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  • Wiley  (129)
  • 2020-2024  (129)
  • Medicine  (129)
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  • Wiley  (129)
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  • 2020-2024  (129)
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  • Medicine  (129)
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  • 1
    Online Resource
    Online Resource
    Development and validation of a mortality predicting scoring system for severe aplastic anaemia patients receiving haploidentical allogeneic transplantation
    Wiley ; 2022
    In:  British Journal of Haematology Vol. 196, No. 3 ( 2022-02), p. 735-742
    Details
    In: British Journal of Haematology, Wiley, Vol. 196, No. 3 ( 2022-02), p. 735-742
    Abstract: Haploidentical allogeneic haematopoietic stem cell transplantation (haplo‐HSCT) is a significant alternative treatment for severe aplastic anaemia (SAA). To improve this process by modifying the risk stratification system, we conducted a retrospective study using our database. 432 SAA patients who received haplo‐HSCT between 2006 and 2020 were enrolled. These patients were divided into a training ( n  = 288) and a validation ( n  = 144) subset randomly. In the training cohort, longer time from diagnosis to transplantation, poorer Eastern Cooperative Oncology Group (ECOG) status and higher haematopoietic cell transplantation‐specific comorbidity index (HCT‐CI) score were independent risk factors for worse treatment‐related mortality (TRM) in the final multivariable model. The haplo‐HSCT scoring system was developed by these three parameters. Three‐year TRM after haplo‐HSCT were 6% [95% confidence interval (CI), 1–21%], 21% (95% CI, 7–40%), and 47% (95% CI, 20–70%) for the low‐, intermediate‐, and high‐risk group, respectively ( P   〈  0·0001). In the validation cohort, the haplo‐HSCT scoring system also separated patients into three risk groups with increasing risk of TRM: intermediate‐risk [hazard ratio (HR) 2·45, 95% CI, 0·92–6·53] and high‐risk (HR 11·74, 95% CI, 3·07–44·89) compared with the low‐risk group ( P  = 0·001). In conclusion, the haplo‐HSCT scoring system could effectively predict TRM after transplantation.
    Type of Medium: Online Resource
    ISSN: 0007-1048 , 1365-2141
    URL: Issue
    URL: Article
    DOI: 10.1111/bjh.v196.3
    DOI: 10.1111/bjh.17916
    RVK:
    XA 34100
    Language: English
    Publisher: Wiley
    Publication Date: 2022
    detail.hit.zdb_id: 1475751-5
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  • 2
    Online Resource
    Online Resource
    TMEM151A Variants Cause Paroxysmal Kinesigenic Dyskinesia: A Large‐Sample Study
    Wiley ; 2022
    In:  Movement Disorders Vol. 37, No. 3 ( 2022-03), p. 545-552
    Details
    In: Movement Disorders, Wiley, Vol. 37, No. 3 ( 2022-03), p. 545-552
    Abstract: Paroxysmal kinesigenic dyskinesia (PKD) is the most common type of paroxysmal dyskinesias. Only one‐third of PKD patients are attributed to proline‐rich transmembrane protein 2 ( PRRT2 ) mutations. Objective We aimed to explore the potential causative gene for PKD. Methods A cohort of 196 PRRT2 ‐negative PKD probands were enrolled for whole‐exome sequencing (WES). Gene Ranking, Identification and Prediction Tool, a method of case–control analysis, was applied to identify the candidate genes. Another 325 PRRT2 ‐negative PKD probands were subsequently screened with Sanger sequencing. Results Transmembrane Protein 151 ( TMEM151A) variants were mainly clustered in PKD patients compared with the control groups. 24 heterozygous variants were detected in 25 of 521 probands (frequency = 4.80%), including 18 missense and 6 nonsense mutations. In 29 patients with TMEM151A variants, the ratio of male to female was 2.63:1 and the mean age of onset was 12.93 ± 3.15 years. Compared with PRRT2 mutation carriers, TMEM151A ‐related PKD were more common in sporadic PKD patients with pure phenotype. There was no significant difference in types of attack and treatment outcome between TMEM151A ‐positive and PRRT2 ‐positive groups. Conclusions We consolidated mutations in TMEM151A causing PKD with the aid of case–control analysis of a large‐scale WES data, which broadens the genotypic spectrum of PKD. TMEM151A ‐related PKD were more common in sporadic cases and tended to present as pure phenotype with a late onset. Extensive functional studies are needed to enhance our understanding of the pathogenesis of TMEM151A ‐related PKD. © 2021 International Parkinson and Movement Disorder Society
