Outcomes in patients with rheumatoid versus osteoarthritis for total hip arthroplasty: A meta-analysis and systematic review.

OBJECTIVE: Total hip arthroplasty(THA) in patients with osteoarthritis(OA) are considered to be a successful procedure, but with little being known about outcomes in patients with rheumatoid arthritis (RA). The aim of this study was to compare rates of THA complications in patients with RA versus OA. METHODS: The PUBMED, EMBASE, and Cochrane Library databases were searched for all available studies comparing the outcomes of THA in RA and OA patients. The quality of the studies was scored using the Newcastle-Ottawa scale (NOS). Meta-analysis was performed using Stata 12.0 software. RESULTS: Twenty-three studies with a total of 877,695 patients were included. Results showed that patients in the RA group were more likely to have revision (OR=1.15, 95% CI, 1.02-1.29; P = 0.019), hip dislocation (OR=2.31, 95% CI, 1.67-3.20; P<0.01), periprosthetic infection (OR=1.44, 95% CI, 1.29-1.61; P<0.01), wound infection (OR=2.15, 95% CI, 1.19-3.90; P = 0.01), and revision for late infection (ES=1.71, 95% CI, 0.54-1.80; P<0.01) than those in the OA group. Subgroup analysis showed no significant difference in revision rates (OR=1.12, 95%CI, 0.94-1.31; P = 0.21) between the two groups in studies published before 2010, while studies published after 2010 showed significantly higher revision rates (OR=1.15, 95%CI, 1.02-1.30; P = 0.04) in the RA group. There were no significant differences in mortality (OR=1.20,95% CI, 0.89-1.61; P = 0.23), deep vein thrombosis (OR=0.52, 95% CI, 0.12-2.34; P = 0.40) between the groups. THA can improve the function and reduce pain of patients with RA. CONCLUSION: Our study indicated that compared with OA, patients with RA are at higher risk of complications after THA. But THA can relieve pain, improve function and the quality of life of patients with RA, and did not increase mortality risk. Therefore, THA can be used as a treatment option for RA patients who failed to respond to conservative treatment. Patients should be fully informed of the benefits and possible risks of surgery, individualized treatment strategy should be made.

Outcomes of hip arthroplasty in Parkinson's disease: a meta-analysis and systematic review.

PURPOSE: Hip arthroplasty in Parkinson's disease (PD) is considered to be associated with higher mortality, perioperative complications, and worse clinical outcomes. The purpose of this study was to evaluate the outcomes of hip arthroplasty in PD. METHODS: The PUBMED, EMBASE, and Cochrane Library databases were searched for all available studies comparing the outcomes of hip arthroplasty in PD and non-PD patients. The quality of the studies was scored using the Newcastle-Ottawa scale (NOS). Meta-analysis was performed using Stata 12.0 software. RESULTS: Six retrospective cohort studies with a total of 59,878 patients were included. Among them, there were 15,313 patients in the Parkinson's group and 44,565 in the non-Parkinson's group. Results showed that patients in the Parkinson's group were more likely to have periprosthetic fracture (OR = 7.93, 95% CI, 1.96-32.04; P = 0.004), revision surgery (OR = 2.33, 95% CI, 1.37-3.98; P = 0.002), deep vein thrombosis (OR = 2.34, 95% CI, 1.56-3.49; P < 0.01), and urinary tract infection (OR = 1.34, 95% CI, 1.20-1.49; P < 0.01) than those in the non-Parkinson's group. The Parkinson's group had a longer hospital stay (WMD = 0.38, 95% CI, 0.24-0.52; P < 0.01) and higher hospitalization costs (WMD = 2487.08, 95% CI, 225.99-4748.17; P = 0.03), but there was no significant difference in short-term postoperative mortality (OR = 1.07, 95% CI, 0.65-1.73; P = 0.80). Hip arthroplasty can significantly improve the function of patients with PD and reduce pain. CONCLUSION: Although hip arthroplasty in patients with PD is associated with a higher revision rate and potential risk, it does not increase short-term mortality and can reduce pain, improve function, and improve quality of life. The periprosthetic fracture risk and revision rate are significantly higher and should be accounted for when deciding on operative treatment and communicated to patient with PD.

Environmental exposure during pregnancy and the risk of childhood allergic diseases.

