A multiplex recombinase polymerase amplification assay combined with CRISPR/Cas12a for the detection of respiratory syncytial virus and respiratory adenovirus.

OBJECTIVE: Respiratory syncytial virus (RSV) and respiratory adenovirus (ADV) are two common pathogens that cause acute respiratory tract infections in children. We aimed to develop a rapid method for detecting both pathogens simultaneously. METHODS: The recombinase polymerase isothermal amplification (RPA) method was combined with the CRISPR/Cas detection system. The assay's specificity and sensitivity were explored by designing RPA primers and CRISPR RNAs (crRNAs) through multi-sequence comparisons, optimizing the reaction conditions, and using a fluorescent reading device. The consistency of the test results of 160 clinical pharyngeal swab samples was studied using quantitative polymerase chain reaction (qPCR) results as a comparative control. RESULTS: RSV and ADV could be detected at levels as low as 104 copies/mL and 103 copies/mL, respectively, within 50 minutes with no cross-reactivity with other similar pathogens. For the clinical samples, compared with the qPCR method, the sensitivities for RSV and ADV were 98.1% and 91.4%, respectively, and the detection specificities were both 100%. The Kappa values were greater than 0.95, suggesting a high degree of consistency. CONCLUSION: This method for detecting RSV and ADV is rapid, sensitive, and specific. It can accurately detect mixed infections in a timely manner, making it suitable for use in areas with scarce healthcare resources.

Correlation of the emm genotyping and virulence genes with the isolation sites of Group A Streptococcus strains from children with impetigo / 中华实用儿科临床杂志

Objective:To investigate the correlation of the emm genotypes and virulence genes with the isolation sites of Group A Streptococcus (GAS). Methods:It was a retrospective study.The specimens were collected from children with impetigo in Beijing Children′s Hospital, Capital Medical University from 2006 to 2008 for GAS isolation and identification.A total of 24 GAS strains were isolated from 16 children with impetigo, among which 7 pairs of strains were isolated from the throat and skin of 7 children, and 1 pair of strains was isolated from the vulva and skin of one child, and the remaining 8 GAS strains were isolated from the skin pus samples of 8 children.Polymerase chain reaction was applied to detect the emm genotypes and 13 virulence genes ( speA, speB, speC, speF, speG, speH, speI, speJ, speK, speL, speM, smeZ and ssa). The correlation of the emm genotypes and virulence genes with the isolation sites of GAS strains was analyzed. Results:In this study, four emm genotypes were detected, including emm1.0 (15/24), emm12.0 (4/24), emm22.0 (2/24) and emm160.0 (1/24), and one subtype emm12.19 (2/24) was detected as well.The carrying rates of 13 virulence genes speA, speB, speC, speF, speG, speH, speI, speJ, speK, speL, speM, smeZ and ssa were 58.3%, 100%, 91.7%, 100%, 50.0%, 12.5%, 54.2%, 66.7%, 16.7%, 25.0%, 12.5%, 100% and 91.7%, respectively.All strains carried 5 to 11 virulence genes and they all carried speB, speF and smeZ.There were significant differences in the carrying rate of speA and speJ among the strains with different emm genotypes (all P<0.05). There was no significant difference in the distribution of virulence genes between skin isolates and pharyngeal isolates, including the 5 pairs of strains carrying the emm1.0 genotype (all P>0.05). Conclusions:The distribution of virulence gene of GAS in children with impetigo is significantly correlated with the emm genotype, rather than the isolation site.

Quantitative analysis of vessel density in the optic disc and macular of patients with idiopathic optic neuritis / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To assess the changes of vessel density in the optic disc and macular of the affected eye and the uninvolved contralateral eye in patients with idiopathic optic neuritis(ON)and to provide clinical guidance for the treatment and follow-up of idiopathic ON.METHODS: A total of 16 patients with first-episode monocular idiopathic ON ≤3 mo diagnosed between December 2019 and December 2021 were included in this cross-sectional study. The eye of patients was divided into 16 eyes in the affected eye group and 16 eyes in the uninvolved contralateral eye group, and 20 healthy age-matched eyes(n=20)served as controls. Optical coherence tomography angiography(OCTA)was performed in all eyes at 4.5 mm×4.5 mm region of the optical disc and 6 mm×6 mm region of the macular, and blood flow indicators were collected and compared. RESULTS: Compared with the control group and the uninvolved contralateral eye group, the density of all vessels and capillary were reduced in the whole area of optic disc, and all subdivisions of the peripapillary region in the ON group(all P&#x003C;0.05). Compared with the uninvolved contralateral eye group, the density of superficial capillary plexus(SCP)was significantly lower in the whole area of macular and perifovea region, and its all subdivisions of the ON eye, as well as in the superior-hemi and superior subdivision of the parafovea region(all P&#x003C;0.05). Compared with the control group, the density of SCP in the inferior-hemi, nasal, and inferior perifovea region was significantly reduced in the ON affected eye group(all P&#x003C;0.05). Compared with the control group, the whole area of macular and its subdivisions in the uninvolved contralateral eye group showed an increase in the density of SCP(P&#x003C;0.05)and an increase in the density of SCP in the parafovea region(P&#x003C;0.05), but no significant change in the inferior-hemi and nasal subdivisions; the increase in the density of SCP in the perifovea region was only significant in the superior-hemi and superior subdivisions(P&#x003C;0.05).CONCLUSION: Patients with ON in the duration of ≤3 mo may showed a decreased vessel density in all peripapillary subdivisions, and a decreased density of SCP in some subdivisions of the perifovea region, accompanied by an increased density of SCP in some subdivisions of the macular region of the contralateral eyes.

