No records
|
376 |
Dewiasty E., Setiati S., Agustina R., Roosheroe A.G., Abdullah M., Istanti R., de Groot L.C. |
55257705500;14325991900;57214141404;28767975000;7103393434;23496653300;57213769769; |
Prevalence of lactose intolerance and nutrients intake in an older population regarded as lactase non-persistent |
2021 |
Clinical Nutrition ESPEN |
43 |
|
|
317 |
321 |
|
1 |
https://www.scopus.com/inward/record.uri?eid=2-s2.0-85104952533&doi=10.1016%2fj.clnesp.2021.03.033&partnerID=40&md5=afacacb3695eb5029eae5ee3bb8270ab |
Department of Internal Medicine, Faculty of Medicine, Universitas Indonesia, Dr. Cipto Mangunkusumo General Hospital, Jl. Pangeran Diponegoro No 71, Kenari, Senen, Jakarta PusatDKI Jakarta 10430, Indonesia; Division of Human Nutrition and Health, Wageningen University and Research, P.O. Box 176700, Wageningen, AA, Netherlands; Center of Clinical Epidemiology and Evidence-Based Medicine, Faculty of Medicine Universitas Indonesia – Dr. Cipto Mangunkusumo Hospital, Jakarta Indonesia, Jl. Pangeran Diponegoro No 71, Kenari, Senen, Jakarta PusatDKI Jakarta 10430, Indonesia; Department of Nutrition, Faculty of Medicine, Universitas Indonesia, Dr. Cipto Mangunkusumo General Hospital, Jakarta, Jl., Salemba Raya No. 6, Jakarta Pusat, DKI Jakarta 10430, Indonesia; Human Nutrition Research Center, Indonesian Medical Education and Research Institute (HNRC-IMERI), Faculty of Medicine Universitas Indonesia, Jl. Salemba Raya No. 6, Jakarta Pusat, DKI Jakarta 10430, Indonesia |
Dewiasty, E., Department of Internal Medicine, Faculty of Medicine, Universitas Indonesia, Dr. Cipto Mangunkusumo General Hospital, Jl. Pangeran Diponegoro No 71, Kenari, Senen, Jakarta PusatDKI Jakarta 10430, Indonesia, Division of Human Nutrition and Health, Wageningen University and Research, P.O. Box 176700, Wageningen, AA, Netherlands; Setiati, S., Department of Internal Medicine, Faculty of Medicine, Universitas Indonesia, Dr. Cipto Mangunkusumo General Hospital, Jl. Pangeran Diponegoro No 71, Kenari, Senen, Jakarta PusatDKI Jakarta 10430, Indonesia, Center of Clinical Epidemiology and Evidence-Based Medicine, Faculty of Medicine Universitas Indonesia – Dr. Cipto Mangunkusumo Hospital, Jakarta Indonesia, Jl. Pangeran Diponegoro No 71, Kenari, Senen, Jakarta PusatDKI Jakarta 10430, Indonesia; Agustina, R., Department of Nutrition, Faculty of Medicine, Universitas Indonesia, Dr. Cipto Mangunkusumo General Hospital, Jakarta, Jl., Salemba Raya No. 6, Jakarta Pusat, DKI Jakarta 10430, Indonesia, Human Nutrition Research Center, Indonesian Medical Education and Research Institute (HNRC-IMERI), Faculty of Medicine Universitas Indonesia, Jl. Salemba Raya No. 6, Jakarta Pusat, DKI Jakarta 10430, Indonesia; Roosheroe, A.G., Department of Internal Medicine, Faculty of Medicine, Universitas Indonesia, Dr. Cipto Mangunkusumo General Hospital, Jl. Pangeran Diponegoro No 71, Kenari, Senen, Jakarta PusatDKI Jakarta 10430, Indonesia; Abdullah, M., Department of Internal Medicine, Faculty of Medicine, Universitas Indonesia, Dr. Cipto Mangunkusumo General Hospital, Jl. Pangeran Diponegoro No 71, Kenari, Senen, Jakarta PusatDKI Jakarta 10430, Indonesia; Istanti, R., Department of Internal Medicine, Faculty of Medicine, Universitas Indonesia, Dr. Cipto Mangunkusumo General Hospital, Jl. Pangeran Diponegoro No 71, Kenari, Senen, Jakarta PusatDKI Jakarta 10430, Indonesia; de Groot, L.C., Division of Human Nutrition and Health, Wageningen University and Research, P.O. Box 176700, Wageningen, AA, Netherlands |
