Objective To explore the effects of bioactive parts of Xiongma Decoction (parts of ethyl acetate and n-butanol extract) on the CGRP-CRLR/RAMP1 signal pathway so as to clarify its therapeutic mechanism on migraine.Methods We randomly divided 36 male SD rats into 6 groups with 6 in each:blank group,model group,groups of low-,medium-and high-dose Xiongma Decoction bioactive parts,and Sumatriptan group.By giving hypodermic injection of 10 mg/kg nitroglycerin,the migraine rat model was copied;Only 18 rats were found to have positive expressions of CGRP,CRLR,and RAMP1 in TCC with immunohistochemistry staining after heart perfusion.For the remaining 18 rats,TCC was stripped directly from the whole brain and divided into two parts,one part used to detect CGRP,CRLR,RAMP1 mRNA expressions by qPCR,and the other part to detect CGRP,CRLR,RAMP1 protein expressions by Western blot.Results The number of CGRP,CRLR and RAMP1 immunoreactive cells,the mRNA and protein expressions on TCC in model group were effectively increased,compared with those in the blank group (P＜0.05),indicating that the model copying was successful.Compared with those in the model group,the number of CGRP,CRLR and RAMP1 immunoreactive cells in Xiongma Decoction bioactive parts was significantlv decreased,and the expressions of CGRP,CRLR and RAMP1 mRNA and protein were reduced (P＜0.05).Conclusion The bioactive parts of Xiongma Decoction can reduce the activity of CGRP-CRLR/RAMP1 signal pathway in TCC of migraine rats.

AIM: To observe the therapeutic action of Guizhifuling Capsule (Ramuluo Cinnamomi, Poria, Cortex moutan, Semen Persicae, etc.)on hyperplasia of mammary glands in rast, and to explore the new therapeutic effectiveness and its mechanism. METHODS: To inject extrinsic E2 and Pt into muscle to reproduce rats model of hyperplasia of mammary glands, to observe the morphologic changes of nipple in every group, determine extradiol and progesterone content on plasma in experimental rats by radio immunoassay, and study the changes of ER and PR by immunohistochemical method. RESULTS: Compared with the model group, Guizhi Fuling Capsule can markedly abate papilledema, proliferation of lobule, acinus and duct in mammary glands, reduce obviously changes of blood, estradiol on plasma, the ER and PR content of mammary glands and increase progesterone. CONCLUSION:Cuizhi Fuling Capsules have the good therapeutic action on hyperplasia of mammary glands in rats, its mechanism is related to correcting changes of blood, adjusting the blood concentration of sexual hormone in experimental rats and its expression of receptor.

According to the analysis regarding the basic researches and clinical literature of moxibustion smoke in recent ten years, it was found the low-concentration moxibustion smoke had unique application values for air disinfection, infectious diseases and skin diseases, etc. However, certain chemical components of long-time high-concentration moxibustion smoke could cause toxic and adverse effects on respiratory system, immune system and circulation system. Therefore, the safety effects of moxibustion smoke were summarized, hoping to provide references for further clinical researches and evidence-based medicine.

Objective To explore the role of transvertebral transposition of the spinal cord in the treatment of the pa?tients suffering from angular kyphoscoliosis with neurologic deficit. Methods Twelve patients with at least 1 year follow?up, in?cluding 5 females and 7 males, suffering from neurologic deficit secondary to angular kyphoscoliosis underwent transvertebral transposition of the spinal cord and posterior correction from January 2005 to June 2013 were retrospectively analyzed. The initial age averaged 21±14 years. The etiologies of deformity were congenital kyphoscoliosis in 9 patients, idiopathic kyphoscoliosis in 3 patients. Preoperative spinal cord function graded by Frankel criteria was 3 cases of grade C and 9 of grade D. The initial major curve was 79.4°±16.2° with a global kyphosis of 68.3°±25.0°. The change of distance between spinal cord and canal on the convex side at the apex was measured on MR images to evaluate the efficacy of transposition;in addition, Cobb angle on coronal and sagit?tal plane was evaluated before surgery, on discharge and at the last follow?up. Results All patients were followed up for 2 years on average (1-5 years). The post operation spinal function evaluated by Frankel criteria were as follow:with grade C of 1 case, grade D of 4 cases and grade E of 7 (7 cases changed one grade, 1 case changed two grades, 4 cases did not change obvious). At the last follow?up, all the cases got obvious recovery of spinal function, with 1 case of grade C and 11 of grade D (10 cases changed one grade, 2 cases changed two grades). The distance between spinal cord and canal on the convex side at the apex changed from 11.9±6.4 mm to 9.8±6.9 mm. After surgery, the major curve improved to 39.3°±15.1° with 50.3%±20.5%correction rate, and the mean loss of correction was 6.1%±7.5%. The mean kyphosis was 41.3°±16.6°, demonstrating a 39.0%±13.0%correction rate im?mediately after operation, and showing 3.1%±4.2%correction loss at the final follow?up. No neurologic complications or no death occurred intra?and post?operatively. No screw or rod fracture, instrumentation loosening and pseudarthrosis were found during fol?low?up. Conclusion Transvertebral transposition of the spinal cord and posterior correction is a useful procedure for the recovery of neurologic deficit secondary to angular kyphoscoliosis, with good results of deformity correction.