Mechanism of Aurantii Fructus and Its Active Components in Regulating Gastrointestinal Motility： A Review / 中国实验方剂学杂志

Gastrointestinal motility disorder is an important cause of digestive system diseases. Patients often suffer from nausea， vomiting， gastric retention， gastroparesis， constipation， and many other symptoms， and their quality of life is seriously reduced. Prokinetic agents are routinely used in clinical practice， but their long-term use is prone to problems such as reduced efficacy and increased adverse reactions. Since the incidence of gastrointestinal diseases has continued to rise globally in recent years， there is an urgent need for clinical development of safe and effective treatment strategies. Aurantii Fructus， a traditional Chinese medicine， has the effect of smoothing Qi and eliminating distention， and it has been used to treat gastrointestinal diseases for thousands of years. In modern clinical practice， it is mainly used for the treatment and auxiliary treatment of various gastrointestinal diseases such as functional dyspepsia， functional constipation， and irritable bowel syndrome. The efficacy is remarkable， and no adverse reactions have been reported at conventional doses. Therefore， it can greatly improve the symptoms of patients with gastrointestinal diseases and improve their quality of life. Modern research has revealed that there are many active components in Aurantii Fructus， among which flavonoids have the highest content and the most types. Flavonoids are the main active components in Aurantii Fructus to regulate gastrointestinal motility. Aurantii Fructus and its active components can affect gastrointestinal hormones， neural pathways， Cajal mesenchymal cells， and other multiple mechanisms. They can adjust gastrointestinal motility and correct gastrointestinal motility disorders， showing potential application value in the treatment of gastrointestinal motility disorders. However， a comprehensive analysis of Aurantii Fructus in this aspect is still lacking. This study summarized the pharmacological activities of active components of Aurantii Fructus extract and its flavonoids， volatile oils， alkaloids， and coumarin on the regulation of gastrointestinal motility and explored the latest research progress on its mechanism. Finally， the adverse reactions of Aurantii Fructus were summarized. It aims to provide a scientific basis for the research and clinical application of Aurantii Fructus and its active components in the regulation of gastrointestinal motility.

Correlation of ARK5 expression in peripheral blood circulating tumor cells with curative efficacy and prognosis for gemcitabine chemotherapy in pancreatic cancer patients / 中华胰腺病杂志

Objective:To detect the expression of ARK5 in peripheral blood circulating tumor cells (CTCs) from pancreatic cancer patients and explore its correlation with the efficacy and prognosis for gemcitabine chemotherapy.Methods:A total of 175 peripheral blood samples of pancreatic cancer patients who were treated in the Department of Hepatobiliary Surgery, Second Affiliated Hospital of Jiaxing University from January 2016 to June 2021 were collected. CTCs were enriched by nano-microfluidic chip technology. The expression of ARK5 in CTCs was detected by immunofluorescence. According to the expression of ARK5, the patients were divided into two groups: positive group and negative group. The differences on clinicopathological features, the efficacy of chemotherapy, median survival and progression-free survival time between the two groups were compared.Results:CTCs were enriched in 98 of 175 patients (55.6%), including 70 ARK5 positive and 28 ARK5 negative patients. There were no significant differences on clinical features between the two groups, and the two groups were comparable. In the 70 ARK5 positive patients, 64 patients (91.4%) were resistant to gemcitabine, while only 12 of the 28 ARK5 negative patients (42.8%) were resistant to gemcitabine. The incidence of gemcitabine resistance in ARK5 positive patients was significantly higher than that in ARK5 negative patients, and the difference was statistically significant ( P<0.001). The median survival time was 11.5 months in ARK5 positive group and 14 months in ARK5 negative expression group, and the progression-free survival time was 6 months in ARK5 positive expression group and 8 months in negative expression group. The survival time of ARK5 positive group was significantly shorter than that of ARK5 negative group, and the difference was statistically significant ( P<0.05). Conclusions:The pancreatic cancer patients with ARK5 positive CTCs have significantly higher incidence of gemcitabine resistance and shorter survival time than those with ARK5 negative CTCs. Detection of ARK5 expression in CTCs may be a new method to judge chemotherapy efficacy and prognosis for pancreatic cancer patients.