BACKGROUND: Allergic diseases are one of the most common and important diseases that can exert hazardous effects on children's health. The prevalence of allergic diseases in childhood is gradually increasing all over the world in recent decades. Known causes of these diseases include anomalous immune responses and allergic inflammatory reactions, but the causes of allergic diseases in childhood are complex. DATA SOURCES: PubMed, Cochrane Library, Embase and Web of Science were searched for articles focusing on environmental exposure during pregnancy and the risk of childhood allergic diseases, including asthma and atopic dermatitis, and the possible underlying mechanism. RESULTS: In terms of environmental factors, allergic diseases in childhood are closely related to environmental chemical exposure during pregnancy, including bisphenols, phthalates acid esters, perfluorochemicals, polybrominated diphenyl ethers, and polychlorinated biphenyls. However, allergic diseases in childhood are also closely associated with maternal dietary nutrition, maternal intake of drugs, such as acetylsalicylic acid (aspirin), paracetamol and antibiotics, and maternal lifestyle. CONCLUSIONS: Several harmful environmental factors during pregnancy can result in the interruption of the function of helper T cells (Th1/Th2), cytokines and immunoglobulins and may activate allergic reactions, which can lead to allergic diseases during childhood.

[Formation history of Dao-di herbs in Dabie Mountains].

Dabie Mountains, a unique transition area of subtropical and warm temperate zone, span Anhui, Hubei and Henan pro-vinces with a humid and suitable climate. It is rich in traditional Chinese medicine resources including many Dao-di herbs, and has a profound culture of traditional Chinese medicine with many herbalists in the past. This paper combed the historical geography of Dabie Mountains and development vein of medicinal materials recorded in history, and a textual research on the historical evolution of Dao-di herbs and special herbs in Dabie Mountains was carried out. The administrative region of Dabie Mountains has been changing constantly in history, which includes 25 counties and districts of six cities in Anhui, Henan and Hubei provinces at present. Dabie Mountains abound in a variety of Chinese herbal medicines, which have been recorded in herbal works in the past dynasties and local chronicles in detail. The recorded Dao-di herbs Dendrobium huoshanense, Artemisia argyi, and Poria cocos are well-known in China, and the output of Gastrodia elata, Ganoderma lucidum, and P. cocos is in the forefront of the country. Additionally, there are still many local special herbs emerging in modern times. In a word, this paper reviewed development of historical geography and ancient records of medicinal materials in Dabie Mountains, and made a textual research on the Dao-di herbs and special herbs, will provide a reference for the mo-dern research and intellectual property protection of Dao-di herbs in Dabie Mountains.

The impact of diabetes mellitus on carotid artery stenting: a meta-analysis.

Diabetes is considered to be one of the important factors affecting the prognosis of patients undergoing carotid endarterectomy (CEA), but its effect on carotid stenting (CAS) has not been determined. We performed this analysis to evaluate the impact of diabetes mellitus (DM) on carotid stenting. Studies were searched in the MEDLINE, EMBASE, and Cochrane Library databases for all available studies comparing the outcomes of diabetic and nondiabetic patients who underwent CAS. A meta-analysis was performed using the Mantel-Haenszel model to calculate odds ratios (ORs) with RevMan 5.3 software. Five studies with high quality were ultimately included. A total of 3364 patients underwent CAS, including 1042 diabetic patients and 2322 nondiabetic patients. Meta-analysis showed that there was no statistically significant difference in the risk of perioperative stroke(OR,1.20;95%CI,0.74-1.97;P = 0.46;I2 = 24%), transient ischemic attack(TIA)(OR,1.46;95%CI,0.80-2.66;P=0.22;I2=37%), myocardial infarction(MI)(OR,1.12;95%CI,0.47-2.67;P=0.80;I2=0%), mortality (OR,1.77;95%CI,0.91-3.42;P = 0.09;I2=0%), and composite of stroke or death (OR,1.26;95%CI,0.87-1.81;P = 0.22;I2=35%) between diabetic and nondiabetic patients receiving CAS. Sensitivity analysis showed that the meta-analysis results of all outcome events were stable. Compared with nondiabetic patients, diabetes did not increase the risk of the following: perioperative stroke, TIA, MI, mortality, and composite of stroke or death in patients receiving CAS. This study may be used to guide carotid artery stenosis patients with diabetes to choose the optimal treatment option. However, due to the limited number of studies and the different technical levels of institutions, the results should be interpreted caution.

Validation of the predictive model for seizure recurrence after withdrawal of antiepileptic drugs.

