Objective To identify the correlativity between SCN4A V781I mutation and hypokalemic periodic paralysis(HOKPP).Methods The SCN4A V781I mutation was screened on the members of a hypokalemic periodic paralysis family(including 3 patients and 14 healthy relatives), 71 sporadical hypokalemic periodic paralysis patients and 100 healthy adults with the PCR sequencing and the incision enzyme techniques.Results The mutation existed not only in all the patients but also in 4 healthy relatives, including 1 male and 3 females, in the hypokalemic periodic paralysis family.Seven of 71 sporadieal hypokalemic periodic paralysis patients and 7 of 100 health adults indicated the SCN4A V781I mutation.There was no significant difference in the SCN4A V781I mutation rate between the hypokalemic periodic paralysis patients and the healthy adults(X~2=0.452, P=0.502).Conclusions SCN4A V781I mutation maybe is an innocent polymorphism.There is little correlativity between SCN4A V781I and hypokalemic periodic paralysis.

Objective To evaluate the usefulness of real-time three-dimensional transesophageal echocardiography (RT-3D TEE) in the perventricular device occlusion of the ventricular septal defect (VSD).Methods Sixty patients underwent perventricular device occlusion of VSD were divided into two groups.Group A:30 patients,3D images of VSD were obtained intraoperatively by RT-3D TEE.3D images were analyzed and the maximal and minimum area and maximal diameter of VSD were measured in the cardiac cycle.The size of selected VSD occluder was the diameter calculated from the maximal area based on the formula πD2/4 and plus 1～2 mm.Simultaneously,two dimensional transesophageal echocardiography (2D TEE) images of VSD were obtained and measured on standard views.Group B:other 30 patients,intraoperative 2D TEE was performed to measure the diameter of VSD on standard views.The selection VSD occluder was based on the maximal diameter plus 1～2 mm.Results The VSDs shape showed oval,class round and irregular by RT-3D TEE imaging.There was a significant difference between the maximal area and smallest area of VSD appeared in the cardiac cycle obtained by RT-3D TEE (P<0.01).There was a significant difference between the maximal diameter by measurement of RT-3D TEE and 2D TEE (P<0.01).Of all 30 patients with evaluation of VSD size based on the RT-3D TEE,1.1 times attempts of device occlusion were performed on one patient (33/30).Of the other 30 cases with evaluation of VSD size by 2D TEE,2 cases were failed with device occlusion because of instant residual shunt.Of the remaining 28 cases,1.43 attempts of device occlusion were performed on one patient (40/28).Conclusions RT-3D TEE can accurately reveal the size of VSD,and aid in the selection of VSD occluder in the device occluding procedure of VSD.

