Objective To study the chemical constituents of Salvia miltiorrhiza f. alba Methods The compounds were isolated by chromatographic techniques and their structures were elucidated by spectroscopic analysis. Results Four compounds were obtained from the plant and identified as 8, 9-dihydro-1, 6-dimethylfuro [3, 2-c] naphtha [2, 1-e] oxepine-10, 12-dione (Ⅰ), tanshindiol C (Ⅱ), salviolone (Ⅲ), 1, 2, 6, 7, 8, 9-hexahydro-1, 6, 6-trimethyl-3, 11-dioxanaphtho [2, 1-e] azulene-10, 12-dione (Ⅳ). Conclusion Compound Ⅰ is a new one, named salmilalbanone (8, 9-dihydro-1, 6-dimethylfuro [3, 2-c] naphtha [2, 1-e] oxepine-10, 12-dione), while compounds Ⅱ—Ⅳ are isolated from the plant for the first time.

Objective To explore the rule of acupoints selection of acupuncture and moxibustion in treating Alzheimer disease based on TCM Knowledge Service and Sharing System (V1.2.8); To provide references for the clinic. Methods Literature about acupuncture and moxibustion for the treatment of Alzheimer disease in CNKI, Choingqing Weipu, Wanfang database, and CBM from 1st January, 1996 to 1st January, 2016 was searched. Excel2007 was used to establish database and enter into TCM Knowledge Service and Sharing System (V1.2.8). The data were analyzed by data mining method such as frequency analysis, association rule analysis, point-to-pair analysis and core prescription analysis. Results After screening, 125 acupuncture prescriptions were included, involving 108 acupoints and 998 total acupoints selection frequency. Baihui had the highest frequency (78 times, 62.40％). The core acupoint combinations were Zusanli-Baihui, Zusanli-Sanyinjiao, and Baihui-Sishencong. The main core prescription was Bauhui, Zusanli, Sishencong, Shenmen, Taixi, Sanyinjiao, Neiguan, Taichong, Fenglong, Dazhui, Fengchi, Shenshu, Shenting, Xuanzhong. The second core prescription was Hegu, Yintang, Tanzhong, Xuehai, Geshu, Ganshu, Pishu, Qihai, Yanglingyuan, and Shangxing. Conclusion The rules of acupoints selection analyzed by TCM Knowledge Service and Sharing System (V1.2.8) for Alzheimer disease can be used to guide clinical application, but with modification according to symptoms.

Objective Applyingthe traditional Chinese medical knowledge service and sharing system, to explore the medication rules of ankylosing spondylitis in the medical records.Methods The ankylosing spondylitis medical records in the traditional chinese medical knowledge service and sharing system has been searched and 149 prescriptions were identified. The medication frequency, association rules, herbal combination and the cluster analyses were explored.Results Among the 149 prescriptions with 210 drugs, the use of high frequency drugs wereGouji, Baishao, Danggui, Duzhong, Weilingxian, and other medication whose frequency was more than 20 times were good at tonifying liver and kidney, activating blood circulation, clearing heat and promoting diuresis, dispelling cold and removing dampness. The high frequency of drug combinations included Sangjisheng and Gouji, Duzhong and Gouji, Baishao and Gouji, Guizhi and Baishao, Gancao and Danggui.Conclusions The treatment of ankylosing spondylitis with Chinese medicine is mainly based on tonifying liver and kidney, supplemented with drugs for warming Kidney Yang, dispelling cold and dehumidification, promoting blood circulation and removing blood stasis, clearing heat and promoting diuresis, dispelling wind and cold.

We retrospectively analyzed clinical and imaging data of 26 children with split cord malformations (SCMs). Based on Pang's classification, 14 SCMs were defined as type Ⅰ and 12 as type Ⅱ.Neural function was markedly improved in 20 patients postoperatively. Three of 4 children who did not undergo surgical treatment had neural function deteriorated. Two children lost follow-up. We suggest that Pang's Classification of SCMs may be useful in describing pathological changes and guiding surgical procedure; imaging examine (including MRI, CT and X-ray) would play a significant role in confirmed SCMs diagnosis; and surgical operation should focus on eliminate and prevent spinal cord damnification.

