Browsing by Author "Kanokwan Boonyapisit"

Filter results by typing the first few letters
Now showing 1 - 12 of 12
  • No Thumbnail Available
    PublicationMetadata only
    Can we predict final outcome of internal medicine residents with in-training evaluation.
    (2011-02-01) Nitipatana Chierakul; Supot Pongprasobchai; Kanokwan Boonyapisit; Yingyong Chinthammitr; Manop Pithukpakorn; Adisak Maneesai; Apiradee Srivijitkamol; Pornpan Koomanachai; Ajchara Koolvisoot; Tawesak Tanwandee; Chairat Shayakul; Udom Kachintorn; Mahidol University
    To assess the predictive value of in-training evaluation for determining future success in the internal medicine board certifying examination. Ninety-seven internal medicine residents from Faculty of Medicine Siriraj Hospital who undertake the Thai Board examination during the academic year 2006-2008 were enrolled. Correlation between the scores during internal medicine rotation and final scores in board examination were then examined. Significant positive linear correlation was found between scores from both written and clinical parts of board certifying examination and scores from the first-year summative written and clinical examinations and also the second-year formative written examination (r = 0.43-0.68, p < 0.001). Monthly evaluation by attending staffs was less well correlated (r = 0.29-0.36) and the evaluation by nurses or medical students demonstrated inverse relationship (r = -0.2, p = 0.27 and r = -0.13, p = 0.48). Some methods of in-training evaluation can predict successful outcome of board certifying examination. Multisource assessments cannot well extrapolate some aspects of professional competences and qualities.
  • No Thumbnail Available
    PublicationMetadata only
    Congenital myasthenic syndromes in the Thai population: Clinical findings and novel mutations
    (2020-10-01) Nalinee Pattrakornkul; Chupong Ittiwut; Ponghatai Boonsimma; Kanokwan Boonyapisit; Chaiyos Khongkhatithum; Oranee Sanmaneechai; Kanya Suphapeetiporn; Vorasuk Shotelersuk; Chulalongkorn University; King Chulalongkorn Memorial Hospital, Faculty of Medicine Chulalongkorn University; Faculty of Medicine, Ramathibodi Hospital, Mahidol University; Faculty of Medicine, Siriraj Hospital, Mahidol University
    © 2020 Elsevier B.V. Congenital myasthenic syndromes (CMS) comprise a heterogeneous group of genetic disorders of the neuromuscular junction. Next generation sequencing has been increasingly used for molecular diagnosis in CMS patients. This study aimed to identify the disease-causing variants in Thai patients. We recruited patients with a diagnosis of CMS based on clinical and electrophysiologic findings, and whole exome sequencing was performed. Thirteen patients aged from 2 to 54 years (median: 8 years) from 12 families were enrolled. Variants were identified in 9 of 13 patients (69%). Five novel variants and two previously reported variant were found in the COLQ, RAPSN and CHRND gene. The previously reported c.393+1G>A splice site variant in the COLQ gene was found in a majority of patients. Five patients harbor the homozygous splice site c.393+1G>A variant, and two patients carry compound heterozygous c.393+1G>A, c.718–1G>T, and c.393+1G>A, c.865G>T (p.Gly289Ter) variants. The novel variants were also found in RAPSN (p.Cys251del, p.Arg282Cys) and CHRND (p.Met481del). Molecular diagnosis in CMS patients can guide treatment decisions and may be life changing, especially in patients with COLQ mutations.