Efficacy and safety of dapagliflozin combined with metformin in treatment of type 2 diabetes mellitus / 中国医师进修杂志

Objective:To analyze the efficacy and safety of dapagliflozin combined with metformin in the treatment of type 2 diabetes.Methods:A prospective research method was adopted. A total of 60 patients with type 2 diabetes who were treated in Huainan Chaoyang Hospital from January 2019 to December 2021 were collected as research objects, and the above patients were divided into the observation group (30 cases) and the control group (30 cases) according to the random number table method. After admission, they were treated with oral metformin sustained-release tablets combined with exercise and diet control. On this basis, the observation group was treated with dapagliflozin, while the control group was treated with glimepiride. The blood glucose-related indexes after 3 months of treatment, blood lipid indexes after 1 month of treatment, and adverse reactions were compared between the two groups of patients.Results:After 3 months of treatment, the fasting blood glucose (FBG), 2 h postprandial blood glucose (2 h PBG) and glycosylated hemoglobin (HbA 1c) of the two groups were significantly lower than those before treatment, observation group: (6.60 ± 1.01) mmol/L vs. (7.76 ± 1.82) mmol/L, (10.43 ± 2.74) mmol/L vs. (14.05 ± 3.84) mmol/L, (5.90 ± 1.56)% vs. (8.46 ± 2.07)%; control group: (6.77 ± 0.95) mmol/L vs. (7.82 ± 1.38) mmol/L, (10.17 ± 2.23) mmol/L vs. (14.01 ± 2.63) mmol/L, (6.14 ± 1.51)% vs. (8.73 ± 1.58)% ( P<0.05), but there was no difference in FBG, 2 h PBG and HbA 1c between the two groups ( P>0.05). The total cholesterol (TC) and low-density lipoprotein cholesterol (LDL-C) in the observation group were significantly lower than those in the control group: (5.02 ± 0.98) mmol/L vs. (5.71 ± 0.77) mmol/L, (2.81 ± 0.69) mmol/L vs. (3.39 ± 0.87) mmol/L ( P<0.05). There was no difference in adverse reactions ( P>0.05). Conclusions:For patients with type 2 diabetes mellitus, on the basis of metformin sustained-release therapy, whether combined with dapagliflozin or glimepiride therapy has good hypoglycemic effect, but dapagliflozin has more advantages in improving blood lipids.

Application of typical tasks-based mind mapping in nursing teaching for children with autism spectrum disorder / 中华医学教育探索杂志

Objective:To explore the application of typical tasks-based mind mapping in nursing teaching for children with autism spectrum disorder (ASD).Methods:A total of 102 nursing students who were involved in the nursing of children with ASD in Hunan Children's Hospital were selected and divided into control group and observation group according to the teaching methods. Fifty-one students in the control group were provided with conventional teaching, while 51 students in the observation group were provided with typical tasks-based mind mapping teaching. The students in the two groups were assessed for performance, self-directed learning ability score, and overall literacy at completion of the nursing course. SPSS 22.0 was used for the t test. Results:The scores of theoretical examination[(92.34±4.07) vs. (89.92±3.61)], nursing note writing[(91.07±3.84) vs. (88.60±3.59)], and operational examination[(90.47±2.98) vs. (88.52±2.73)] were significantly higher among students in the observation group than among those in the control group ( P<0.05); after the internship, students in the two groups had significantly increased scores in interpersonal relationships, learning awareness, learning strategies, learning behaviors, and learning evaluation, and the observation group had better performance than the control group in the above indices ( P<0.05); after the internship, students in the two groups had significantly increased scores in problem solving, interpersonal communication, critical thinking, and self-leadership, and the observation group had better performance than the control group in the above indices ( P<0.05). Conclusion:The application of typical tasks-based mind mapping in nursing teaching for children with ASD can improve nursing students' academic performance, enhance their self-directed learning, and improve their overall literacy.