PURPOSE: The purpose of this study was to validate the practicability of Lamberink's prediction model in risk assessment of antiepileptic drug (AED) withdrawal in a real, seizure-free population and to find a practical cutoff value to guide clinical withdrawal. METHODS: A group of seizure-free patients from West China Hospital was recruited. Each patient had been seizure-free for at least two years. The seizure recurrence risk among the patients was calculated by an online AED withdrawal risk calculator. The predictive ability of Lamberink's model was assessed by analyzing discrimination and calibration with receiver operating characteristic (ROC) curves and calibration plots, respectively. RESULTS: A total of 184 seizure-free patients received risk evaluation, all of whom were followed up for at least two years or had an earlier report of seizure relapse. Of these patients, 128 patients were followed up for at least five years or had an earlier report of relapse within five years. Sixty-two of 184 (33.7%) patients relapsed within two years, while 81 of 184 (44.0%) patients relapsed within five years after the start of AEDs' withdrawal. Cox regression analyses showed that seizure duration before remission and the age of seizure onset were independent predictors of relapse at two years. For predictors of recurrence at five years, seizure duration before remission, age at onset, and withdrawal were significant. For discrimination, ROC curve analysis showed that the area under the curve (AUC) for the seizure recurrence within two and five years was 0.605 (95% confidence interval [CI]: 0.518-0.692, p = 0.02) and 0.656 (95% CI: 0.563-0.749, p = 0.003), respectively. For calibration, it was poor in two-year prediction; the observed number was considerably lower than the predicted number. However, the calibration plot showed good calibration with the five-year prediction except for the second, fourth, and eighth deciles. With a cutoff two-year recurrence risk of 47%, the model had a sensitivity of 0.758 and a specificity of 0.410; the largest Youden index was 1.168. With a cutoff five-year recurrence risk of 77%, the model had a sensitivity of 0.358 and a specificity of 0.979; the largest Youden index was 1.337. CONCLUSIONS: Lamberink's prediction model has a general discrimination ability. The model overestimated the actual recurrence events when predicting the two-year recurrence risk, but it showed relatively good calibration with five-year prediction. The cutoff value found in this study may be used to guide patients and clinicians towards a decision regarding the withdrawal of AEDs. The model appears to be a useful tool for predicting seizure recurrence for the five-year recurrence risk.

Linguistic Characteristics of Mandarin-Speaking Huntington's Disease Patients.

Objective Linguistic problem is common in Huntington's disease (HD) patients. It has been studied before in native speakers of alphabetic languages, such as English. As a hieroglyphic language, Chinese differs from alphabetic languages in terms of phonology, morphology, semantics and syntax. We aimed to investigate the linguistic characteristics of manifest HD in native speakers of Mandarin. Meanwhile, we expected to explore the linguistic differences associated with cortical or subcortical pathology.Methods Five HD patients and five Alzheimer's disease (AD) patients matched in age, gender, disease course and educational level were enrolled. All the participants were Mandarin native speakers. All finished history inquiry, physical examination, basic test, genetic test and neuropsychological assessment. Language evaluation was performed by Aphasia Battery of Chinese.Results HD patients had a mean disease course of 5.4±2.97 (range, 2-10) years. They showed a linguistic disorder close to transcortical motor aphasia. They exhibited prominent phonological impairment, as well as slight semantic and syntactic abnormality. Tonic errors were found in speech. Character structural errors and substitutions were detected in writing. In comparison, AD patients showed a more severe linguistic impairment, characterized by global aphasia with more semantic errors. Conclusion Mandarin-speaking HD patients have a transcortical motor aphasia-like disturbance with prominent phonological impairment, whereas AD patients have a more severe global aphasia with salient semantic impairment.

Therapeutic effect of intravenous levetiracetam in status epilepticus: A meta-analysis and systematic review.

PURPOSE: We performed this analysis to evaluate the efficacy and safety of intravenous levetiracetam (IV LEV) in patients with status epilepticus. METHOD: Studies were searched in MEDLINE, EMBASE, and the Cochrane Central Register of Controlled Trials for available randomized controlled trials(RCTs) comparing the efficacy and/or safety of IV LEV with other antiepileptic drugs (AEDs). Meta-analysis was performed using the random-effects model to calculate risk ratio with the RevMan 5.3 software. RESULTS: Six RCTs with a total of 543 patients were included. There was no significant differences in clinical seizure cessation and hospital mortality, either between IV LEV and IV phenytoin (PHT) or between IV LEV and IV valproate (VPA). Compared with IV PHT, IV LEV had a lower risk of poor neurological outcome. For IV LEV compared with IV lorazepam (LOR), no significant difference in efficacy was found, but IV LEV patients had significantly lower need for ventilatory assistance. Adding IV LEV to clonazepam (CNP), compared with adding placebo showed no significant differences in seizure cessation at 15 min. CONCLUSIONS: Our results suggested that IV LEV was comparable to IV PHT,VPA, or LOR in efficacy, and IV LEV as add-on therapy of CNP had no superiority in seizure cessation than CNP plus placebo. IV LEV may have a better tolerability than other AEDs do. More RCTs are needed to validate the role of IV LEV in status epilepticus.

