Traditional Chinese medicine of Polygonum cuspidatum has been utilized in China for thousands of years. Its primary active compound, polydatin, exhibits a variety of pharmacological effects including the regulation of glucose and lipid metabolism, suppression of cough and asthma, as well as antibacterial and anti-inflammatory properties. However, conventional methods for polydatin production are inadequate to satisfy the market demand. This study aims to explore the green and efficient preparation of polydatin. With resveratrol as the substrate, we efficiently synthesized polydatin by using the triple mutant IGW (Y14I/I62G/M315W) of the glycosyltransferase UGT_BS based on a strategy of two-enzyme coupled with one-pot and realized the recycling of uridine diphosphate-glucose (UDPG). The conditions of the two-enzyme reaction were optimized. Under the conditions of 35 ℃, pH 8.0, IGW: AtSuSy1 activity ratio of 3:4, dimethyl sulfoxide (DMSO) volume fraction of 5%, uridine diphosphate (UDP) concentration of 0.10 mmol/L, and sucrose concentration of 0.6 mol/L, the conversion of 2 mmol/L resveratrol reached 80.6% within 1 h, and the proportion of polydatin was over 90%. This study achieved the recycling of UDPG via a two-enzyme coupling system and shortened the reaction time. At the same time, the fed-batch strategy was adopted, and the yield of polydatin reached 6.28 g/L after 24 h in the one-pot coupling reaction, which provided a new strategy for green and efficient preparation of polydatin.
Stilbenes/chemistry* ; Glucosides/biosynthesis* ; Resveratrol ; Fallopia japonica/chemistry* ; Glycosyltransferases/genetics*

Objective:To report a family of Zellweger spectrum disorders (ZSD) caused by mutations in the PEX10 gene and to increase the level of awareness of the disease among clinicians. Methods:The clinical and genetic data of a brother-sister co-morbidity family with ZSD due to PEX10 gene mutation admitted to the Department of Neurology, Qilu Hospital of Shandong University in July 2022 were collected and a literature review was performed. Results:The proband was a 24-year-old female who was admitted to the Qilu Hospital of Shandong University due to unstable walking for 8 years and aggravated for 1 year with squatting effort. Cranial magnetic resonance imaging (MRI) showed cerebellar atrophy, electromyography suggested peripheral neuropathy with motor and sensory involvement, and pure tone hearing threshold measurement showed neurogenic deafness. The 15-year-old brother of the proband presented with "unsteadiness in walking and difficulty in squatting for 2 years". His cranial MRI and electromyography were similar to those of the proband, and his laboratory results suggested abnormal liver function. The whole exon sequencing results of the proband suggested a compound heterozygous mutation in the PEX10 gene with c. 992G>A(p. R331Q) and c. 988T>C(p. C330R) mutations and both were likely pathogenic mutations and respectively from her parents. And her brother also carried the above variants. A total of 9 case reports in English literature (1 of which was published by domestic scholars) were retrieved from major domestic and international databases on PEX10 gene mutations causing ZSD, with a total of 15 patients, most of whom had childhood and adolescent onset. The most common initial symptom was slowly progressed ataxia. The majority of patients showed cerebellar atrophy on cranial MRI and peripheral neuropathy was found in the most patients.The 2 patients were suggested to have phytic acid-free food, at the same time, they bagan taking L-arginine and ursodeoxycholic acid. Fortunately,both the ataxia symptom and liver function of them were dramatically alleviated 3 months later. Conclusions:For the patients with unexplained ataxia with polyneuropathy and abnormal liver function,the possibility of ZSD should be considered. Phytic acid-free food, L-arginine and ursodeoxycholic acid supplement may be beneficial for the ZSD patients.

Objective To ascertain the levels and distributions of individual dose of radiologic workers arising from external exposure in medical institutions in Yangpu District during 2018—2020, so as to provide basic data for the prevention of occupational radiation diseases. Methods Based on the individual dose monitoring specifications for occupational external exposure, the individual dose of radiologic workers was monitored by the thermoluminescence dosimetry systems, and the data were collected and analyzed. Results A total of 2082 people were monitored from 2018 to 2020, and the average annual effective dose per capital was 0.20 mSv·a⁻¹. The average annual effective dose per capital in 2020 was lower than that in 2018 and 2019, and the difference was statistically significant (P < 0.05). The average annual effective dose of 4 radiologic workers exceeded 5 mSv·a⁻¹ per capital. The average annual effective dose of diagnostic radiology was 0.20 mSv·a⁻¹, which was higher than that of dental radiology and other medical applications, and the difference was statistically significant (P < 0.05). The average annual effective dose per capital of nuclear medicine was 0.34 mSv·a⁻¹, which was higher than that of other medical categories, and the difference was statistically significant (P < 0.05). The average annual effective dose per capital in unrated hospitals was 0.14 mSv·a⁻¹, which was lower than that in tertiary hospitals and secondary hospitals, and the difference was statistically significant (P < 0.05). Conclusion The average annual effective dose per capital of radiologic workers in medical institutions in Yangpu District is generally kept at a low level. The radiation protection of radiologic workers in nuclear medicine, secondary and tertiary hospitals should be strengthened to reduce the occupational external exposure doses to the lowest level as possible.