Construction of human TCRP1 gene knockout chronic myeloid leukemia K562 cell line using CRISPR/Cas9 technology and its biological function detection / 中国医师杂志

Objective:To select human chronic myeloid leukemia (CML) cell line K562 as the experimental object, and use lentivirus mediated CRISPR/Cas9 gene editing technology to construct a stable CML cell line K562/TCRP1-KO that knocks out the tongue cancer resistance related protein 1 (TCRP1) gene; and through functional tests such as cell proliferation, apoptosis, and drug sensitivity, compare the phenotypic differences between K562/TCRP1-KO and control cells (K562/cas9-CTL), and preliminarily explore the possible mechanism of TCRP1 gene involvement in the pathogenesis of CML.Methods:The small guide RNA (sgRNA) targeting TCRP1 was designed at a specific location. After annealing, the oligonucleotide fragments were recombined with the linearized Cas9 expression vector, and the lentivirus packaging system was transfected into 293T cells. The purified virus was collected and infected with K562 cells. Positive polyclons were screened for puromycin pressure, and monoclonal K562/TCRP1-KO was further screened by limited dilution method. Stable cell lines were successfully knocked out by sanger sequencing and Western blot detection; Simultaneously, K562 cells transfected with lentiCRISPR vector were constructed as control cell lines (K562/cas9-CTL); Using cell counting method, cell counting kit 8 (CCK8) method, imatinib (IM) gradient dilution method, and flow cytometry cell proliferation, drug sensitivity, and apoptosis analysis were performed on K562/TCRP1-KO and K562/cas9-CTL, respectively.Results:The sgRNA-Cas9 recombinant plasmid vector for TCRP1 knockout was successfully constructed, and after transfection into 293T cells, TCRP1 knockout monoclonal cell lines were successfully screened using limited dilution method. Compared with K562/cas9-CTL cells, the proliferation ability of K562/TCRP1-KO cells was significantly reduced, IM drug sensitivity was significantly enhanced, and the process of cell apoptosis was significantly accelerated (all P<0.05). Conclusions:A CML cell line with TCRP1 knockout was successfully constructed using CRISPR/Cas9. TCRP1 may act as a cancer related gene to affect the proliferation, IM resistance, and apoptosis process of CML cells.

Research progress on anti-factor H antibody associated atypical hemolytic uremic syndrome / 中华实用儿科临床杂志

Atypical hemolytic uremic syndrome (aHUS) is a rare acute and critical disease in childhood, which is easy to lead to acute kidney injury, and has a high mortality rate and chronic kidney disease incidence if not diagnosed and treated in time.According to the etiology, aHUS can be classified into hereditary and acquired.Anti-factor H antibody associated aHUS is acquired and mainly occurs in children aged 5 to 15 years.Anti-factor H antibody associated aHUS is strongly associated with homozygous deletion of the complement H related protein 1/3 (CFRH1/3) gene.In recent years, there have been significant advances in the etiology, genetics, and immunology of aHUS, especially the treatment of Eculizumab, which has greatly improved the prognosis of the disease.However, at present, there are still problems that need to be solved in the pathogenesis, diagnosis, treatment and prognosis of antibody-related aHUS, and this article will discuss the above content and put forward corresponding prospects to provide reference for clinical and scientific research.

Clinical characteristics and prognosis of anti-factor H antibody associated-atypical hemolytic uremic syndrome in children / 中华实用儿科临床杂志

Objective:To summarize the clinical data of anti-factor H antibody-associated atypical hemolytic uremic syndrome (aHUS) in children, and analyze the risk factors for disease recurrence and poor prognosis.Methods:A prospective cohort study was conducted on 52 children with anti-factor H antibody-associated aHUS in Beijing Children′s Hospital, Capital Medical University from November 2011 to November 2021.Patient information about the genetic background, clinical and renal pathological characteristics, treatment, and prognosis were collected.Then, the disease recurrence and prognosis were analyzed using the survival curve and Cox regression model. Results:In 52 children, there were 33 males and 19 females.The average age of onset for aHUS was 2.4-12.8 years, and 92.3%(48/52) of the children developed symptoms at the age of 4-12 years.The copy numbers of complement factor-H-related 1 (CFHR1) and complement factor-H-related 3 (CFHR3) genes were calculated in 42 children.Among the 42 cases, 18 cases (42.9%) had CFHR1 homozygous deletion, and 83.3% (15/18) of them also had CFHR3 homozygous deletion.All the patients were given plasma therapy.Besides, 76.9% (40/52) of the children were treated with immunosuppressive therapy (steroid and/or immunosuppressant) at the first onset of the disease.About 86.5%(45/52 cases) of the patients received immunosuppressive therapy in the course of disease, and the immunosuppressive treatment lasted for 6-20 months in total.The median follow-up time was 58 (28, 91) months.Among 52 patients, only 12 patients (23.1%) suffered disease recurrence.The relapse-free survival rate in children with CFHR1 homozygous deletion was significantly lower than that in children with non-homozygous deletion ( χ2=4.700, P=0.030). The relapse-free survival rate in children with CFHR1 and CFHR3 homozygous deletions was also significantly lower than that in other children ( χ2=4.181, P=0.041). At the end of the follow-up, 73.1%(38/52) of the children had normal renal function and no persistent proteinuria or hypertension.23.1%(12/52 cases) of the children had persistent proteinuria and/or hypertension.One child had Stage 3-4 chronic kidney disease, and 1 child was dialysis dependent. Conclusions:Anti-factor H antibody-associated aHUS is prone to occur in children aged between 4-12 years old, who respond well to plasma therapy and immunosuppressive therapy.Children with anti-factor H antibody-associated aHUS and CFHR1 and CFHR3 homozygous deletions have a high recurrence rate.Treatment with immunosuppressive therapy and assessment of the copy number of CFHR1 and CFHR3 genes in the early stage of the disease are important for preventing disease recurrence and improving prognosis.

