Chromosome Aberrations ; Cytogenetic Analysis ; standards ; Humans ; Multiple Myeloma ; diagnosis ; genetics

Objective To evaluate the efficacy and safety of near-infrared light combined with tacrolimus 0.1％ ointment in the treatment of facial glucocorticoid-dependent dermatitis.Methods A total of 68 patients with facial glucocorticoid-dependent dermatitis were enrolled from Department of Dermatology of Yantai Yuhuangding Hospital between December 2014 and December 2015,and randomly and equally divided into treatment group and combination group by a random number table.The treatment group was treated topically with tacrolimus ointment twice a day for 4 weeks.Besides the treatment with tacrolimus ointment,the combination group was irradiated with near-infrared light once a week for 4 sessions.After 4-week treatment,improvement in clinical manifestations such as itching and burning sensation was evaluated,so was the therapeutic effect.Results The combination group showed significantly higher response rate (85.3％ [29/34]) compared with the treatment group (61.8％ [21/34],x2 =4.84,P ＜ 0.05).Additionally,response rates for itching and burning sensation,erythema,scales and papules were all significantly higher in the combination group than in the treatment group (x2 =4.84,6.35,8.42 and 5.52,respectively,all P ＜ 0.05).Conclusion Near-infrared light combined with tacrolimus ointment is effective and safe for the treatment of facial glucocorticoid-dependent dermatitis.

Objective To study the correlation between the electrophysiological features and mechanical ventilation and long?term outcome in Guil?lain?Barré syndrome(GBS)patients. Methods Electrophysiological and clinical data were retrospectively collected,and compared between venti?lated and not ventilated patients,as well as among each subtype of GBS. Results Totally 38 GBS patients were included in the study,among which 12(32%)were ventilated. The p/d CMAP ratio of the common peroneal nerve was significantly lower in ventilated group compared to not ventilated group(48.7±15.3 vs 80.8±24.0,P=0.005). AIDP was the most common subtype in ventilated patients compared with AMAN and undetermined (46%vs 0%and 9%,P=0.027). The long?term outcome score of AMAN was significantly lower than AIDP(4.3±1.3 vs 2.5±0.9,P=0.028). Conclusion Electrophysiological testing was predictive for mechanical ventilation and long?term outcome:low p/d CMAP ratio of the common pero?neal nerve was helpful for predicting mechanical ventilation,AIDP was prone to develop respiratory failure and had a worse outcome compared to AMAN.

Objective To retrospectively study the clinical and electrodiagnostic features in subacute combined degeneration of spinal cord (SCD)patients. Methods The clinical and electrodiagnostic recordings of all SCD patients conformed in our Neurology Department from Janu?ary 2013 to July 2015 were retrospectively reviewed. Totally 29 patients diagnosed as SCD,whom received no treatment before admitted in our hos?pital,were recruited for the study. Correlation analysis between serum level of vitamin B12(VB12),course of disease and degree of neural lesion was performed,and the electrodiagnostic features were summarized and analyzed. Results The course of disease had significant correlation with the degree of neural lesion(P=0.001),but there was no significant relevance between serum VB12 level and neural damage(P>0.05). Electrodi?agnostic examination revealed:The abnormal rate of motor nerve(15.20%)was lower than that of sensory nerve(42.75%,P<0.001)and the up?per limb nerve(27.1%)was lower than that of lower limb nerve(30.87%,P=0.578). There was no remarkable difference of abnormal rate in all kinds of nerves of motor and sensory nerve(P1=0.320,P2=0.755). In the analysis of the parameters of electromyograph,there was statistical signif?icance of the abnormal rates of compound muscle action potential(CAMP)between each motor nerve(P=0.005). There was statistical signifi?cance of the abnormal rates of CAMP and no function between each sensory nerve(all P<0.001). Conclusion The course of disease has signifi?cant correlation with the degree of neural lesion. Sensory nerve is tended to be betreffend than motor nerve. Phil.always nervous are more likely to be affected than others in motor nerve at CAMP. Tibial nerve is easier to be influenced at CAMP in sensory nerve ,and the lesion is more serious.