[Identification of Corydalis yanhusuo,C. turtschaninovii,C. decumbens by allele-specific PCR].

To establish a DNA molecular markers method for identification of Corydalis yanhusuo,C. turtschaninovii and C. decumbens,the mat K,trn G and psb A-trn H sequences of 56 samples from 14 species of C. yanhusuo,C. turtschaninovii,C. decumbens and their related species were obtained by sequencing. The SNP loci were obtained by Bio Edit 7. 2. 2 software. The primers for AS-PCR identification were designed based on the mutation sites,and the conditions of PCR were optimized to identify C. yanhusuo,C. turtschaninovii,and C. decumbens according to the specific bands. The results showed that the amount of template( 0. 6-1 200 ng)and annealing temperature( 42-60 ℃) had little influence on the amplification results,and the number of cycles had much influence on the amplification results. When the number of cycles was 20,the specific bands of 297 bp( mat K),353 bp( trn G) and 544 bp( mat K) were amplified from C. yanhusuo,C. turtschaninovii and C. decumbens,respectively. The method established in this study had a minimum detection limit of 6 ng for C. yanhusuo,60 ng for C. decumbens and less than 0. 6 ng for C. turtschaninovii. Thus,the allelespecific PCR method established in the research can specifically identify C. yanhusuo,C. turtschaninovii,and C. decumbens.

Tissue-Specific Metabolite Profiling on the Different Parts of Bolting and Unbolting Peucedanum praeruptorum Dunn (Qianhu) by Laser Microdissection Combined with UPLC-Q/TOF⁻MS and HPLC⁻DAD.

BACKGROUND: Qianhu is a traditional Chinese medicine. It is thought that Qianhu roots will harden after bolting and not be suitable for medicinal purposes. Bolting Qianhu and unbolting Qianhu are referred to as "Xiong Qianhu" and "Ci Qianhu," respectively. In this study, the properties, microscopic and chemical characteristics of Ci Qianhu and Xiong Qianhu roots were compared using fluorescence microscopy, laser microdissection coupled with ultra-high-performance liquid chromatography-quadrupole/time-of-flight mass spectrometry, and high-performance liquid chromatography with diode-array detection. RESULTS: Microscopy results showed that the area of secondary xylem in the root increased after bolting, with the cork and secretory canals showing strong fluorescence intensity. A total of 34 peaks, mostly pyranocoumarins, were identified in the tissues of Ci Qianhu and Xiong Qianhu. The secretory canals contained the highest variability of coumarins, whereas the secondary xylem contained the least coumarins. Moreover, seven coumarins, especially the pyran- coumarin, decreased after bolting. Generally, both before and after bolting, coumarin level was the highest in the bark, followed by the middle part, and the lowest in the inner part. CONCLUSION: Thus, it was indicated that the area of secondary xylem increased after bolting, however the coumarin variant and content decreased in the secondary xylem of Qianhu. The result shows that the quality of Qianhu decreases after bolting, which supports the viewpoint that Xiong Qianhu is not suitable for medicinal use.

Determination of the species status of Fallopia multiflora, Fallopia multiflora var. angulata and Fallopia multiflora var. ciliinervis based on morphology, molecular phylogeny, and chemical analysis.