Application of optical coherence tomography angiography in optic nerve diseases / 中华实验眼科杂志

Optical coherence tomography angiography (OCTA) can be used to obtain retinal and choroidal blood flow images of optic disc and macular area, and evaluate the vascular morphology and blood perfusion of different layers in different areas of optic disc and macular area.It provides rich possibilities for the description and quantification of optic nerve diseases, the exploration of disease pathogenesis, and the development and evaluation of new treatments.In recent years, OCTA has played an important role in the diagnosis and treatment of optic nerve diseases.It is helpful in the diagnosis of optic neuritis, ischemic optic neuropathy, papilledema secondary to idiopathic intracranial hypertension, and to some extent to evaluate the visual function of affected eyes.The vascular morphology and quantitative analysis of the optic disc and macular area by OCTA may be of value in discriminating optic disc swelling from various etiologies and different types of anterior ischemic optic neuropathy, and facilitate further exploration of the pathogenesis of optic nerve diseases.This article reviewed the application status, recent progress and limitations of OCTA in the diagnosis, differential diagnosis and pathogenesis of optic nerve diseases.OCTA is still not in the stage of meaningful clinical use in neuro-ophthalmology, but its application can be wider as there are more meaningful researches and findings.

Clinical, Pathological and Genetic Analysis of Alport Syndrome in Children / 罕见病研究

  Objective  To explore the phenotype-genotype correlation of Alport syndrome in children.  Methods  Retrospectively analyze the clinical and pathological features of 55 patients with Alport syndrome with COL4A mutations detected by second-generation sequencing, who were treated at Beijing Children's Hospital from January 2016 to December 2020.  Results  A total of 55 children with Alport syndrome were included. All cases had hematuria, including 31 cases (56.4%) with gross hematuria and 24 cases (43.6%) with microscopic hematuria. A total of 39 (70.9%) patients also had proteinuria. Extrarenal manifestations were pres- ent in 12 patients (21.8%). 36(65.4%) patients had a family history of Alport syndrome. 32 patients underwent pathological examination and 23 of them had the specific pathological changes of Alport syndrome. In 55 cases, 36 (65.4%) were diagnosed as X-linked Alport syndrome, 5(9.1%) were diagnosed as autosomal recessive Alport syndrome, 10(18.2%) were diagnosed as autosomal dominate Alport syndrome, and 4(7.3%) were diagnosed as digenic Alport syndrome. Missense mutations in COL4A genes accounted for 62.5%, 67.5% of missense mutations resulted in glycine substitution. There were statistical significances in proteinuria degree and hearing loss between male and female patients with XLAS (P < 0.05) as well as statistical significance in the degree of proteinuria between autosomal recessive Alport syndrome and autosomal dominate Alport syndrome (P=0.044), and there was critical statistical significance in the age of onset. There was statistical significance in hearing loss between children with renal impairment and children with normal renal function (P=0.001).  Conclusions  Most of the pathogenic variants in COL4A genes that cause Alport syndrome result in glycine substitutions. The degree of proteinuria and hearing loss of males with XLAS were greater than those of females. The degree of proteinuria in autosomal recessive Alport syndrome was greater than that of children with autosomal dominate Alport syndrome, and the age of onset was earlier than that of autosomal dominate Alport syndrome. Renal manifestation was more severe in children with hearing loss. The early clinical manifestations of Alport syndrome are diverse and pathological manifestations may be atypical. The application of next-generation sequencing can reduce misdiagnosises of Alport syndrome.

