BACKGROUNDRopivacaine and levobupivacaine have been introduced into obstetric analgesic practice with the proposed advantages of causing less motor block and toxicity compared with bupivacaine. However, it is still controversial whether both anesthetics are associated with any clinical benefit relative to bupivacaine for labor analgesia. This study aimed to compare the analgesic efficacy, motor block and side effects of bupivacaine, ropivacaine and levobupivacaine at lower concentrations for patient-controlled epidural labor analgesia.

METHODSFour hundred and fifty nulliparous parturients were enrolled in this randomized clinical trial. A concentration of 0.05%, 0.075%, 0.1%, 0.125% or 0.15% of either bupivacaine (Group B), ropivacaine (Group R) or levobupivacaine (Group L) with sufentanil 0.5 microg/ml was epidurally administered by patient-controlled analgesia mode. Effective analgesia was defined as a visual analogue scale score was

RESULTSThere were no significant differences among groups in the numbers of effective analgesia, pain scores, hourly local anesthetic amount used, sensory and motor blockade, labor duration and mode of delivery, side effects and maternal satisfaction (P>0.05). The relative median potency was bupivacaine/ropivacaine: 0.828 (0.602-1.091), bupivacaine/levobupivacaine: 0.845 (0.617-1.12), ropivacaine/levobupivacaine: 1.021 (0.774-1.354), respectively. However, a significantly less number of effective analgesia and higher hourly local anesthetic use were observed in the concentration of 0.05% than those of >or=0.1% within each group (P<0.05).

CONCLUSIONSUsing patient-controlled epidural analgesia, lower concentrations of bupivacaine, ropivacaine and levobupivacaine with sufentanil produce similar analgesia and motor block and safety for labor analgesia. The analgesic efficacy mainly depends on the concentration rather than the type of anesthetics.

Adult ; Amides ; therapeutic use ; Analgesia, Epidural ; methods ; Analgesia, Obstetrical ; methods ; Analgesia, Patient-Controlled ; methods ; Anesthetics, Local ; therapeutic use ; Bupivacaine ; analogs & derivatives ; therapeutic use ; Female ; Humans ; Labor Pain ; drug therapy ; Labor, Obstetric ; Pregnancy ; Sufentanil ; therapeutic use ; Young Adult

OBJECTIVETo investigate the mutation characteristics of ATP13A2 gene in Chinese patients with familial autosomal recessive early-onset parkinsonism (AREP).

METHODSMutations of ATP13A2 gene were screened by polymerase chain reaction combined with DNA direct sequencing in patients with familial AREP.

RESULTSNo pathogenic mutations in ATP13A2 gene were detected in this group. Six reported polymorphisms were identified. They were IVS6+70A>G, IVS12+66A>G, m.1849C>T, IVS20-56 G>A, m2671C>T and m2824G>A.

CONCLUSIONATP13A2 gene mutations may be rare in Chinese patients with familial autosomal recessive early-onset parkinsonism.

Adult ; Age of Onset ; Asian Continental Ancestry Group ; genetics ; Base Sequence ; China ; epidemiology ; DNA Mutational Analysis ; Female ; Humans ; Male ; Middle Aged ; Molecular Sequence Data ; Mutation ; Parkinsonian Disorders ; epidemiology ; genetics ; Pedigree ; Polymorphism, Genetic ; Proton-Translocating ATPases ; genetics

OBJECTIVETo establish a stable, accurate and intuitive method for detecting the CAG trinucleotide repeats of MJD1 gene.

METHODSThe CAG trinucleotide polymorphism of the MJD1 gene was analyzed by recombinant DNA technology and DNA sequencing in 35 spinocerebellar ataxia 3/Machado-Joseph disease (SCA3/MJD) patients from Mainland China.

RESULTSThe range of the CAG repeat of the 35 patients was 65-81 (mean = 72.96 +/- 4.24). The CAG repeats contained two CAAs and one AAG variations in the CAG motif in all the patients and majority of the healthy controls. There was a CGG/GGG polymorphism at the 3' end of the CAG repeat. The GGG allele was consistently associated with smaller CAG repeats in healthy controls. On the other hand, the CGG allele consistently existed in the patients.

CONCLUSIONRecombinant DNA technology can stably, accurately and intuitively detect the CAG trinucleotide repeat of the MJD1 gene. It should be used as a major technique to diagnose the SCA3/MJD and analyze the polymorphism of CAG sequence.

