Objective:We conducted a quality analysis and a survey of current situation of portable blood glucose meters from hospitals and patient sources in Zhejiang Province..Methods:The study is a retrospective cross-sectional study that included 252 portable blood glucose meters, of which 35 portable blood glucose meters (4 in provincial hospitals, 29 in municipal hospitals, and 2 in county-level hospitals) selected for the "Zhejiang Province Portable Blood Glucose Meter Flight Quality Control Inspection" project in April 2023, 126 portable blood glucose meters collected for the "Zhejiang Hospital Portable Blood Glucose Meter Whole Hospital Comparison" project in January 2024, and 91 portable blood glucose meters collected from patients in the "Zhejiang Hospital Home Portable Blood Glucose Meter Convenience Service" project from 2021 to 2023. By comparing the blood glucose results with the reference analyzer, evaluate the qualification rate of portable blood glucose meters between hospitals and patients, imported and domestic brands, and analyze the distribution of blood glucose concentration intervals for unqualified portable blood glucose meters. Conduct a survey questionnaire on the use of portable blood glucose meters by patients to analyze their trust in the results, quality control execution rate, comparison awareness rate, and comparison execution rate.Results:The overall qualification rate of portable blood glucose meters in hospitals (95.7%) was higher than that of portable blood glucose meters use by patients (70.3%), and the difference was statistically significant ( χ 2=31.94 , P<0.001). Among the unqualified blood glucose meters, the unqualified blood glucose concentration is mainly concentrated in the high concentration range (65.9%). Among imported brands, the qualification rate of blood glucose meters from hospitals(99.3%) is higher than that from patients(74.0%), and the difference is statistically significant ( χ 2=36.97, P<0.001). Meanwhile, 75% of patients fully trust the detection results of the blood glucose meter, but its instrument qualification rate is 69%. The awareness rate of patients regarding the comparison of portable blood glucose meters is 73%, but the execution rate of quality control and standard comparison of portable blood glucose meters by patients is 0. Conclusion:The quality of portable blood glucose meters in Zhejiang Province is uneven, with the highest pass rate for portable blood glucose meters from hospitals. Patients have weak awareness of quality control and comparison of portable blood glucose meters.

Objective:We conducted a quality analysis and a survey of current situation of portable blood glucose meters from hospitals and patient sources in Zhejiang Province..Methods:The study is a retrospective cross-sectional study that included 252 portable blood glucose meters, of which 35 portable blood glucose meters (4 in provincial hospitals, 29 in municipal hospitals, and 2 in county-level hospitals) selected for the "Zhejiang Province Portable Blood Glucose Meter Flight Quality Control Inspection" project in April 2023, 126 portable blood glucose meters collected for the "Zhejiang Hospital Portable Blood Glucose Meter Whole Hospital Comparison" project in January 2024, and 91 portable blood glucose meters collected from patients in the "Zhejiang Hospital Home Portable Blood Glucose Meter Convenience Service" project from 2021 to 2023. By comparing the blood glucose results with the reference analyzer, evaluate the qualification rate of portable blood glucose meters between hospitals and patients, imported and domestic brands, and analyze the distribution of blood glucose concentration intervals for unqualified portable blood glucose meters. Conduct a survey questionnaire on the use of portable blood glucose meters by patients to analyze their trust in the results, quality control execution rate, comparison awareness rate, and comparison execution rate.Results:The overall qualification rate of portable blood glucose meters in hospitals (95.7%) was higher than that of portable blood glucose meters use by patients (70.3%), and the difference was statistically significant ( χ 2=31.94 , P<0.001). Among the unqualified blood glucose meters, the unqualified blood glucose concentration is mainly concentrated in the high concentration range (65.9%). Among imported brands, the qualification rate of blood glucose meters from hospitals(99.3%) is higher than that from patients(74.0%), and the difference is statistically significant ( χ 2=36.97, P<0.001). Meanwhile, 75% of patients fully trust the detection results of the blood glucose meter, but its instrument qualification rate is 69%. The awareness rate of patients regarding the comparison of portable blood glucose meters is 73%, but the execution rate of quality control and standard comparison of portable blood glucose meters by patients is 0. Conclusion:The quality of portable blood glucose meters in Zhejiang Province is uneven, with the highest pass rate for portable blood glucose meters from hospitals. Patients have weak awareness of quality control and comparison of portable blood glucose meters.

Modulating Tankyrases (TNKS), interactions with USP25 to promote TNKS degradation, rather than inhibiting their enzymatic activities, is emerging as an alternative/specific approach to inhibit the Wnt/β-catenin pathway. Here, we identified UAT-B, a novel neoantimycin analog isolated from Streptomyces conglobatus, as a small-molecule inhibitor of TNKS-USP25 protein-protein interaction (PPI) to overcome multi-drug resistance in colorectal cancer (CRC). The disruption of TNKS-USP25 complex formation by UAT-B led to a significant decrease in TNKS levels, triggering cell apoptosis through modulation of the Wnt/β-catenin pathway. Importantly, UAT-B successfully inhibited the CRC cells growth that harbored high TNKS levels, as demonstrated in various in vitro and in vivo studies utilizing cell line-based and patient-derived xenografts, as well as APC^min/+ spontaneous CRC models. Collectively, these findings suggest that targeting the TNKS-USP25 PPI using a small-molecule inhibitor represents a compelling therapeutic strategy for CRC treatment, and UAT-B emerges as a promising candidate for further preclinical and clinical investigations.