A Case of Renal Coloboma Syndrome Caused by Spontaneous Mutation of PAX2 / 罕见病研究

This article reports a case of a child with full-length (paired box family, PAX) PAX2 mutation leading to renal coloboma syndrome. The patient is an 11-year-old boy presented with persistent foamy urine and unexplained renal failure. The boy has suffered from vision decline ever since infancy. Genetic testing confirms the mutation of the PAX2 splice site (c.862-1G > A). Sanger sequencing shows no mutation at this site in his parents and demonstrates a spontaneous mutation. His clinical manifestations also confirms diagnosis of renal coloboma syndrome. The PAX2 mutation was responsible for the boy's progression to end-stage renal disease and extrarenal manifestations.

Progress in Diagnosis and Treatment of Children having Fabry Disease / 罕见病研究

Fabry disease (FD) is a rare progressive X-linked genetic lysosomal storage disorder. Mutations of the GLA gene result in deficiency of α-galactosidase (α-Gal A), and the accumulation of glycosphingolipids, particularly globotriaosylceramide (GL-3) and derivatives deacylated derivative globotriaosylsphingosine (Lyso-GL-3) in multiple tissues of the body systems, eventually leading to lesions in multiple organs. The symptoms commonly seen in childhood include neuropathic pain, gastrointestinal dysfunction, angiokeratoma and cornea verticillata, and others. The fact that early symptoms are not specific usually causes the delay in diagnosis of Fabry disease. Making definite diagnosis needs to involve the activity of α-Gal A, GL-3, Lyso-GL-3, biomarkers, pathology and genetic tests. The early start of treatment using enzyme replacement therapy (ERT) is effective in alleviating the signs and symptoms of Fabry disease and in preventing disease progression.

Chinese experts′ consensus statement on diagnosis, treatment and prevention of Group A Streptococcus infection related diseases in children / 中华实用儿科临床杂志

Group A Streptococcus (GAS) is a very important pathogen, especially for children.On a global scale, GAS is an important cause of morbidity and mortality.But the burden of disease caused by GAS is still unknown in China and also has not obtained enough attention.For this purpose, the expert consensus is comprehensively described in diagnosis, treatment and prevention of GAS diseases in children, covering related aspects of pneumology, infectiology, immunology, microbiology, cardiology, nephrology, critical care medicine and preventive medicine.Accordingly, the consensus document was intended to improve management strategies of GAS disease in Chinese children.

Clinical analysis of acute post-streptococcal glomerulonephritis and C 3 glomerulopathy in children / 中华实用儿科临床杂志

Objective:To investigate the clinical features of acute post-streptococcal glomerulonephritis (APSGN) and C 3 glomerulopathy (C 3G) in children, and to improve the understanding, diagnosis and treatment of C 3G in children with atypical APSGN. Methods:The clinical data of 100 children whose were clinically diagnosed with APSGN and admitted to the Beijing Children′s Hospital, Capital Medical University from January 2016 to December 2021 were collected and retrospectively analyzed.Seventy-three cases finally diagnosed with APSGN were included in the APSGN group and 27 cases with C 3G were classified into the C 3G group.The clinical manifestations, laboratory results, treatment and prognosis of the 2 groups of children were analyzed and compared by the t-test, Mann-Whitney U test and χ2 test. Results:Both APSGN and C 3G patients had a history of streptococcal infection at the early stage of the disease.There was no significant difference in the onset age and gender between the 2 groups (all P>0.05). The clinical manifestations of APSGN and C 3G at the early stage are sometimes difficult to distinguish.However, the incidence rates of gross hematuria (92.6%) and nephrotic proteinuria (66.7%) in the C 3G group were higher than those in the APSGN group (69.8%, 30.1%) ( χ2=5.583, 10.960; all P<0.05). The laboratory test results suggested that compared with the C 3G group, the APSGN group had higher albumin levels [(36.3±7.4) g/L vs.(28.9±6.8) g/L], but lower triglycerides [(1.2±0.6) mmol/L vs.(1.6±0.7) mmol/L], blood urea [(7.6±5.6) mmol/L vs.(14.7±16.3) mmol/L], blood creatinine [(66.2±45.2) μmol/L vs.(120.1±170.3) μmol/L], and urine protein levels [(43.5±58.5) g/24 h vs.(319.2±994.8) g/24 h] ( t=4.655, 2.738, 2.241, 1.624, 1.448; all P<0.05). As for treatment, the use rates of hormones and other immunosuppressants in the C 3G group were higher than those in the APSGN group (59.3% vs.12.3%, 29.6% vs.1.4%) ( χ2=23.15, 19.22; all P<0.05). The follow-up data revealed that compared with the APSGN group, the C 3G group took a longer time for gross hematuria and microscopic hematuria symptoms to disappear, proteinuria test to turn negative and complement C 3 to recover [51.1(14.3, 90.0) d vs.14.9(6.0, 15.5) d; 218.3(60.0, 277.5) d vs.65.5(27.0, 82.5) d; 127.9(60.0, 180.0) d vs.38.2(13.0, 53.6) d; 129.3(55.5, 225.0) d vs.39.1(24.0, 51.0) d] ( U=2.395, 2.730, 2.890, 3.054; all P<0.05). Conclusions:APSGN children with relatively severe clinical manifestations during the acute stage, especially with unrelieved nephrotic proteinuria, should be highly suspected with C 3G.Such patients should be treated with steroids and undergo renal biopsy and complement investigation if necessary, so as to identify the cause early, adjust the treatment and improve their prognosis.