Objective To study the impact of hypothyroidism on sperm motility in male rat.Methods According to body weight,20 male Wistar rats were randomly divided into control group and hypothyroidism group (1 ml/100 g/day,0.1％ propylthiouracil by intragastric administration for 60 days) 10 rats in eachgroup.Body weight of these rats was observed every 3 days.After the last intragastric administration,all rats were killed.The levels of thyroid hormones [total triiodothyronine (T3),total thyroxine (T4),and thyroid stimulating hormone (TSH)] were measured by radioimmunoassay.Sperm motility parameters[average path velocity(VAP),straight fine velocity (VSL),straightness (STR),amplitude of lateral head displacement (ALH),sperm density(p),curvilinear velocity (VCL),linearity(LIN),wobble (WOB),mean angular deviation (MAD) and beat cross frequency (BCF)] were measured by a WLJY-9000 color-detection system.Results Compared with the control groups[(298.20 ± 12.15) g,(344.00 ± 13.73)g],the weights of hypothyroidism group of the 30 days[(239.00 ± 15.02) g] and the 60 days [(232.67 ± 17.86)g] were significantly lower(t =7.704,11.380,all P ＜ 0.05).The levels of T3[(373.3 ± 101.3) ng/L] and T4 [(4.00 ± 0.89) × 103 ng/ml] of hypothyroidism group were significantly decreased compared with that of the control groups [(1000.0 ± 273.5)ng/L,(44.33 ± 7.84)× 103 ng/L,t =5.262,12.520,all P ＜ 0.05].Level of TSH[(5.77 ± 0.89) × 103 U/L] of hypothyroidism group was significantly increased compared with that of the control group[(1.87 ± 0.70) × 103 U/L,t =8.413,P ＜ 0.05].Values of VAP[(27.45 ± 1.59)μm/s],VSL [(21.08 ± 1.10)μm/s],STR[(70.53 ± 3.48)％] and ALH[(1.96 ± 0.26)μm] of hypothyroidism group were significantly increased compared with that of the control groups[(24.38 ± 2.59)μm/s,(17.99±2.06)μm/s,(65.93 ± 2.71)％,(1.53 ± 0.27)μm,t =2.687,2.404,2.420,3.175,all P ＜ 0.05].p[(5.07 ± 0.74)109/L] of hypothyroidism group was significantly decreased compared with that of the control group [(8.76 ± 1.01)109/L,t =6.463,P ＜ 0.05].VCL[(52.83 ± 5.56)μm/s],LIN[(38.58 ± 3.41)％],WOB[(52.64 ± 3.24)％],MAD [(64.21 ± 6.71) radian/s] and BCF [(8.93 ± 0.62) Hz] of hypothyroidism group were not significantly different compared with that of the control groups[(49.92 ± 6.43) μm/s,(36.52 ± 2.73)％,(52.49 ± 3.49)％,(62.77 ± 7.34)radia/s,(9.32 ± 0.61)Hz,t =0.805,1.089,0.037,0.341,1.033,all P ＞ 0.05].Conclusion Hypothyroidism can affect sperm activity in male rats,decrease sperm density and cause damage to the reproductive system.

Objective To study hemodynamics of cerebral blood flow in newborns with asphyxia.Method Bilateral cerebral electrical admittance plethysmogram(BCEAP) was used to explore characteristics of cerebral blood flow in 20 healthy newborns and 20 newborns with asphyxia respectively from first to fourth day after birth.Results Both the ratio of Hs to b-S(Hs/ b-S) and index of admittance differential loop(ADL) Ⅰ+Ⅱ decreased significantly in newborns with asphyxia compared to normal control from first to fourth day after birth(P

Objective To understand the status and main causes of death among children under five years old in Yuyao County from 2013 to 2017, and to provide scientific basis to formulate relevant intervention measures． Methods Data monitoring deaths among children under five years old from 2013 to 2017 in Yuyao were collected. Trend of death, death rank, regional difference and utilization of pre-death health care services for children under 5 years old were retrospectively analysed using SPSS 18.0. Results From 2013 to 2017, the mortality rate in newborns, infants, 1-4 years old children and children under 5 years old (U5MR) decreased in Yuyao county(P=0.016, 0.002, 0.038, ＜0.001). Based on monitored 263 cases of deaths,the majority of deaths of children under 5 years old occurred in infants, accounting for 68.82% (181/263). Congenital malformation and unintentional injury were the main causes of death for children under 5 years old. The deaths of children under 5 years old mainly occurred in medical institutions, and 93.54% of the children were treated before death. The mortality rate of boys under 5 years old was 10.50‰, which was 1.54 times of the mortality of girls ( χ2=11.693, P<0.001). The trend Chi-square test showed that the U5MR in local residents had no obvious downward trend in the past 5 years ( χ2trend =0.195, P=0.658), while the U5MR in floating population significantly decreased with fluctuation ( χ2trend =17.706, P<0.001). Conclusion The key to reduce U5MR in Yuyao is to reduce infant mortality, and the key content of death intervention is to prevent congenital malformations and unintentional injuries. Improving maternal and child health care and developing safety education for migrant children are two effective measures to reduce U5MR.