  • No Thumbnail Available
    PublicationMetadata only
    Efficacy and economic evaluation of delivery of care with tele-continuous EEG in critically ill patients: A multicentre, randomised controlled trial (Tele-cRCT) study protocol
    (2020-03-04) Chusak Limotai; Atiporn Ingsathit; Kunlawat Thadanipon; Oraluck Pattanaprateep; Anuchate Pattanateepapon; Kammant Phanthumchinda; Nijasri C. Suwanwela; Iyavut Thaipisuttikul; Kanokwan Boonyapisit; Ammarin Thakkinstian; Chulalongkorn University; Faculty of Medicine, Ramathibodi Hospital, Mahidol University; Faculty of Medicine, Siriraj Hospital, Mahidol University
    © Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. Introduction Some critically ill patients are confirmed by continuous electroencephalography (cEEG) monitoring that non-convulsive seizure (NCS) and/or non-convulsive status epilepticus (NCSE) are causes of their depressed level of consciousness. Shortage of epilepsy specialists, especially in developing countries, is a major limiting factor in implementing cEEG in general practice. Delivery of care with tele-continous EEG (tele-cEEG) may be a potential solution as this allows specialists from a central facility to remotely assist local neurologists from distant areas in interpreting EEG findings and suggest proper treatment. No tele-cEEG programme has been implemented to help improve quality of care. Therefore, this study is conducted to assess the efficacy and cost utility of implementing tele-cEEG in critical care. Methods and analysis The Tele-cRCT study is a 3-year prospective, randomised, controlled, parallel, multicentre, superiority trial comparing delivery of care through € Tele-cEEG' intervention with € Tele-routine EEG (Tele-rEEG)' in patients with clinical suspicion of NCS/NCSE. A group of EEG specialists and a tele-EEG system were set up to remotely interpret EEG findings in six regional government hospitals across Thailand. The primary outcomes are functional neurological outcome (modified Rankin Scale, mRS), mortality rate and incidence of seizures. The secondary outcomes are cost utility, length of stay, emergency visit/readmission, impact on changing medical decisions and health professionals' perceptions about tele-cEEG implementation. Functional outcome (mRS) will be assessed at 3 and 7 days after recruitment, and again at time of hospital discharge, and at 90 days, 6 months, 9 months and 1 year. Costs and health-related quality of life will be assessed using the Thai version of the EuroQol-five dimensions-five levels (EQ-5D-5L) at hospital discharge, and at 90 days, 6 months, 9 months and 1 year. Ethics and dissemination This study has been approved by the ethics committees of the Faculty of Medicine, Chulalongkorn University, and of Ramathibodi Hospital, Mahidol University, and registered on Thai Clinical Trials Registry. The results will be disseminated in a peer-reviewed journal. Trial registration number TCTR20181022002; preresults.
  • No Thumbnail Available
    PublicationMetadata only
    From international guidelines to real-world practice consensus on investigations and management of status epilepticus in adults: A modified Delphi approach
    (2020-02-01) Chusak Limotai; Kanokwan Boonyapisit; Piradee Suwanpakdee; Suda Jirasakuldej; Kwuanrat Wangponpattanasiri; Sattawut Wongwiangiunt; Tipakorn Tumnark; Panutchaya Noivong; Sirincha Pitipanyakul; Chaiwiwat Tungkasereerak; Phopsuk Tansuhaj; Watchara Rattanachaisit; Polchai Pleumpanupatand; Songchai Kittipanprayoon; Phattarawin Ekkachon; Atiporn Ingsathit; Ammarin Thakkinstian; Chulalongkorn University; Hatyai Hospital; King Chulalongkorn Memorial Hospital, Faculty of Medicine Chulalongkorn University; Faculty of Medicine, Ramathibodi Hospital, Mahidol University; Thailand Ministry of Public Health; Maharaj Nakhon Ratchasima Hospital; Buddhachinaraj Hospital; Faculty of Medicine, Siriraj Hospital, Mahidol University; Phramongkutklao College of Medicine; Prasat Neurological Institute
    © 2020 Elsevier Ltd Objective: To establish a consensus which is practical and ready-to-use on investigations (ISE) and for management of status epilepticus (MSE) in adults using a modified Delphi approach. Patients and methods: A 4-round modified Delphi approach was used. First and second rounds were conducted using Google® survey with structured statements and 6-point Likert scale response. Threshold agreement was set to ≥80%. Third round was a face-to-face meeting aimed to facilitate the development of approach algorithms for ISE and MSE. Fourth round was a final review asking participants to rate the algorithms post completion. Results: The panel consisted of 8 board-certified epileptologists along with 6 neurologists from main regional hospitals across Thailand. Thirty-seven statements for ISE and 68 statements for MSE were used for the Round I survey, 17/37 (45.9%) and 49/68 (72.1%) reached threshold agreement (≥80%). The average absolute-agreement intraclass correlation coefficients for ISE and MSE were 0.82 (95% CI 0.71, 0.89) and 0.81 (95% CI 0.73, 0.87), respectively; indicating good extent of consensus among participants. Upon Round II, further 10/18 (55.6%) for ISE and 10/19 (52.6%) for MSE reached agreement. In Round III, face-to-face point-by-point discussion was performed to generate approach algorithms. All (100%) provided positive responses with the algorithms post completion in Round IV. Conclusion: A practical and ready-to-use consensus using modified Delphi approach on ISE and MSE was developed in a Thai regional hospital context. In real practice, this approach is more suitable and feasible for a localized setting when compared with totally adopting international guidelines.