Status quo of hemoglobin a1c in community-dwelling patients with type 2 diabetes mellitus and its relationship with health literacy / 中国基层医药

Objective:To investigate the status quo of hemoglobin a1c (HbA1c) in community-dwelling patients with type 2 diabetes mellitus and its relationship with health literacy, and analyze the factors that affect HbA1c level.Methods:A total of 134 patients with T2DM who received treatment in Hbxi Hospital Affiliated to Jining Medical University (Shanxian Central Hospital) from February 2019 to February 2020 were included in this study. All patients were divided into standard group (HBA1c < 7%, n = 61) and substandard group (HbA1c ≥ 7%, n = 73) according to HbA1c level. Fasting elbow venous blood was collected to measure relevant biochemical indexes. General data were collected from patients in the two groups. The Chinese Citizen Health Literacy Questionnaire was used to evaluate the health literacy level in patients from the two groups. Results:Serum levels of creatinine, blood uric acid and blood urea nitrogen in the substandard group were significantly higher than those in the standard group (all P < 0.05). Health literacy score in the substandard group was significantly lower than that in the standard group ( P < 0.05). The percentage of patients who smoked or had relevant complications among all included patients in the substandard group was 17.81% and 16.44%, respectively, which were significantly higher than 3.28% and 4.92%,respectively, in the standard group ( χ2 = 7.05, 4.43, both P < 0.05). The percentage of patients who had regular glucose level among all included patients in the substandard group was significantly lower than that in the standard group (63.93% vs. 80.82%, χ2 = 4.82, P < 0.05). Pearson analysis results showed that HbA1c level was significantly negatively correlated with health literacy score, with the correlation coefficient r of -0.80. Logistic regression model results showed that serum levels of creatinine and blood uric acid were independent risk factors for substandard level of HbA1c, with an OR value of 3.81 (95% CI: 1.60-9.09) and 1.03 (95% CI:1.01-1.06), respectively. Health literacy score was a protective factor for substandard level of HbA1c, with an OR value of 0.23 (95% CI: 0.10-0.54). Conclusion:Less than 50% of community-dwelling patients have standard HbA1c levels. Health literacy score is remarkably negatively correlated with HbA1c level. Health literacy score is a protective factor for HbA1c level, while serum creatinine and blood uric acid are independent risk factors for substandard HbA1c.

Clinical analysis of methylmalonic acidemia presented with hemolytic uremic syndrome in children / 中华实用儿科临床杂志

Objective:To understand clinical characteristics, treatment effects and prognosis of children with methylmalonic acidemia (MMA) presented with hemolytic uremic syndrome(HUS).Methods:The medical records of children with MMA were collected in Beijing Children′s Hospital, Capital Medical University from January 2012 to January 2019, the clinical manifestations, laboratory, imaging material, inspection results, renal pathological, gene analysis, treatment effect, and prognosis of MMA children with renal damage were analyzed, and were followed-up for 1-7 years.Results:Thirty cases were diagnosed as MMA with secondary renal damage.Eight cases(26.67%) showed as MMA-HUS.Age was from 1 month and 14 days to 12 years and 10 months old.There were 4 males and 4 females.The concentration of urine methylmalonic acid increased by 10-62 times.All were combined with hyperhomocysteine(HCY). The level of serum methylmalonic acid(1.5-11.8 mg/L), propylene carnitine(6.33-9.77 μmol/L)and the ratio of propylene /ethylene carnitine (0.24-0.29)were increased.Manifested as the mental and physical development retardation, anemia, jaundice, renal dysfunction, platelet reduction, hematuria, proteinuria in 8 cases, hypertension in 6 cases, frequent vomiting and convulsions in 2 cases.Two cases had a positive family history.Renal pathology showed that mesangial cells and mesangial matrix proliferation broadening, electron dense deposits no mesangial area, renal tubular epithelial cell swelling degeneration, immunofluorescence was negative.Two cases were genetically analyzed. One case was a CblC type MMACHC compound heterozygous mutation[c.80A>G(p.Q27R); c.217C>T(p.R73X)] and CblX type HCFC1 heterozygous mutation [c.3757G>A(p.R1253C)] double mutation; 1 case was a CblC type MMACHC compound heterozygous mutation[c.365A>T(p.H122L); c.609 G>A(p.W203X)]. Children diagnosed were treated with vitamin B 12, etc.Four cases of children gave up.The others, after treatment, were improved. Conclusions:MMA-HUS might be associated with multiple organ failure.Early diagnosis was the key, timely treatment could effectively control the disease, improve the prognosis.It should be followed up for ever.

