Clinical characteristics of 111 cases with mucopolysaccharidosis ⅣA / 中华儿科杂志

Objective: To analyze the clinical characteristics of patients with Mucopolysaccharidosis ⅣA (MPS ⅣA). Methods: A retrospective study was conducted on 111 patients with MPS ⅣA in Xinhua Hospital of Shanghai Jiao Tong University School of Medcine from December 2008 to August 2020, confirmed by enzyme activity and genetic testing. General situation, clinical manifestations and enzyme activity test results were analyzed. According to the clinical manifestations, it can be divided into severe, intermediate and mild group. The independent sample t test was used to compare the birth body length and weight of children with that of normal boys and girls, and group comparisons of enzyme activities were evaluated by median test. Results: One hundred and eleven unrelated patients, 69 males and 42 females, were classified into 3 subtypes: severe (n=85), intermediate (n=14), and mild (n=12). The age at symptom onset were 1.6 (1.0, 3.0) years, and at diagnosis were 4.3 (2.8, 7.8) years. Skeletal manifestations were observed in all patients and consisted mainly of pectus carinatum (96/111, 86.5%), motor dysfunction (78/111, 70.3%), spinal deformity (71/111, 64.0%), growth retardation (64/111, 57.7%), joint laxity (63/111, 56.8%) and genu valgum (62/111, 55.9%). Eighty-eight patients (88/111, 79.3%) with MPS ⅣA were also along with non-skeletal manifestations, mainly including snoring (38/111, 34.2%), coarse faces (34/111, 30.6%), and visual impairment (26/111, 23.4%). The most common skeletal manifestation was pectus carinatum (79 cases), and non-skeletal manifestation was snoring (30 cases) and coarse faces (30 cases) in severe patients, pectus carinatum (13 cases) and snoring (5 cases) in intermediate type, motor dysfunction (11 cases) and snoring (3 cases) and visual impairment (3 cases) in mild patients. The height and weight of severe patients began to fall below -2 s at 2-<5 years and 5-<7 years, respectively. At the age of 10-<15 years, the standard deviation score of the height of severe patients reached (-6.2±1.6) s in males and (-6.4±1.2) s in females, and the score of weight got (-3.0±1.1) s in males and (-3.5±0.5) s in females. The height of intermediate patients began to fall below -2 s at the age of 7-<10 years, and the standard deviation score of height were -4.6 s and -3.6 s in 2 males, and -4.6 s and -3.8 s in 2 females at the age of 10-<15 years. The weight remained within -2 s in 72.0% (18/25) of intermediate patients compared to age-matched healthy children. In the mild patients with MPS ⅣA, the mean standard deviation score of height and weight was within -2 s. The enzyme activities of mild patients (2.02 (1.05, 8.20) nmol/(17 h·mg)) were both significantly higher than that of intermediate (0.57 (0.47, 0.94) nmol/(17 h·mg)) and severe (0.22 (0, 0.59) nmol/(17 h·mg)) patients (Z=9.91, 13.98, P=0.005, 0.001), and the enzyme activity of intermediate patients was significantly higher than that of severe patients (Z=8.56, P=0.010). Conclusions: The clinical manifestations of MPS ⅣA are charactered by pectus carinatum, motor function impairment, spinal deformity and growth retardation. The clinical characteristics, growth rate and enzyme activity differ among the 3 subtypes of MPS ⅣA.

Analysis of Chinese Medical Syndrome Features of Ischemic Stroke Based on Similarity of Symptoms Subgroup / 中国结合医学杂志