Objective To explore the effects of PDCA-based self-management intervention model on health behavior and medication adherence in aged patients with percutaneous coronary intervention (PCI). Methods Totally 130 aged patients treated by PCI were randomly divided into the intervention group and the control group with 65 patients. The patients in the control group received routine health education, and the patients in the intervention group received PDCA-based self-management intervention model. All patients were investigated with Coronary Heart Disease Self-Management Behavior Scale (CSMS) and Health Promoting Lifestyle Profile (HPLP) and Medication Compliance Scale (MMAS-8) 3 months and 6 months after discharge. Results Six months after discharge, the score of self-management and healthy behavior and medication adherence were 96.98 ± 14.12, 131.86 ± 16.53, 7.18 ± 0.69 respectively in the intervention group, and the score of them were 86.04 ± 11.78, 105.33 ± 10.97, 5.69 ± 1.29 respectively in the control group, and the difference was statistically significant (t=10.981, 10.793, 7.438, P<0.05 or 0.01). Conclusions PDCA-based self-management intervention model is a patient-centered, problem-oriented, dynamic and interactive health education intervention. It may be helpful in improving PCI patients′ health behavior and medication adherence after discharge. And it may establish lasting self-management skills, and is worthy of application and promotion.

OBJECTIVETo investigate changes in the adherent ability, the expression of adhesion related proteins Pyk2 and paxillin during HL-60 cells differentiation into granulocyte-monocyte induced by low-dose (LD) bufalin in combination with all-trans retinoic acid (ATRA), and to explore the effects of bortezomib on cellular adhesion and the expression of Pyk2 and paxillin.

METHODSThe expression of CD11b was detected by flow cytometry, cellular adherence ability by MTT assay, and the expressions of Pyk2, paxillin and tubulin by Western blot.

RESULTSThe combination of 5 nmol/L bufalin and 30 nmol/L ATRA induced HL-60 cells differentiation in a time-dependent manner, the percentages of CD11b positive cells treated for 2 d and 4 d being (20.0 +/- 2.8)% and (75.0 +/- 5.3)%, respectively, with the increasing of cellular adherence ability. Meanwhile the expressions of Pyk2 and Paxillin were also up-regulated in a time-dependent manner. Bortezomib suppressed HL-60 cell adhesion in a dose-dependent manner. At concentrations of 1 nmol/L and 10 nmol/L the adherence level were (7.8 +/- 0.1)% and (5.3 +/- 0.3)%, respectively, with down-regulation of Pyk2 but not Paxillin.

CONCLUSIONPyk2 is involved in the regulation of cellular adherence function. Bortezomib might inhibit HL-60 cells adhension function by down-regulation of Pyk2 expression.

Boronic Acids ; pharmacology ; Bortezomib ; Bufanolides ; pharmacology ; Cell Adhesion ; drug effects ; Cell Proliferation ; drug effects ; Focal Adhesion Kinase 2 ; metabolism ; HL-60 Cells ; Humans ; Paxillin ; metabolism ; Pyrazines ; pharmacology ; Tretinoin ; pharmacology

Cells, Cultured ; Chemokine CCL4 ; Chemotaxis, Leukocyte ; drug effects ; Endothelial Cells ; cytology ; metabolism ; Homocysteine ; pharmacology ; Humans ; Macrophage Inflammatory Proteins ; biosynthesis ; genetics ; Monocytes ; physiology ; RNA, Messenger ; biosynthesis ; genetics ; Umbilical Veins ; cytology

Background Leber hereditary optic neuropathy (LHON)is a common inherited eye disease,which generally affects young adults with bilateral loss of central vision.Mutation frequency of Leber hereditary has not been fully clarified. Objective This study was to investigate the mutation frequency of mitochondrial NDI gene associated with LHON in Chinese population. Methods The proposal of the study was approved by Ethic Committee of Wenzhou Medical College.Written informed consent was obtained from each subject initial of this trial.Eight hundred and ninety-four LHON patients and 134 normal subjects were collected.Genomic DNA was extracted from peripheral blood leukocytes of the all participants.Polymerase chain reaction (PCR) was used to amplify and sequence analysis of the mitochondrial ND1 gene was performed and aligned with revised Cambridge Reference Sequence(rCRS) of mitochondrial DNA.Then mutated gene frequency was screened and analyzed. Results Mutational analysis of mitochondrial ND1 gene in 894 LHON patients revealed the presence of G3316A,T3394C,G3460A,C3497T,G3635A,G3733A,and T4216C.11.19％ LHON patients (100/894 ) were found to be associated with the gene mutations mentioned above,and 3.24％ patients (29/894) showed the co-occurrence of three primary mutations.Mutation frequencies in LHON patients were 2.57％,2.23％,1.45％,3.80％,0.67％,0.11％,0.34％,respectively,and G3316A,T3394C,C3497T and T4216C also were detected in 134 normal controls with the mutation frequencies of 4.48％,2.99％,4.48％ and 1.49％,respectively.Mutation frequency analysis showed an insignificant difference in the mutations of G3316A,T3394C,C3497T and T4216C between LHON patients and normal controls (x2 =0.926,P=0.336;x2 =0.052,P=0.820; x2 =0.142,P=0.707;P=0.129).G3376A,G3496T,G3700A,A4136G,T4160C and C4171A were absent in Chinese LHON patients. Conclusions Mitoehondrial ND1 gene in LHON is a mutational hotspot in Chinese population,11.19％ (100/894)associated with LHON was caused by ND1 gene mutation.G3635A,G3733A may be rare pathological mutation in Chinese population.However,G3316A,T3394C,C3497T and T4216C are insufficient to produce the clinical phenotype,but they may play a synergic role for penetrance and phenotypic manifestation in LHON.

