Objective To study the clinical and imaging features of Marinesco Sjgren syndrome.Methods The clinical,laboratorial and imaging information of a patient with Marinesco Sjgren syndrome (28 years old, female) was reported.Results The clinical findings included besides the major features of the syndrome, e.g. ataxia, cataracts, growth deficiency and mental retardation,chronic renal deficiency, hypoparathyroidism and hypogonadism. Karyotype of chromosome was 46,XX,1q +,22s +. CT scan showed stripped path like high density in both lenticular nucleus, cerebellar atrophy; MRI showed cerebellar vermis and hemispheres of small volume. Other abnormal superatentorial findings were showed in MRI, including enlarged third, fourth and lateral ventricles,and expanded cistern;pachygyria in left temporal lobe and a thin corpus callosum lap.Conclusion The major features of Marinesco Sjgren syndrome were growth deficiency and mental retardation,congenital cataracts, cerebellar ataxia, etc.Skull MRI might be helpful in the early diagnosis of the disorders.

OBJECTIVETo explore the mechanisms involved in the ligustrazine alleviation of the pulmonary artery hypertension (PAH) in patients of chronic obstructive pulmonary disease (COPD) associated with chronic cor pulmonale (CCP) during exacerbation.

METHODSSeventy patients of COPD and CCP with acute exacerbation were randomly and equally divided into control group and treatment group. The control group received standard treatment with antibiotics, antiasthmatic and expectorant medications, and oxygenation; and the ligustrazine treatment group received ligustrazine treatment (80 mg/d; i.v.; for 2 weeks) in addition to the standard treatment. Before and at the end of 2 week treatment, the clinic responses of the two regimens were evaluated, plasma levels of endothelin-1 (ET-1) and nitric oxide (NO) were determined; arterial oxygen partial pressure (PaO2, mean pulmonary arterial pressure (mPAP), outflow tract of right ventricle (RVOT), and internal diameter of right ventricle (RV) were measured.

RESULTSGood clinic benefits were achieved in both the standard and ligustrazine regimens, plasma level of ET-1, values of mPAP, RV and RVOT decreased significantly, plasma level of NO and PaO2 values decreased (all P < 0.01 vs pre-treatment to all parameters). Compared with the control group, ligustrazine greatly enhanced the clinic efficacy from 77.1% to 97.1% (P < 0.05), and also resulted in more significant changes of all these parameters (P < 0.01 vs control group for all parameters). For both groups, the levels of plasma ET-1 were positively correlated with values of mPAP, RVOT, and RV (r = 0.710, 0.853, and 0.766, respectively, all P = 0.000), and negatively correlated with plasma NO and PaO2 (r = - 0.823, and - 0.752, respectively, all P = 0.000).

CONCLUSIONLigustrazine is effective in treating pulmonary artery hypertension during acute exacerbation of COPD and CCP in patients from the plateau area. The observed changes in the plasma levels of NO and ET-1 in response to ligustrazine treatment suggest that ligustrazine may act through the selective effect on pulmonary blood vessels to enhance the synthesis and release of NO and suppress those of ET-1 from lung vascular endothelial cells, thus reducing pulmonary artery pressure and decreasing pulmonary arterial hypertension.

Altitude ; Blood Gas Analysis ; Chronic Disease ; Endothelin-1 ; blood ; Humans ; Hypertension, Pulmonary ; drug therapy ; Nitric Oxide ; blood ; Pulmonary Artery ; physiopathology ; Pulmonary Disease, Chronic Obstructive ; drug therapy ; Pyrazines ; therapeutic use ; Respiration