OBJECTIVE@#To derive the Chinese medicine (CM) syndrome classification and subgroup syndrome characteristics of ischemic stroke patients.@*METHODS@#By extracting the CM clinical electronic medical records (EMRs) of 7,170 hospitalized patients with ischemic stroke from 2016 to 2018 at Weifang Hospital of Traditional Chinese Medicine, Shandong Province, China, a patient similarity network (PSN) was constructed based on the symptomatic phenotype of the patients. Thereafter the efficient community detection method BGLL was used to identify subgroups of patients. Finally, subgroups with a large number of cases were selected to analyze the specific manifestations of clinical symptoms and CM syndromes in each subgroup.@*RESULTS@#Seven main subgroups of patients with specific symptom characteristics were identified, including M3, M2, M1, M5, M0, M29 and M4. M3 and M0 subgroups had prominent posterior circulatory symptoms, while M3 was associated with autonomic disorders, and M4 manifested as anxiety; M2 and M4 had motor and motor coordination disorders; M1 had sensory disorders; M5 had more obvious lung infections; M29 had a disorder of consciousness. The specificity of CM syndromes of each subgroup was as follows. M3, M2, M1, M0, M29 and M4 all had the same syndrome as wind phlegm pattern; M3 and M0 both showed hyperactivity of Gan (Liver) yang pattern; M2 and M29 had similar syndromes, which corresponded to intertwined phlegm and blood stasis pattern and phlegm-stasis obstructing meridians pattern, respectively. The manifestations of CM syndromes often appeared in a combination of 2 or more syndrome elements. The most common combination of these 7 subgroups was wind-phlegm. The 7 subgroups of CM syndrome elements were specifically manifested as pathogenic wind, pathogenic phlegm, and deficiency pathogens.@*CONCLUSIONS@#There were 7 main symptom similarity-based subgroups in ischemic stroke patients, and their specific characteristics were obvious. The main syndromes were wind phlegm pattern and hyperactivity of Gan yang pattern.

Astragalin Inhibits Apoptosis of Cerebral Cortex Neurons in APP/PS1 Mice / 中山大学学报(医学科学版)

ObjectiveTo investigate the effect of Astragalin （AST） on apoptosis of cerebral cortex neurons in APP/PS1 transgenic mice. MethodsEighteen six-month-old male APP/PS1 transgenic mice were randomly divided into APP/PS1 group， APP/PS1+ 40 mg/kg AST group and APP/PS1+ 20 mg/kg Donepezil （DNP） group， with six mice in each group. At the same time， six male C57BL/6 mice were selected as the normal control group. After intraperitoneal injection of AST once a day and continuous administration for one month， we used Tunel staining to detect the apoptosis of neurons in the cerebral cortex of APP/PS1 mice； immunofluorescent staining to examine the expression of apoptosis-related proteins Bax， Bcl-2， Caspase9 and Cleaved-Caspase3 in the cerebral cortex neurons of APP/PS1 mice； Western blot method to evaluate the changes of the expression of Bax， Bcl-2， Caspase9 and Caspase3. ResultsTunel staining showed that 40 mg/kg AST and 20 mg/kg DNP both reduced the apoptosis of neurons in the cerebral cortex of APP/PS1 mice， AST with more significant inhibition effect. Immunofluorescent staining revealed that 40 mg/kg AST and 20 mg/kg DNP both inhibited the expression of Bax， Caspase9， and Cleaved-Caspase3， and icreased the expression of Bcl-2 in the cerebral cortex neurons of APP/PS1 mice. Western blot results further confirmed that 40 mg/kg AST and 20 mg/kg DNP both down-regulated the expression of Bax （P < 0.05， P < 0.05）， Caspase9 （P < 0.005， P < 0.05） and Caspase3 （P < 0.0001， P < 0.0001） ， and up-regulated the expresstion of Bcl-2 （P < 0.05， P < 0.05） in the cerebral cortex neurons of APP/PS1 mice. ConclusionsAST can inhibit the apoptosis of cerebral cortex neurons in APP/PS1 mice.

Analysis of variation trend in health workforce equity allocation in China / 北京大学学报(医学版)

OBJECTIVE@#To analyze the long-term trends of the changes in the equity of China's health workforce allocation to provide a reference for the more balanced and orderly development of China's health system.@*METHODS@#The Gini coefficient was used to evaluate the degree of equity in the allocation of health workforce between regions, and the Gini coefficients for the allocation of doctors and nurses based on population and regional gross domestic product (GDP) distribution were calculated respectively.@*RESULTS@#In 2019, the number of licensed (assistant) physicians per 1 000 population in China was 2.77, and the number of registered nurses per 1 000 population was 3.18. The Gini coefficient for the distribution of licensed (assistant) physicians by population was 0.141 in 2002, decreasing to 0.081 by 2014 and then remained stable. The Gini coefficient for the distribution of registered nurses by population was 0.164 in 2002 and decreased to 0.066 in 2018. The Gini coefficient for the distribution of licensed (assistant) physicians by GDP was 0.236 in 2002, decreased to 0.169 in 2013, then increased to 0.183 and remained stable. The Gini coefficient for the distribution of registered nurses by GDP was 0.206 in 2002, decreased to 0.150 in 2013, and then increased each year to 0.180 in 2019. The equity of the allocation of registered nurses by population was worse than the equity of the allocation of licensed (assistant) physicians in 2002, and in 2016, for the first time, exceeded that of licensed (assistant) physicians.@*CONCLUSION@#Equity in the allocation of health workforce across China has improved, but the improvement in equity between regions has hit a bottleneck, with health workforce allocation in the western regions still relatively scarce. Although nursing workforce allocation equity caught up with licensed (assistant) physicians, the number of licensed (assistant) physicians is close to that of developed western countries, while there is a large gap in registered nurses. It is recommended that the relevant authorities make good long-term planning for health workforce, further increase the policy for the introduction of health workforce in the western region, and increase the supply of healthcare services in the western region with the help of digital transformation of healthcare and internet healthcare. At the same time, they should further increase investment in resources for higher nursing education and actively plan to cope with the ageing population.