  • No Thumbnail Available
    PublicationMetadata only
    Mutation analysis of the GNE gene in distal myopathy with rimmed vacuoles (DMRV) patient in Thailand
    (2006-12-01) Teerin Liewluck; Theeraphong Pho-Iam; Chanin Limwongse; Wanna Thongnoppakhun; Kanokwan Boonyapisit; Natte Raksadawan; Kumiko Murayama; Yukiko K. Hayashi; Ichizo Nishino; Tumtip Sangruchi; Mahidol University; Bangkok International Hospital; National Institute of Neuroscience, Kodaira
    Distal myopathy with rimmed vacuoles (DMRV) is an early-adult-onset, distal myopathy caused by a mutation of the UDP-N-acetylglucosamine 2 epimerase/N-acetylmannosamine kinase (GNE) gene. We herein report four Thai patients with DMRV who carried compound heterozygous mutations of the GWE gene including three novel (p.G89R, p.P511T, and p.I656N) and two known mutations (p.A524V and p.V696M). All patients shared p.V696M in one allele. Our study demonstrates the mutation spectrum of the GNE gene in Thai patients with DMRV. © 2006 Wiley Periodicals, Inc.
  • No Thumbnail Available
    PublicationMetadata only
    A novel mutation of the GNE gene in distal myopathy with rimmed vacuoles: A case with inflammation
    (2014-01-01) Jantima Tanboon; Kanjana Rongsa; Manop Pithukpakorn; Kanokwan Boonyapisit; Chanin Limwongse; Tumtip Sangruchi; Mahidol University; Faculty of Medicine, Siriraj Hospital, Mahidol University
    Distal myopathy with rimmed vacuoles (DMRV) is an autosomal recessive or sporadic early adult-onset myopathy caused by mutations in the UDP-N-acetylglucosamine 2-epimerase and N-acetylmannosamine kinase (GNE) gene. Characteristic pathologic features of DMRV are rimmed vacuoles on muscle biopsy and tubulofilamentous inclusion in ultrastructural study. Presence of inflammation in DMRV is unusual. We report a sporadic case of DMRV in a 40-year-old Thai man who presented with slowly progressive distal muscle weakness. Gene analysis revealed a compound heterozygous mutation of the GNE gene including a novel mutation c.1057A>G (p.K353E) and a known mutation c.2086G>A (p.V696M). The latter is the most common mutation in Thai DMRV patients. The muscle pathology was compatible with DMRV except for focal inflammation. © 2014 S. Karger AG, Basel.