Objective To explore the mechanisms underlying supraliminal and subliminal N-back task in the children with symptoms of attention-deficit/hyperactivity disorder (ADHD).Methods Thirty-five ADHD children and thirty-three matched normal controls were enrolled.Working memory was measured with the different reaction time of N-back task.Results The error number of the subliminal 2-back task in ADHD group ((4.49± 1.20) times) was worse than that of the healthy controls ((3.79±-1.14) times), and the difference was significant(t=-2.459, P=0.017).In ADHD group,the error number of 1-back task and reaction time of 2-back task in subliminal task ((4.77±1.09) times, (1 447.49±271.35) ms) were worse than those of supraliminal task ((3.09± 1.63) times, (1 315.48±229.63) ms), and the difference was significant between the two tasks (t=4.755, P=0.000;t=-2.281, P=0.029).But there were no significant findings in healthy controls(P＞0.05).Conclusion The present study suggests that the working memory is impaired in ADHD children.

OBJECTIVETo investigate the clinical effect of bridging fixation with locking plate for the Seinsheimer type V subtrochanteric femoral fracture.

METHODSFrom March 2009 to September 2014,18 cases of Seinsheimer type V subtrochanteric femoral fracture were treated by open reduction and bridging fixation with locking plate through proximal and distal approach including 16 males and 2 females with an average age of 41 years old ranging from 22 to 67 years old. Among them, 12 cases caused by traffic accident, 5 cases by falling, 1 case by heavy aboving. All cases were fresh and closed fractures. Time between injury and operation was from 4 to 9 days with an average of 6.2 days. Of them, 11 cases were fixed with reverse LISS and the other 7 cases were fixed with anatomical locking plates of proximal femur.

RESULTSThe mean time of operation was 110 min (ranged from 90 to 155 min). The mean blood loss during operation was 425 ml (ranged from 350 to 650 ml) and 16 cases got blood transfusion which was meanly 300 ml. The mean hospital time was 14 days (ranged from 12 to 18 days). The mean duration of followed up was 11.8 months (ranged from 8 to 22 months). The mean time of bone union was 6.6 months (ranged from 5 to 8 months). There was not any complication such as infection, implant failure, hip varus, external rotation deformity of low limb or fat embolism. The Sanders hip scores were 53.22 ± 6.48, the result was excellent in 12 cases and good in 6 cases at the last follow-up.

CONCLUSIONUnder the principle of biological osteosynthesis, treatment of Seinsheimer type V subtrochanteric femoral fracture with bridging locking plate fixation has such advantages as high mechanism, less interference of blood supply, stable fixation and little complication. It is a safe and idea way for the treatment of the Seinsheimer type V subtrochanteric femoral fracture.

Adult ; Aged ; Bone Plates ; Female ; Fracture Fixation, Internal ; methods ; Hip Fractures ; surgery ; Humans ; Male ; Middle Aged

Objective： This study was designed to determine the serum carcinoembryonic antigen (CEA), soluble Fas (sFas), and interleukin-6 (IL-6) levels in colorectal cancer and analyze their clinical significance. Methods： Serum sFas and IL-6 were tested in pre-operated and post-operated 162 patients with colorectal cancer using double antibody sandwich ELISA method, serum CEA and immune indicators were also examined. Results： Levels of serum CEA, sFas and IL-6 [(32.52± 10.81)μ g/L, (20.97± 8.19) ng/L, (36.87± 11.20) ng/L, respectively] in the colorectal cancer patients with the positive rate (sFas： 53.7％ ;IL-6： 67.91％ ; CEA： 41.98％ ) were significantly higher than those in healthy control group and benign patients （ P<0.01), the post operated patients showed a lower levels CEA, sFas, and IL 6 than the pre operation patients （ P< 0.01） , serum levels of sFas and IL 6 in post operated patients were the lowest. Serum sFas level was correlated with tumor metastases, serum concentration of IL 6 was correlated strongly with tumor size and metastases (P< 0.01). Serum concentration of sFas was correlated negatively with lymphocyte transformated rate, NK or LAK killing rate, and had marked correlation among CEA, IL 6, and sFas. Conclusion： The positive rates of serum sFas and IL 6 in the colorectal carcinoma patients were higher than those of the serum CEA. Serum sFas and IL 6 levels reflected indirectly situation of proliferation and metastases in tumors. sFas may inhibit the patients immune function and tumor cells apoptosis, and IL 6 may accelerate proliferation of the colorectal carcinoma cells. These results indicate that combinative measuring of the serum levels of CEA, sFas, and IL 6 may be an useful method for clinical diagnosis, disease prognosis, and treatment effect in colorectal patients.