Mechanism of Aconiti Kusnezoffii Radix Processed with Chebulae Fructus Against H9c2 Cardiomyocyte Toxicity Based on TRPV1 Channel / 中国实验方剂学杂志

ObjectiveTo explore the role of transient receptor potential vanilloid 1 （TRPV1） channel in reducing cardiomyocyte toxicity of Aconiti Kusnezoffii Radix processed with Chebulae Fructus. MethodH9c2 cardiomyocytes cultured in vitro were used as a model to assess cell viability by methyl thiazolyl tetrazolium （MTT） assay， the expression of TRPV1 mRNA was detected by real-time fluorescence quantitative polymerase chain reaction （Real-time PCR）， and the leakage rate of lactate dehydrogenase （LDH）， the changes of nucleus， reactive oxygen species （ROS）， mitochondrial membrane potential and Ca2+ contents were detected by enzyme linked immunosorbent assay （ELISA）. ResultCompared with the blank group， when the concentration was ≥0.5 g·L-1， the cell viability was significantly decreased （P<0.01）， the leakage rate of LDH， the release of ROS and Ca2+ were increased， the mitochondrial membrane potential was decreased， and the nucleus was pyknosis or even broken in raw Aconiti Kusnezoffii Radix and Aconiti Kusnezoffii Radix processed with Chebulae Fructus groups. When the concentration was ≥0.5 g·L-1， compared with the same mass concentration of raw Aconiti Kusnezoffii Radix group， the cell viability increased significantly （P<0.01）， the leakage rate of LDH， the release of ROS and Ca2+ decreased， the mitochondrial membrane potential increased， and the nuclear morphology improved in Aconiti Kusnezoffii Radix processed with Chebulae Fructus group. Application of the same mass concentration of raw Aconiti Kusnezoffii Radix to H9c2 cardiomyocytes pretreated with the TRPV1 inhibitor BCTC significantly increased cell viability， decreased leakage rate of LDH， ROS and Ca2+ release， increased mitochondrial membrane potential and improved nuclear pyknosis compared with untreated H9c2 cardiomyocytes. Application of the same mass concentration of Aconiti Kusnezoffii Radix processed with Chebulae Fructus to H9c2 cardiomyocytes pretreated with BCTC decreased cell viability， increased LDH leakage rate， ROS and Ca2+ release， reduced mitochondrial membrane potential compared with untreated H9c2 cardiomyocytes. Real-time PCR results showed that both raw Aconiti Kusnezoffii Radix and Chebulae Fructus decoction could increase the expression of TRPV1 mRNA in cardiomyocytes in a concentration dependent manner. ConclusionRaw Aconiti Kusnezoffii Radix can induce cardiomyocyte apoptosis and cardiotoxicity by activating TRPV1 channel， while Aconiti Kusnezoffii Radix processed with Chebulae Fructus can attenuate the toxicity through TRPV1 channel， which may be related to the synergistic effect of acid components in Chebulae Fructus and alkaloids in Aconiti Kusnezoffii Radix on TRPV1 channel.

In Vitro Fatigue Test of Lung Volume Reduction Loop / 中国医疗器械杂志

Lung volume reduction loop uses bronchoscopic lung volume reduction(BLVR) technology to compress and collapse the necrotic emphysema tissue and exhaust the internal gas to achieve the purpose of lung volume reduction to treat emphysema. After the lung volume reduction loop is implanted into the human body, the compressed part of the lung tissue tends to expand with breathing, which makes the lung volume reduction loop expand into a linear trend periodically. Fatigue resistance is one of the most important performance indexes of the lung volume reduction loop. In the paper, Z-direction vibration fatigue machine was used to simulate the changes of human respiratory cycle movement to test the fatigue performance of lung volume reduction loop, which can provide some reference for the test method of in vitro fatigue performance of lung volume reduction related products in the future.