  • No Thumbnail Available
    PublicationMetadata only
    Oral L-ornithine-L-aspartate for patients with overt hepatic encephalopathy treated with lactulose: A randomized, double-blinded, placebo-controlled trial
    (2018-04-01) Supot Nimanong; Inchaya Sansak; Tawesak Tanwandee; Phunchai Charatcharoenwitthaya; Watcharasak Chotiyaputta; Kanokwan Boonyapisit; Siwaporn Chainuvati; Faculty of Medicine, Siriraj Hospital, Mahidol University
    © 2018, Medical Association of Thailand. All rights reserved. Objective: Lactulose is widely used for treating patients with hepatic encephalopathy [HE] despite of no strong evidence of its effectiveness. L-ornithine-L-aspartate [LOLA] can lower serum ammonia level and improve mental state in patients with mild HE. We evaluated the add-on benefit of oral LOLA in patients with grade II-III HE those treated with optimal dose of lactulose. Materials and Methods: In a randomized, double-blinded, placebo-controlled trial, cirrhotic patients with grade II and III HE according to West-Haven criteria those treated with lactulose were stratified randomized by serum creatinine level to receive LOLA (3 grams thrice daily) or placebo for 7 days. The primary outcome was the improvement of mental status, and secondary outcomes were the improvement of the number connection test [NCT], plasma ammonia level, asterixis, electroencephalogram [EEG], portal systemic encephalopathy index [PSEI] and safety. Results: Thirty-five patients with overt HE (grade II25 patients and grade III 10 patients) were assigned to LOLA group (n = 18) or placebo group (n = 17). The LOLA group tended to have improvement of mental status by 90% compared to 84.6% of placebo group at day 7 (p = 1.0) by per-protocol analysis. Both groups also had similar improvement in mental status at day 4, asterixis, EEG and PSEI. Dose of lactulose and daily bowel movement were comparable in both groups. Treatment of infection which was the main precipitating factor, strongly correlated with better mental state in day 7 (p = 0.025). No serious adverse effect of LOLA was found. Conclusion: There was no add-on benefit of oral LOLA for cirrhotic patients with overt HE who were treated with an optimal dose of lactulose. The more effective treatment of HE was to correct the precipitating factors especially infection.
  • No Thumbnail Available
    PublicationMetadata only
    Pathogenic Variants in the Myosin Chaperone UNC-45B Cause Progressive Myopathy with Eccentric Cores
    (2020-12-03) Sandra Donkervoort; Carl E. Kutzner; Ying Hu; Xavière Lornage; John Rendu; Tanya Stojkovic; Jonathan Baets; Sarah B. Neuhaus; Jantima Tanboon; Reza Maroofian; Véronique Bolduc; Magdalena Mroczek; Stefan Conijn; Nancy L. Kuntz; Ana Töpf; Soledad Monges; Fabiana Lubieniecki; Riley M. McCarty; Katherine R. Chao; Serena Governali; Johann Böhm; Kanokwan Boonyapisit; Edoardo Malfatti; Tumtip Sangruchi; Iren Horkayne-Szakaly; Carola Hedberg-Oldfors; Stephanie Efthymiou; Satoru Noguchi; Sarah Djeddi; Aritoshi Iida; Gabriella di Rosa; Chiara Fiorillo; Vincenzo Salpietro; Niklas Darin; Julien Fauré; Henry Houlden; Anders Oldfors; Ichizo Nishino; Willem de Ridder; Volker Straub; Wojciech Pokrzywa; Jocelyn Laporte; A. Reghan Foley; Norma B. Romero; Coen Ottenheijm; Thorsten Hoppe; Carsten G. Bönnemann; Universite Grenoble Alpes; Université de Strasbourg; International Institute of Molecular and Cell Biology; Instituut Born-Bunge; Göteborg University, Sahlgrenska Academy; University of Newcastle upon Tyne, Faculty of Medical Sciences; Università degli Studi di Genova; University of Cologne; National Institute of Neuroscience, Kodaira; UCL Queen Square Institute of Neurology; National Center of Neurology and Psychiatry Kodaira; IRCCS Istituto Giannina Gaslini - Ospedale Pediatrico; National Institute of Neurological Disorders and Stroke (NINDS); Hopital Raymond Poincare; Universitair Ziekenhuis Antwerpen; Northwestern University Feinberg School of Medicine; Universiteit Antwerpen; Università degli Studi di Messina; Faculty of Medicine, Siriraj Hospital, Mahidol University; The University of Arizona; Centre Hospitalier Universitaire de Grenoble; Amsterdam UMC - Vrije Universiteit Amsterdam; Fundacion Hospital de Pediatria Professor Dr. Juan P. Garrahan; Sorbonne Universite; The Newcastle Upon Tyne Hospitals NHS Foundation Trust; Broad Institute; Defense Health Agency; Institute of Myology