BACKGROUNDMutations in fumarylacetoacetate hydrolase (FAH) gene can lead to tyrosinemia type 1 (HT1), a relatively rare autosomal recessive disorder. To date, no molecular genetic defects of HT1 in China have been described. We investigated a Chinese family with a HT1 child to identify mutations in FAH.

METHODSDNA sequencing was used for mutations screening in FAH gene. Real-time polymerase chain reaction (PCR) was performed to determine the FAH gene expression level. To confirm the presence of degradation by the nonsense-mediated mRNA decay pathway (NMD), the fragments containing R237X mutations were analyzed by primer introduced restriction analysis-polymerase chain reaction (PIRA-PCR) and cDNA sequencing. Finally, the effects of the mutations reported in this study were predicted by online softwares.

RESULTSA boy aged 3 years and 8 months was diagnosed clinically with HT1 based on his manifestations and biochemical abnormalities. Screening of FAH gene revealed two heterozygous mutations R237X and L375P transmitted from his mother and father respectively. In this pedigree, the amount of FAH mRNA relative to a healthy control was 0.44 for the patient, 0.77 for his mother and 1.07 for his father. Moreover, both PIRA-PCR and cDNA sequencing showed significant reduction of the FAH mRNA with R237X nonsense mutation. The missense mutation of L375P was not reported previously and prediction software showed that this mutation decreased the stability of protein structure and affected protein function.

CONCLUSIONSThis is the first case of HT1 analyzed by molecular genetics in China. The R237X mutation in FAH down- regulates the FAH gene expression, and the L375P mutation perhaps interrupts the secondary structure of FAH protein.

Child, Preschool ; China ; Humans ; Hydrolases ; genetics ; Male ; Molecular Sequence Data ; Mutation ; Mutation, Missense ; genetics ; Nonsense Mediated mRNA Decay ; genetics ; Real-Time Polymerase Chain Reaction ; Tyrosinemias ; genetics

OBJECTIVETo verify the clinical efficacy of acupuncture combined with auricle cutting method for treatment of blood stasis-type psoriasis.

METHODSFifty-six cases of blood stasis-type psoriasis were randomly divided into a combined therapy group, a auricle cutting group, an acupuncture group and a control group, 14 cases in each one. Based on regular treatment of TCM decoction in four groups, the combined therapy group was treated with acupuncture and auricle cutting method, and the auricle cutting group was treated with sham-acupuncture and auricle cutting, and the acupuncture group was treated with acupuncture and sham auricle cutting, and the control group was treated with sham-acupuncture and sham auricle cutting. The acupuncture was applied at Dazhui (GV 14), Feishu (BL 13), Ganshu (BL 18) and Geshu (BL 17), etc., and manipulated with routine technique; in the sham acupuncture, the needle was inserted into dermis layer so that the needles could be swung without being dropped out. In the auricle cutting, erbeixin (P1) of unilateral auricle was selected and cut by Chan needle to perform bloodletting; in the sham auricle cutting, the neighborhood approximately 0.5 cm next to erbeixin (P) of auricle was selected as cutting area. The treatment was given once a day, seven days as a treatment session for totally two sessions. Psoriasis area and severity index (PASI) before and after treatment was observed and efficacy of each group was compared.

RESULTSThe effective rate was 57.1% (8/14) in the combined therapy group, which was superior to 14.3% (2/14) in the auricle cutting group, 7.1% (1/14) in the acupuncture group and 0.0% (0/14) in the control group (all P < 0.05). The scores of PASI were all decreased in each group after the treatment (all P < 0.05), which was the most significant in the combined therapy group (all P < 0.05). After factorial analysis, the main effect was P < 0.05 in the auricle cutting, P < 0.05 in the acupuncture and P < 0.05 in interaction effect of combined therapy.

CONCLUSIONThe scores of PASI of blood stasis-type psoriasis could be effectively reduced by acupuncture, auricle cutting method and TCM decoction, among which the interaction effect of auricle cutting and acupuncture combined with TCM decoction is the most significant.

Acupuncture Therapy ; Adolescent ; Adult ; Aged ; Bloodletting ; Combined Modality Therapy ; Ear ; blood supply ; Female ; Humans ; Male ; Middle Aged ; Psoriasis ; blood ; therapy ; Treatment Outcome ; Young Adult