Relationships among Fallopia multiflora (Thunb.) Haraldson., F. multiflora var. angulata (S. Y. Liu) H. J. Yan, Z. J. Fang & Shi Xiao Yu., and F. multiflora var. ciliinervis (Nakai) Yonekura & H. Ohashi. were determined based on macroscopic and microscopic morphology, molecular phylogeny, and chemical analysis. The macroscopic and microscopic morphologies of root tubers or rhizomes, stems, and leaves were compared among the three taxa. The content of 11 chemical components (catechin, polydatin, stilbene glucoside, emodin, emodin-8-O-ß-D-glucopyranoside, rhein, chrysophanol, aloe-emodin, quercetin, physcion, and resveratrol) in the three taxa was determined by HPLC, and the chemical diversity was further evaluated by principal component and hierarchical cluster analyses. Molecular phylogenies were mapped using two chloroplast markers (matK and the psbA-trnH intergenic region) and a nuclear ribosomal marker [internal transcribed spacer 2 (ITS2) region]. Analyses of macroscopic and microscopic morphological characteristics revealed that the subterranean organs of F. multiflora and F. multiflora var. angulata are root tubers, whereas those of F. multiflora var. ciliinervis are rhizomes. In the phylogenetic trees, F. multiflora and F. multiflora var. angulata were clustered into a clade based on the combine matK + psbA-trnH sequence, with neighbour-joining, maximum likelihood, and Bayesian inference bootstrap support values of 99, 85, and 0.99, respectively. In addition, there were obvious differences in the chemical compositions of F. multiflora, F. multiflora var. angulata and F. multiflora var. ciliinervis. The root tubers of F. multiflora contain higher levels of stilbene glucoside and catechin, but lower levels of polydatin and anthraquinone compounds. In contrast to F. multiflora, the rhizomes of F. multiflora var. ciliinervis contain higher levels of polydatin and anthraquinone compounds, but lack stilbene glucoside. The content of all 11 assessed components was lower in F. multiflora var. angulata than in F. multiflora and F. multiflora var. cillinervis. Principal component and hierarchical cluster analyses revealed that F. multiflora and F. multiflora var. angulata individuals were clustered into a single clade, whereas F. multiflora var. ciliinervis individuals were clustered into a single clade separate from that containing F. multiflora and F. multiflora var. angulata individuals. On the basis of the results of our morphological, molecular phylogeny, and chemical analyses, we tentatively conclude that F. multiflora var. ciliinervis is an independent species, whereas F. multiflora var. angulata should be considered as a variety of F. multiflora.

Predictors of seizure reduction outcome after vagus nerve stimulation in drug-resistant epilepsy.

PURPOSE: To evaluate the predictors of seizure reduction outcome after vagus nerve stimulation (VNS) in patients with drug-resistant epilepsy (DRE). METHODS: A meta-analysis was performed using relevant research from databases such as PubMed, Embase, Cochrane Online Library, and Clinicaltrials.gov. Studies were selected according to predefined inclusion and exclusion criteria. The quality of studies was evaluated by using the Newcastle-Ottawa Scale (NOS) scale. All data was pooled by STATA 12.0 software for meta-analysis. RESULTS: The review considered 1281 articles, and 16 articles with NOS score ≥6 were included in the analysis. The meta-analysis showed that at 6 m, 1, 2, 3, 4, 6 and 12 years after implantation, 33.99, 43.42, 46.50, 63.31, 52.71, 54.64, 70.37 and 82.90% of patients exhibited >50% reduction of seizure frequency after VNS. The duration of epilepsy showed a significant difference between the good responders and poor responders (p = 0.038), whereas age at VNS implantation (p = 0.305), age at seizure onset (p = 0.530), seizure type (p = 0.11), etiology (p = 0.187), and history of previous epilepsy surgery (p = 0.075) were not predictors of seizure reduction outcome after VNS. Several features about the electroencephalogram (EEG) feature and heart rhythm complexity (HRV) have not been analyzed by a sufficient number of studies. CONCLUSIONS: DRE patients with shorter duration of epilepsy may be better candidates for VNS rather than those who are younger at onset and implantation. Several EEG or HRV features may have predictive value but more research is needed.

[Growth rings in roots of seven Sect. Paeonia species and its application on identification of Paeoniae Radix Rubra].

The growth years of medicinal materials are closely related to their quality, and "Herb-chronology" has been used to determine the growth years of perennial dicotyledonous plants in recent years. On the basis of conventional paraffin section and freehand section, the anatomical study on roots of seven Sect. Paeonia species and main roots of cultivated Paeonia lactiflora was conducted in this paper. The results showed that, there existed some differences in microstructure of the seven species such as P. lactiflora, P. obovata, P. veitchii, P. mairei, P. anomala, P. sinjiangensis and P. anomala var. intermedia, and this could be used to distinguish different species. In the roots of seven Sect. Paeonia species, distinct growth rings were formed because that the different diameters or density of xylem vessels in the secondary xylem formed clusters and arranged interrupted rings in tangential direction. There were growth rings in the main roots of P. lactiflora cultivated 1-4 years in Siping, Jilin, which were all consistent with their growth years. Due to the similar growth characteristics between wild Sect. Paeonia species and cultivated P. lactiflora, the growth rings can provide a basis for the age identification and lay the foundation for the quality evaluation of Paeoniae Radix Rubra.

