. Variants in the coding regions of the three genes and transcriptional splicing sites were screened by using Mutation Surveyor^TM software. RESULTS: The Clauss method showed that Fg:C was significantly reduced in the proband and her father, whilst her mother and son were normal. With the DFg-PT method, the proband, her parents and son were all within the normal range. The Fg:C/Fg:Ag ratio of the proband and her father was lower than 0.7, whilst her mother and son were above 0.7. No significant change in the prothrombin time, activated partial thromboplastin clotting time and thrombin time was noted. Two genetic variants were detected, which included a homozygous missense variant in the FGA gene [c.991A>G (p.Thr331Ala)], which was predicted to be benign, and a heterozygous missense variant of the γ chain of the FGG gene [c.1211C>G (p.Ser404Phe)], which is located in a conserved region and unreported in the CLINVAR/HGMD/EXAC/1000G databases and literature. CONCLUSION This pedigree has conformed to the autosomal dominant inheritance of CD. The c.1211C>T (p.Ser404Phe) missense variant of the γ chain of the FGG gene probably underlay the pathogenesis of CD in this pedigree. The variant was unreported previously and named as "Fibrinogen Harbin II Ser404Phe".
Female ; Humans ; Afibrinogenemia/congenital* ; East Asian People ; Fibrinogen/genetics* ; Mothers ; Mutation ; Pedigree

Uveal melanoma (UM) is the most common intraocular malignancy in adults. Recently, great progresses have been made in the diagnosis, treatment and prognosis of UM, however, nearly 50% of patients still develop liver metastases, which severely affects on the survival of UM patients. Whether UM patients will benefit from the immune checkpoint blockade similarly as the cutaneous melanoma (CM)? Whether the specific gene mutations targeting UM could improve the anti-tumor efficacy? Whether chimeric antigen receptor T cell or T cell receptor T cell immunotherapy is effective to UM patients with liver metastases? How about the combinational therapies in UM and the clinical effects? This review summarizes the anti-tumor research and novel treatment options of UM, analyzes the current achievements and problems.

Objective:To investigate frailty in older patients with comorbidities and explore related risk factors.Methods:A cross-sectional study was conducted with an enrollment of 746 patients aged 65 years or older with comorbidities in the Wanshoulu Road area of Beijing from April 2019 to December 2020.A total of 617 patients with comorbidities were finally included, aged(85.6±4.8)years, including 358 women(58.0%); According to the FRAIL scale, 617 patients with comorbidities were divided into a frail group(156 cases, 25.3%)and a non-frail group(461 cases, 74.7%). Demographic data and information on comorbidities were collected.Univariate and multivariate Logistic regression analyses of risk factors were conducted.Results:Among 617 patients with comorbidities, the common chronic diseases in descending order were hypertension(497 cases, 80.6%), coronary heart disease(375 cases, 60.8%), osteoporosis(357 cases, 57.9%), osteoarthritis(281 cases, 45.5%), type 2 diabetes(211 cases, 34.2%), stroke and/or transient ischemic attack(193 cases, 31.3%), chronic lung disease(144 cases, 23.3%), tumor(133 cases, 21.6%), chronic kidney disease(92 cases, 14.9%), and heart failure(58 cases, 9.4%). Univariate logistic regression analysis showed that age, type 2 diabetes, coronary heart disease, heart failure, chronic lung disease, stroke/transient ischemic attack, cancer and osteoarthritis were influencing factors for frailty( P<0.05). Multivariate logistic regression analysis showed that age, type 2 diabetes, heart failure, chronic lung disease, cancer and osteoarthritis were risk factors for frailty( OR=1.076, 1.806, 3.275, 3.371, 1.640, 2.227, all P<0.05). Conclusions:Old age, type 2 diabetes, heart failure, chronic lung disease, tumor and osteoarthritis are closely related to frailty in elderly patients with comorbidities.Proactive and effective prevention and intervention should be instituted to target risk factors for frailty to reduce the occurrence of adverse outcomes.

