Objective To explore the feature of electrical physiology and the key aspect of diagnosis in early stage of spinal muscular atrophy in infants.Method The back clinical data of the musle change of electrical physiology within 25 cases were analyzed.Results The children were ill in 1 year old,with the symptom that four limbs present symmetry and paralysing slowly,low limbs being more serious than upper limbs,the close and the distant of the four limbs.The levels of creatine kinase(CK) and lactate dehydrogenase(LDH) in serum were normal and the case unconcentrated was not seen rarely.The show of electromyogram(EMG) was that three limbs presenting wide-range losing nerve electric potential in peace state,when straingently the deadline was long and the motor unit potential(MUP) was high,when strain highly the eletric potential was decreased;the speed of nerve conduction was common.Muscle inspection was typical nervine myatrophy.Conclusion The clinical feature,the changes of muscle electrical physiology and the muscle inspection are valuable for diagnosis so as to afford reliable evidence for further gene diagnosis.J Appl Clin Pediatr,2006,21(3):167

Objective:This work aims to explore the long-term efficacy and complications of late-course accelerated hyperfrac-tionation (LCAHF) for treating nasopharyngeal carcinoma. Methods:A total of 58 patients who consulted from December 2005 to May 2008 and histologically proven nasopharyngeal carcinoma at initial diagnosis were randomized into an LCAHF group (experimental group) and a conventional fractionation (CF) group (control group). The treatment dose for both groups was 2 Gy per fraction once dai-ly, 5 days a week. After the 40 Gy to 50 Gy dose, the dosage in the LCAHF group was increased to two daily doses at 1.5 Gy per frac-tion 6 h apart, 5 days a week. The total dose in this group was 73 Gy to 76 Gy, the total dose in the CF group was 70 Gy to 76 Gy, with the total course of the treatment shortened by 0.5 weeks to 1.5 weeks in the former group. Results:The 5-year control rates of the naso-pharyngeal cancers was 86% in the LCAHF group and 59% in the CF group (P=0.021), with statistically significant differences be-tween the two groups. The late complications slightly increased in the LCAHF group than in the CF group, but the differences were not statistically significant. Conclusion:LCAHF treatment improves the local control of nasopharyngeal carcinoma without increasing the incidence of long-term complications.

Diagnostic Errors ; Female ; Humans ; Infant, Newborn ; Inhalation ; Male ; Organophosphate Poisoning ; Poisoning ; diagnosis

Endotoxins are lipopolysaccharides ( LPS) , major component of out wall in Gram-negative bacteria. Often they are considered as "prime criminals" of triggering systemic inflaming reaction such as sepsis, bacteremia and so on. It was found in recent years that bactericidal/permeability-increasing protein, a 55kDa member of lipid-related protein family, was a kind of natural molecule of anti-infection and it has special endotoxin-neutralising activity and antibacterial activity. Comprehensive phase II/III clinical trials demonstrated the feasibility and safety of recombinant BPI. So pharmaceutical corporations are attracted to try to apply it to therapy.

Objective To study the molecular biology of rifampin-depending M. Tuberculosis. Methods The seguence (a 319-bp DNA fragment) of rpoB gene were analyzed by automated DNA sequencing machine. (2) The fingerprints of genomic DNA were obtained by random amplified polymorphic DNA (RAPD) fingerprinting. (3)The protein electrophoresis of bacterium by SDS-polyacrylamide gel (SDS-PAG).(4) The cases of pulmonary tuberculosis by rifampin-depending strains were retrospectively analyzed. Results (1) rpoB gene sequenced: The point mutationrate of rifampin-depending strainswas 96.7%(29/30) and that of rifampin-residtant strains 81.1%(30/37), P

Background and purpose:Ara-C is one of the most effective and common agents in the treatment of acute nonlyphocytic leukemia. Telomerase is a unique complex of ribonucleoprotein. It plays an important role in the pathogenesis and development of cancer. In this study, we investigate the changes of mRNA expression of telomerase subunits in HL-60 cells induced by Ara-c and try to come up with a theory that could help to assess the efficacy of Ara-C. Methods:The combinations of various Ara-C concentration and the incubation time were used to treat HL-60. The ratios of apoptotic cell to necrosis cell were determined by flow cytometry and the expressions of telomerase subunits mRNA were evaluated by RT-PCR.Results:① There was no influence on transcription of telomerase subunits gene after HL-60 cells was cultured with 0~0.2ug/ml Ara-C for 12 hours;② 2ug/ml and 10ug/ml of Ara-C could down regulate the expression of hTERT from 0.80+0.07 to 0.50+0.04 and 0.39+0.03, not hTR and hTP1;③ with longer incubation with 10ug/ml of Ara-C, the percentage of apoptosis could be increased. The maximal induction of apoptosis (18.16+4.25%) could be reached at 12hrs treatment of Ara-C, then gradually decreased later on. The rate of necrosis increased with time, the maximal percentage(57.94+12.03%) of necrosis was observed at 48hrs of incubation time with drug. The mRNA level of hTERT gene also decreased along with the cultured time , the lowest value (0.18+0.03) has been documented at 48hrs time point, but not hTR、TP1.Conclusions:① Ara-C could down-regulate the expression of hTERT mRNA in a dose-and time-dependent manner, but not hTR、hTP1;② There might be no relationship between the percentage of apoptosis induced by Ara-C apoptosis and the expression of telomerase hTERT gene mRNA, but a close relationship between necrosis and the expression of hTERT mRNA has been found.

