Type Ⅰ sialidosis is a neurosomatic disorder related to the storage of lysosomal and induced by shortage of neuraminidase. It is an autosomal recessive disorder, maybe heterogeneous in its onset, clinical manifestations and prognosis. A case of type Ⅰ sialidosis with a missense mutation in the α-N-acetyl-neuraminidase (NEU1) gene is reported. The patient was characterized by reduced visual acuity, ataxia and subcortical myoclonus. Although the macular cherry red spots were not detected in the male patient, his bilateral visual evoked potential showed severely prolonged latencies of P100, which was consistent with continuous decline of his visions. Finally, he was treated with carbamazepine and clonazepam with moderate improvement in the symptom of myoclonus. In order to make the definite diagnosis, the importance of a clinical history integrating all the patient′s clinical manifestations and the mutation in NEU1 gene was highlighted. Regardless of being an uncommon disorder, the burden for those patients with sialidosis was significant. Therefore, this diagnosis in the relevant setting should always be considered.

Objective: To analyze the epidemiological characteristics of hospitalized children with burn in Ningbo from 2013 to 2018,and to provide evidence for developing intervention strategies. Methods: The pediatric burn cases,discharged from the Ningbo Women & Children's Hospital from 2013 to 2018,were registered using the Ningbo Hospitalized Injury Monitoring Report Card,and their distributions of time,places,groups,involved body parts and prognosis were analyzed. Results: There were 3 862 pediatric burn inpatients included in this study,with 2 977（77.08%）cases aged 1-3 years and 2 532（65.65%）nonlocal cases. About 898（23.25%）cases occurred during 9:00-12:00 a.m. and 1 833（47.46%）cases occurred during 17:00- 21:00 p.m. Burns predominantly occurred at home,with 3 810（98.65%）cases. The top three body regions involved were multiple regions,lower limbs and upper limbs with 1 820（47.13%）,835（21.62%）and 541 cases（14.01%）. The proportions of involving multiple regions declined with age（Psingle<0.05）. The proportion of involving multiple regions in nonlocal children was higher than that of local children（P<0.05）. Conclusion Burn is one of the leading causes of child injury-related hospitalization in Ningbo. Home is the main burn scene. Nonlocal and 1-3 year-old children were especially at high risk of burns.

Objective: To analyze the trend of the fall death rate in Ningbo,and to provide reference for prevention and control of fall death. Methods : The data of fall mortality in Ningbo from 2002 to 2018 was collected to calculate the crude mortality rate,standardized mortality rate,age specific mortality rate and the proportion of fall death types. Joint Point Regression was used to evaluate annual percentage change（APC）of fall mortality. Results : The average annual crude and standardized mortality rate of fall in Ningbo was 16.81/100 000 and 11.18/100 000,showing an upward trend from 2002 to 2018（P<0.05）. The standardized mortality rate of fall was 11.57/100 000 in men and 10.41/100 000 in women,and the difference was statistically significant（P<0.05）,both showing an upward trend from 2002 to 2018（P<0.05）. The standardized mortality rate of fall was 11.07/100 000 in urban residents and 11.25/100 000 in rural residents,and the difference was statistically significant（P<0.05）,both showing an upward trend from 2002 to 2018（P<0.05）. The mortality rate of fall increased with age（P<0.05）. The mortality rate of fall showed a small peak in children less than five years old（1.81/100 000）,and began to rise rapidly in people aged 65 years or over and peaked again in people aged 85 years or over. There were 11 141 cases died of falling on the same plane,accounting for 68.25%. Fall deaths occurred mainly at home,with 11 829 cases（72.46%）. Conclusion The mortality rate of fall in Ningbo increased from 2002 to 2018. The males,urban residents,people aged less than five years and aged 65 years or older have high risk of death from fall,who were targets for prevention and control strategies of fall.

