Neonatal hyperinsulinism/hyperammonemia syndrome is a genetic disease result from glutamate dehydrogenase gene mutations.The clinical manifestations are hypoglycemia,hyperinsulinemia and mild hyperammonemia.Hypoglycemia may occur quickly due to eating protein.It is a rare neonatal disease that was easily ignored or delayed diagnosis and treatment causing serious sequelae of nervous system.This review summarized pathogenesis,clinical manifestation and diagnosis of the disease.

Objective To study the therapeutic effects and related mechanism of Tongmai Yizhi granules on vascular dementia in rats. Methods Bilateral carotid artery ligation was used to establish rat model of vascular dementia (VD). The rats were randomly divided into 6 groups: sham operated,the model control,piracetam (0. 375 g·kg-1 ·d-1 ) as positive con-trol,low dose (2. 5 g·kg-1 ·d-1 ),middle dose(5. 0 g·kg-1 ·d-1 ),and high dose (10. 0 g·kg-1 ·d-1 ) of Tongmai Yizhi granules. Each group was intragastrically administered with 10 mL·kg-1 of corresponding medications for 31 days after the VD model was established. The sham group was given with 0. 9% NaCl solution. Y-electric maze was used to test the learning and memory function of rats at the second and fourth weeks. On the day 32,hippocampal tissues were collected for pathological analy-sis by microscope. The activities of SOD and content of MDA in serum and tissue homogenate were tested. Results Compared with model control group,each dose of Tongmai Yizhi granules obviously shortened the incubation period of VD rats in electric maze test,and accuracy of learning and memory was improved (P<0. 05 or P<0. 01). The activity of SOD was decreased,and the content of MDA was increased after treatment with Tongmai Yizhi granules (P < 0. 01). Hippocampal neuronal cells were disar-ranged,and the number of cone cells was decreased significantly in the model control group. In contrast,the necrosis and degener-ation of hippocampal neurons were alleviated in all Tongmai Yizhi treatment groups. Conclusion Tongmai Yizhi granule can apparently improve learning and memory function of VD rats. It can also improve the activity of SOD and reduce the content of MDA in serum and hippocampus,effectively remove free radicals,and alleviate the injury of hippocampal pyramidal cells.

BACKGROUNDTo investigate the relationship between micrometastasis of bone marrow and neoadjuvant chemotherapy and prognosis in patients with stage III non-small cell lung cancer (NSCLC).

METHODSSixty-five patients with stage III NSCLC were randomly divided into group A (32 patients treated with preoperative neoadjuvant chemotherapy plus operation) and group B (33 patients treated with operation and postoperative chemotherapy as control group). Expression of CK19 mRNA and CEA mRNA was detected in bone marrow samples from the rib segments of all patients obtained from operation by RT-PCR. The relationship between survival duration and CK19 and CEA expression was analyzed.

RESULTSThe positive rates of CK19 mRNA expression were 18.8%(6/32) and 45.5%(15/33) in group A and B, respectively (P=0.033), and the positive rates of CEA were 25.0%(8/32) and 51.5% (17/33) in group A and B, respectively (P= 0.041). A significant positive correlation was observed between CK19 and CEA expression (r s=0.671,P < 0.001). The response rates of neoadjuvant chemotherapy were 0%(0/5) and 56.5%(13/23) in patients with CK19(+)/CEA(+) and CK19(-)/CEA(-), respectively (P=0.044), and the median survival duration were 11 and 27 months, respectively (P=0.000 6). Cox's model showed that the response to neoadjuvant chemotherapy and the expression of CK19 or CEA were significantly prognostic factors in group A.

CONCLUSIONSNeoadjuvant chemotherapy can reduce the possibility of bone marrow micrometastasis in stage III NSCLC patients. Bone marrow micrometastasis may indicate a poorer prognosis for NSCLC.

