Objective To explore the electrophysiological charaterstics of upper extremities nerves on the patients with Hirayama disease (HD),amyotrophic lateral sclerosis (ALS),and distal cervical spondylotic amyotrophy (DCSA).Methods The data of electrophysiological examination of the upper limbs of 87 patients with HD,83 with ALS and 28 with DCSA were reviewed retrospectively.Seventy-two patients with HD among 87 had unilateral upper limb's amyotrophy and the other 15 ones had bilateral amyotrophy.There were 30 patients had unilater upper limb's amyotrophy and 53 ones had bilateral amyotrophy from the group of patients with ALS; 20 patients with DSCA were affected unilaterally and 8 ones were bilaterally affected.Results Compound muscle action potential (CMAP) evoked by ulnar stimulation had a lower ampititude compared with that evoked by median stimulation in HD patients.In ALS cases that was just the opposite.However,the CMAPs were similar in DCSA cases.The mean ratio of CMAP amplitude by ulnar stimulation to by median stimulation was 0.58±0.40 in HD group; 2.28±1.25 in ALS and 1.31±0.63 in DCSA.The differences in the three groups were statistical significance.The U/M CMAP ratio was less than 0.6in 62 patients with HD,3 with ALS and 1 with DCSA,and more than 1.7 in 73 cases (57 ALS,12 HD and 4 DCSA).Conduction velocities (CV) of the sensory and motor nerves,the amplitude of the sensory nerve action potential in bilateral limbs,and the CMAP amplitude of the unaffected limb were normal in all cases.Conclusion This study could concluded that the severity of amyotropy in hypothenar mucles were higher than that in thenal muscles in patients with HD; there was just opposite in ALS cases and similar in DSCA.

Objective To explore the characteristics of proinsulin secretion in latent autoimmune diabetes in adults (LADA). Methods Fasting and 2 h sera in oral glucose tolerance test from 36 LADA patients, 37 type 2 diabetic patients and 43 healthy controls were collected to test glucose, proinsulin (PI) and C-peptide (CP) by radioimmune assay. Glutamie acid decarboxylase antibodies (GAD-Ab) were determined by radioligand assay.Results (1) Fasting proinsulin (FPI) and 2 h proinsulin (PPI) level in LADA patients were lower than those in type 2 diabetic patients (P<0.05), being both significantly inereasad compared with healthy controls (P<0.05 or P<0.01); The ratios of FPI/FCP and PPI/PCP (%) in LADA were beth significantly higer than those of type 2 diabetes mellitus and controls (P<0.05 or P<0.01); (2) LADA type-1 (GAD-Abe>0.3) patients showed lower PI levels(P<0.05 or P<0.01) and higher PI/CP ratio (all P<0.05) than LADA type-2 (0.05≤GAD-Ab<0.3); Meanwhile, there were no significant differences in above parameters between LADA type-2 and type 2 diabetes meUitus (P>0.05). (3) GAD-Ab index was negatively correlated with FPI and PPI in LADA group (r=-0.236 and-0.268, both P<0.05), and positively correlated with PPI/PCP (r=0.254, P=0.030).Meanwhile BMI was positively correlated with FPI, PPI and PI/CP in type 2 diabetes mellitus (all P<0.01). No factor entered the multiple regression analysis for predieting the hyperproinsulinemia and dispropriately elevated proinsulin levels in LADA patients. (4) According to the 99.5 th percentile of proinsulinemia in the healthy controls, which is defined as the cutoff point dispropriately elevated proinsulin levels, the proportion of subjects with fasting dispropriately elevated proinsulin levels (FPI/FCP) were 77.8%, 62.2% and 2.3% in LADA, type 2 diabetes meUitus and controls respectively, and PPI/PCP 83.3%, 51.4% and 2.3% respectively. Conclusion LADA patients, as well as type 2 diabetic patients, all showed hyperproinsulinemia and disproportionately elevated proiasulin levels that were one of characteristics of defective β-cell function. Moreover, disproportionately elevated nproinsulin level is more evident in LADA patients than that in type 2 diabetics and this may be related to humoral immunity.

The purpose of education is to cultivate talents who can master the ability of self-learning lifelong. With the rapid development of multimedia technology, the knowledge carrier represented by micro curriculum plays a very important role in improving students' self-learning ability. In traditional Chinese medicine college, due to the short of time, weak learning foundation, the ability of self-learning is hard to improve in the modern medical courses such as biochemistry. This is not conducive to the cultivation of modern talents of Chinese medicine. In this paper, we chose the biochemistry teaching in TCM college as an example, and discuss how we can make the application of micro courses reasonably in the teaching process. This study was regarded as a starting to improve the students' self-learning ability effectively.

OBJECTIVETo investigate results of total knee arthroplasty using the long-stem tibial component combined with metallic wedge of knee prosthesis for the treatment of proximal defects.