Background Clinical research showed that the corneal endothelial cell density value from different corneal specula microscopies exist diversity.The relevant literature of SP02,Tomey EM-3000 and SP3000P is still seldom up to now. Objective This research was to assess the repeatability of endothelial cell density measurements by SP02,Tomey EM-3000 and SP3000P respectively and the agreement among 3 kinds of endothelial microscopes.MethodsFifty-four healthy volunteers with the age 17-38 years old were included this research.The written informed consent was obtained from each subject before examination.The corneal endothelial cell densities in the right eyes were analyzed with SP02,Tomey EM-3000 and SP3000P respectively for 3 times under the automatic mode,and the analytical procedure of SP3000P measurement were divided into automatic mode SP3000P (A) and manual correction modes SP3000P( M).The repeatability of each specula microscopy was analyzed by calculating the intraclass correlation coefficients (ICC) and coefficient of variation ( CV ),and the 95％ confidence intervals and plotting Bland-Altman graphs were used to analyze the agreement among these methods.ResultsThe mean corneal endothelial cell densities in the population ＜24 years were significantly higher than the ones ≥ 24 years (t =3.692,P＜0.05 ),but no statistical difference was found between different gender ( t =0.335,P =0.739 ).The mean corneal endothelial cell densities were ( 3058 ± 260 ),( 2954 ± 229 ),( 2668 ± 258 ),( 2734 ± 268 ) cell/mm2 ; the ICCs were 0.957,0.940,0.972 and 0.972 and the CV were 0.063,0.061,0.056,0.058 for SP02,Tomey EM-3000,SP3000P (A) and SP3000P ( M ) respectively.The 95％ confidence intervals were ( - 100.8 - 306.8 ),( 162.6 - 617.4 ),( 109.9-494.1 ) and ( -0.6 - 132.6 ) cell/mm2 for between SP02 and Tomey EM-3000,SP3000P ( A ) and SP02,SP3000P(A) and Tomey EM-3000,SP3000P(A) and SP 3000P(M) respectively.ConclusionsSP02,Tomey EM-3000 and SP3000P(A) have good repeatability in the measurement of corneal endothelial cell density,however the outcome is different.Therefore,it is not interchangeable for the detection of corneal endothelial cell density.The differences of corneal endothelial cell density obtained from these instruments shall be paid high attention for their differences.SP3000P(A) and SP3000P(M) can be used interehangeably and SP3000P(A) is a preferable choice due to its convenience and quickness.

Background Central corneal thickness (CCT)is an important parameter to evaluate corneal healthy status and is crucial for surgical planning.However,clinical study found that the center of cornea does not correspond to the thinnest point of cornea.Thus,it is essential to characterize the minimum corneal thickness(MCT) and its location.Objective Present study was to determine the thickness and location of MCT and its relationship to the fellow eye using Pentacam High Resolution technique.Methods The 564 eyes from 282 Chinese myopic patients were reviewed in this study.The CCT,MCT,pupillary central corneal thickness(PCT) and x-y coordinate of thinnest point were bilaterally measured.Written informed consent was obtained prior to the ocular biomedical measurement.Results CCT was (540.07±31.78) μm in the right eyes and (539.24±31.06) μm in the lefi eyes; PCT was (540.25±30.75)μm in the right eyes and (539.48±31.00)μm in the left eyes.MCT was (537.87± 31.91)μm in the right eyes and (536.35±31.24)μm in the left eyes,showing significant differences in all the parameters between the right eyes and left eyes expect for PCT(CCT:P=0.046;PCT:P=0.065 ;MCT:P=0.000).The C CT,PCT and M CT were significantly correlated between the right eyes and left eyes (r =0.97,0.97,0.98,P＜ 0.01).Bland-Altman plot showed a good consistence between the both eyes.The mean distance from the center was (0.50±0.21) mm in the right eyes and (0.56±0.22)mm in the left eyes,showing a significant difference (P =0.000).The difference between CCT and MCT was approximately (2.20±1.74)μm in the right eyes and (2.88±1.75) μm in the left eyes.The location of MCT in both the right eyes and left eyes tended to symmetry along the vertical midline.The distance between the R (x,y) to transformed L (x,y) was (0.29 ± 0.17)mm and the angular distance was (28.28±28.21)degree.Conclusions This study offers a range of MCT and its location in Chinese myopic patients.The difference exists between the CCT and MCT in bilateral eyes.The location of the thinnest point tends to be symmetrical along the vertical midline between the both eyes.The changes of these parameters may be helpful for the diagnosis of some corneal diseases.