Objective To observe the effects of epidural anesthesia and subarachnoid anesthesia on puerperants and fetus perioperatively undergoing cesarean delivery after epidural labor analgesia.Methods A retrospective cohort study was conducted to select and analyze the relevant clinical data of women who had epidural labor anal-gesia and were converted to cesarean delivery at Beijing Tiantan Hospital.According to the anesthesia method of cesarean section,the participants were divided into the epidural anesthesia(EA)group and the subarachnoid an-esthesia(SA)group.That were aimed to compare the effects of the two anesthesia methods on the mother and fe-tus perioperatively.Results Compared with women in the epidural anesthesia group,women in the subarachnoid anesthesia group had a better anesthesia outcome,visual analogue scale(VAS)scores,frequency(%)[VAS scores 0-3:5(25%)vs.14(70%);VAS scores 4-6:12(60%)vs.6(30%);and VAS scores 7-10:3(15%)vs.6(30%)],and higher perioperative complication rate.There was nonsignificance difference in perio-perative maternal bleeding,neonatal Apgar score,and neonatal umbilical artery blood gas analysis between the two groups.Conclusions In women with epidural analgesia undergoing cesarean section,the anesthesia effect of subarachnoid anesthesia is better than that of the epidural anesthesia.The anesthesiologist must pay attention to prevent complications in the perioperative period.

This study was aimed to investigate the aggregation of rehydrated-lyophilized platelets. The aggregation rate of fresh and rehydrated-lyophilized platelets were measured by using thrombin, ristocetin, ADP and collagen as inductors and APACT2 aggregameter; the effects of intra- and extra-cellular trehalose on maximum aggregation rate of rehydrated-lyophilized platelets were detected by using ADP as an inductor. The results showed that the aggregation rate of fresh platelets was all about 100%, while aggregation rate of rehydrated lyophilized platelets was (70.17 +/- 7.36)%, (15.3 +/- 2.81)%, (68.67 +/- 6.86)%, (64.67 +/- 11.6)% respectively, when the concentration of thrombin, ristocetin, ADP and collagen was 1 U/ml, 1.6 mg/ml, 20 micromol/L and 2 microg/ml. The maximum aggregation rates of rehydrated-lyophilized platelets in intra- and extra-cellular trehalose, extracellular trehalose and blank control groups were (66.0 +/- 4.69)%, (25.3 +/- 2.42)% and (11.5 +/- 1.87)% (P < 0.01), meanwhile there was significant difference of rehydrated-lyophilized platelet aggregation rate between intra- and extra-cellular trehalose and extracellular trehalose groups (P < 0.01). It is concluded that the concentrations of thrombin (1 U/ml), ristocetin (1.6 mg/ml), ADP (20 micromol/L) and collagen (2 microg/ml) are optimal for platelets aggregation tests, the internal and extracellular trehalose significantly enhance the aggregation of rehydrated-lyophilized platelets.
Blood Platelets ; cytology ; drug effects ; metabolism ; Blood Preservation ; methods ; Freeze Drying ; methods ; Humans ; Platelet Aggregation ; physiology

Objective:Healthy life expectancy (HLE), which combines life expectancy with health, is an essential comprehensive measure of life length and quality. This article aimed to systematically review the methods for defining and measuring HLE and describe application studies published, providing a reference for decision makers to select and develop methods suitable for China's conditions to measure HLE.Methods:Seven Chinese and English literature databases were searched up to May 7, 2022, and several related reviews and bibliography were manually retrieved. Systematic reviews and empirical research were included concerning HLE indicators and measurement of HLE. Information including the study area, type of the study, study population, HLE index, measurement method, data sources, and results from application studies published in the last five years were extracted. The evolution of the definition of HLE, the scope of different indicators, the measurement scale of health, and measurement methods, were all collected. Results of the empirical research related to measurement methods of indicators were summarized. The study followed the scoping review framework and was written according to the PRISMA-ScR statement.Results:A total of 84 articles were included, including 13 reviews, 17 original studies related to HLE index definition, ten original studies related to index measurement, and 44 empirical studies conducted in the past five years. There were as many as 20 indicators related to HLE, and each scale had its emphasis. A total of ten methods measuring HLE were identified, which vary in the definition of health, whether using weight, and the data type. The most commonly used indicators in the past five years were disability-free life expectancy and HLE. For the method of HLE calculation, Sullivan's method was mainly used for cross-sectional data, and the multistate life table was mainly used for longitudinal data.Conclusions:There are various definitions and measurement methods of HLE, but none are suitable for all scenarios. To summarize the HLE concept, health evaluation techniques, measurement methods, and application studies published worldwide can provide a reference for the localization of HLE measurement in China.