Single center investigation of anemia in children with chronic kidney disease stage 3-5D / 中华肾脏病杂志

Objective:To explore the clinical characteristics of chronic kidney disease (CKD) at the stage 3-5D in children with renal anemia, and provide reference data for standardized diagnosis and treatment.Methods:A single-center retrospective study was conducted to collect clinical data in children with CKD at Beijing Children's Hospital Affiliated to Capital Medical University from January 2016 to December 2018. The patients were divided into CKD stage 3 group, stage 4 group and stage 5 group according to estimated glomerular filtration rate. The indexes of anemia among the groups were compared. Data on anemia indicators, treatment, and anemia improvement in maintenance dialysis children at stage 5D were analyzed.Results:A total of 171 children with CKD were included in the study. The hemoglobin levels in CKD stage 3 group, stage 4 group and stage 5 group were (126.4±20.5) g/L, (90.8±26.0) g/L and (78.7±18.4) g/L, respectively, and there was a statistical difference among the groups ( χ2=61.982, P<0.001; trend test F=71.061, P<0.001). The incidences of anemia in children with CKD stage 3, stage 4 and stage 5 were 27.3% (9/33), 83.3% (25/30) and 95.4% (105/108), respectively. Mild, moderate and severe anemia in children with CKD stage 3 accounted for 15.2%(5/33), 12.1% (4/33) and 0(0), respectively. Mild, moderate and severe anemia in children with CKD stage 4 accounted for 26.7% (8/30), 50.0% (15/30) and 6.7% (2/30), respectively. Mild, moderate and severe anemia in children with CKD stage 5 accounted for 21.3%(23/108), 60.2%(65/108) and 15.8%(17/108), respectively. Anemia type was mostly normocytic anemia. The hemoglobin of 30 children with CKD stage 5D at the initial stage of dialysis was (79.3±16.3) g/L. Twenty-three children with CKD stage 5D received erythropoietin combined with oral iron or intravenous iron therapy. The hemoglobin compliance rates in children with maintenance dialysis in initial phase, 1 month, 2 months and 3 months were 6.7% (2/30), 16.7%(5/30), 63.3%(19/30) and 90.0%(27/30), respectively. The correction time for anemia was (2.5±1.0) months. Twelve children with CKD stage 5D received iron sucrose infusion, and no adverse reaction occurred. Conclusions:Renal anemia has a high incidence in children with CKD. Early and standardized treatment is of great significance to improve outcome of renal anemia. Venous iron infusion is a safe and effective treatment method for children with maintenance dialysis.

An empirical study on the evaluation system of clinical nurse training in hospitals of traditional Chinese and Western medicine based on post competency / 中华医学教育探索杂志

Objective:To evaluate the discrimination, reliability and validity of the evaluation system of clinical nurse training based on post competency in hospitals of traditional Chinese and Western medicine.Methods:From August 2018 to January 2019, 271 clinical nurses from a three-A hospital were selected by convenient sampling method. The competency of clinical nurses was assessed on the spot by case tracking method. The discrimination, reliability and validity of the evaluation system were tested by item analysis, Cronbach's α coefficient and confirmatory factor analysis.Results:The evaluation system had good discrimination, reliability and validity. Among the four scales of the evaluation system, there were significant differences in the high score and the low score of all items ( P < 0.05). The Cronbach's α coefficient of each dimension of the four scales was 0.769-0.898. Four structural equation model diagrams were established, the AVE (average variance extracted) of each dimension was 0.51-0.74, factor load was 0.53-0.93, C.R. (composite reliability) was 0.79-0.91, and the discrimination validity was up to the standard. Conclusion:This system provides a reference for the establishment of scientific, objective, measurable and homogeneous clinical nurse training evaluation tools.