Objective To measure and compare the cerebral blood flow(CBF)of children with autism spectrum disorder(ASD),global developmental delay(GDD),and ASD with GDD groups via arterial spin labeling(ASL)technique,and to evaluate the diag-nostic value of CBF values.Methods ASL images of ASD,GDD,and ASD with GDD groups of children were firstly acquired,and the CBF values of frontal lobe,temporal lobe,parietal lobe,occipital lobe,striatum and thalamus region of interest(ROI)were fur-ther measured,respectively.One-way analysis of variance or Kruskal-Wallis H test was used to compare the differences in CBF values among these three groups,and the receiver operating characteristic(ROC)curve was used to analyze the efficacy of CBF values in distinguishing ASD with GDD from without GDD.Results ASD with GDD had significantly lower CBF values in the left and right frontal lobes than those with ASD or GDD alone,and the differences were statistically significant(P<0.05).The CBF values in the left and right frontal lobes effectively distinguished ASD with GDD from without GDD[area under the curve(AUC)>0.7].Conclusion ASL technique can noninvasively assess CBF in children with or without GDD,helping to understand the pathophysiology of ASD with GDD and improving diagnostic accuracy.

ObjectiveTo observe the effectiveness and safety of the Chinese herbal medicine Mingshi Granules （明视方颗粒） for low myopia in children with heart yang insufficiency. MethodsA multicentre， prospective， double-blind randomised controlled study was conducted， in which 290 children with low myopia from 8 centres were randomly divided into 145 cases in the treatment group and 145 cases in the control group， and the treatment group was given education， dispensing glasses， and Chinese herbal medicine Mingshi Granules， while the control group was given education， dispensing glasses， and granules placebo. Both Mingshi Granules and placebo granules were taken orally， 1 bag each time， twice daily， 4 weeks of oral intake and 2 weeks of rest as 1 course of treatment， a total of 4 courses of treatment （24 weeks）. Equivalent spherical lenses， best naked-eye distance visual acuity， ocular axis， corneal curvature K1， adjustment amplitude， traditional Chinese medicine （TCM） symptom scores， calculate the amount of progression of equivalent spherical lenses， were observed at the 12th and the 24th week of treatment， at the 36th week and 48th week of follow-up， resectively， the control rate of myopia progression was evaluated at the 24th week， and safety indexes were observed before treatment. ResultsThe amount of progression of equivalent spherical lenses was lower in the treatment group than in the control group at the 48-week follow-up （P＜0.05）. The control rate of myopia progression at 24 weeks after treatment in the treatment group was higher （57.60%， 72/125） than that in the control group （44.63%， 54/121） （P＜0.05）. The best naked-eye distance visual acuity at 36-week follow-up in the treatment group was higher than that in the control group （P＜0.05）. Equivalent spherical lenses were significantly lower in both groups at all observation time points compared with pre-treatment （P＜0.05）， and were higher in the treatment group than in the control group at the 48-week follow-up （P＜0.05）. The ocular axes of both groups were significantly higher at each observation time point after treatment and at follow-up compared with before treatment （P＜0.05）. The amount of eye axis growth in the treatment group was lower than that in the control group at 24 weeks after treatment and at the 48-week follow-up （P＜0.05）. Corneal curvature K1 was significantly lower in the treatment group at the 24th week of treatment compared to pre-treatment （P＜0.05）. The magnitude of adjustment in the treatment group was significantly higher at the 36-week follow-up and at the 48-week follow-up than before treatment （P＜0.05）. The scores of white/dark complexion， white coating thin pulse， fatigue and total TCM symptom scores of children in both groups at the 12th， 24th， 36th and 48th weeks of follow-up were significantly lower than those before treatment （P＜0.05）； the scores of blurred vision at the 24th and 36th weeks of follow-up were significantly lower than those before treatment （P＜0.05）； and the scores of blurred vision in the treatment group at the 48th week of follow-up were signi-ficantly lower than those before treatment （P＜0.05）. In the treatment group， the score of fatigue was higher than that of the control group at the 36-week follow-up， and the score of blurred vision was lower than that of the control group at the 48-week follow-up （P＜0.05）. No adverse reactions or obvious abnormalities of the safety indexes were observed of the two groups during the treatment. ConclusionChinese herbal medicine Mingshi Granules showed the effect of controlling the progression of low myopia， improving the best naked eye distance visual acuity， slowing down the growth of the eye axis， improving some of the TCM symptoms， with good safety.