Objective To investigate the surgical diagnosis and treatment of late vitamin K deficiency intracranial hemorrhage caused by biliary atresia. Methods Clinical data of six cases of biliary atresia with late vitamin K deficiency intracranial hemorrhage were collected in the Department of Neurosurgery of Tianjin Children’s Hospital from January 2000 to December 2013. Data were analyzed to identify the biliary atresia as soon as possible in the treatment of intracranial hemorrhage and prolonged jaundice in children. Results Six cases (1 male, 5 female), mean age was (16.0±2.6) days, and were treated with external drainage of intracranial hematoma and infusion therapy. In the treatment, children were found jaundice exacerbation and doubted about biliary atresia. After consultation by general surgeons, children were transferred to the department of general surgery for further treatment at an average age of (29.1±1.2) days, and were diagnosed as biliary atresia by intraoperative cholangiography. Conclusion Pediatric neurosurgeon should have a sufficient understanding and make an early diagnosis to late vitamin K deficiency intracranial hemorrhage caused by biliary atresia, to avoid delaying the optimal treatment time of biliary atresia.

OBJECTIVE: To explore the molecular etiology for a Chinese family affected with beta-ureidopropinoase deficiency. METHODS: Genomic DNA was extracted from the peripheral blood samples of family members. All exons and flanking intron regions of the UPB1 gene were amplified by PCR and detected by direct sequencing. The pathogenicity of identified mutation was analyzed using Polyphen2 and SIFT software. RESULTS: Compound heterozygous mutations of the UPB1 gene, including c.853G>A (p.A285T) and c.917-1G>A, were discovered in the proband, which were inherited respectively from his mother and father. Bioinformatics analysis suggested that this novel mutation was damaging. CONCLUSION The compound heterozygous mutations of the UPB1 gene probably underlie the beta-ureidopropinoase deficiency in the infant. Discovery of c.853G>A also enriched the mutation spectrum of the UPB1 gene.
Abnormalities, Multiple ; genetics ; Amidohydrolases ; deficiency ; genetics ; Asian Continental Ancestry Group ; Brain Diseases ; genetics ; China ; Exons ; Humans ; Infant ; Introns ; Movement Disorders ; genetics ; Mutation ; Pedigree ; Purine-Pyrimidine Metabolism, Inborn Errors ; genetics

Metabolic-associated fatty liver disease (MAFLD), which is previously known as non-alcoholic fatty liver disease (NAFLD), represents a major health concern worldwide with limited therapy. Here, we provide evidence that ferroptosis, a novel form of regulated cell death characterized by iron-driven lipid peroxidation, was comprehensively activated in liver tissues from MAFLD patients. The canonical-GPX4 (cGPX4), which is the most important negative controller of ferroptosis, is downregulated at protein but not mRNA level. Interestingly, a non-canonical GPX4 transcript-variant is induced (inducible-GPX4, iGPX4) in MAFLD condition. The high fat-fructose/sucrose diet (HFFD) and methionine/choline-deficient diet (MCD)-induced MAFLD pathologies, including hepatocellular ballooning, steatohepatitis and fibrosis, were attenuated and aggravated, respectively, in cGPX4-and iGPX4-knockin mice. cGPX4 and iGPX4 isoforms also displayed opposing effects on oxidative stress and ferroptosis in hepatocytes. Knockdown of iGPX4 by siRNA alleviated lipid stress, ferroptosis and cell injury. Mechanistically, the triggered iGPX4 interacts with cGPX4 to facilitate the transformation of cGPX4 from enzymatic-active monomer to enzymatic-inactive oligomers upon lipid stress, and thus promotes ferroptosis. Co-immunoprecipitation and nano LC-MS/MS analyses confirmed the interaction between iGPX4 and cGPX4. Our results reveal a detrimental role of non-canonical GPX4 isoform in ferroptosis, and indicate selectively targeting iGPX4 may be a promising therapeutic strategy for MAFLD.