Coronary artery disease (CAD) is a multifactorial disease in which inflammation plays a central role.This study aimed to investigate the association of inflammatory markers such as the neutrophil to lymphocyte ratio (NLR),the Global Registry of Acute Coronary Events (GRACE) score with in-hospital mortality of elderly patients with acute myocardial infarction (AMI) in an attempt to explore the prognostic value of these indices for elderly AMI patients.One thousand consecutive CAD patients were divided into two groups based on age 60.The laboratory and clinical characteristics were assessed retrospectively by reviewing the medical records.The NLR and GRACE score were calculated.In the elderly (≥60 years),patients with non-ST-elevation myocardial infarction (NSTEMI) and ST-elevation myocardial infarction (STEMI) had significantly higher NLR than did those with unstable angina (UA) and stable angina pectoris (SAP) (P＜0.01).The NLR was considerably elevated in older AMI patients compared with their younger counterparts (＜60 years) (P＜0.05).In elderly AMI patients,the NLR was considerably higher in the high-risk group than in both the low-risk and medium-risk groups based on the GRACE score (P＜0.05 and P＜0.01,respectively),and the NLR was positively correlated with the GRACE score (r=0.322,P＜0.001).Either the NLR level or the GRACE score was significantly higher in the death group than in the surviving group (P＜0.05).By curve receiver operator characteristic curve (ROC) analysis,the optimal cut-off levels of 9.41 for NLR and 174 for GRACE score predicted in-hospital death [ROC area under the curve (AUC) 0.771 and 0.787,respectively,P＜0.001].It was concluded that an elevated NLR is a potential predictor of in-hospital mortality in elderly patients with AMI.

OBJECTIVETo investigate the diagnostic methods and reasonable treatment of Peutz-Jeghers syndrome (PJS).

METHODSClinical data of six patients with PJS were reviewed.

RESULTSRepeated abdominal pain, intussusception and intestinal polyp with bleeding were main manifestations. Four patients father,three patients grandfather and one patients mother were diagnosed with PJS. Three patients had family history of cancer. Case 4 and case 5 underwent laparotomy for many times because of intussusceptions caused by polyps or recurrent abdominal pain. Case 1 and case 4 had polyps synchronous with adenoma, and case 2 had polyp with gastric cancer. Main treatment included polyp resection and partial small intestinal and colon resection.

CONCLUSIONSPatients with PJS have family history of cancer and a high incidence of polyp recurrence of small intestine. Surgical intervention is the first choice regimen. Surveillance should be emphasized on gastrointestinal tract and other potential malignant organs in PJS patients.

Adolescent ; Adult ; Female ; Humans ; Intestine, Small ; surgery ; Male ; Pedigree ; Peutz-Jeghers Syndrome ; diagnosis ; genetics ; surgery

Cerebral Palsy ; metabolism ; Child ; Child, Preschool ; Female ; Gas Chromatography-Mass Spectrometry ; Humans ; Infant ; Infant, Newborn ; Male ; Metabolism, Inborn Errors ; diagnosis ; genetics

Animals ; Cryopreservation ; Liver ; Liver Transplantation ; Swine ; Swine, Miniature ; Time Factors ; Warm Ischemia

To study the chemical constituents of K. oblongifolia, silica gel column chromatography, MCI and Sephadex LH-20 were used to separate the 70% acetone extract of the stems of K. oblongifolia. The structures of the isolated compounds have been established on the basis of physicochemical and NMR spectroscopic evidence as well as ESI-MS in some cases. Twenty compounds were obtained and identified as heteroclitalignan A (1), kadsulignan F (2), kadoblongifolin C (3), schizanrin F (4), heteroclitalignan C (5), kadsurarin (6), kadsulignan O (7), eburicol (8), meso-dihydroguaiaretic acid (9), kadsufolin A (10), tiegusanin M (11), heteroclitin B (12), (7'S)-parabenzlactone (13), angeloylbinankadsurin B (14), propinquain H (15), quercetin (16), kadsulignan P (17), schizanrin G (18), micrandilactone C (19) and (-)-shikimic acid (20). Compouds 1, 5, 8, 11-15, 18 and 20 were isolated from this plant for the first time. Toxicity of compounds 1-10 were evaluated with zebrafish model to observe the effect on its embryonic development and heart function. The results showed that compounds 7, 9 and 10 caused edema of zebrafish embryo and decreased the heart rate of zebrafish, which exhibited interference effect on heart development of zebrafish.
Animals ; Embryo, Nonmammalian ; drug effects ; Guaiacol ; analogs & derivatives ; toxicity ; Kadsura ; chemistry ; Lignans ; toxicity ; Plant Extracts ; toxicity ; Quercetin ; toxicity ; Triterpenes ; toxicity ; Zebrafish ; embryology