Objective To report the development of clinical symptoms in a Chinese family with autosomal dominant progressive external ophthalmoplegia(adPEO).Methods Electromyologram and muscle biopsy were performed in the proband and 4 family members with the disease.Results The proband was a 57 year-old woman,who developed bilateral ptosis after the age of 30,external ophthalmoplegia after the age of 35 years old,weakness of extremities at the age of 37 years old and bulb palsy with palmus at the age of 47 years old.In the family there were 20 male and female members from five generations.All of them complained about bilateral ptosis between 26—33 years old,external ophthalmoplegia(12/15)and weakness of all extremities(14/15)between 35—45,facial and masticatory weakness(9/9)as well as dysphagia(8/9)between 44—60,accompanied with heart lesions(4/7)after 50 years old.Some patients died due to cardiac impairment.Electromyologram showed myopathic abnormalities in the examined patients. The main myopathological changes were ragged red fibers,cytochrome c oxidase negative fibers and ragged blue fibers in succinate dehydrogenase staining.Conclusions The adPEO started from extra-ocular muscles to limbs,finally facial and bulbar muscles.Heart lesions were presented in late stage and lead to death in some members.The developing process of symptoms suggested that we should pay more attention to cardiac manifestations in this disease.

Diagnostic Errors ; Epilepsy ; diagnosis ; Ethylene Dichlorides ; poisoning ; Humans ; Male ; Young Adult

The UHPLC-LTQ-Orbitrap high resolution mass spectrometer was used to explore the chemical compositions in safflower. The rapid separation of the compositions was conducted by the UHPLC, following by high resolution full scan and MS2 scan, under the positive and negative ion mode. The chemical formula of compositions were deduced by full scan data in less than 5, then the potential structures were confirmed by the MS2 data. Forty-nine compounds were detected, of which 26 was identified, and 5 compounds was validated by the standard substances.
Carthamus tinctorius ; chemistry ; Chromatography, High Pressure Liquid ; methods ; Drugs, Chinese Herbal ; chemistry ; isolation & purification ; Molecular Structure ; Tandem Mass Spectrometry ; methods

Objective To analysis the clinical manifestations of mtDNA A3243G mutation in adulthood.Methods The clinical features were investigated in 36 cases (28 patients from 5 families with the mutation and 8 sporadic cases),in whom mtDNA A3243G mutation was confirmed genetically in 23 cases (15 cases from 5 mutation families and 8 sporadic cases).Cranium radiology was performed in 14 cases.Muscal biopsies were performed in l0 cases.Results Among 28 cases in the 5 family,there were 9 cases (32.1%) with stroke like episodes,17 cases (60.7%) with diabetic mellitus and 16 cases (57.1%) with deafness.Such symptoms usually combined with each other and rarely existed alone. Cardiomyopathy and renal failure were uncommon.In the 23 cases with mtDNA A3243G mutation,14 cases (61.0%) had mitochondria] myopathy,encephalopathy,lactic acidosis,and stroke-like episodes (MELAS),mostly presenting cognitive abnormalities,dysarthria or aphasia and headache,3 cases (13.0%) were asymptomatic carriers,2 cases (8.7%) had autonomic dysfunction,2 cases (8.7%) had diabetic mellitus with or without nerve deafness,1 case (4.3%) had diabetic mellitus with infertilitas and cardiomyopathy,respectively.Cranial radiological images revealed the changes more commonly in the temporal and occipital lobes and less frequently in the frontal lobes.Ragged red fibers were confirmed in 9 of 10 cases with muscle biopsies.The proportion of mutant mtDNA A3243C was not significantly different between MEALS (28.75%?13.69%) and non-MELAS (25.08%?11.54%).Conclusions mtDNA A3243G mutation mainly results in the lesions in the central nerve system,pancreatic island and acoustic nerve in adulthood.Heart and kidney are less frequently involved.Cognitive abnormalities,aphasia and headache are the major symptoms of adult MELAS.Families have with more than 1 patient with diabetic mellitus and deafness,indicating that the mutation is other than MELAS mutation.We should pay more attention to the non-MELAS symptoms in the families with mtDNA A3243G mutation.