Adolescent ; Adult ; Ataxin-3 ; Base Sequence ; Female ; Genetic Engineering ; methods ; Humans ; Machado-Joseph Disease ; genetics ; Male ; Middle Aged ; Molecular Sequence Data ; Nerve Tissue Proteins ; genetics ; Nuclear Proteins ; genetics ; Polymorphism, Genetic ; Repressor Proteins ; genetics ; Sequence Analysis, DNA ; Trinucleotide Repeats ; Young Adult

Objective To explore the status, characteristics and factors in relation to occupational stress for medical staffs in tertiary general hospitals. Methods A total of 2460 medical staff were sampled in five tertiary general hospitals in Beijing, with their occupational stress levels evaluated with the Occupational Stress Inventory. Results The top ten stressors as found ranked as heavy duty, high risk exposure, high workload, low wages, setbacks in the health care management system, insufficient staffing, excessively frequent inspections and examinations, strained doctor-patient relationship, price inflation, frequent overtime, and pressure from continuous learning. Occupational stress is seen as moderate and above by 95.2% of the surveyed. Differences in age, gender, marital status, professional title, education, work experience, as well as those of different organizations, departments, professions, and duty were found to be statistically significant in regard of professional stress. Conclusions Stress management should be in place targeting demographic and stress characteristics. Effective measures are recommended to alleviate the pressure on medical staff, in order to maintain their physical and mental health, hence improving their work efficiency and organizational cohesion.

Adult ; Asian Continental Ancestry Group ; DNA Mutational Analysis ; Female ; Humans ; Male ; Middle Aged ; Parkinsonian Disorders ; genetics ; Pedigree ; Proton-Translocating ATPases ; genetics ; Young Adult

Adolescent ; Adult ; Aged ; Female ; Humans ; Kidney Failure, Chronic ; mortality ; Male ; Middle Aged ; Prognosis ; Renal Replacement Therapy ; Retrospective Studies ; Survival Rate ; Vasculitis ; complications ; mortality

Objective To determine a stable, exact and direct detection method for expanded nucleotide repeat sequences of the causative gene in patients with hereditary spinocerebellar ataxias (SCAs).Methods Quantitative fluorescent-polymerase chain reaction,8%denaturing polyacrylamide gel electrophoresis(PAGE),capillary electrophoresis(CE)and recombinant DNA technology by direct sequencing technique were employed to detect the CAG-repeat numbers of abnormal allele in 50 patients diagnosed as having SCA3/MJD.And then,the differences of CAG repeat numbers detected by CE and recombinant DNA technology were statistically analyzed.Results Of the 50 patients with SCA3/MJD detected by 8%denaturing PAGE,the expanded CAG repeat numbers measured by CE and recombinant DNA technology ranged from 63 to 74(69.56±2.12)and from 67 to 80(73.72±3.29),respectively.Significantly decreasedtendency was showed in the mean CAG repeat numbers of 69.56±2.12 using CE as compared with that in those of 73.72±3.29 using recombinant DNA technology,(t=-9.61,P=0.000). Conclusion Denaturing PAGE and CE can be used as preliminary screening for nucleotide repeat numbers,while the exact numbers depend on the recombinant DNA and direct sequencing technologies.Recombinant DNA technology combined with direct sequencing is a predominant,stable,exact and direct method to detect the repeat numbers of SCAs and analyze the polymorphism of nucleotide sequence.

OBJECTIVETo report a Chinese Charcot-Marie-Tooth disease type 2 (CMT2) family.

METHODSAll the members in the family were studied clinically,and 6 patients were studied electrophysiologically. Sural nerve biopsy was performed in the proband. PMP22 gene duplications were detected by highly polymorphic short tandem repeat. Point mutation analysis of PMP22, MPZ and NEFL gene was screened by PCR-SSCP combined with DNA direct sequencing. A genome-wide screening was carried out to the family.