Objective Human milk of mothers with positive hepatitis B surface antigen (HBsAg) contains hepatitis B virus (HBV). However, breastfeeding does not increase the risk of mother-to-infant transmission of HBV. Previous investigations demonstrated that breast milk has a property of binding with HBsAg. This study aimed to identify the component in human milk that can bind to HBsAg. Methods This study was performed in Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School, from June 2015 to February 2017. Human milk samples from two postpartum women with negative HBV markers and two control samples of cow milk and goat milk were analyzed by Far-Western blot, in which highly purified recombinant yeast HBsAg was used to bind with whey proteins. Based on the results of mass-spectrum analysis, competition inhibition test was used to confirm the functioning component. Results Far-Western blot showed remarkable protein bands at the relative molecular weight of about 80 000 in both lanes of human milk, but none in the lane of cow or goat milk. Mass-spectrum analysis of the protein band indicated there were proteins sharing 28.4%-93.4% homology in amino acid sequences with five proteins with the highest homology to lactoferrin (93.4%). Further Far-Western blot with purified recombinant lactoferrin showed that lactoferrin could bind to the recombinant HBsAg. Competition inhibition test suggested that the purified recombinant lactoferrin inhibited the binding of HBsAg to its antibody in a dose-dependent manner. Conclusions This study confirms the capability of lactoferrin in human milk to combine with HBsAg, suggesting that lactoferrin can bind to HBV. Further study on whether lactoferrin can inhibit the infectivity of HBV would be valuable to clarify the reason for not increasing the risk of mother-to-infant transmission of HBV by breastfeeding.

Objective To summarize the clinical , pathological and genetic characteristics of three patients with caveolin-3 associated myopathy and review the literatures .Methods The clinical data of three patients with caveolin-3 associated myopathy were investigated .With informed consent , we performed muscle biopsy and genetic analysis of CAV 3 and PTRF genes.Results All the three patients presented with percussion/pressure-induced rapid contraction , percussion-induced muscle mounding and mechanically induced muscle rippling.Besides, case 1 had weakness and atrophy of hand muscles .Case 2, who manifested with muscle hyperexcitability at onset , developed weakness and atrophy of distal part of lower limbs.Case 3 showed normal muscle strength and tone .All of them had myalgia or tenderness .Muscle biopsy revealed mild myogenic changes in two patients and a muscular dystrophic pattern in one . Immunohistochemical staining of caveolin-3 revealed an even deficiency in case 1 and a mosaic deficiency in cases 2 and 3.Gene analysis revealed a missense mutation ( c.80G>A, p.R27Q) in CAV3 gene of case 1. No mutations were identified in cases 2 and 3.Conclusions There is phenotypic variability in patients with caveolin-associated myopathy , including limb-girdle syndrome , rippling muscle disease , distal myopathy , muscle hypertrophy , idiopathic hyperCKemia and cardiomyopathy .Muscle biopsy and caveolin-3 staining should be performed for the above patients with muscle rippling .

Objective To explore the correlation between inelligence and speech development of school age children with cerebral palsy.Methods 46 school age children with cerebral palsy were evaluated with China Rehabilitation Research Centre Sign-Significance test (S-S)and Wechsler Intelligence Scale. The correlation between S-S and Wechsler Intelligence Scale was analyzed. Results The intelligence quotient decreased in 36 cases (78.26%), mostly mild to moderate. The development of speech delayed in 36 cases (78.26%). The outcomes of Wechsler Intelligence Scale significantly correlated with those of S-S Test (P<0.001). Conclusion There is a correlation between intelligence and speech development of school age children with cerebral palsy.

Objective To explore the surface electromyography (sEMG) assist for the rehabilitation training for children with cerebral palsy. Methods 60 children with spastic diplegia were divided into treatment group (n=30) and control group (n=30). All the children received comprehensive rehabilitation, while the rehabilitation program were designed with the assist of sEMG in the treatment group. They were assessed with Gross Motor Function Measure (GMFM) before and 3 months after rehabilitation. Results There was no statistically improvement in dimension A, B, and C of GMFM (P>0.05), but was in the total score and the dimension D and E (P<0.0001). There was no statistical difference in dimension A, B, and C between 2 groups (P>0.05), but was in the total score and the dimension D and E (P<0.01) after rehabilitation. Conclusion The sEMG might be helpful for the rehabilitation for children with cerebral palsy.