Clinical Practice Guideline for Allergic Rhinitis Treatment with Acupuncture / 中国结合医学杂志

With reference to international guidelines for the development of tools-Grading of Recommendations Assessment, Development and Evaluation (GRADE) system approach and reasoning, this practice guideline has been drafted reflecting the characteristics of acupuncture to improve effectiveness and safety of acupuncture treatment for allergic rhinitis. This guideline includes outlining the acupuncture diagnosis and treatment principles for allergic rhinitis, suggesting recommendations and related evidence for the acupuncture treatment of allergic rhinitis, and defining operating methods and precautions for the acupuncture treatment of allergic rhinitis.

Comparative Study of Cytotoxicity and Anti-inflammatory Effects between Raw Aconitium kusnezoffii and Aconitium kusnezoffii Processed with Terminalia chebula / 中国药房

OBJECTIVE：To comp are cytotoxicity and anti-inflammatory effects of raw Aconitium kusnezoffii and A. kusnezoffii processed with Terminalia chebula . METHODS ：Using H 9c2 cardiomyocytes isolated from rat as subjects ，CCK-8 assay was used to detect the effects of 0.5，1，2，4，6，8，10 mg/mL raw A. kusnezoffii and A. kusnezoffii processed with T. chebula on cell inhibition rate after cultured for 4，8，12，24 h. Hoechst 33258 staining was used to observe the effects on cell morphology characteristics after treated with 2，4，6 mg/mL raw A. kusnezoffii and A. kusnezoffii processed with T. chebula . Using macrophages RAW264.7 cells as subjects ，CCK-8 assay was used to detect the effects of 0.05，0.1，0.25，0.5，0.75，1，1.5，2 mg/mL raw A. kusnezoffii and A. kusnezoffii processed with T. chebula on cell survival rate after cultured for 24 h. ELISA assay was used to detect the effects of 0.05，0.1，0.25，0.5 mg/mL raw A. kusnezoffii and A. kusnezoffii processed with T. chebula on the release of NO ， TNF-α and IL-6 in RAW 264.7 inflammation cells induced by LPS. RESULTS ：When the mass concentration was 0.5，1 mg/mL， neither raw A. kusnezoffii and A. kusnezoffii processed with T. chebula had no inhibitory effect on H 9c2 cells. When the mass concentration was 2 mg/mL，the inhibitory effects of A. kusnezoffii processed with T. chebula on H 9c2 cells was higher than that of raw A. kusnezoffii （P＜0.05 or P＜0.01）；fluorescence intensity of cells treated for 24 h was stronger than that of raw A. kusnezoffii，but its nucleus was intact. When the mass concentration was 4-10 mg/mL，the inhibitory rate of A. kusnezoffii processed with T. chebula on H 9c2 cells at different time points （except for 24 h culture of 8，10 mg/mL）was significantly lower than raw A. kusnezoffii （P＜0.05 or P＜0.01）. The characteristics of cell morphology also showed that the fluorescence intensity of raw A. kusnezoffii group at 4，6 mg/mL was stronger than that of A. kusnezoffii processed with T. chebula group，and the cell nucleus fragmentation was more serious in the raw A. kusnezoffii group. 0.05-0.5 mg/mL raw A. kusnezoffii and A. kusnezoffii processed with T. chebula had no toxicity to RAW264.7 cells. 0.25，0.5 mg/mL raw A. kusnezoffii and 0.1，0.25，0.5 mg/mL A. kusnezoffii processed with T. chebula showed significant inhibitory effect on the release of NO ，0.05，0.1，0.25，0.5 mg/mL raw A. kusnezoffii and A. kusnezoffii processed with T. chebula showed significant inhibitory effect on the release of TNF-α and IL-6 in RAW 264.7 cell（P＜0.05 or P＜ 0.01）. The inhibitory effects of A. kusnezoffii processed with T. chebula at the same concentration on the release of NO was better than that of raw A. kusnezoffii ，but poorer than raw A. kusnezoffii in the inhibitory effects on the release of TNF-α and IL-6. CONCLUSIONS：The toxicity of A. kusnezoffii can be reduced after processed with T. chebula ，especially the toxicity of it in medium or high concentration and short-term use is lower than that of raw A. kusnezoffii . At the same time ，the anti-inflammatory effect of A. kusnezoffii processed with T. chebula is comparable to that of raw A. kusnezoffii at the same concentration.