    Type of Medium: Online Resource
    ISSN: 0885-3185 , 1531-8257
    URL: Issue
    URL: Article
    DOI: 10.1002/mds.v37.3
    DOI: 10.1002/mds.28865
    RVK:
    XA 10000
    Language: English
    Publisher: Wiley
    Publication Date: 2022
    detail.hit.zdb_id: 2041249-6
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  • 3
    Online Resource
    Online Resource
    Efficacy of Intensive Acupuncture Versus Sham Acupuncture in Knee Osteoarthritis: A Randomized Controlled Trial
    Wiley ; 2021
    In:  Arthritis & Rheumatology Vol. 73, No. 3 ( 2021-03), p. 448-458
    Details
    In: Arthritis & Rheumatology, Wiley, Vol. 73, No. 3 ( 2021-03), p. 448-458
    Abstract: To assess the efficacy of intensive acupuncture (3 times weekly for 8 weeks) versus sham acupuncture for knee osteoarthritis (OA). Methods In this multicenter, randomized, sham‐controlled trial, patients with knee OA were randomly assigned to receive electroacupuncture (EA), manual acupuncture (MA), or sham acupuncture (SA) 3 times weekly for 8 weeks. Participants, outcome assessors, and statisticians were blinded with regard to treatment group assignment. The primary outcome measure was response rate, which is the proportion of participants who simultaneously achieved minimal clinically important improvement in pain and function by week 8. The primary analysis was conducted using a Z test for proportions in the modified intent‐to‐treat population, which included all randomized participants who had ≥1 post‐baseline measurement. Results Of the 480 participants recruited in the trial, 442 were evaluated for efficacy. The response rates at week 8 were 60.3% (91 of 151), 58.6% (85 of 145), and 47.3% (69 of 146) in the EA, MA, and SA groups, respectively. The between‐group differences were 13.0% (97.5% confidence interval [97.5% CI] 0.2%, 25.9%; P = 0.0234) for EA versus SA and 11.3% (97.5% CI −1.6%, 24.4%; P = 0.0507) for MA versus SA. The response rates in the EA and MA groups were both significantly higher than those in the SA group at weeks 16 and 26. Conclusion Among patients with knee OA, intensive EA resulted in less pain and better function at week 8, compared with SA, and these effects persisted though week 26. Intensive MA had no benefit for knee OA at week 8, although it showed benefits during follow‐up.
    Type of Medium: Online Resource
    ISSN: 2326-5191 , 2326-5205
    URL: Issue
    URL: Article
    DOI: 10.1002/art.v73.3
    DOI: 10.1002/art.41584
    RVK:
    XA 10000
    RVK:
    XA 30325
    Language: English
    Publisher: Wiley
    Publication Date: 2021
    detail.hit.zdb_id: 2754614-7
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  • 4
    Online Resource
    Online Resource
    Disease Progression in Patients with Parkin ‐Related Parkinson's Disease in a Longitudinal Cohort
    Wiley ; 2021
    In:  Movement Disorders Vol. 36, No. 2 ( 2021-02), p. 442-448
    Details
    In: Movement Disorders, Wiley, Vol. 36, No. 2 ( 2021-02), p. 442-448
    Abstract: There was a paucity of follow‐up studies in the disease progression of early‐onset PD patients with Parkin mutations ( Parkin ‐EOPD). Here we conducted a longitudinal study to investigate the progression of motor and cognitive features of Parkin ‐EOPD patients. Methods Genetic analysis was performed via target sequencing and multiplex ligation‐dependent probe amplification. Thirty patients carrying homozygous or compound heterozygous Parkin mutations with at least 2 follow‐up revisions were investigated as the Parkin ‐EOPD group. Fifty‐two patients with at least 2 follow‐up revisions, who did not have any known causative PD mutations, GBA or LRRK2 risk variants, a heterozygous Parkin mutation or 2 Parkin mutations without a segregation test, were defined as the genetically undefined EOPD (GU‐EOPD) group. A linear mixed‐effect model was implemented to evaluate longitudinal changes in motor symptoms and cognition. Results At baseline, the Parkin ‐EOPD group had a lower Unified Parkinson's Disease Rating Scale score (UPDRS‐III) (off‐medication) than the GU‐EOPD group, without significant differences in cognition. A longitudinal study showed the estimated progression rate per year (standard error) of the