Objective:To explore the genetic basis of a myopathic patient with pathological characteristics including tubular aggregates and vacuoles.Methods:Next generation sequencing was carried out for the patient, and candidate variant was verified by Sanger sequencing.Results:Genetic testing revealed that the patient has harbored a heterozygous c. 730G>C (p.D244H) variant of Calsequestrin 1 ( CASQ1) gene. The same variant was not found in his unaffected parents. Based on guidelines from the American College of Medical Genetics and Genomics, the variant was rated as pathogenic (PS1+ PM2+ PP3). Conclusion:The novel c. 730G>C (p.D244H) variant of the CASQ1 gene probably underlay the myopathy in this patient. Above finding has enriched the mutational spectrum of the CASQ1 gene.

Objective:To summarize the clinical manifestations, electrophysiological, muscle magnetic resonance imaging (MRI), pathological, and genetic characteristics of 8 patients with Emery-Dreifuss muscular dystrophy (EDMD) to improve the recognition and diagnosis of EDMD.Methods:Eight patients with EDMD confirmed by gene analysis admitted to Hebei Medical University Third Hospital from 2011 to 2022 were enrolled. The detailed clinical symptoms, neurophysiological examination, electrophysiological changes (electromyography and electrocardiography), skeletal muscle MRI characters, skeletal muscle pathological features and gene mutations were analyzed retrospectively.Results:The age of onset ranged from 2.0 to 6.0 (3.6±1.2) years. All patients had insidious onset and progressive development. Muscle weakness was the first symptom for 7 cases that manifested as difficulty in squatting and walking up stairs. Later, spinal ankylosis and joint contracture occurred. One patient had scoliosis as the first symptoms. Abnormal electrocardiogram was found in 4 cases. The electromyography of all patients showed myogenic damage. Muscle biopsy demonstrated dystrophic features in 1 patient, and other myopathic features, including a variation in muscle fiber size, a marked increase in internal nuclei, and, smaller diameter of typeⅠfibers. Next-generation sequencing result showed that 6/8 cases carried 4 LMNA heterozygous mutations (c.1583C>G, c.1357C>T, c.148C>T, c.1336A>G); 1/8 case carried EMD hemizygous mutation (c.501C>G); 1/8 carried SYNE1 heterozygous mutation (c.4364G>A). Conclusions:EDMD has highly clinical and genetical heterogeneity. The onset age is usually in childhood. The first symptom is characterized by weakness of lower limbs and abnormal walking posture. Electromyography shows myogenic lesion. Skeletal muscle MRI shows selective fat infiltrations. Muscle biopsy pathology lacks characteristic pathological findings. It is difficult to make diagnosis and differential diagnosis by clinical manifestations and auxiliary examination in the early stage of the disease. The second generation sequencing technology can improve the early diagnosis rate of EDMD.

This article reported two cases of myopathy type very long-chain acyl coenzyme A dehydrogenase deficiency patients, whose clinical manifestations were mainly repeated rhabdomyolysis. In case 1, with fluctuating muscle weakness and myalgia, pathology of skeletal muscle biopsy showed a small amount of degenerative and necrotic muscle fibers, and some muscle fibers had slightly increased fat components. ACADVL gene complex heterozygous mutation was found by second-generation sequencing. Case 2 showed increased polyacylcarnitine and decreased free carnitine by tandem mass spectrometry. Clinical onset muscle weakness, muscle pain and repeat rhabdomyolysis suggested to consider myopathy type very long-chain acyl coenzyme A dehydrogenase deficiency. Because of no specific performance in lower limb muscle magnetic resonance imaging and skeletal muscle biopsy pathology, the case needed to be differentiated from other metabolic myopathy, and tandem mass spectrometry detection and the second generation sequencing are helpful to diagnosis and differential diagnosis.

The main pathogenesis of liver failure is immune damage and uncontrolled inflammatory response. Glucocorticoids have strong immunosuppressive and anti-inflammatory effects, and are considered to be useful for the treatment of liver failure. However, the results of many clinical studies have shown that the application of glucocorticoids in patients with liver failure cannot effectively improve the prognosis, but instead increases the chance of infection and endangers life. Provided that, it seems reasonable to assume that glucocorticoid resistance exists in patients with liver failure. This article analyzes the mechanism by which P-glycoprotein reverses glucocorticoid transport, intracellular glucocorticoid signaling pathway dysfunction and related gene mutations when the inflammatory response is uncontrolled. In addition, we also evaluated the sensitivity of glucocorticoids in patients with liver failure, so as to provide theoretical basis for efficacy and medication timing.

Liver failure is a severe liver disease syndrome, with massive or sub-massive liver tissues necrosis, and it develops rapidly, with poor clinical prognosis and high mortality. In recent years, autophagy role in the liver failure has received increasing attention. The study of the role of regulatory mechanism of autophagy is of great significance for the in-depth study of the prevention and treatment of liver failure. Based on the research progress of liver failure at home and abroad, this paper explores and summarizes the relationship between autophagy with necrosis and apoptosis, as well as the mechanism and expression level of autophagy in each stage of liver failure, with hope to provide reference for the in-depth research and clinical treatment of liver failure.