A comparative study on enzyme production ability of microorganism producing γ-aminobutyric acid in processing of Sojae Semen Praeparatum at different pH and temperatures / 中草药

Objective: To lay a foundation for elucidating the mechanism of microbial regulation of γ-amino butyric acid (GABA) formation in the processing of Sojae Semen Praeparatum. Methods: The ability of glutamic acid decarboxylase and protease (neutral protease, alkaline protease and acid protease) production of Bacillus subtilis, Enterococcus avium, Enterococcus faecium, Bacillus amyloliquefaciens, Aspergillus niger, Aspergillus flavus, Aspergillus tamarii, Penicillium citrinum, Cladosporium tenuissimum, Phanerochaete sordida, Rhizopus oryzae, Sporidiobolus salmonicolor in different pH and temperature conditions were determined by Berthelot colorimetry and Folin phenol method. Results: The GAD and protease activities of the twelve strains were stronger at pH 5-7 and temperature 28-37 ℃. The highest GAD enzyme activity from A. flavus was 41.97 U/h, the optimum pH was 7 and the temperature was 28 ℃, followed by S. salmonicolor, A. niger, R. oryzae, and B. subtilis, respectively. The enzyme activities were 29.04, 25.78, 22.42 and 19.43 U/h. The highest neutral protease activity of B. subtilis was 24.80 U/mL, the optimum pH was 7 and the temperature was 37 ℃, followed by B. amyloliquefaciens, R. oryzae and E. avium. The enzyme activities were 16.86, 12.51 and 9.18 U/mL. The highest alkaline protease activity of B. amyloliquefaciens was 13.29 U/mL, the optimum pH was 7 and the temperature was 34 ℃, followed by B. subtilis and R. oryzae. The enzyme activities were 8.86 and 6.20 U/mL, respectively. The ability of 12 kinds of microorganisms to produce acid protease was generally poor. Conclusion: The optimal pH and temperature of the 12 kinds of microorganisms selected for this experiment are basically consistent with the natural processing of Sojae Semen Praeparatum. Among them, fungi have stronger GAD production capacity and bacteria have stronger neutral protease production capacity. The high GABA concentration of Sojae Semen Praeparatum is caused by the joint action of multiple strains.

Polypeptide compound urantide inhibiting expression of type i collagen in rat heart of atherosclerosis / 解剖学报

Objective To observe the effect of the peptide compound urantide on the expression of type I collagen (Col I ) in the heart tissue of rats with atherosclerosis ( As) , and to explore its mechanism of prevention and treatment of heart damage in As rats. Methods Sixty healthy male 3-week-old SPF Wistar rats were selected. The As model was established by intraperitoneal injection of vitamin D3(VD3) to damage the arterial intima and high-fat diet. They were randomly divided into normal group, As model group, simvastatin group and urantide (3 days, 7 days, 14 days) groups. HE staining and Masson trichrome staining were used to observe the morphology and collagen fiber expression of rat hearts. Immunohistochemistry, Western blotting and Real-time PCR were used to detect the expression of Col I protein and gene in rat heart. Results Compared with the normal group, pathological phenomena such As myocardial cell degeneration, intercellular infiltration of a large number of neutrophils, scattered foam cells and hyperemia and hemorrhage were observed in the heart tissues of the As model group. Meanwhile, collagen fibers increased, and the gene and protein expression levels of Col I increased. Compared with the As model group, the cardiac pathological phenomena were effectively alleviated after the treatment with urantide. With the extension of the administration time, the collagen fibers decreased, and the gene and protein expression levels of Col I were gradually down-regulated, especially the effect was the best when the drug was given for 14 days. Conclusion Urantide can inhibit the expression of Col I in As heart to reduce myocardial interstitial damage, and has a protective effect on the heart of As rats.