UPDRS‐III score (off‐medication) was lower in the Parkin ‐EOPD group (0.203 [0.3162] points per year) than in the GU‐EOPD group (1.056 [0.3001] points per year). The difference in the UPDRS‐III score rate between the 2 groups was 0.853 (0.4183) ( P = 0.042). The Parkin ‐EOPD group showed better maintenance of spatial processing ability compared with the GU‐EOPD group ( P = 0.027). Conclusion Parkin ‐EOPD patients showed a slower deterioration of motor symptoms and a better spatial processing ability than GU‐EOPD patients, which suggests that subtyping according to genetic features can help predict PD progression. © 2020 International Parkinson and Movement Disorder Society
    Type of Medium: Online Resource
    ISSN: 0885-3185 , 1531-8257
    URL: Issue
    URL: Article
    DOI: 10.1002/mds.v36.2
    DOI: 10.1002/mds.28349
    RVK:
    XA 10000
    Language: English
    Publisher: Wiley
    Publication Date: 2021
    detail.hit.zdb_id: 2041249-6
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  • 5
    Online Resource
    Online Resource
    A new multimodal, i mage‐guided , robot‐assisted, interstitial brachytherapy for the treatment of head and neck tumors–A preliminary study
    Wiley ; 2020
    In:  The International Journal of Medical Robotics and Computer Assisted Surgery Vol. 16, No. 5 ( 2020-10), p. 1-5
    Details
    In: The International Journal of Medical Robotics and Computer Assisted Surgery, Wiley, Vol. 16, No. 5 ( 2020-10), p. 1-5
    Abstract: Interstitial brachytherapy (BT) is becoming an accepted treatment option for head and neck cancer patients for whom surgery poses high risks. Multimodal, image‐guided, robotic surgery has the potential to allow precise seed implantation into tumors. Our aim was to introduce a new multimodal, image‐guided surgical robot during the performance of interstitial BT for the treatment of tumors in the head and neck regions. Methods Clinical data for three patients were analyzed, retrospectively; patients had received 125 I seed implantations from July 2019 to October 2019. Multimodal, image‐guided, robotic surgery was performed in all patients. Postoperative computed tomography data were imported to software to evaluate the accuracy of the seed position and the operation times. Results The mean placement error of the 125 I seed was 1.9 ± 0.74 mm. The mean operation time is 47 minutes. Conclusion The experimental results showed that the Remebot has promise for use during BT for the head and neck.
    Type of Medium: Online Resource
    ISSN: 1478-5951 , 1478-596X
    URL: Issue
    URL: Article
    DOI: 10.1002/rcs.v16.5
    DOI: 10.1002/rcs.2133
    RVK:
    XA 49983
    Language: English
    Publisher: Wiley
    Publication Date: 2020
    detail.hit.zdb_id: 2156187-4
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  • 6
    Online Resource
    Online Resource
    The Phenotypic and Genetic Spectrum of Paroxysmal Kinesigenic Dyskinesia in China
    Wiley ; 2020
    In:  Movement Disorders Vol. 35, No. 8 ( 2020-08), p. 1428-1437
    Details
    In: Movement Disorders, Wiley, Vol. 35, No. 8 ( 2020-08), p. 1428-1437
    Abstract: Paroxysmal kinesigenic dyskinesia is a spectrum of involuntary dyskinetic disorders with high clinical and genetic heterogeneity. Mutations in proline‐rich transmembrane protein 2 have been identified as the major pathogenic factor. Objectives We analyzed 600 paroxysmal kinesigenic dyskinesia patients nationwide who were identified by the China Paroxysmal Dyskinesia Collaborative Group to summarize the clinical phenotypes and genetic features of paroxysmal kinesigenic dyskinesia in China and to provide new thoughts on diagnosis and therapy. Methods The China Paroxysmal Dyskinesia Collaborative Group was composed of departments of neurology from 22 hospitals. Clinical manifestations and proline‐rich transmembrane protein 2 screening results were recorded using unified paroxysmal kinesigenic dyskinesia registration forms. Genotype‐phenotype correlation analyses were conducted in patients with and without proline‐rich transmembrane protein 2 mutations. High‐knee exercises were applied in partial patients as a new diagnostic test to induce attacks. Results Kinesigenic triggers, male predilection, dystonic attacks, aura, complicated forms of paroxysmal kinesigenic dyskinesia, clustering in patients with family history, and dramatic responses to