RESULTExcept 2 who had weakness and atrophy in both proximal and distal muscles of the lower limbs, all patients presented muscle wasting and a predominating weakness of distal parts of the lower limbs, and mild to moderate sensory impairments. In 6 patients who were subjected to elctrophysiological examinations, median-nerve conduction velocity (NCV) of the median nerve was normal. Electromyograms (EMGs) revealed signs of denervation with large motor unit potentials, fibrillation potentials and positive sharp waves. Sural nerve biopsy of the proband confirmed the presence of axonal neuropathy with an important loss of large myelinating fibers and a large number of clusters with mostly thinly myelinated axons. PMP22, MPZ and NEFL gene mutations were not found. The results of genome-wide screening revealed a linkage of CMT2 to a locus at chromosome 12q24.

CONCLUSIONThe results are consistent with the diagnosis of CMT2. This family represents a rare genetic type of CMT2 which can be designated as CMT2L.

Adolescent ; Adult ; Asian Continental Ancestry Group ; Charcot-Marie-Tooth Disease ; genetics ; pathology ; Chromosomes, Human, Pair 12 ; genetics ; Electromyography ; Female ; Humans ; Male ; Pedigree

OBJECTIVETo assist the establishment of platform and provide the reference standard for mutation detection in spinocerebellar ataxia (SCA) subtypes 1, 2, 3, 6, 7, 8, 10, 12, 17 and dentatorubral-pallidoluysian atrophy (DRPLA) in Chinese Han population.

METHODSThe nucleotide repeat numbers of the 9 SCA subtypes and DRPLA were detected using fluorescence-PCR and capillary gel electrophoresis technique in 300 healthy Chinese Han individuals.

RESULTSAmong the 300 healthy controls, the range of the CAG trinucleotide repeat number was 17 to 35 in SCA1, 14-28 in SCA2, 13-41 in SCA3/MJD, 4-16 in SCA6, 5-17 in SCA7, 5-21 in SCA12, 23-41 in SCA17, and 12-33 in DRPLA; and the range of CTA/CTG trinucleotide repeat number on SCA8 locus was 12-43 and the range of ATTCT pentanucleotide repeat number on SCA10 locus was 9-32. Of which, the 12 CTA/CTG repeats of SCA8, 9 ATTCT repeats of SCA10, 23 CAG repeats of SCA17 were the shortest normal repeat number, while the 41 CAG repeats of SCA3/MJD, 32 CAG repeats of SCA10 were the largest normal number that have not been reported.

CONCLUSIONThe normal ranges of polynucleotide repeats of different subtypes of SCA vary with geographical areas and ethnicities. It might be associated with the genetic and ethnic backgrounds. This is the first normal reference standard of polynucleotide repeat number of these ten SCA subtypes in Chinese Han.

Adult ; Asian Continental Ancestry Group ; ethnology ; genetics ; Base Sequence ; Case-Control Studies ; Humans ; Male ; Middle Aged ; Molecular Sequence Data ; Myoclonic Epilepsies, Progressive ; ethnology ; genetics ; Spinocerebellar Ataxias ; ethnology ; genetics ; Trinucleotide Repeat Expansion

OBJECTIVETo study the audiological characteristics of newborns and infants who failed hearing screening.

METHODSOne hundred and six infants failed hearing screening received follow-up study with routine audiological evaluations (auditory brainstem response, distortion product otoacoustic emission, tympanometry and visual reinforcement audiometry).

RESULTSSixty-five infants (61.3%) of this group were normal hearing subjects and 39(36. 8% ) of the infants had hearing loss. Two cases (1.9%) received follow-up by phone. Fifteen cases (14.2%) with conductive hearing loss and 24 cases (22.6%) with sensorineural hearing loss. Thirteen (12.3%), 14 (13.2%), 6 (5.7%), and 6 (5.7%) cases were found to be mild, moderate, severe and profound hearing loss respectively. Diagnosis of hearing loss in the thirty-nine infants conducted a prevalence of 0.264% (39/14 785) of congenital hearing loss (both binaural and monaural). The hearing level of those cases with severe and profound hearing loss basically did not change, but that of cases with mild and moderate hearing loss changed.

CONCLUSIONSEarly identification and intervention of infants with severe and profound hearing loss by 6 months of age were successful. Infants with mild and moderate hearing loss should be followed up to six or eight months and received routine audiologic evaluations.

Audiometry, Evoked Response ; China ; Evoked Potentials, Auditory, Brain Stem ; Female ; Follow-Up Studies ; Hearing Loss ; diagnosis ; epidemiology ; Humans ; Infant ; Infant, Newborn ; Male ; Neonatal Screening ; Prevalence