Background and aims: Energy and nutrient intakes of community-dwelling older adults in Indonesia are inadequate whereby milk consumption is among the lowest in the world. Lactose intolerance is probably one of the reasons for such low milk consumption, but information on the burden of this problem and its consequences for dietary intake is lacking. We obtained data on the prevalence of lactose intolerance and dietary intakes in Indonesian older outpatients, thereby comparing dairy users and non-dairy users. Methods: A cross-sectional study was conducted in 2019 involving 103 community-dwelling older adults in the outpatient geriatric clinic, Dr. Cipto Mangunkusumo Hospital, Jakarta. A structured questionnaire was used to categorize participants as dairy- or non-dairy users. Food records were collected to assess nutrients intake from the diet. The prevalence of lactose intolerance (LI) was estimated based on the results of the hydrogen breath test (HBT) and on symptoms of lactose malabsorption. The difference in LI prevalence between dairy- and non-dairy user group was presented as a prevalence ratio (with 95% confidence interval). Chi-square tests, t-test, and Mann–Whitney tests were used to evaluate differences in demographic and clinical characteristics, as well as in nutrient intake profiles between dairy and non-dairy users. Results: The prevalence of lactose intolerance amounted to 66% (57–75%), 54% (37–70%), and 73% (61–84%) in the total population, dairy- and non-dairy users, respectively. Lactose intolerance tended to be higher among non-dairy users (PR 1.36 95% CI 0.99–1.89). On the other hand, we found no pronounced differences in symptoms of lactose intolerance between dairy and non-dairy users. The overall mean protein, calcium, vitamin D, and vitamin B12 intakes of the older adults were low. Intakes of protein, calcium, vitamin D and vitamin B12 were higher among dairy than among non-dairy users. Conclusions: This study uncovered the large size of the lactose intolerance problem in Indonesian older adults. Especially in non-dairy users, the intakes of proteins and some micronutrients are a concern. Strategies to tackle lactose intolerance are most relevant as to open the door for more nutrient-dense foods in the diet of Indonesian older adults. © 2021 European Society for Clinical Nutrition and Metabolism |
Indonesia; Lactase non-persistent; Lactose intolerance; Nutrients intake; Older adults |
calcium; cyanocobalamin; lactase; vitamin D; adult; aged; Article; cross-sectional study; dietary intake; female; human; hydrogen breath test; lactose intolerance; male; middle aged; Mini-Nutritional Assessment; nutrient intake; nutritional assessment; nutritional status; prevalence; structured questionnaire; vitamin intake |
Elsevier Ltd |
24054577 |
|
34024534 |
Article |
Q2 |
659 |
7601 |
|
|
379 |
Pranata R., Lim M.A., Huang I., Yonas E., Henrina J., Vania R., Lukito A.A., Nasution S.A., Alwi I., Siswanto B.B. |
57201973901;57216039756;57208576645;57201987097;57218482646;57208328436;57213835420;57189373134;15055173800;14422648800; |
Visceral adiposity, subcutaneous adiposity, and severe coronavirus disease-2019 (COVID-19): Systematic review and meta-analysis |
2021 |
Clinical Nutrition ESPEN |
43 |
|
|
163 |
168 |
|
9 |
https://www.scopus.com/inward/record.uri?eid=2-s2.0-85104309308&doi=10.1016%2fj.clnesp.2021.04.001&partnerID=40&md5=e8122f7c8b69f82d927a6b4a78d83d7a |