METHODSFrom January 2011 to May 2013, 10 patients (11 knees) were treated with total knee arthroplasties using the long-stem tibial component with metallic tibial wedge of knee prosthesis. All the patients were female and the average age was 67 years old (ranged, 60 to 77 years old). All the patients were osteoarthritis. All the patients were classified as T2A style. The patients were evaluated according to knee score system (KSS).

RESULTSAll the patients were followed up for 12 months on average (ranged 3 to 29 months). The clinical outcome was assessed using KSS score, including knee pain score, knee stability score, knee range of motion score and knee walking score, knee stairs score. There were significantly differences at 6 weeks, 3 months, 6 months and 12 months between pre-and postoperative KSS score.

CONCLUSIONThe mechanical stability of tibial fixation in primary TKA is significantly increased by using the long-stem tibial component with metallic wedge of knee prosthesis, even in the presence of poor proximal bone.

Aged ; Arthroplasty, Replacement, Knee ; Female ; Humans ; Knee Joint ; physiopathology ; surgery ; Knee Prosthesis ; Male ; Osteoarthritis, Knee ; physiopathology ; surgery ; Range of Motion, Articular ; Tibia ; abnormalities ; physiopathology ; surgery

Objective To investigate the effect of folic acid intervention on plasma homocysteine (Hcy) metabolic changes and pulse wave velocity(PWV)in patients with type H hypertension. Methods Patients(hos-pitalized from March 2014 to December in our hospital)with H type hypertension were randomly divided into treat-ment group and control group randomly ,and were given routine antihypertensive drug therapy. Treatment group was given oral folic acid 0.8 mg,1 times a day,the control group was given placebo,1 times a day. All patient were treated for 12 months. Changes of plasma Hcy and PWV levels were observed. Results 432 patients(Han nationality)with type H hypertension were enrolled in this study with the age of 61.7 ± 13.6 years old and the ratio of men and women is 1.3:1. The most common diseases were coronary heart disease and type 2 diabetes mellitus. 2 groups were treated for a period of 12 months,with follow-up time from 6 to 10 months(average duration in 8 months). After treatment,the difference between plasma Hcy(Z=-7.63,P=0.000)and PWV(Z=-3.16,P=0.002)levels of the two groups were statistically significant. Conclusion Folic acid intervention can significantly reduce the level of plasma Hcy in patients with type H hypertension ,slow down the progression of atherosclerosis and reduce the risk factors of cardiovascular disease.

Background The human transforming growth factor beta-induced gene (TGFBI) is the first determined pathogenic gene to corneal dystrophy.But the molecular genetic mechanism is completely unknown.The study of concerning role of TGFBI is very important for us understand the physiological function of cornea,and the pathogenesis of corneal dystrophy.Objective The vector of human transforming growth factor beta-induced gene (TGFBI) in eukaryotic expression was constructed and transfected into the human corneal epithelial cells in order to explore its influence on the growth of human corneal epithelial cells.Methods Total RNA was extracted from normal donor cornea tissue and cDNA was obtained by reverse transcription.TGFBI cDNA was synthesized by reverse transcription-PCR and cloned into pCMV-N-HA vector and identified by sequencing with PCR and EcoRV,XhoI double restriction endonuclease.The cells were grouped into recombinant pCMV-N-HA-TGFBI plasmid group,pCMVN-HA plasmid group,non-transfected group and pGFP-C2 transfected group.The recombinant pCMV-N-HA-TGFBI plasmid was transfected to human corneal epithelial cells and identified by observing the expression of enhanced green fluorescence protein(EGFP) in the cells.The TGFBI mRNA and proteins were harvested from the cells for real-time PCR analysis and Western blot assay respectively in 58 hours after transfection.The growth of the transfected cells was assessed by Cell Counting Kit-8.The expressions of matrix metalloproteinase(MMP) and tissue inhibitors of matrix metalloproteinase (TIMP) proteins and their mRNA in transfected cells were detected using SYBR fluorescence realtime PCR analysis and Western blot assay.Results The sequencing result of pCMV-N-HA-TGFBI positive clone plasmid showed that amplified TGFBI eDNA inserted into the vector at the correct sequence.EGFP was expressed in transfected cells in 48 hours after transfer of pGFP-C2 with the transfer efficacy 70％.The expression intensity of TGFBI mRNA was significantly higher in recombinant pCMV-N-HA-TGFBI plasmid group compared with pCMV-N-HA plasmid group and non-transfected group,and TGFBI protein was expressed in recombinant pCMV-N-HA-TGFBI plasmid group.No significant difference was found in the A450value among recombinant pCMV-N-HA-TGFBI plasmid group,pCMV-N-HA plasmid group and non-transfected group ( F=3.34,P＞0.05 ).The mRNA level of MMP1,MMP3in the transfected cells was significant elevated but that of TIMP1 was declined in the recombinant pCMV-N-HA-TGFBI plasmid group compared with pCMV-N-HA plasmid group and non-transfected group (all P ＜ 0.05 ).Meanwhile,the expressions of MMP1,MMP3 and TIMP1 proteins appeared the same tendency( all P＜0.05).Conclusions Eukaryotic expression vector harboring human TGFBI eDNA can be successfully constructed and efficiently overexpressed in human corneal epithelial cells.TGFBI gene is involved in the physical and pathological conditions of human corneal epithelial cells by regulating the activity of MMP1,MMP3 and TIMP1.The results offer a new approach for the study of the role of TGFBI in pathogenesis of corneal transparency.