antiepileptic treatment were the prominent features in this multicenter study. Clinical analysis showed that proline‐rich transmembrane protein 2 mutation carriers were prone to present at a younger age and have longer attack duration, bilateral limb involvement, choreic attacks, a complicated form of paroxysmal kinesigenic dyskinesia, family history, and more forms of dyskinesia. The new high‐knee‐exercise test efficiently induced attacks and could assist in diagnosis. Conclusions We propose recommendations regarding diagnostic criteria for paroxysmal kinesigenic dyskinesia based on this large clinical study of paroxysmal kinesigenic dyskinesia. The findings offered some new insights into the diagnosis and treatment of paroxysmal kinesigenic dyskinesia and might help in building standardized paroxysmal kinesigenic dyskinesia clinical evaluations and therapies. © 2020 International Parkinson and Movement Disorder Society
    Type of Medium: Online Resource
    ISSN: 0885-3185 , 1531-8257
    URL: Issue
    URL: Article
    DOI: 10.1002/mds.v35.8
    DOI: 10.1002/mds.28061
    RVK:
    XA 10000
    Language: English
    Publisher: Wiley
    Publication Date: 2020
    detail.hit.zdb_id: 2041249-6
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  • 7
    Online Resource
    Online Resource
    Application of 5G technology to conduct tele‐surgical robot‐assisted laparoscopic radical cystectomy
    Wiley ; 2022
    In:  The International Journal of Medical Robotics and Computer Assisted Surgery Vol. 18, No. 4 ( 2022-08)
    Details
    In: The International Journal of Medical Robotics and Computer Assisted Surgery, Wiley, Vol. 18, No. 4 ( 2022-08)
    Abstract: The aim of this study was to test the effectiveness, safety and stability of the 5G communication technology in clinical laparoscopic telesurgery. Methods An ultra‐remote radical cystectomy (network communication distance of nearly 3000 km) was performed on patient diagnosed with T2N0M0 stage bladder cancer using a domestically produced “MicroHand” surgical robot. Results The network delay, operative time, blood loss, intraoperative complications, postoperative recovery, and hospitalisation time were recorded. The 5G network was used throughout the operation, with an average total delay of 254 ms. The operation went well and the patient recovered smoothly. Conclusions Ultra‐remote clinical laparoscopic surgery can be performed safely and smoothly. More importantly, our model can provide insights for promoting the future development of telesurgery in China.
    Type of Medium: Online Resource
    ISSN: 1478-5951 , 1478-596X
    URL: Issue
    URL: Article
    DOI: 10.1002/rcs.v18.4
    DOI: 10.1002/rcs.2412
    RVK:
    XA 49983
    Language: English
    Publisher: Wiley
    Publication Date: 2022
    detail.hit.zdb_id: 2156187-4
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  • 8
    Online Resource
    Online Resource
    Reshaping the murine immunoglobulin heavy chain repertoire with bovine DH genes
    Wiley ; 2022
    In:  Immunology Vol. 165, No. 1 ( 2022-01), p. 74-87
    Details
    In: Immunology, Wiley, Vol. 165, No. 1 ( 2022-01), p. 74-87
    Abstract: Having a limited number of VH segments, cattle rely on uniquely long DH gene segments to generate CDRH3 length variation (3–70 aa) far greater than that in humans or mice. Bovine antibodies with ultralong CDRH3s ( 〉 50 aa) possess unusual structures and abilities to bind to special antigens. In this study, we replaced most murine endogenous DH segments with bovine DH genes, generating a mouse line termed B‐DH. The use of bovine DH genes significantly increased the length variation of CDRH3 and consequently the Ig heavy chain repertoire in B‐DH mice. However, no ultralong CDRH3 was observed in B‐DH mice, suggesting that other factors, in addition to long DH genes, are also involved in the formation of ultralong CDRH3. The B‐DH mice mounted a normal humoral immune response to various antigens, although the B‐cell developmental paradigm was obviously altered compared with wild‐type mice. Additionally, B‐DH mice are not predisposed to the generation of autoantibodies despite the interspecies DH gene replacement. The B‐DH mice reported in this study provide a unique model to answer basic questions regarding the synergistic evolution of DH and VH genes, VDJ recombination and BCR selection in B‐cell development.