    © 2020 The myosin-directed chaperone UNC-45B is essential for sarcomeric organization and muscle function from Caenorhabditis elegans to humans. The pathological impact of UNC-45B in muscle disease remained elusive. We report ten individuals with bi-allelic variants in UNC45B who exhibit childhood-onset progressive muscle weakness. We identified a common UNC45B variant that acts as a complex hypomorph splice variant. Purified UNC-45B mutants showed changes in folding and solubility. In situ localization studies further demonstrated reduced expression of mutant UNC-45B in muscle combined with abnormal localization away from the A-band towards the Z-disk of the sarcomere. The physiological relevance of these observations was investigated in C. elegans by transgenic expression of conserved UNC-45 missense variants, which showed impaired myosin binding for one and defective muscle function for three. Together, our results demonstrate that UNC-45B impairment manifests as a chaperonopathy with progressive muscle pathology, which discovers the previously unknown conserved role of UNC-45B in myofibrillar organization.
  • No Thumbnail Available
    PublicationMetadata only
    Poliomyelitis and post-poliomyelitis syndrome
    (2014-11-01) Kanokwan Boonyapisit; Mahidol University
    © 2014 Springer Science+Business Media New York. All rights are reserved. Poliomyelitis and the post-poliomyelitis syndrome are often evaluated and cared for by neuromuscular specialists and in neuromuscular clinics. This chapter covers the etiology and pathogenesis, clinical manifestations, diagnosis, evaluation, and management of these disorders.
  • No Thumbnail Available
    PublicationMetadata only
    Siriraj Acute Stroke Unit: 10 years experience.
    (2012-02-01) Songchai Chinwatanakul; Kanokwan Boonyapisit; Darakul Pornsriniyom; Naraporn Proyoonwiwat; Vorapan Senanarong; Rungsan Chaisevikul; Chotipat Danchaivijitr; Watanachai Chotinaiwatarakul; Sith Sathornsumetee; Niphon Poungvarin; Mahidol University
    Stroke is the second most common cause of death and leading cause of adult disability worldwide. The recent publication guidelines suggest that there are treatment strategies for optimizing the management of acute stroke patients including thrombolytic therapy, antiplatelet drugs and the establishment of a stroke unit. In Thailand, the first stroke unit has been established since May 1997 and was named Siriraj Acute Stroke Unit (SASU). The authors retrospectively analyzed the data of stroke patients who were admitted in the SASU from May 1997 to May 2007, as well as hyper acute stroke (within 3 hours after onset). The statistical analysis was performed by using SPSS 11.0. There were 2,109 patients admitted to the SASU during 10 year-period. The mean age of all patients was 65.35 years (range 14-94 years, median 69.2 years). Stroke subtypes were classified as infarction (including transient ischemic attack) in 1799 patients (86.7%) and hemorrhage in 310 patients (13.26%). The most common stroke mechanism was small vessel disease (38.97%). The mainly ischemic stroke distribution was middle cerebral artery territory (77.14%). Risk factors of stroke were as follow: hypertension (61.79%), diabetes mellitus (35.47%), hyperlipidaemia (46.58%), smoking (21.02%) and prior stroke or coronary heart disease (23.74%). Mortality rate was 3.4% mainly due septicemia (26 patients). The mean total hospital stay of stroke patients at the SASU was 13.81 days (ranging from 1-120 days). There were thirty hyper-acute ischemic stroke patients who received intravenous thrombolytic therapy (between August 2005 and May 2007) for which the mean age was 70.7 years (ranges 48-88 years, median 74.5 years). Mean initial National Institutes of Health Stroke Scale (NIHSS) was 14.27. Intracranial hemorrhage after intravenous thrombolytic therapy was found in 8 patients which include 3 patients with symptomatic hemorrhage. The standard of care in SASU is not inferior to other stroke unit worldwide. Admission of acute stroke patient to the SASU provides a better chance of survival as well as a shorter length of hospital stay.