Objective: To investigate the trends in incidence and mortality of gastric cancer in Ningbo City, Zhejiang Province from 2011 to 2022, so as to provide insights into improving gastric cancer control strategy. Methods: The incidence and mortality of gastric cancer in Ningbo City from 2011 to 2022 were collected through Ningbo Municipal Chronic Disease and Cause of Death Monitoring System. The incidence and mortality of gastric cancer were calculated, and standardized by the data from the Sixth Chinese National Population Census in 2020 (Chinese-standardized rate) and the world standard population first introduced by Segi in 1960 (world-standardized rate). The trends in incidence and mortality of gastric cancer were evaluated using annual percent change (APC) and average annual percent change (AAPC). Results : The crude incidence of gastric cancer was 45.69/105 in Ningbo City from 2011 to 2022, with no significant changing patterns seen during the study period (AAPC=-0.02%, P>0.05), and the Chinese- and world-standardized incidence of gastric cancer was 28.61/105 and 21.87/105, which both appeared a tendency towards a decline (AAPC=-3.19% and -3.05%, both P<0.05). The crude, Chinese-standardized and world-standardized mortality rates of gastric cancer were 28.56/105, 17.07/105 and 12.57/105, respectively, all showing a tendency towards a decline (AAPC=-3.00%, -6.26% and -6.34%, all P<0.05). The Chinese- and world-standardized incidence and mortality of gastric cancer all appeared a tendency towards a decline in urban (AAPC=-2.72%, -2.53%, -5.91% and -5.96%, all P<0.05) and rural areas (AAPC=-3.61%, -3.53%, -6.79% and -6.89%, all P<0.05), and the Chinese- and world-standardized incidence and mortality of gastric cancer were significantly higher among urban residents than among rural residents. The Chinese- and world-standardized incidence and mortality of gastric cancer all appeared a tendency towards a decline among men (AAPC=-3.18%, -3.00%, -5.82% and -5.91%, all P<0.05) and women (AAPC=-2.98%, -2.90%, -7.12% and -7.12%, all P<0.05), and the Chinese- and world-standardized incidence and mortality of gastric cancer was significantly higher among men than among women. In addition, the crude incidence and mortality of gastric cancer both appeared a tendency towards a rise with age among residents in Ningbo City (both P<0.05). Conclusions The incidence and mortality of gastric cancer both appeared a tendency towards a decline in Ningbo City from 2011 to 2022; however, the incidence and mortality remained high. Males and urban residents should be given a high priority for gastric cancer control, and gastric cancer screening should be strengthened among individuals at ages of 40 years and older.

Objective: To investigate the trends in mortality of malignant tumors in Ningbo City, Zhejiang Province from 2002 to 2022, so as to provide the evidence for formulating malignant tumor control strategies in Ningbo City. Methods The data regarding the mortality of malignant tumors in Ningbo City from 2002 to 2022 were collected through the Ningbo Municipal Death Cause Monitoring System, and the crude mortality and age-specific mortality of malignant tumors were calculated in Ningbo City. The mortality of malignant tumors was standardized by the population of the sixth National Population Census in China in 2010 (Chinese-standardized mortality) and the world standard population in 1960 (world-standardized mortality). The trends in mortality of malignant tumors were evaluated with annual percent change (APC) and average annual percent change (AAPC). Methods: The data regarding the mortality of malignant tumors in Ningbo City from 2002 to 2022 were collected through the Ningbo Municipal Death Cause Monitoring System, and the crude mortality and age-specific mortality of malignant tumors were calculated in Ningbo City. The mortality of malignant tumors was standardized by the population of the sixth National Population Census in China in 2010 (Chinese-standardized mortality) and the world standard population in 1960 (world-standardized mortality). The trends in mortality of malignant tumors were evaluated with annual percent change (APC) and average annual percent change (AAPC). Results: The crude mortality of malignant tumors was 186.43/105 to 221.24/105 in Ningbo City from 2002 to 2022, which showed a tendency towards a rise (AAPC=0.76%), and both the Chinese- (AAPC=-2.64%) and world-standardized mortality (AAPC=-2.74%) appeared a tendency towards a decline (all P<0.05). The world-standardized mortality of malignant tumors presented three changes in Ningbo City from 2002 to 2022, with a more remarkable decline from 2011 to 2018 (APC=-3.53%) than from 2002 to 2011 (APC=-2.10%) and from 2018 to 2022 (APC=-2.00%) (all P<0.05). The annual decline in mortality of malignant tumors was higher in men (Chinese-standardized mortality: AAPC=-2.68%; world-standardized mortality: AAPC=-2.75%) than in women (Chinese-standardized mortality: AAPC=-2.45%; world-standardized mortality: AAPC=-2.57%), and higher in urban areas (Chinese-standardized mortality: AAPC=-2.85%; world-standardized mortality: AAPC=-2.92%) than in rural areas (Chinese-standardized mortality: AAPC=-2.45%; world-standardized mortality: AAPC=-2.57%) (all P<0.05). The mortality of malignant tumors appeared a tendency towards a rise with age in Ningbo City, with the highest mortality in residents at ages of 85 years and older (1 447.13/105). Death from malignant tumors were responsible for 31.86% of all causes of death in Ningbo City, and the five most common causes of cancer death included lung cancer, liver cancer, gastric cancer, colorectal cancer and esophageal cancer. In addition, the world-standardized mortality of pancreatic cancer (AAPC=3.92%), prostate cancer (AAPC=4.71%), and cervical cancer (AAPC=1.60%) appeared a tendency towards a rise in Ningbo City (all P<0.05). Conclusions The crude mortality of malignant tumors appeared a tendency towards a rise in Ningbo City from 2002 to 2022, while the standardized mortality showed a tendency towards a decline. Management of malignant tumors should be given a high priority among men and rural residents, and lung cancer, liver cancer, gastric cancer, colorectal cancer and esophageal cancer should be emphasized.