The study is to report the establishment of a method of screening the antitumor compounds based on the dynamic bio-response profile of cells to make up for the shortages of conventional end-point tests such as tedious operation and low sensitivity. Based on the principle of electric impedance of cells, the real-time cell electronic sensing (RT-CES) system was used to monitor the effect of epirubicin (EPI), cisplatinum (DDP) and carboplatin (CBP) on the growth of HepG2 cells, with the cell index (CI), half maximal inhibitory concentration (IC50) and detachment curve as evaluation indexes. Meanwhile, cell counting kit-8 (CCK-8) and microscopy were applied for verification. The results showed that CI curve could sensitively real-time profile the inhibitory effect of model drugs on HepG2 cells. The IC50 of EPI, DDP and CBP were 0.53 +/- 0.04, 9.79 +/- 0.26 and 597.00 +/- 3.79 microg x mL(-1), respectively. What's more, the significant differences of detachment curves of the three drugs indicated that their functional mechanisms might be different, this is consistent with the literature. The RT-CES system with non-invasive, label-free and real-time characteristics could be used to monitor the bio-response profile of the three drugs to HepG2 cells, allowing to qualitatively and quantitatively distinguish the antitumor activities of the three drugs, and could be a complementary method for the present screening of antitumor compounds.
Antineoplastic Agents ; pharmacology ; Biosensing Techniques ; methods ; Cell Count ; Cell Line, Tumor ; Cisplatin ; pharmacology ; Drug Screening Assays, Antitumor ; Electric Impedance ; Humans

This study aims at trying to establish a novel method of sterility test for injections based on biothermodynamics, in order to overcome the deficiencies of routine sterility tests such as long detecting cycle, low sensitivity and prone to misjudgments. A biothermodynamics method was adopted to rapidly detect the microorganism contamination of injections by monitoring the heat metabolism during the growth of microbe. The growth rate equal to or greater than zero and the heat power difference of P(i) and P(0) with three folds higher than the noise of baseline were chosen as indexes to study the heat change rule of microbe. In this way, the effectiveness of the new method to detect strains required by conventional sterility test or in injection samples was also investigated. Results showed that the Gram-positive bacteria, Gram-negative bacteria and fungi demanded by sterility testing methodology could be detected by biothermodynamics method within 10 hours, with the sensitivity lower than 100 CFU x mL(-1). Meanwhile, this method was successfully applied to the sterility test of Compound Yinchen injection (FFYC), Shuanghuanglian powder injection (SHL) and Compound Triamcinolone injection (TAND) which were sterilized with different degrees. Therefore, the biothermodynamics method, with advantages of fast detection and high sensitivity, could be a complementary solution for conventional sterility tests.
Anti-Inflammatory Agents ; administration & dosage ; chemistry ; Drug Contamination ; Drugs, Chinese Herbal ; administration & dosage ; chemistry ; Fungi ; isolation & purification ; Gram-Negative Bacteria ; isolation & purification ; Gram-Positive Bacteria ; isolation & purification ; Hot Temperature ; Injections ; Microbiological Techniques ; methods ; Sensitivity and Specificity ; Sterilization ; Triamcinolone ; administration & dosage ; chemistry