Objective To discuss current detection of food intolerance in health check-up populations and the influence of food intolerance on immune system and inflammation parameters.Methods Serum levels of 14 kinds of food-specific IgG antibodies were analyzed by using enzyme linked immunosorbent assay (ELISA) among 257 healthy check-up adults.White blood cell count,eosinophils,globulin,erythrocyte sedimentation rate (ESR),C-reactive protein (CRP),rheumatoid factor (RF),antinuclear antibodies (ANA) and T cell function were compared between the positive group and the negative group.Student's t test and Chi-square test were used for data analysis.Results Food-specific IgG antibodies were found in 134 participants (52.14％).The most common intolerant foods were egg,crab and milk.Moderate to severe food intolerance was caused by egg or milk.There was significant difference in eosinophils and serum globulin between the positive and the negative group (t =-0.07,x2 =8.91,both P ＜0.05).However,no difference was observed in white blood cell count,ESR,CRP,RF,ANA and T cell function between two groups (all P ＞ 0.05).Conclusion Eosinophils and serum globulin may significantly increased in people with food intolerance,although white blood cell count,ESR,CRP,RF,ANA and T cell function could be normal.

Objective To investigate the pathological features of blood vessel inflammation in facioscapulohumeral muscular dystrophy ( FSHD ) and the role of vasculitis on the pathogenesis of FSHD. Methods The clinical manifestations and myopathological features of 26 FSHD patients were retrospectively analyzed and summarized. All of the patients were divided into 2 groups; inflammatory infiltration group and non-inflammatory infiltration group. The latter was further divided into 3 subgroups;endomysial inflammation subgroup, perivasculitis subgroup and transmural vasculitis subgroup.Immunohistochemical staining were carried out in inflammatory infiltration group with anti-CD3, anti-CD4,anti-CD8,anti-CD20 and anti-SMA antibody. The control group was composed of 10 dermatomyositis ( DM)cases and 10 polymyositis ( PM) cases. Results The age of onset was (25. 2 ± 12. 6) years old and the average course was (7. 8 ±7. 3) years. The sex ratio of male to female was 1.6: 1. Five of them had family history. The main clinical features were progressive weakness and atrophy of facial, shoulder girdles and proximal upper limbs muscles. The lower distal limbs and (or) lower distal limbs and pelvic girdle muscles were involved in 18 cases. The main pathological features were shown as followed. Seventeen of them had focal inflammatory cell infiltration, including endomysial inflammation (4/17) , perivasculitis (7/17) , and transmural vasculitis (6/17). Immunohistochemical staining confirmed the major types of inflammatory cells were CD4* T lymphocytes and CD20B lymphocytes, which was familiar with DM. While in PM, CD8+ T lymphocytes were dominant The proportionality of residual muscle fibers obviously decreased in inflammatory infiltration group ( 48. 0% ± 23. 6% ) than non-inflammatory infiltration group ( 94. 3% ±3. 1% , T = 198. 000, P = 0. 000). As to CK levels, there were no significant deviation. Conclusions Obvious inflammatory cell infiltration can be seen in FSHD, the locations of inflammatory cells are endomyosium inflammation, perivasculitis and transmural vasculitis. Transmural vasculitis indicates vascular pathological factor may have something to do with pathogenesis of FSHD.

Objective To investigate use of oral antihypertensive drugs among community hypertensive patients in Shanghai and find out factors related to their unreasonable use to direct their clinical use. Methods Seven hundred and three hypertensive patients were surveyed with questionnaire by stratified cluster sampling at three neighborhoods and one village of Dahua community, Baoshan district, Shanghai during April to June 2009 to understand their use of antihypertensive agents, including kinds and forms of drugs, rationale of drug use. Results Five hundred and eighty-two (82. 8% ) of 703 hypertensive patients interviewed were using antihypertensive drugs, 271 (38. 5% ) of them used only one kind of non-compound antihypertensive drug, 182 (25.9%) used one kind of compound agent, 311 (53.4%) used two or more kinds of drugs in combination, including 117 patients ( 16. 8% ) used two kinds of agents combined and 12 patients ( 1.6% ) used three kinds of agents combined. Two hundred and sixty-six (47.5%) patients took orally calcium-channel blockers and 205 ( 35.2% ) used compound agents. Conclusions Frequency of combined use of two or more kinds of antihypertensive agents is reasonable and significantly higher than that of use of one kind of drug at Dahua community in Shanghai. Calcium channel blocker plays a predominant role in treatment for hypertension, non-long-acting compound agents are used in a higher proportion. But,guidelines for hypertension prevention and treatment are not so well complied with in local hypertensive patients. So, it is suggested that training for community physicians and management for standard use of antihypertensive agents at community should be strengthened further.