Acupuncture for secondary dysmenorrhea of adenomyosis: a prospective case-series study / 中国针灸

OBJECTIVE@#To observe the clinical effect of acupuncture by stages on secondary dysmenorrhea of adenomyosis through prospective case-series study.@*METHODS@#A total of 36 cases of adenomyosis patients with moderate-to-severe dysmenorrhea were treated with acupuncture by stages. The acupoints of Diji (SP 8), Sanyinjiao (SP 6), Ciliao (BL 32) and Shiqizhui (EX-B 8) were selected and acupuncture was given once a day during menstrual period; the acupoints of Guanyuan (CV 4), Zigong (EX-CA1), Sanyinjiao (SP 6) and Zusanli (ST 36) ect. were selected and acupuncture was given twice per week during non-menstrual period. All the treatment was given for three menstrual cycles. The visual analogue scale (VAS), Cox menstrual symptom scale (CMSS), Endometriosis Health Profile-5 (EHP-5) scores and the menstrual blood volume of pictorial blood loss assessment chart (PBAC) were observed before treatment and at the 1st, 2nd and 3rd menstrual cycle into treatment. Before treatment and at the 3rd menstrual cycle into treatment, the volume of uterus was measured by transvaginal ultrasound and the correlation among the quality of life, the severity of pain and symptoms was analyzed.@*RESULTS@#The VAS, CMSS and EHP-5 scores at the 1st, 2nd and 3rd menstrual cycle into treatment were lower than those before treatment (0.05). Compared before treatment, at the 1st, 2nd and 3rd menstrual cycle into treatment, the PBAC scores were reduced in patients with PBAC>100 points (<0.01). Compared between 2nd and 1st menstrual cycle into treatment, between 3rd and 2nd menstrual cycle into treatment, the VAS, CMSS scores were all decreased (<0.01, <0.05). There was a significant positive correlation between the severity score of CMSS and EHP-5 at the corresponding time points of the 1st, 2nd and 3rd menstrual cycle into treatment (<0.01).@*CONCLUSION@#The acupuncture by stages has significant analgesic effect in patients with secondary dysmenorrhea of adenomyosis, and has the advantages of relieving the menstruation-related symptoms, regulating menstrual blood volume and improving the quality of life.

A retrospective study of cryptococcal infection after renal transplantation and literature review / 中华器官移植杂志

Objective:To explore the clinical characteristics, diagnosis and treatment of cryptococcal infection after renal transplantation.Methods:The clinical data were analyzed retrospectively for 17 hospitalized cases of cryptococcal infection after kidney transplantation from January 2003 to December 2019. The relevant parameters included site of infection, clinical manifestations, complications, comorbidities, treatments and outcomes. The average time to infection after transplantation was (7.9±5.4) years, the median baseline level of creatinine was 137(75-741) μmol/L. Concurrent conditions included hypertension (n=15, 88.2%), diabetes (n=6, 35.3%) and chronic hepatitis (n=9, 52.9%). The most common site of infection was central nervous system (88.2%), followed by lungs (29.4%) and skin (17.6%).Results:The clinical manifestations were diverse. Most patients received amphotericin B liposome and/or fluconazole as an initial option. The outcomes were curing (n=17, 58.8%), death from cryptococcal infection (n=5, 29.4%), partial relief (n=1, 5.9%) and stable disease (n=1, 5.9%). Among 10 curative cases, 2 cases died from other causes and 4 cases returned to hemodialysis with graft loss.Conclusions:Cryptococcosis is typically a late-occurring infection in kidney transplant recipients. Many factors, such as complications, nonstandard antifungal treatment, immune dysbalance, have adverse prognoses. Strengthening follow-ups, dealing with complications, validating the diagnosis early, interdepartmental cooperations, standardizing antifungal therapy and balancing immune status may improve the outcomes of cryptococcosis after kidney transplantation.

The treatment of the congenital nasolacrimal duct obstruction / 国际眼科杂志(Guoji Yanke Zazhi)

@#Congenital nasolacrimal duct obstruction(CNLDO)has an incidence rate of 5.7% and there are many clinical treatments for the disease. But these treatments have been taken very randomly. Therefore, it is still a controversial topic on how to choose the best treatment. This paper will discuss the treatments of CNLDO so as to know the characteristics of each treatment for better clinical services.

