Objective:To summarize and analyze the clinical phenotype and genotype characteristics of atypical hemolytic uremic syndrome (aHUS) in Chinese children.Methods:It was a retrospective study. The clinical data and genetic results of 6 children with aHUS admitted to Children's Hospital Affiliated to Capital Institute of Pediatrics from May 2016 to October 2022 were analyzed, and literature on Chinese aHUS children with genetic screening data by searching databases such as Wanfang, CNKI, and PubMed were reviewed and summarized. Through literature search, the children with aHUS were divided into genetic variation group and non-genetic variation group according to the results of genetic testing, and the differences of clinical phenotype, laboratory examination, follow-up and outcomes were compared between the two groups. Logistic regression method was used to analyze the risk difference of disease recurrence, end-stage kidney disease and death between genetic variation group and non-genetic variation group.Results:Among the 6 aHUS children in this center, there were 1 male and 5 females, with onset age of 7 months to 10 years old. Four patients had gene variations, including 1 patient of complement factor H ( CFH) gene variation, 1 patient of C3 gene variation, and 2 patients of CFHR1 combined with CFHR3 gene variation. Six children had gross hematuria, proteinuria, hypertension and decreased complement C3. The mean values of serum creatinine in 4 genetic variation and 2 non-genetic variation children were 153.9 μmol/L and 214.3 μmol/L, respectively; the mean values of estimated glomerular filtration rate were 26.4 ml·min -1·(1.73 m 2) -1 and 28.9 ml·min -1·(1.73 m 2) -1, respectively; the mean values of hemoglobin were 81 g/L and 57 g/L; the mean values of platelet were 46×10 9/L and 71×10 9/L; the mean values of lactic dehydrogenase were 2 408 U/L and 2 106 U/L, respectively; there were 1 and 2 cases of positive CFH antibody, and 1 and 1 case of nervous system complication, respectively. Ninety-seven aHUS children were retrieved including the reported 6 cases in this center, with 60 males and 37 females, and median onset age of 5 years old. The positive detection rate of genetic variation was 58.8% (57/97). The main type of genetic variation was CFHR gene variation (43.9%, 25/57), followed by CFH gene variation (33.3%, 19/57).There was no significant difference in onset age, sex distribution, proportions of gross hematuria, massive proteinuria, hypertension, complement C3 decline, positive CFH antibody and treatment method, platelet, and lactic dehydrogenase between genetic variation group and non-genetic variation group (all P>0.05). Compared with the genetic variation group, non-genetic variation group had higher serum creatinine ( Z=2.311, P=0.021) and lower hemoglobin ( Z=-2.636, P=0.008). The median follow-up time in genetic variation group and non-genetic variation group was 1 year and 2 years, respectively. The proportions of non-remission and recurrence in the genetic variation group were significantly higher than those in non-genetic variation group ( χ2=12.016, P=0.002; χ2=4.689, P=0.030). Logistic regression analysis showed that the recurrence risk of aHUS in children with genetic mutations was higher than that in children with non-genetic mutations ( OR=2.807, 95% CI 1.014-7.772). Conclusions:The main type of aHUS gene variation in Chinese children is CFHR gene variation, and the children with gene variation have poor prognosis and a higher risk of recurrence.

Multiple sclerosis(MS)is a systemic inflammatory illness of the central nervous system that involves demyelinating lesions in the myelin-rich white matter and pathology in the grey matter.Despite signifi-cant advancements in drug research for MS,the dis-ease's complex pathophysiology makes it difficult to treat the progressive forms of the disease.In this study,we identified a natural flavonoid compound icariin(ICA)as a potent effective agent for MS in ameliorating the deterioration of symptoms including the neurological defi-cit score and the body weight in a murine experimental autoimmune encephalomyelitis(EAE)model.These improvements were associated with decreased demyelin-ation in the corpus callosum and neuron loss in the hippo-campus and cortex confirmed by immunohistochemistry analysis.Meanwhile,it was observed that the activation of microglia in cerebral cortex and hippocampus were inhibited followed by the neuroinflammatory cytokines downregulation such as IL-1β,IL-6 and TNF-α after ICA treatment,which was probably attributable to the sup-pression of microglial NLRP3 inflammasome activation.Additionally,molecular docking also revealed the binding force of ICA to NLRP3 inflammasome protein complexes in vitro.Taken together,our findings have demonstrated that ICA,as pleiotropic agent,prevents EAE-induced MS by improving demyelination and neuron loss,which inter-feres with the neuroinflammation via microglial NLRP3 inflammasome activation.

AIM: To evaluate the pharmacokinetics and bioequivalence of cetirizine hydrochloride tablets under fasting and fed conditions in Chinese healthy subjects. METHODS: This was a randomized, open-label, double-sequence, two-period, crossover designed study, and healthy subjects enrolled and administrated a single dose of 10 mg test and reference cetirizine hydrochloride tablets in each period under fasting or fed condition. The plasma concentrations of cetirizine were determined by a validated LC-MS/MS method. The pharmacokinetic parameters were calculated with WinNonlin 6.3 and the bioequivalence was evaluated through SAS 9.4 software. RESULTS: In the fasting condition, the major pharmacokinetic parameters of cetirizine of test and reference formulations were as follows, C

Objective:To understand the subjective sensation of professional status of nurses in the ward so as to provide a basis for nursing managers.Methods:From December 2018 to February 2019, convenience sampling was used to select 410 nurses from 8 Class Ⅲ Grade A hospitals in 5 cities, including Beijing, Shijiazhuang, Taiyuan and Changsha, etc. A self-designed Subjective Sensation of Ward Nurses' Professional Status Questionnaire was used to investigate nurses.Results:Among 410 nurses, 68.0% (279/410) of nurses experienced workplace violence in the past year; 55.4% (227/410) of nurses were satisfied or very satisfied with their current work; 71.2% (292/410) of nurses had no plans to change jobs in the near future. 37.1% (152/410) of nurses would recommend others to study or work in nursing.Conclusions:The subjective sensation of professional status of nurses in the ward need to be improved. Nurses' exposure to workplace violence cannot be ignored, and satisfaction needs to be further improved, and the profession identity still needs to be strengthened.