Objective To investigate the axonal lesion in chronic inflammatory demyelinating polyneuropathy(CIDP).Methods Eighteen patients had undergone sural nerve biopsy.The clinical and electrophysiological distinction based on the different pathological changes were analyzed.Results Five patients with demyelination predominance which presented myelinated fiber with thin myelin.Three of them showed also mild axonal degeneration.Eight patients with axonal lesion predominance which presented Wallerian degeneration and regeneration of myelinated fibers.Three patients with mixed myelin and axon lesion of myelinated fibers and two with mild lesion.There was no significant difference between CIDP predominantly with axonal lesion and demyelination.Electrophysiological examination shows both axonal lesion and demyelination feature in some of the 2 types patients at the same time.Conclusions Axonal lesion is a common pathological change in CIDP and should not be considered as an exclusive criterion in diagnosis of the disease.Infiltration of macrophages is a common change.

OBJECTIVETo observe the impact of tonifying Qi traditional Chinese medicines contained in Yiqi Qingwen Jiedu mixture on mRNA expression of lung inflammatory cytokines and pulmonary pathological injury of mice infected by influenza virus, in order to discuss the mechanism of tonifying Qi traditional Chinese medicines against pulmonary immune inflammatory injury of infected mice.

METHODIn different time phases after mice were infected with influenza virus FM1, the RT-PCR method was adopted to observe the impact of tonifying Qi traditional Chinese medicines contained in Yiqi Qingwen Jiedu mixture on five inflammatory cytokines TNF-α, IL-1, IL-6, IL-10 and IFN-γ, and the changes in pulmonary pathological injury of mice with viral pneumonia after intervention with tonifying qi traditional Chinese medicines.

RESULT(1) Tonifying Qi traditional Chinese medicines significantly reduced the mRNA expression of TNF-α at 1-5 d and IL-1 mRNA expression at 7 d, may increase IL-1 mRNA expression in mouse lung at 3 d, significantly reduced IL-6 mRNA expression in mouse lung and increased IL-10 mRNA expression at 3-7 d, and significantly increased IFN-γ mRNA expression at 1 d. (2) Tonifying Qi traditional Chinese medicines could significantly inhibited and repaired pulmonary immune inflammatory injury of mice infected by FM1, which was most remarkable at 3-7 d after the infection with influenza virus FM1.

CONCLUSIONTonifying Qi traditional Chinese medicines contained in Yiqi Qingwen Jiedu mixture could resist pulmonary immune inflammatory injury and repair inflammatory injury by regulating the mRNA expression of imbalance inflammatory cytokines of organisms infected with influenza virus.

Animals ; Drugs, Chinese Herbal ; administration & dosage ; Humans ; Influenza A virus ; drug effects ; immunology ; Influenza, Human ; drug therapy ; genetics ; immunology ; Interferon-gamma ; genetics ; immunology ; Interleukin-1 ; genetics ; immunology ; Interleukin-10 ; genetics ; immunology ; Interleukin-6 ; genetics ; immunology ; Lung ; immunology ; virology ; Male ; Mice ; Mice, Inbred BALB C ; Tumor Necrosis Factor-alpha ; genetics ; immunology

OBJECTIVETo establish a method to determine underivatized endogenous amino acids in brain tissues after cerebral ischemia based on RRLC-QQQ.

METHODDiamonsil chromatographic column C18 (4.6 mm x 250 mm, 5 microm) was adopted to determine 12 amino acids in 15 min, with acetonitrile-0.1% formic acid for gradient elution. The flow rate was set at 0.5 mL x min(-1). With ESI as the ion source, positive ion scanning mode was adopted for multi-reaction monitoring.

RESULTEach amino acid standard curve (AAs) showed good linear relationship within the detection range (r > 0.996), with the limit of detection of less than 11%, the limit of quantitation of less than 3.09 microg x L(-1). The RSD of intra- and inter-day precisions at high, middle and low concentrations were less than 11%.

CONCLUSIONThe determination results of actual samples showed that compared with the levels of AAs of the sham operation group, all of the remaining amino acids apart from N-acetyl-aspartate increased in brain tissues. Some amino acids showed significant changes in a time-dependent manner after the operation. The method is so simple, rapid and sensitive that it can be used for finding biological metabolite markers of cerebral ischemia, and exploring cerebral ischemia molecular mechanisms and synergistic mechanism of combined administration of multi-component traditional Chinese medicines.