Very-long chain acyl coenzyme A dehydrogenase deficiency / 中国实用儿科杂志

Very long chain acyl-CoA dehydrogenase deficiency(VLCADD)is a disorder involving the initial step of fatty acid beta-oxidation in the mitochondrial matrix. VLCADD can present at various ages,from the neonatal period to adulthood,with symptoms including hypoglycemia,rhabdomyolysis,skeletal muscle weakness and cardiomyopathy,and poses the greatest risk of complications during intercurrent illness or after prolonged fasting. Early diagnosis,treatment,and surveillance can reduce mortality. The most common diagnostic evaluation methods are plasma acylcarnitine profiles and ACADVL gene molecular testing. Functional testing,including white blood cell or fibroblast enzyme assay,is a useful diagnostic adjunct if molecular sequencing alone is insufficient to deter-mine the diagnosis or uncharacterized mutations are identified. Treatment emphasizes the avoidance of fasting and often includes a specialized diet that is high carbohydrate/low longchain fat which is supplemented by medium chain triglycerides(MCT).very-long chain acyl coenzyme A dehydrogenase

Paying attention to screening,diagnosis and treatment of fatty acid oxidation disorders / 中国实用儿科杂志

Fatty acid oxidation disorders(FAOD)include more than 10 kinds of diseases,they all belong to autosomal recessive diseases and are common inherited metabolic diseases. Onset age of the patients with FAOD are from newborn to adult. The clinical manifestations were nonspecific,mainly manifested as liver disease,cardiomyopathy and muscle diseases. Detection of free carnitine and acylcarnitines in blood by tandem mass spectrometry and detection of gene mutations are important methods for diagnosis of such diseases. Tandem mass screening for neonatal screening is helpful for early diagnosis and early treatment of FAOD. Primary carnitine deficiency and multiple acyl-CoA dehydrogenase deficiency can be treated by specific therapeutic drugs with good effect. There are no specific drugs for other diseases,which need symptomatic treatment.

Screening and identification of GABA-producing microbes in fermentation process of Sojae Semen Praeparatum / 中国中药杂志

A high-content GABA was found in Sojae Semen Praeparatum(SSP), which is a famous traditional Chinese medicine and officially listed in Chinese Pharmacopoeia. To screen out and identify GABA-producing microbes from samples at different time points during the fermenting process of SSP, traditional microbiological methods combined with molecular biological methods were used to study the predominant GABA-producing microorganisms existing in the fermenting process of SSP. This study would lay a foundation for further studying the processing mechanism of SSP. The fermenting process of SSP was based on Chinese Pharmacopoeia(2010 edition), and samples were taken at different time points during the fermenting process of SSP. The bacteria and fungi from samples at different time points in the fermenting process of SSP were cultured, isolated and purified by selective medium, and dominant strains were selected. The dominant bacteria were cultured in the designated liquid medium to prepare the fermentation broths, and GABA in the fermentation broth was qualitatively screened out by thin-layer chromatography. The microbial fermentation broth with GABA spots in the primary screening was quantitatively detected by online pre-column derivatization and high performance liquid chromatography established in our laboratory. GABA-producing microorganisms were screened out from predominant strains, and their GABA contents in fermentation broth were determined. The DNA sequences of GABA-producing bacteria and fungi were amplified using 16S rDNA and 18S rDNA sequences by PCR respectively. The amplified products were sequenced, and the sequencing results were identified through NCBI homology comparison. Molecular biological identification was made by phylogenetic tree constructed by MEGA 7.0 software. Through the homology comparison of NCBI and the construction of phylogenetic tree by MEGA 7.0 software, nine GABA-producing microorganisms were screened out and identified in this study. They were Bacillus subtilis, Enterococcus faecium, E. avium, Aspergillus tamarii, A. flavus, A. niger, Cladosporium tenuissimum, Penicillium citrinum and Phanerochaete sordida respectively. For the first time, nine GABA-producing microorganisms were screened out and identified in the samples at different time points during the fermenting process of SSP in this study. The results indicated that multiple predominant GABA-producing microorganisms exist in the fermenting process of SSP and may play an important role in the formation of GABA.

Impact of vacuum sealing drainage on outcomes of patients with post-sternotomy mediastinitis: a systematic review / 中国实用护理杂志

Objective To examine the impact of vacuum sealing drainage on clinical outcomes of patients with post-sternotomy mediastinitis after cardiac surgery. Methods A systematic search were performed in Cochrane Library, Pubmed, Embase, China Biology Medicine(CBM), WanFang, VIP database. The quality of articles was critically appraised and data were extracted by 2 reviewers independently. Meta-analysis were conducted for the eligible researches. Results Fourteen cohort studies were inclued finally. Patients treated with VSD had significantly lower in-hospital mortalityand lower re-infection compared to those treated without VSD. While there had no significant defferences in length of ICU stay(days) and in-hospital stay (days) between VSD group and control group. Conclusions VSD therapy was associated with lower re-infection and in-hospital mortality than other conventional methods in patients with post-sternotomy mediastinitis after cardiac surgery and those results should be further tested in future research and practice.