Objective:To study the duration of invasive mechanical ventilation (MV) and its influencing factors after neonatal abdominal surgery under general anesthesia in neonatal intensive care unit (NICU).Methods:From January 2018 to December 2020, neonates received abdominal surgery under general anesthesia and needed endotracheal intubation and MV after surgery in NICU of our hospital were retrospectively studied. According to MV duration, the neonates were assigned into <72 h group and ≥72 h group. Multivariate logistic regression was used to analyze the risk factors of postoperative MV duration.Results:A total of 113 neonates were enrolled, including 57 male (50.4%) and 56 female (49.6%). The gestational age was (35.7±3.6) weeks, the birth weight was (2 497±933) g, the average operation age was 9.9(3.6, 22.2) d and the average hospital stay was 22.0(12.0,37.0) d. Congenital intestinal obstruction (37/113, 32.7%) was the most common diagnoses on discharge, followed by neonatal necrotizing enterocolitis(28/113,24.8%) and gastrointestinal perforation (18/113,15.0%). The duration of operation was 80.0 (55.8,117.3) min. All neonates needed MV with endotracheal intubation. The duration of postoperative respiratory support was 30.0(7.0,84.5) h. 48 neonates (42.5%) had endotracheal intubation removed within 24 h after surgery. Multivariate logistic regression analysis showed that preoperative respiratory support ( P=0.004), congenital heart disease( P=0.013) and intravenous midazolam ( P=0.032) were independent risk factors for prolonged postoperative MV. Conclusions:The need of preoperative respiratory support, congential heart disease and intravenous midazolam were independent risk factors for the duration of postoperative MV after neonatal abdominal surgery under general anesthesia.

OBJECTIVE: To diagnose and fine map a deletion in chromosome region 2q37. METHODS: G-banded chromosomal karyotyping, multiplex ligation-dependent probe amplification (MLPA), single nucleotide polymorphism array (SNP-array), and fluorescence in situ hybridization (FISH) were carried out in conjunct for the analysis. RESULTS: The patient was found to have karyotype of 46,XY,del(2)(q3?), MLPA revealed one copy number of both CAPN10-3 and ATG4B-7 genes from the 2q37.3 region, Both parents were found to be normal upon chromosome karyotyping and MLPA. SNP-array has found a 9.7 Mb deletion in the 2q37.1.37.3 region. FISH analysis has confirmed there is a single copy for 2q37.3. CONCLUSION Combination of MLPA, FISH and SNP-array have enabled accurate diagnosis for the patient, and also provided more clues for the correlation of genotype with the phenotype of the disease, and a basis for genetic counseling.
Chromosome Banding ; Chromosome Deletion ; Humans ; In Situ Hybridization, Fluorescence ; Karyotyping ; Phenotype

Objective To analyze the epidemiological characteristics and incidence trend of gonorrhea in Hubei Province, and to provide reference for scientific formulation of prevention and control measures. Methods Based on the surveillance data of gonorrhea from 2010 to 2021, three-way distribution and ARIMA model were used for data analysis and incidence prediction. Results From 2010 to 2021, the reported incidence rate fluctuated between 3.01/100 000-7.07/100 000, and the average annual reported incidence rate was 4.62/100 000. The reported incidence rate showed the characteristics of ^“first fall and then rise, and then fall and rise again^”, and the peak incidence period was from June to December. The male to female ratio of reported cases was 5.78:1, and the number of reported cases in the age group of 20-39 years old accounted for 62.43% of the total number of cases. The reported cases were mainly housework and unemployed, farmers, and unknown occupation. The severity of the regional incidence was divided into 5 categories by the Q-type clustering, and the most serious category included Shennongjia Forest District, Huangshi City, and Wuhan City. The ARIMA model predicted the incidence rate to be in good agreement with the actual incidence rate, with a predicted number of 3 343 cases in 2022. Conclusion At present, gonorrhea in Hubei Province is still at a high prevalence level. There are obvious differences in gender, age, occupation, and regional distribution. The ARIMA model is suitable for predicting the incidence of gonorrhea, and it is predicted that the incidence will increase slightly in 2022.