    Type of Medium: Online Resource
    ISSN: 0019-2805 , 1365-2567
    URL: Issue
    URL: Article
    DOI: 10.1111/imm.v165.1
    DOI: 10.1111/imm.13407
    RVK:
    XA 10000
    Language: English
    Publisher: Wiley
    Publication Date: 2022
    detail.hit.zdb_id: 2006481-0
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  • 9
    Online Resource
    Online Resource
    Evaluation of the Effect of Sodium‐Glucose Cotransporter 2 Inhibition on Fracture Risk: Evidence From Mendelian Randomization and Genetic Association Study
    Wiley ; 2023
    In:  Journal of Bone and Mineral Research
    Details
    In: Journal of Bone and Mineral Research, Wiley
    Abstract: This study aims to evaluate the causal effect of sodium‐glucose cotransporter 2 (SGLT2) inhibition on bone mineral density (BMD), osteoporosis, and fracture risk using genetics. Two‐sample Mendelian randomization (MR) analyses were performed utilizing two sets of genetic variants as instruments (six and two single‐nucleotide polymorphisms [SNPs]) associated with SLC5A2 gene expression and glycated hemoglobin A1c levels. Summary statistics of BMD from the Genetic Factors for Osteoporosis consortium (BMD for total body, n  = 66,628; femoral neck, n  = 32,735; lumbar spine, n  = 28,498; forearm, n  = 8143) and osteoporosis (6303 cases, 325,717 controls) and 13 types of fracture (≤17,690 cases, ≤328,382 controls) data from the FinnGen study were obtained. One‐sample MR and genetic association analyses were conducted in UK Biobank using the individual‐level data of heel BMD ( n  = 256,286) and incident osteoporosis (13,677 cases, 430,262 controls) and fracture (25,806 cases, 407,081 controls). Using six SNPs as the instrument, genetically proxied SGLT2 inhibition showed little evidence of association with BMD of total body, femoral neck, lumbar spine, and forearm (all p  ≥ 0.077). Similar results were observed using two SNPs as instruments. Little evidence was found for the SGLT2 inhibition effect on osteoporosis (all p  ≥ 0.112) or any 11 major types of fracture (all p  ≥ 0.094), except for a nominal significance for fracture of lower leg ( p  = 0.049) and shoulder and upper arm ( p  = 0.029). One‐sample MR and genetic association analysis showed that both the weighted genetic risk scores constructed from the six and two SNPs were not causally associated with heel BMD, osteoporosis, and fracture (all p  ≥ 0.387). Therefore, this study does not support an effect of genetically proxied SGLT2 inhibition on fracture risk. © 2023 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).
    Type of Medium: Online Resource
    ISSN: 0884-0431 , 1523-4681
    URL: Article
    DOI: 10.1002/jbmr.4880
    RVK:
    XA 52230
    Language: English
    Publisher: Wiley
    Publication Date: 2023
    detail.hit.zdb_id: 2008867-X
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  • 10
    Online Resource
    Online Resource
    Clinical utiliy of a model‐based piperacillin dose in neonates with early‐onset sepsis
    Wiley ; 2022
    In:  British Journal of Clinical Pharmacology Vol. 88, No. 3 ( 2022-03), p. 1179-1188
    Details
    In: British Journal of Clinical Pharmacology, Wiley, Vol. 88, No. 3 ( 2022-03), p. 1179-1188
    Abstract: Early‐onset sepsis (EOS) is a common disease in neonates with a high morbidity and mortality rate. Piperacillin/tazobactam has been used extensively and empirically for EOS treatment without clinically validated dosing regimens, although the population pharmacokinetics (PPK) of piperacillin in neonates has been reported. Therefore, we wanted to study the effectiveness and tolerance of a PPK model‐based dosing regimen of piperacillin/tazobactam in EOS patients. Methods A prospective, single‐centre, phase II clinical study of piperacillin/tazobactam in neonates with EOS was conducted. The dosing regimen (90 mg·kg −1 , q8h) was determined based on a previous piperacillin PPK model in young infants using NONMEM v7.4. The pharmacodynamics (PD) target (70%fT  〉  MIC, free drug concentration above MIC during 70% of the dosing interval) attainment was calculated using NONMEM combined with an opportunistic sampling design. The clinical treatment data were collected. Results A total of 52 neonates were screened and 49 neonates completed their piperacillin/tazobactam treatment course and were included in this analysis. The median (range) values of postmenstrual age were 33.57 (range 26.14–41.29) weeks. Forty‐seven (96%) neonates reached their PD target. Eight (16%) neonates experienced treatment failure clinically. The mean (SD, range) duration of treatment and length of hospitalization were 100.1 (62.2, 36.2–305.8) hours and 31 (30, 5–123) days. There were no obvious adverse events and no infection‐related deaths occurred in the first month of life. Conclusions A model‐based dosing regimen of piperacillin/tazobactam was evaluated clinically, was tolerated well and was determined to be effective for EOS treatment.
    Type of Medium: Online Resource
    ISSN: 0306-5251 , 1365-2125
    URL: Issue
    URL: Article
    DOI: 10.1111/bcp.v88.3
    DOI: 10.1111/bcp.15058
    RVK:
    XA 33650
    Language: English
    Publisher: Wiley
    Publication Date: 2022
    detail.hit.zdb_id: 1498142-7
    SSG: 15,3
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