A Randomized Controlled Clinical Trial of Treatment of Moderate and Severe Persistent Allergic Rhinitis of Spleen Deficiency Pattern by Herbal Cake-partitioned Moxibustion Combined with Rhinocort Nasal Spray / 针刺研究

OBJECTIVE: To evaluate the clinical effect of herbal cake-partitioned moxibustion combined with medication in the treatment of moderate and severe persistent allergic rhinitis (PAR) patients of spleen deficiency pattern. METHODS: Sixty patients with moderate and severe PAR of spleen deficiency pattern were randomized into medication (Rhinocort) group and herbal cake-partitioned moxibustion combined with medication group (combination group, n=30 cases in each). Medicinal cake-partitioned moxibustion was applied to Yintang (GV 29, for about 20 min), Shenque(CV 8, about 50 min), bilateral Zusanli (ST 36, about 40 min)and Hegu(LI 4, about 40 min)once every other day for successive 8 weeks. All the patients received treatment with Rhinocort nasal spray, 2 jets/day for each nostril (64 µg/jet) for 8 weeks. The patients' conditions (symptoms of nasal sufferings, sleeping, sniffle, eye, emotion, etc.) were assessed by visual analogue scale (VAS) and rhino conjunctivitis quality of life questionnaire (RQLQ, 24 items of 7 aspects, 0-6 points/item), respectively. The spleen deficiency syndrome score was determined according to "Guiding Principles for Clinical Research of New Drugs of Chinese Materia Medica" (2002). The therapeutic effect was assessed by referring to "the Diagnostic and Therapeutic Principles and Recommended Schemes for Allergic Rhinitis" (2004). RESULTS: Following the treatment, the scores of VAS, RQLQ, spleen deficiency syndrome were significantly decreased on the 4thand the 8th week of treatment in both groups in comparison with those of their own individual pre-treatment (P0.05). CONCLUSION: Herbal cake-partitioned moxibustion combined with hormone (Rhinocort) nasal spray is effective in relieving symptoms of moderate and severe PAR patients of spleen deficiency syndrome, and has a better post-effect.

Analysis of development trend of Chinese medicine health products based on history of western health products development / 中国药理学与毒理学杂志

Because of the rapidly rhythm of modern society, fast food and take-out become the main dining methods for most of young people,while the outside foods always have few types of foods and nutrition is not complete. Nutrient imbalances may cause many diseases such as gastrointestinal diseases,cardiovascular diseases,diabetes,obesity,and cancer.In this environment,health products have emerged.At present,there are a wide variety of health care products at home and abroad,which are broadly divided into three categories:traditional vitamins,emerging nutrient products,and extracts of natural plant active ingredients.In the early 1970s,the sales of health products in the United States had reached 170 million dollars.At present,it has nearly 100 billion dollars,which is almost 1/3 of the total food sales, people's demand for health products is increasing rapidly. In recent years, Chinese medicine health products become more popular in western,in fact, Chinese medicine health products have a long history of application in China and have a good reputation in the folk. Obviously, Chinese medicine health products have great potential for development.So this article mainly compared the de-velopment and state of health products between China and west countries.Based on the development of health products in western countries, this article analyzes the development trend and prospects for the development of Chinese medicine health products. It is roughly divided into three parts. The first part introduces the development reasons and history of western health products development.The sec-ond part introduces the history of Chinese health products and the current situation of Chinese medi-cine health products. The third part guesses the development trend of Chinese medicine health prod-ucts and provides some development ideas. The purpose of this airticle is to provide reference and ideas for the future research and development of traditional Chinese medicine health products.

Research on harmless disposal method and equipment for medical waste in mobile hospital / 医疗卫生装备

Objective To explore harmless disposal method and equipment for the medical waste in the mobile hospital. Methods The characteristics and harmless disposal methods were analyzed,and then a pyrolysis scheme was proposed for the medical waste in the mobile hospital.The scheme took considerations on equipment miniaturization,vehicle mounting as well as gas cleaning. Results A medical waste pyrolysis vehicle was developed for the mobile hospital, which had the performances met the requirements of 300 persons/d casualty throughput,environment protection and etc.Conclusion The developed vehicle can be used for harmless disposal of the medical waste in the mobile hospital, and is worthy promoting practically.