Amino Acids ; metabolism ; Animals ; Brain ; metabolism ; Brain Ischemia ; metabolism ; Chromatography, High Pressure Liquid ; methods ; Male ; Rats ; Rats, Sprague-Dawley ; Reproducibility of Results ; Tandem Mass Spectrometry ; methods

To study the chemical constituents of Alchornea trewioides, silica gel column chromatography, Sephadex LH-20, reverse phase ODS column chromatography, MCI and semi-preparative HPLC were used to separate the 95% EtOH extract of the root of Alchornea trewioides. The structures were elucidated on the basis of spectroscopic studies including ESI-TOF-MS, 1H NMR, 13C NMR, HSQC and HMBC. Eight phenolic acids were obtained and identified as 1-O-galloyl-6-O-vanilloyl-beta-glucose (1), gallic acid (2), ethyl gallate (3), syringic acid (4), glucosyringic acid (5), erigeside C (6), 3, 4-dimethoxyphenyl-(6'-O-alpha-L-rhamnosyl)-beta-D-glucopyranoside (7) and 3, 4, 5-trimethoxyphenyl-(6'-O-galloyl)-O-beta-D-glucopyranoside (8). Among them, compound 1 is a new compound, compounds 4-8 are isolated from the genus Alchornea for the first time, and the others are isolated from the plant for the first time.
Benzoates ; chemistry ; isolation & purification ; Euphorbiaceae ; chemistry ; Gallic Acid ; analogs & derivatives ; chemistry ; isolation & purification ; Glucose ; analogs & derivatives ; chemistry ; isolation & purification ; Glucosides ; chemistry ; isolation & purification ; Hydroxybenzoates ; chemistry ; isolation & purification ; Molecular Structure ; Phenols ; chemistry ; isolation & purification ; Plant Roots ; chemistry ; Plants, Medicinal ; chemistry

OBJECTIVETo investigate chemical constituents from folk herb Pilea cavaleriei subsp. cavaleriei.

METHODThe compounds were separated and purified by silica gel, Sephadex LH-20 and the like. The structures were identified by spectral methods such as (1)H, (13)C-NMR and MS.

RESULTSeventeen compounds were isolated and identified as benzoic acid (1), 4-hydroxy benzalde-hyde (2), coumaric acid(3), protocatechuic acid (4), gallic acid (5), 4-hydroxy benzoic acid (6), 3-indole carboxaldehyde (7), 3-indole carbo-xylicacid (8), 4-methyl-(1,2,3) -triazole(9), uracil(10), nicotinamide (11), (2S,E)-N-[2-hydroxy-2-(4-hydroxy phenyl) ethyl] ferulamide (12), (+) -dehydrovomifoliol (13), hentriantane (14), beta-sitosterol (15), palmitic acid (16), daucossterol (17) , respectively.

CONCLUSIONAll compounds were obtained from the genus for the first time.

Dextrans ; chemistry ; isolation & purification ; Drugs, Chinese Herbal ; chemistry ; isolation & purification ; Molecular Structure ; Sitosterols ; chemistry ; isolation & purification ; Urticaceae ; chemistry

OBJECTIVETo investigate the possibility and the limit in increasing the survival area of the random skin flap by extremely increasing the ratio of its length and width within 24 hours.

METHODSSD rats (n = 20) were chosen for this study. The rats were randomly divided into: subject group and control one. Pre-made skin flap was prepared as design. The subject group was carried out rapid pre-fabricated skin flap formation training. No training was performed in control group. The changes in perfusion value of micro-circulation inside skin flap were monitored during the whole process, and micro-circulation parameters of the skin flap were used to evaluate whether its blood circulation network was mature or not.

RESULTSTraining of pre-made skin flap at 18th hour, the perfusion value of its micro-circulation was basically stable, Skin flap formation was finished at 24th hour. Survival area in control group was (68.25 +/- 0.18)% and in subject group was (97.25 +/- 0.24)% (P < 0.01). There was a significant difference between the two groups.

CONCLUSIONSWithin short time, it is possible to establish micro-circulation in skin flap which exceeds the limit set by traditional theory. Digitalized judgment can be used to monitor the fast formation of super-big skin flap. This method is reliable and can increase the survival rate of random skin flap.

Animals ; Female ; Male ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Skin ; blood supply ; metabolism ; Skin Transplantation ; Surgical Flaps ; Time Factors