OBJECTIVETo observe the anaphylaxis and hemocytolysis of 6 kinds of tween-80 injection from different source.

METHODThe Hartley albinism guinea pig was used to carry on the active systematic anaphylaxis (ASA) and the passive cutaneous anaphylaxis (PCA). The in vitro hemolytic experiment and the hemocytolysis was observed by means of spectrophotometry on the domestic rabbit.

RESULTThe result of ASA and PLA of 6 kinds of tween-80 injection from different source assumed the negative. In observation time, the temolysis rate of 3 kinds of tween-80 injection are more than 5%, while the others are also more than 5% only in the highly concentrated test tube.

CONCLUSIONSix kinds of tween-80 injection from different source have not caused the immune-mediated anaphylaxis, but it may have hematolysis tendency on intravenous injection. The hemocytolysis of tween-80 may not be entirely caused by the impurities. It is worthy of further study that the physical and chemical properties of the product itself and the undeserved concentration is doubtful whether there is also some internal relations with the generation of hemolytic.

Anaphylaxis ; chemically induced ; Animals ; Chemistry, Pharmaceutical ; Female ; Guinea Pigs ; Hemolysis ; drug effects ; Injections ; Male ; Passive Cutaneous Anaphylaxis ; drug effects ; Polysorbates ; administration & dosage ; adverse effects ; chemistry ; Rabbits

Objective To establish nasopharyngeal carcinoma cell line (CNE1) with eIF1 gene stable over-expression and to study its effects on the proliferation and migration of nasopharyngeal carcinoma cells.Methods EIF1 over-expression vector was constructed by adopting the pEGFPC1 eukaryotic expression system for transfecting nasopharyngeal carcinoma CNE1 cells.Thus the stably transfected EIF1-elF1 and its control cells were obtained.The over-expression situation of eIF1 in these cells was verified by real time fluorescence quantitative PCR(qPCR) and Western blot.The proliferation and migration activity of CNE1-eIF1 cells were tested by adopting the cell proliferation and migration tests.Results The enzyme digestion electrophoresis identification and sequencing showed that the pEGFPC1-eIF1 eukaryotic expression vector was successfully constructed.After mRNA and protein expression identification,compared with the reloading plasmid transfection group,the eIF1 gene mRNA and protein expression levels in nasopharyngeal carcinoma cell line CNE1 stably over-expressing eIF1 were up-regulated by 2.85 folds and 2.58 folds respectively (P＜ 0.05),while its proliferation and migration activities were down-regulated by 55 ％ and 36 ％ respective (P＜ 0.05).Conclusion The nasopharyngeal carcinoma cell line over-expressing elF1 is successfully constructed,the eIF 1 over-expression could significantly down-regulate the proliferation and migration activities of nasopharyngeal carcinoma cells,suggesting that eIF1 has potential anti-tumor effect.

OBJECTIVETo investigate the effects of refining temperatures and amounts of sheep's oil used in processing Epimedii Folium on Kedney-yang deficiency rats.

METHODThe corticosterone was subcutaneous injected to establish the kidney yang deficiency rat model. With the temperatures and amounts of sheep's oil were 250 degrees C/30%, 120 degrees C/30% and 120 degrees C/20% respectively, the crude drug and three kinds of pressed Epimedii Folium were extracted by water and used as examined samples while total flavonoid of Epimedii Folium was used as positive control. After examined samples and control samples were intragastirc administrated, the pharmacologic action was analyzed.

RESULTAs compared to crude drug, all of the aqueous extracts of processed Epimedii Folium have stronger effect of warming kidney and enhancing yang, especially the Epimedii Folium processed by sheep's oil with refining temperatures 120 degrees C and amounts of sheep's oil 30%. Its mechanism might be related to improving the insufficiency of hypothalamic-pituitary-adrenal-thymus (HPAT) axis suppression.

CONCLUSIONThe refining temperature of sheep's oil can affect the quality of excipients and processed drugs. The results may be useful in explaining the mechanism of Epimedii Folium processing and establishment of pharmaceutical standard of sheep's oil used as processing excipients.