  • No Thumbnail Available
    PublicationMetadata only
    Sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance (SLONM-MGUS): An alternative treatment using cyclophosphamide-thalidomide-dexamethasone (CTD) regimen
    (2018-07-01) Theerawat Kumutpongpanich; Weerapat Owattanapanich; Jantima Tanboon; Ichizo Nishino; Kanokwan Boonyapisit; National Institute of Neuroscience, Kodaira; Faculty of Medicine, Siriraj Hospital, Mahidol University
    © 2018 Elsevier B.V. Sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance is a rare subacute adult-onset myopathy. Without appropriate treatment, the prognosis is unfavorable and can be fatal. Various efficacious treatment options have been reported. High dose melphalan followed by autologous stem cell transplantation is the most used option with favorable outcome. Nevertheless, potentially safer alternative regimens await exploration. Here, we report the case of sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance in a 33-year-old man with significant clinical improvement and complete remission of monoclonal gammopathy after 5 cycles of cyclophosphamide, thalidomide, and dexamethasone regimen. The regimen may be considered as an alternative option for patients with sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance that are ineligible for upfront high-dose melphalan with autologous stem cell transplantation or that are being treated in resource-limited settings. Longer-term follow-up is needed to determine the long-term effectiveness of the cyclophosphamide, thalidomide, and dexamethasone regimen.
  • No Thumbnail Available
    PublicationMetadata only
    Sw2-year outcomes of subthalamic deep brain stimulation for idiopathic Parkinson's disease
    (2010-05-01) Sarun Nunta-Aree; Bunpot Sitthinamsuwan; Kanokwan Boonyapisit; Apichart Pisarnpong; Mahidol University
    Background: Deep brain stimulation of the subthalamic nucleus (STN-DBS) is the recent surgical treatment of choice for patients with idiopathic Parkinson's disease (PD) complicated by motor fluctuation and disabling dyskinesia. Objective: To study 2 years clinical outcomes, changes of medication and complications following STN-DBS in patients with advanced PD. Material and Method: Twenty-seven patients with 2-year follow-up and complete data were enrolled for retrospective evaluation of Unified Parkinson's Disease Rating Scale (UPDRS) and levodopa equivalent dose (LED). Postoperative UPDRS at 6-month, 1-year and 2-years were compared with the preoperative corresponding UPDRS. Postoperative LED at 2 years was compared with the preoperative baseline. Statistical analysis was performed with paired t-test. Additionally, 62 patients with STN-DBS were enrolled for evaluation of treatment complications. Results: Of 27 patients with complete 2-years follow-up, preoperative dopamine challenge test showed 50.6% improvement of motor score (UPDRS axis III). Mentation, behavior and mood (UPDRS axis I) were not significantly improved in each subscore, but significantly improved in the total score. Marked improvement of activities of daily living (UPDRS axis II) and complications of therapy (UPDRS IV) was found. Two-year postoperative motor score (UPDRS axis III) during "off medication-on stimulator" showed progressive and dramatic improvement by mean of 59.83%. The present study also revealed significant improvement of motor score (UPDRS axis III) during "on medication-on stimulator" in some items. A significant 33.4% reduction of LED was noted. Of 62 patients with bilateral STN-DBS, there was 1 asymptomatic intracerebral hemorrhage (0.8% per side), 2 speech difficulty (3.2%), 1 transient confusion (1.6%), 2 transient hypomania (3.2%), 1 stimulation induced hemiballism (1.6%), 1 wound infection (1.6%) and 1 lead malposition (0.8% per side). Conclusion STN-DBS is a safe and effective treatment for PD complicated by motor fluctuation or dyskinesia. The operative outcomes show long-term improvement of activities of daily living, motor function and reduction of medication and drugrelated complications.