Faculty of Medicine, Universitas Pelita Harapan, Tangerang, Indonesia; Department of Internal Medicine, Faculty of Medicine, Universitas Padjadjaran, Hasan Sadikin General Hospital, Bandung, Indonesia; Faculty of Medicine, Universitas YARSI, Jakarta, Indonesia; Balaraja General Hospital, Tangerang, Indonesia; Division of Plastic, Reconstructive and Aesthetic, Department of Surgery, Faculty of Medicine, Udayana University, Sanglah General Hospital, Bali, Indonesia; Department of Cardiology and Vascular Medicine, Siloam Hospitals Lippo Village, Tangerang, Indonesia; Division of Cardiology, Department of Internal Medicine, Faculty of Medicine, Universitas Indonesia/ Cipto Mangunkusumo National General Hospital, Jakarta, Indonesia; Department of Cardiology and Vascular Medicine, Faculty of Medicine Universitas Indonesia, National Cardiovascular Center Harapan Kita, Jakarta, Indonesia |
Pranata, R., Faculty of Medicine, Universitas Pelita Harapan, Tangerang, Indonesia; Lim, M.A., Faculty of Medicine, Universitas Pelita Harapan, Tangerang, Indonesia; Huang, I., Faculty of Medicine, Universitas Pelita Harapan, Tangerang, Indonesia, Department of Internal Medicine, Faculty of Medicine, Universitas Padjadjaran, Hasan Sadikin General Hospital, Bandung, Indonesia; Yonas, E., Faculty of Medicine, Universitas YARSI, Jakarta, Indonesia; Henrina, J., Balaraja General Hospital, Tangerang, Indonesia; Vania, R., Faculty of Medicine, Universitas Pelita Harapan, Tangerang, Indonesia, Division of Plastic, Reconstructive and Aesthetic, Department of Surgery, Faculty of Medicine, Udayana University, Sanglah General Hospital, Bali, Indonesia; Lukito, A.A., Faculty of Medicine, Universitas Pelita Harapan, Tangerang, Indonesia, Department of Cardiology and Vascular Medicine, Siloam Hospitals Lippo Village, Tangerang, Indonesia; Nasution, S.A., Division of Cardiology, Department of Internal Medicine, Faculty of Medicine, Universitas Indonesia/ Cipto Mangunkusumo National General Hospital, Jakarta, Indonesia; Alwi, I., Division of Cardiology, Department of Internal Medicine, Faculty of Medicine, Universitas Indonesia/ Cipto Mangunkusumo National General Hospital, Jakarta, Indonesia; Siswanto, B.B., Department of Cardiology and Vascular Medicine, Faculty of Medicine Universitas Indonesia, National Cardiovascular Center Harapan Kita, Jakarta, Indonesia |
Background and aims: Body mass index (BMI) has previously been shown to increase mortality and disease severity in patients with COVID-19, but the pooled effect estimate was heterogeneous. Although BMI is widely used as an indicator, it cannot distinguish visceral from subcutaneous fat. This systematic review and meta-analysis aimed to investigate the association between visceral adiposity, subcutaneous fat, and severe COVID-19. Methods: We performed a systematic literature search using the databases: PubMed, Embase, and EuropePMC. Data on visceral fat area (VTA), subcutaneous fat area (SFA), and total fat area (TFA) were collected. The outcome of interest was severe COVID-19. We used a REML random-effects model to pool the mean differences and odds ratio (OR). Results: There were 5 studies comprising of 539 patients. Patients with severe COVID-19 have a higher VTA (mean difference 41.7 cm2 [27.0, 56.4], p < 0.001; I2: 0%) and TFA (mean difference 64.6 cm2 [26.2, 103.1], p = 0.001; I2: 0%). There was no significant difference in terms of SFA between patients with severe and non-severe COVID-19 (mean difference 9.3 cm2 [-4.9, 23.4], p = 0.199; I2: 1.2%). Pooled ORs showed that VTA was associated with severe COVID-19 (OR 1.9 [1.1, 2.2], p = 0.002; I2: 49.3%). Conclusion: Visceral adiposity was associated with increased COVID-19 severity, while subcutaneous adiposity was not. Prospero id: CRD42020215876. © 2021 European Society for Clinical Nutrition and Metabolism |