OBJECTIVE:To establish the HPLC fingerprints for Huobahuagen tablets intermediate. METHODS:HPLC was per-formed on Inertsil ODS-4 column with mobile phase consisted of acetonitrile-0.1% phosphoric acid (gradient elution) at the flow rate of 0.75 mL/min. The detection wavelength was set at 220 nm,and column temperature was 30 ℃. The sample size was 10 μL. Using wilforgine as reference,HPLC chromatograms of 10 batches of samples were determined. Common peak identification and similarity evaluation were performed by using Similarity Evaluation System for TCM Chromatographic Fingerprint (2004 A edi-tion). RESULTS:There were 25 common peaks in HPLC chromatograms of 10 batches of samples,and similarity degrees were higher than 0.9. After validated,HPLC chromatograms of 10 batches of samples were in good agreement with control fingerprints. CONCLUSIONS:The established fingerprint can provide reference for identification and quality evaluation of Huobahuagen tab-lets intermediate.

OBJECTIVETo assess the association of copy number variations of SMN1, SMN2, NAIP, GTF2H2 and H4F5 genes with clinical classification of spinal muscular atrophy in children, and determine the copy number of the SMN gene among pregnant women. A carrier screening was also performed in Sichuan province.

METHODSThe copy number variations of the above genes among 53 confirmed SMA patients were determined with MLPA technique. The copy number variations were analyzed by the Fisher's exact test. Deletion of exon 7 in the SMN1 gene was screened with denaturing high performance liquid chromatography (DHPLC) for 427 pregnant women.

RESULTSAmong the 53 cases of type I, II, and III SMA patients, the rate of homozygous deletion of both exons 7 and 8 of the SMN1 gene were 100%, 94.44% and 87.50%, respectively, whereas those of homozygous deletion of exon 7 of SMN1 gene were 0, 5.56%, and 12.50%, respectively. The patients with 1, 2, 3, and 4 copies of exon 7 of the SMN2 gene were 11.32%, 67.92%, 13.21% and 7.55%, respectively. The patients with 0, 1, and 2 copies of exon 5 of NAIP gene were 11.32%, 62.26%, and 26.42%, respectively. No deletion was detected in GTF2H2 or H4F5 genes. The heterozygous loss rate of exon 7 in SMN gene in the pregnant women population of Sichuan region was approximately 2.11%.

CONCLUSIONCopy number variations of SMN2 and NAIP genes in patients are related to SMA clinical types (P < 0.05). In contrast, there was no relationship between SMA clinical types and deletion of exons 7 and 8 in the SMN1 gene (P > 0.05). Analysis of copy number change in SMN1 gene can assist SMA carrier screening. However, when the general population without SMA family history is screened for disease-causing genes, it should be noted that the type "2+0" carriers may affect the screening result, and the result should be interpreted with caution.

Adolescent ; Child ; Child, Preschool ; DNA Copy Number Variations ; Female ; Genetic Carrier Screening ; Humans ; Infant ; Male ; Neuronal Apoptosis-Inhibitory Protein ; genetics ; Spinal Muscular Atrophies of Childhood ; genetics ; Survival of Motor Neuron 1 Protein ; genetics

Objective To survey the quality of life of disabled people in Shanghai Jing'an district and to analyze the influencing factors.Methods A face-to-face interview was conducted among 1 831 disabled people from 5 communities of Jing'an district between September 2015 and January 2016.The quality of life was evaluated with Modified Barthel Index (MBI),12-Item Short Form Health Survey (SF-12) and Patient Health Questionnaire (PHQ).Results There were 941 males and 870 females among 1 815 qualified participants.There were 433 cases of visual disability (23.86％),222 cases of hearing disability (12.23％),20 cases of language disability(1.10％),742 cases of limb disability (40.88％),169 cases of intelligent disability (9.31％),198 cases of mental disability (10.91％) and 31 cases (1.71％) of multiple disabilities.The SF-12 showed that the mean scores of both physical aspect and mental aspect were 50.0 ± 10.0.Multiple linear regression showed showed that the low self-care ability of daily life (β =0.24,P =0.00),depression (β =-9.65,P =0.00) and high serum lipids (β =-2.36,P =0.02) were associated with the quality of life.Conclusion The study indicates that the quality of life of the disabled people in Jing'an district is generally low,which is affected by various factors.