Animals ; Drugs, Chinese Herbal ; administration & dosage ; Hypothalamo-Hypophyseal System ; drug effects ; Kidney Diseases ; drug therapy ; physiopathology ; Male ; Oils ; administration & dosage ; Pituitary-Adrenal System ; drug effects ; Rats ; Rats, Sprague-Dawley ; Sheep ; Technology, Pharmaceutical ; Yang Deficiency ; drug therapy ; physiopathology

Objective:To explore the effects of genotypes of one-carbon metabolism (OCM)-related enzyme single nucleotide polymorphisms (SNPs) sites and anti-epileptic drugs on OCM metabolite levels in epileptic patients, and to screen valproic acid (VPA) teratogenic susceptibility genes.Methods:Three hundred and seventy-two epileptic patients, admitted to our hospital from January 2019 to December 2020, were enrolled in the study; patients taking VPA, levetiracetam (LEV), lamotrigine (LTG) or oxcarbazepine (OXC) for more than 6 months without attack during regular medication were classified as VPA group ( n=95), LEV group ( n=61), LTG group ( n=57) and OXC group ( n=70); firstly diagnosed epileptic patients who had never taken antiepileptic drugs or had not taken antiepileptic drugs in the previous 6 months were assigned into blank control group ( n=89). Plasma folic acid (FA), vitamin B12 (VitB 12) and homocysteine (Hcy) levels were determined by automatic chemiluminescence immunoassay, and genotypes of OCM-related enzyme SNPs sites were detected by Sequenom iPLEX. Results:(1) As compared with LEV group and blank control group, VPA group had significantly decreased FA level and significantly increased Hcy level ( P<0.05). (2) Patients with DNA methyltransferase (DNMT) 3a rs12987326(-178G>A) GA type had significantly higher Hcy level than those with GG type ( P<0.05); patients with DNMT1 rs2288350(82G>C) GC type had significantly higher Hcy level than those with GG type ( P<0.05); patients with DNMT1 rs75616428 (55850G>C) GC type had significantly lower VitB 12 level than those with GG type ( P<0.05). Patients with DNMT1 rs1863771(128G>A) GA+AA type had significantly higher FA level than those with GG type, patients with folate receptor 2 rs2298444(59T>C) CT+CC type had significantly higher Hcy level than those with TT type, patients with 5,10-methylenetetrahydrofolate reductase rs1801131(1298A>C) AC+CC type had significantly higher Hcy level than those with AA type, and patients with DNMT3a rs6722613(2327C>T) CT+TT type had significantly lower VitB 12 level than those with CC type ( P<0.05). Conclusions:Decreased FA and increased Hcy levels can be noted in epileptic patients who used VPA; some gene variations in SNPs of OCM also affect the OCM metabolite levels in epileptic patients. Epileptic patients during pregnancy should avoid using VPA or detecting SNPs genotypes before medication to reduce the incidence of fetal malformation.

OBJECTIVETo investigate the changes and mechanism of angiopoietin1 (Ang1) in murine bone marrow during G-CSF induced mobilization of hematopoietic stem/progenitor cell.

METHODSThe proportion of Lin-Sca1⁺cKit⁺ (LSK) cells in peripheral blood of C57BL/6 mice before and after G-CSF mobilization was detected by flow cytometry. Expression changes of Ang1 and osteocalcin (OCN) during HSC mobilization were determined by immunohistochemistry, enzyme linked immunosorbent assay (ELISA) and real-time fluorescence quantitative PCR. The number of osteoblasts in the bone marrow was counted under the microscope.

RESULTSAfter treated with G-CSF, the proportion of LSK cells in peripheral blood significantly increased from the controls (0.04 ± 0.01)% to (0.61 ± 0.05)% at day 5 (P<0.05). Before G-CSF mobilization, the endosteum cells expressed higher level of OCN and Ang1 than that of bone marrow nucleated cells. The mRNA expression level of OCN was significantly reduced from 28.64 ± 8.61 in the controls to 12.55 ± 7.06 on day 3 and 4.75 ± 1.62 on day 5, and the expression level of Ang1 also declined from 2.84 ± 0.95 in the controls to 0.93 ± 0.30 on day 3 and to 0.92 ± 0.22 on day 5 after G-CSF mobilization. The number of endosteum osteoblasts was significantly decreased after mobilization (P<0.05). The Ang1 expression was decreased in the BM after mobilization. The serum OCN was significantly reduced from (24.11 ± 3.17) ng/ml in the controls to (9.96 ± 2.16) ng/ml on day 3 and (8.43 ± 2.62) ng/ml on day 5, and the Ang1 also declined from (2.24 ± 0.52) ng/ml in the controls to (1.21±0.38) ng/ml on day 3 and (0.90±0.24) ng/ml on day 5.

CONCLUSIONIn G-CSFinduced HSPC mobilization, the bone marrow osteoblasts retraction causes reduction of Ang1, and the reduction of Ang1 may contribute to HSPC mobilization.

Animals ; Bone Marrow ; Bone Marrow Cells ; Granulocyte Colony-Stimulating Factor ; Hematopoietic Stem Cell Mobilization ; Hematopoietic Stem Cells ; Mice ; Mice, Inbred C57BL ; Osteoblasts ; RNA, Messenger