Objective To introduce improved program for traction wire production in endoscopic submucosal dis-section assisted by oral traction. Methods A retrospective analysis was performed on 40 patients who received en-doscopy intervention. Through the use of improved traction and normal traction, they were divided into experimental group and control group. Then analyze and compare their clinical data like gender, age, traction line installed perfect time, fixed lesions after hemostatic clip off times, one-time complete resection, enbloc resection rate, bleeding and perforation and other complications and other clinical conditions. Results The improvement time was (53.30 ±12.85) s in experimental group, it was significantly shorter than that in control group (105.00 ± 11.68) s ( = 3.42, <0.05). The experimental group fixed lesions after hemostatic clamp off times were significantly less than that in con-trol group (χ2=2.37, <0.05). Conclusions Using innovative methods, adequate preoperative preparation, the op-erator's tacit understanding of nursing cooperation, close attention to the disease after surgery is the key to achieve the desired results of endoscopic surgery.

Objective To analyze the antioxidant activities of extracts from Scutellaria baicalensis Georgi. by different solvents, and to determine the contents of total flavonoids. Methods By employing colorimetric method, contents of total flavonoids in different extracts were determined. The free radical scavenging activity of each extract was measured by DPPH method. Results Different extracts from Scutellaria baicalensis Georgi. by different solvents were provided with DPPH free radical scavenging activities, of which the ethyl alcohol extracts were the highest in scavenging activities. In addition, total flavonoids content of ethyl alcohol extracts was significantly higher than that of others. Conclusion The antioxidant activity of extracts from Scutellaria baicalensis Georgi. was related to the content of total flavonoids in it.

Objective To explore the association between urinary lactate/creatinine ratio and neonatal asphyxia,and to clarify the application value of urinary lactate/creatinine ratio in forecasting the hypoxie-ischemic encephalopathy (HIE)in neonates.Methods Using case-control study design method,40 cases of neonatal asphyxia infants were selected as asphyctic group and 40 healthy infants were used as control group.The urinary lactate/creatinine ratio in the first day after birth, urinary N-Acetyl-β-D-glucosaminidase (NAG)/creatinine and the score of Apgar of 80 infants were detected.The relationship between the urinary lactate/creatinine ratio,urinary NAG/creatinine ratio, as well as the score of Apgar and HIE were analyzed and compared in asphyctic group and control group.Results The Apgar scores at 1 and 5 min in asphyctic group were significantly lower than those in control group (P<0.01). The urinary lactate/creatinine ratio and urinary NAG/creatinine ratio in asphyctic group 1 d after birth were significantly higher than those in control group(P<0.01).There was significantly negative correlation between the urinary lactate/creatinine ratio and the 1-min and 5-min Apgar scores(r=-0.636,P<0.001;r=-0.883,P<0.01),but there was a significantly positive correlation between the urinary lactate/creatinine ratio and urinary NAG/creatinine ratio(r=0.433,P<0.01).Conclusion The urinary lactate/creatinine ratio may have decisive roles in prognosis evaluation of neonatal asphyxia,and so as the important foundation for the prediction of HIE.

Objective To evaluate the effects and the safety of peroral endoscopic myotomy (POEM) for achalasia (AC).Methods POEM was performed on 7 AC patients from November 2010 to November 2011.Preoperative dysphagia grade,postoperative complications and short-term and long-term efficacy were recorded.Results POEM was performed successfully on all the 7 patients.The operation time were ranging from 46 to 113 minutes,mean 73 ± 20 minutes.Subcutaneous emphysema and mediastinal emphysema occurred in 2 patients,but cured after treatment.The 1-12 month follow-up showed dysphagia of all the patients was obviously improved.Conclusion POEM is efficient and safe to relieve dysphagia of AC patients.

Objective To analyze the related factors of local recurrence in patients with rectal cancer after Dixon operation. Methods A retrospective analysis of clinical data of 100 patients from September 2005 to September 2007 in our hospital by TME standard surgicai treatment of low rectal cancer was carried out. Results Of 100 patients, the pre-sacral recurrence rate was 10. 0%, with gender, age, tumor size, tunor invasion lumen circumference,depth of invasion,lymph node metastasis and Duke's stage has nothing to do with the gross type,histological type and lumen,peritoneal tumor cell shedding,and as an independent prognostic factor. Of 90 patients without local recurrence,the 5-year survival rate was 76. 0% ,haff of the survival of 62 months;10 cases of local recurrence,5-year survival rate was 5. 0%, half of the survival of 24 months, suggested that local recurrence in patients influenced the prognosis. Conclusion The low local recurrence of rectal cancer related factors were gross type,histological type and the intestine, peritoneal tumor cells shed, seriously affect the prognosis of patients with local recurrence.