OBJECTIVE: To investigate characteristics of molecular etiology of children with profound sensorineural hearing loss in Hubei province, and to provide reference for deafness treatment and genetic counseling. METHOD: Three hundred and six children with profound sensorineural hearing loss in Hubei province were enrolled, their genomic DNA were extracted from peripheral blood and a deafness gene test chip was used to screen nine hot spot mutation in the GJB2, GJB3, SLC26A4, and mitochondria 12SrRNA gene. All patients with SLC26A4 gene mutation were given temporal bone CT scan. RESULT: One hundred and thirty-two (43.14%) out of 306 children were found carrying at least one pathogenic gene mutation. The mutation rates of GJB2, SLC26A4 and mitochondria DNA 12SrRNA gene were 29.41% (90/306), 13.72% (42/306) and 0.65% (2/306), respectively. None out of 306 children was detected GJB3 gene mutation. Thirty-six patients carrying SLC26A4 gene mutation were detected enlarged vestibular aqueduct by CT scan. CONCLUSION Mutations of GJB2 and SLC26A4 gene are two major pathogenic gene for genetic hearing loss in children. 235delC mutation is the main mutation type, followed by IVS7-2A> G mutation type. The screening of SLC26A4 gene common mutations contribute to the diagnosis of enlarged vestibular aqueduct syndrome.
Adolescent ; Child ; Child, Preschool ; China ; Connexin 26 ; Connexins ; genetics ; DNA Mutational Analysis ; Deafness ; genetics ; Female ; Genetic Testing ; Humans ; Infant ; Male ; Membrane Transport Proteins ; genetics ; Mutation ; Oligonucleotide Array Sequence Analysis ; Sulfate Transporters

Objective To explore the activated brain region of acute epilepsy in cat model induced by pentylenetetrazol(FFZ)with manganese enhanced-functional MR imaging(ME-fMRI),and evaluate the application of ME-fMRI on localization of the activated brain.Methods Forty cats were divided into 4 groups by random number table method as epileptic A and B groups as well as control A and B groups. Cats of epileptic groups were injected with PTZ(55 mg/kg)intramuscularly,and those of control groups were injected with the saline at same dose.The behavior change in the epileptic and control group A was observed and electroencephalogram(EEG)was also undertaken.Cats of epileptic and control group B were performed ME-fMRI,and the percentage of the enhanced signal intensity was then calculated.Results After injection with PTZ(55 mg/kg)intramuscularly,epileptic seizure was all evoked,and then EEG recording showed spike-wave and polyspike-wave complexes.The neocortex of cats of epileptic group B was diffusely phanero-enhanced on ME-fMRI.The percent enhancement of signal intensity in cortex of frontal lobe,parietal lobe and occipital lobe was(34.6?5.7)% and that in cortex of temporal lobe with(22.9? 6.5)%,whereas those of control group B with(14.9?4.5)% and(11.6?3.2)% respectively.And there was significant difference between the above different localization of the brain in the two groups (t=-10.43,-5.46 respectively,P

The aim of this study was to examine the effects of endoplasmic reticulum (ER) stress on aldosterone (Aldo)-induced apoptosis of endothelial cells. Glucose-regulated protein 78 (GRP78) and C/EBP homologous protein (CHOP, a hallmark of ER-associated apoptosis) were used to evaluate ER stress. Western blotting and real-time PCR were used to analyze indicators of ER molecule. Apoptosis was detected by annexin V/propidium iodide staining and flow cytometry. Human umbilical vein endothelial cells (HUVECs) were stimulated with different concentrations of Aldo for different durations. Aldo promoted apoptosis of HUVECs and induced ER stress, as evidenced by increased expression of GRP78 and CHOP. siRNA knockdown of CHOP attenuated Aldo-mediated apoptosis. These results indicate that ER stress may be involved in Aldo-induced apoptosis of HUVECs.
Aldosterone ; pharmacology ; Apoptosis ; drug effects ; Endoplasmic Reticulum Stress ; drug effects ; Gene Expression Regulation ; drug effects ; Heat-Shock Proteins ; biosynthesis ; Human Umbilical Vein Endothelial Cells ; cytology ; metabolism ; Humans ; Transcription Factor CHOP ; biosynthesis

Adolescent ; Biopsy ; Cystic Adenomatoid Malformation of Lung, Congenital ; diagnosis ; diagnostic imaging ; therapy ; Diagnosis, Differential ; Diagnostic Errors ; adverse effects ; prevention & control ; Humans ; Infant ; Lung Diseases ; diagnosis ; diagnostic imaging ; therapy ; Male ; Middle Lobe Syndrome ; diagnosis ; diagnostic imaging ; surgery ; Tomography, X-Ray Computed ; Treatment Outcome