Objective To observe the therapeutic effects of the co-administration of γ-aminobutyric acid (CABA), sodium dimercaptopropane sulfonate (Na-DMPS) and vitamin B6 in large doses on liver and heart of rats with acute tetramine intoxication, and compare their separate effects of either GABA or Na-DMPS alone with those of the triad combination. Method Thirty rats were randomized into control group (n = 6), tetramine intoxication without treatment group (n = 6), tetramine intoxication treated with GABA group (n = 6), tetramine intoxication treated with Na-DMPS group (n = 6) and tetramine intoxication treated with triad combination (GABA + Na-DMPS + vitamin B6, GNDV n = 6) group. Samples of blood, liver tissue and heart tissue of rats with acute tetramine intoxication were collected immediately two hours after medication with different drugs. Serum alanine aminotrasferase (ALT), aspartate aminotransferase (AST), creatine kinase (CK) and creatine kinase isoenzyme (CK-MB) were measured, and the pathological changes of liver tissue and heart tissue were observed under microscope. Results The symptoms of poisoning were apparently relieved and the latency for convulsion/muscular twitch were obviously delayed in poisoned rats treated with GABA, Na-DMPS and GNDV separately. Furthermore, combination group showed the latent period delayed longer than either GABA or Na-DMPS groups The GABA, Na-DMPS and GNDV significantly lowered the serum levels of ALT, AST, CK and CK-MB in rats with tetramine intoxication, and those serum levels of enzymes were lower in GNDV group than those in either GABA group or Na-DMPS group. However, there were no difference in those serum enzymes between GABA group and Na-DMPS group. Moreover, the intoxicated rats treated with combination treatment had the slightest pathological changes in liver and heart (GNDV < GABA or Na-DMPS). Conclusions The co-administration of γ-aminobutyric acid, sodium demercaptopropane sulfonate and vitamin B6 in large doses for the treatment of tetramine intoxication is a method of choice.

BackgroundIn an ideal eye there would be no light scattering at all,but the eye media is not optically ideal.Intraocular straylight causes a veil of light and reduction in the contrast of the retinal image and thus decrease the quality of vision.ObjectiveThe present study was to investigate the repeatability and reproducibility of C-Quant straylight meter( Oculus,Germany)in measuring retinal straylight of myopia and post-laser in situ keratomileusis(LASIK) corneas.MethodsThis is a prospective research.The consecutive 35 eyes of 21 myopic patients and 34 eyes of 22 patients who received LASIK were included in this trail.Retinal straylight was measured for 7 times at a period of time and analyzed quantificational to evaluate the repeatability of measurement.Thirty-eight eyes of 19 patients were measured again at 3-7 days for 3 times at a period of time to assess the reproducibility of C-Quant straylight meter.The mean standard difference (SD) and coefficient of variation (CV) were used as the credibility evaluation.This clinical study complied with Helsinki Declaration and the informed consent was obtained prior to the medical procedure.ResultsThe straylight Log(s) of 7 times measurement were all less than 0.95.The mean Log(s) were 0.92±0.12 and 0.93±0.17 respectively in myopia group and post-LASIK group,without statistically significant differences among 7 times measurement( F=0.335,P=0.812;F=1.000,P=0.409).The mean SD for the 7 times measurement was 0.07 Log units.SD and CV increased with the number of measurements.The differences of mean SD and CV between 3 times result and 6 times result were significant different (t =-2.080,P =0.045;t =-2.190,P =0.035 ).No difference was found between different time periods( t =-0.531,P=0.598 ).The difference of the results between two measurements from the same patient was 0.013.ConclusionsC-Quant is a noncontact,noninvade,rapid and convenient method for the measurement of straylight in myopia and post-LASIK eyes due to the high repeatability and reproducibility.