In vitro neuraminidase inhibition assays and ultrafiltration liquid chromatography with diodearray detector coupled to time of flight mass spectrometer (UPLC-DAD-TOF-MS) were combined to screen bioactive compounds inhibiting neuraminidase from Isatidis Radix. By comparing the compounds from Isatidis Radix before and after ultrafiltration, we found that arginine, goitrin and adenosinea can bind with neuraminidase, and the binding degree of the three compounds were (36.23 +/- 1.12)%, (32.54 +/- 1.02)% and (9.38 +/- 0.47)%, respectively. The IC50 of arginine and goitrin were (1.16 +/- 0.02), (1.20 +/- 0.02) g x L(-1), respectively. While the IC50 of adenosinea was higher than 500 g x L(-1). The results showed that arginine and goitrin might be the main compounds with antiviral activity of Isatidis Radix. This study may provide a useful method for the screening of bioactive compounds and quality control of Isatidis Radix.
Antiviral Agents ; analysis ; pharmacology ; Arginine ; analysis ; pharmacology ; Drug Evaluation, Preclinical ; Drugs, Chinese Herbal ; analysis ; pharmacology ; Isatis ; chemistry ; Mass Spectrometry ; Neuraminidase ; antagonists & inhibitors ; metabolism ; Orthomyxoviridae ; drug effects ; enzymology ; Oxazolidinones ; analysis ; pharmacology ; Plant Roots ; chemistry ; Ultrafiltration ; Viral Proteins ; antagonists & inhibitors ; metabolism

OBJECTIVETo develop a scoring system to measure the severity of the multiple organ dysfunction syndrome (MODS).

METHODSThe clinical data of patients with MODS were collected and analyzed prospectively. Seven indicators were screened out to assess the functions of seven organs. Each indicator was scored from 0 to 4 points, with the 0 point representing the normal organ function, and 1 point to 4 points representing the dysfuntion to failure of the organ. Acording to the index and points a severity scoring system were developed.

RESULTSSeven indicators including the systolic pressure, oxygenation index, conscious state, peripheral blood platelet count, blood total bilirubin, and serum creatinine were used to represent the functions of seven organs. The seven organs included cardiovascular system, lung, brain, coagulative system, liver, kidney, and gastrointestinal tract. The severity scoring system were composed of seven indicators and their points. The total score was 24 points. The mortality increased along with the increase of the points (P<0.001).

CONCLUSIONThis scoring system can be used to assess the severity of the MODS.

Humans ; Multiple Organ Failure ; diagnosis ; mortality ; physiopathology ; Severity of Illness Index

OBJECTIVETo identify enterovirus 71 (EV71) strains isolated from patients with hand, foot and mouth disease (HFMD) in Guangdong and Fujian provinces from 2000 to 2001 by using phylogenetic analysis.

METHODSAll 25 samples were first tested for enteric viruses by RT-PCR using enterovirus specific primers EV-1 and EV-2, and then were identified for EV71 by RT-PCR using EV71 specific primers 159S and 162A. The amplicons of 485bp segment (part of the VP1 gene) were cloned into pGEM-T and sequenced. A phylogenetic tree was constructed by comparison of the sequences with other 12 EV71 strains isolated from China, Japan, Hungary, and the United States including the prototype BrCr.

RESULTSThe positive rate of EV71 was about 20%. The sequence analysis showed that the new isolate (GZH2000) shared 94%-96% nucleotide identity with three strains isolated in 1998 and 2000, and 91% with a strain isolated in 1987 from Chinese mainland, but shared only 82%-84% homology with EV71 isolates studied abroad.

CONCLUSIONSEV71 is one of the important pathogens of HFMD in south China. The strains isolated from mainland were closely related with most isolates from Taiwan, but different from most EV71 strains reported abroad. The symptoms of EV71 infection in mainland were not as intensive as those described in Taiwan's outbreak.

Adolescent ; Child ; Child, Preschool ; China ; Enterovirus ; genetics ; isolation & purification ; Hand, Foot and Mouth Disease ; virology ; Humans ; Infant ; Phylogeny ; Sequence Analysis ; Sequence Homology, Nucleic Acid