Variation of DNA methylation patterns associated with gene expression in rice (Oryza sativa) exposed to cadmium.

We report genome-wide single-base resolution maps of methylated cytosines and transcriptome change in Cd-exposed rice. Widespread differences were identified in CG and non-CG methylation marks between Cd-exposed and Cd-free rice genomes. There are 2320 non-redundant differentially methylated regions detected in the genome. RNA sequencing revealed 2092 DNA methylation-modified genes differentially expressed under Cd exposure. More genes were found hypermethylated than those hypomethylated in CG, CHH and CHG (where H is A, C or T) contexts in upstream, gene body and downstream regions. Many of the genes were involved in stress response, metal transport and transcription factors. Most of the DNA methylation-modified genes were transcriptionally altered under Cd stress. A subset of loss of function mutants defective in DNA methylation and histone modification activities was used to identify transcript abundance of selected genes. Compared with wide type, mutation of MET1 and DRM2 resulted in general lower transcript levels of the genes under Cd stress. Transcripts of OsIRO2, OsPR1b and Os09g02214 in drm2 were significantly reduced. A commonly used DNA methylation inhibitor 5-azacytidine was employed to investigate whether DNA demethylation affected physiological consequences. 5-azacytidine provision decreased general DNA methylation levels of selected genes, but promoted growth of rice seedlings and Cd accumulation in rice plant.

Eight novel mutations in the ABCD1 gene and clinical characteristics of 25 Chinese patients with X-linked adrenoleukodystrophy.

BACKGROUND: X-linked adrenoleukodystrophy (X-ALD) is a fatal neurodegenerative disease caused by mutations in the adenosine triphosphate-binding cassette D1 (ABCD1) gene. This study aimed to retrospectively investigate the clinical characteristics of 25 patients with X-ALD including members of large pedigrees, to analyze ABCD1 gene mutations, the effect of gene novel variants on ALD protein (ALDP) structure and function, and to expand gene mutation spectrum of Chinese patients. METHODS: Twenty-five male patients diagnosed with X-ALD were enrolled in this study. The clinical characteristics of the patients were retrospectively summarized by reviewing medical records or telephone consultation. ABCD1 gene mutations were analyzed. The pathogenicity of novel missense variants was analyzed using cobalt constraint-based multiple protein alignment tool, polymorphism phenotyping, sorting intolerant from tolerant, Align-Grantham variation and Grantham deviation, and Swiss-Program Database Viewer 4.04 software, respectively. RESULTS: Childhood cerebral form ALD (CCALD) is the most common phenotype (64%) in the 25 patients with X-ALD. The progressive deterioration of neurological and cognitive functions is the main clinical feature. The demyelination of the brain white matter and elevated plasma very long chain fatty acids (VLCFAs) were found in all patients. Different phenotypes were also presented within family members of the patients. Twenty-two different mutations including 8 novel mutations in the ABCD1 gene were identified in the 25 patients. Of the mutations, 63.6% were missense mutations and 34.8% located in exon 1. The amino acid residues of three novel missense mutations in eight species were highly conserved, and were predicted to be "probably" damaging to ALDP function. The other five novel mutations were splice, nonsense, deletion or duplication mutations. CONCLUSIONS: CCALD is the most common phenotype (64%) in our patients with X-ALD. Eight novel mutations in the ABCD1 gene identified are disease-causing mutations. Brain magnetic resonance imaging and plasma VLCFA determination should be performed for the patients who present with progressive deterioration of neurological development.

[Distribution laws of 5 compounds in rhizome and root of Polygonum cuspidate].

To understand the distribution and accumulation rules of polydatin, resveratrol, anthraglycoside B, emodin and physicion in different tissue structure of rhizome and root of Polygonum cospidatum, the content of 5 active compounds were analyzed simultaneously by HPLC, based on plant anatomy and histochemistry. The rhizome and root consist of different tissues, with an increased diameter, the proportions of the secondary xylem and phloem have increased. Resveratrol and polydatin mainly distributed in the pith, the secondary phloem and periderm of rhizome, and the secondary phloem and periderm of the root, while emodin and anthraglycoside B concentrated in the secondary structure and pith of rhizome mostly. In different thickness of the measured samples, the total contents of 5 compounds were correspondingly higher in thinner rhizome and root than those in the coarse ones.