As a new group of stem cells found in the human breast in the 21st century, stem cells in human breast milk have the potential to differentiate into ectoderm, mesoderm, and endoderm cells.It is not only rich in sources, but also can be obtained in a non-invasive way.At present, there are relatively few ethical disputes.First, as a potential source of stem cells, it has a great prospect in the field of alternative medicine and regenerative medicine.Second, it can promote newborns early growth and development, repair the damaged tissues, and prevent as well as treat some early diseases.This review summarized stem cells from human breast milk, from aspects of distribution, acquisition, and functional characteristics, and discussed their application in newborns.

OBJECTIVE: To explore the clinical and genetic characteristics of three children with KBG syndrome. METHODS: Clinical data of the three children from two families who have presented at the First Affiliated Hospital of Zhengzhou University between October 2019 and September 2020 and their family members were collected. Trio-whole exome sequencing (trio-WES) and Sanger sequencing were carried out. RESULTS: All children had feeding difficulties, congenital heart defects and facial dysmorphism. The sib- pair from family 1 was found to harbor a novel de novo heterozygous c.6270delT (p.Q2091Rfs*84) variant of the ANKRD11 gene, whilst the child from family 2 was found to harbor a novel heterozygous c.6858delC (p.D2286Efs*51) variant of the ANKRD11 gene, which was inherited from his mother who had a mild clinical phenotype. CONCLUSION The heterozygous frameshift variants of the ANKRD11 gene probably underlay the disease in the three children. Above findings have enriched the spectrum of the ANKRD11 gene variants.
Female ; Child ; Humans ; Abnormalities, Multiple/genetics* ; Intellectual Disability/genetics* ; Bone Diseases, Developmental/genetics* ; Tooth Abnormalities/genetics* ; Facies ; Repressor Proteins/genetics* ; Mothers ; Mutation

Background: Gastric cancer is one of the common gastrointestinal malignancies. Early diagnosis can reduce the mortality rate significantly. In the Chinese consensus published in 2017, the New Gastric Cancer Screening Scoring System was recommended to be used for risk stratification of gastric cancer. Aims: To preliminarily explore the value of the New Gastric Cancer Screening Scoring System in early gastric cancer screening in asymptomatic community population. Methods: At several communities in Suzhou City Xiangcheng District, a questionnaire survey was conducted in asymptomatic community residents willing to accept voluntary serum tests to collect information on high risk factors of gastric cancer. Serum pepsinogen (PG), PGⅡ, gastrin 17 (G-17) and Helicobacter pylori (Hp) IgG were tested simultaneously. Risk stratification of gastric cancer was carried out in accordance with the New Gastric Cancer Screening Scoring System. Gastroscopy was recommended for moderate to high risk individuals. Results: A total of 540 asymptomatic individuals completed the study, of which 11 were categorized as high risk (2.0%), 168 as moderate risk (31.1%), and 361 as low risk (66.9%). Sixty-four moderate to high risk individuals completed the gastroscopy with a response rate of 35.8%. Four precancerous lesions were detected (6.2%), including 3 gastric low-grade intraepithelial neoplasia and 1 duodenal adenoma. No gastric cancer was detected. Conclusions: The New Gastric Cancer Screening Scoring System is useful for risk stratification of gastric cancer in asymptomatic population and may provide a basis for further endoscopic examination. However, the value of this scoring system in low risk areas of gastric cancer needs to be further verified.

【Objective】 To understand the current situation of blood components distribution in domestic prefecture-level blood stations through analyzing the components distribution data of 24 prefecture-level blood stations in China. 【Methods】 The data of components distribution of 24 blood stations from 2017 to 2020 as well as the data of blood deployment of 24 blood stations from 2019 to 2020 were collected and analyzed. 【Results】 From 2017 to 2020, positive annual growth in red blood cells, plasma and cryoprecipitate was observed in 22, 19 and 15 out of the 24 blood stations, and the annual growth median rate of above three components was 5.24%, 3.80% and 3.25%, respectively. Among the 24 prefecture-level blood stations, 23 carried out the preparation of cryoprecipitate. 【Conclusion】 The distribution of red blood cells, cryoprecipitate and plasma in prefecture-level blood stations is increasing year by year. However, there is a overstock of plasma, and most blood stations need blood employment.