OBJECTIVETo detect potential mutations of fibrillin-1 (FBN1) gene in a child with Marfan syndrome (MFS) and explore its molecular pathogenesis.

METHODSThe 66 exons of the FBN1 gene were analyzed by direct sequencing. SIFT and PolyPhen-2 were used to predict the structural and functional changes at the protein level.

RESULTSA novel heterozygous mutation c.3998 G>A (p.Cys1333Tyr) was found in exon 32 in the child. The same mutation was not found among his unaffected family members and 683 healthy controls. Multiple sequence alignment showed that this novel mutation was located in a highly conserved region of the FBN1 protein across various species and may induce structural change to a functional domain.

CONCLUSIONThe novel c.3998G>A (p.Cys1333Tyr) mutation of the FBN1 gene probably predisposed the MFS in the child. Above finding has enriched the spectrum of FBN1 mutations.

OBJECTIVE: To screen for MYOC gene variants among sporadic patients with primary open angle glaucoma (POAG). METHODS: For 398 patients with POAG, Sanger sequencing was applied to detect potential variants of the MYOC gene. RESULTS: Eight patients (2.0%) were found to harbor variations of the MYOC gene. These included five types of variants, among which c.667C>T (p.Pro223Ser) and c.1138G>T (p.Asp380Tyr) were novel. c.382C>T (p.Arg128Trp), c.1109C>T(p.Pro370Leu) and c.1130C>A (p.Thr377Lys) were previously associated with POAG. Alignment of amino acid sequences of MYOC proteins of various species revealed that the two novel variants have occurred at highly conserved positions. c.1138G>T was predicted to be possible pathogenic by Bioinformatic analysis. CONCLUSION Two novel variants of the MYOC gene were detected among sporadic POAG patients, which enriched its variant spectrum.
Cytoskeletal Proteins ; genetics ; Eye Proteins ; genetics ; Glaucoma, Open-Angle ; genetics ; Glycoproteins ; genetics ; Humans ; Mutation

Objective:To explore the effects of health education based on evidence-based practice using the knowledge to action model (KTA) on patients with osteoporosis and lumbar vertebral fractures.Methods:Totally 108 patients with osteoporosis and lumbar vertebral fractures admitted in the the Xingtai General Hospital of North China Medical and Health Group from October 2017 to October 2019 were selected and divided into an observation group and a control group according to the random number table, with 54 patients in each group. Patients in both groups received routine treatment and care. Patients in the control group received routine health education, while patients in the observation group received health education received health education based on evidence-based practice using KTA. The rehabilitation status [Oswestry dysfunction index (ODI) , Barthel index] between the two groups of patients, as well as the knowledge level (osteoporosis knowledge questionnaire) , living habits and quality of life [The World Health Organization Quality of Life Brief Version (WHOQOL-BREF) ] before and after the intervention were compared.Results:After 3 months of intervention, the ODI of the two groups decreased while the Barthel index increased ( P< 0.05) ; and the ODI of the observation group was lower than that of the control group, while the Barthel index score was higher than that of the control group, with statistically significant differences ( P< 0.05) . After 3 months of intervention, the risk factors, exercise knowledge, calcium knowledge scores and total scores of the osteoporosis knowledge questionnaire in the two groups increased, with statistically significant differences ( P< 0.05) , which were higher in the observation group than in the control group, with statistically significant differences ( P< 0.05) . After 3 months of intervention, the ratio of smoking and drinking in the observation group was lower than that in the control group, and the ratio of exercise and calcium supplementation was higher than that in the control group, with statistically significant differences ( P< 0.05) . After 3 months of intervention, the scores in all dimensions of the WHOQOL-BREF of the two groups increased ( P< 0.05) , and the scores in the physical, psychological and social dimensions of the observation group were higher than those of the control group, with statistically significant differences ( P< 0.05) . Conclusions:The health education based on evidence-based practice using KTA in patients with osteoporosis and lumbar vertebral fractures helps to improve patients' related knowledge levels, living habits, rehabilitation effects, and quality of life.