Adiposity; Coronavirus; Obesity; Visceral fat; Visceral fat area |
Article; artificial ventilation; body composition; body mass; cardiovascular disease; coronavirus disease 2019; critical illness; diabetes mellitus; disease exacerbation; disease severity; human; hypertension; intensive care unit; intra-abdominal fat; meta analysis; mortality; Newcastle-Ottawa scale; nonhuman; obesity; observational study; respiratory tract intubation; Severe acute respiratory syndrome coronavirus 2; shock; subcutaneous fat; systematic review; abdominal obesity; aged; body mass; comorbidity; complication; female; intra-abdominal fat; male; metabolism; middle aged; severity of illness index; subcutaneous fat; Adiposity; Aged; Body Mass Index; Comorbidity; COVID-19; Female; Humans; Intra-Abdominal Fat; Male; Middle Aged; Obesity; Obesity, Abdominal; SARS-CoV-2; Severity of I |
Elsevier Ltd |
24054577 |
|
34024509 |
Article |
Q2 |
659 |
7601 |
|
|
638 |
Wiyono L., Rocha I.C.N., Cedeño T.D.D., Miranda A.V., Lucero-Prisno Iii D.E. |
57207889054;57406221700;57224097138;57222664687;56051373800; |
Dengue and COVID-19 infections in the ASEAN region: a concurrent outbreak of viral diseases |
2021 |
Epidemiology and health |
43 |
|
|
e2021070 |
|
|
1 |
https://www.scopus.com/inward/record.uri?eid=2-s2.0-85122549130&doi=10.4178%2fepih.e2021070&partnerID=40&md5=0876d7707a72acb2031f56c5f9268d61 |
Faculty of Medicine, Universitas IndonesiaJakarta, Indonesia; School of Medicine, Centro Escolar University, Manila, Philippines; Department of Global Health and Development, London School of Hygiene and Tropical Medicine, London, United Kingdom; Faculty of Management and Development Studies, University of the Philippines Open University, Los Baños, Philippines |
Wiyono, L., Faculty of Medicine, Universitas IndonesiaJakarta, Indonesia; Rocha, I.C.N., School of Medicine, Centro Escolar University, Manila, Philippines; Cedeño, T.D.D., School of Medicine, Centro Escolar University, Manila, Philippines; Miranda, A.V., Faculty of Medicine, Universitas IndonesiaJakarta, Indonesia; Lucero-Prisno Iii, D.E., Department of Global Health and Development, London School of Hygiene and Tropical Medicine, London, United Kingdom, Faculty of Management and Development Studies, University of the Philippines Open University, Los Baños, Philippines |
Member countries of the Association of Southeast Asian Nations (ASEAN) have faced dengue outbreaks for decades, and the region has one of the highest rates of dengue globally. Outbreaks continue to occur concurrently with the coronavirus disease 2019 (COVID-19) pandemic in the 10 ASEAN countries. Both infectious diseases pose a tremendous burden in these countries related to both infection control and the economy. Increases in the number of dengue cases occurred in part due to disruptions in the pathogen-host-vector relationship caused by changes in human behaviour in response to the COVID-19 pandemic. The spread of dengue was further aggravated by the implementation of lockdowns and social distancing policies. These measures limited the coverage of dengue preventive programs and delayed the medical management of both diseases due to co-infection and misdiagnosis. It is of the utmost importance for the population to remain aware of both diseases, and dengue vector control strategies must be devised to properly address outbreaks using digitalization and remote surveillance. Similarly, critical triage algorithms and further research are also needed to combat co-infection and misdiagnosis. Controlling the spread of COVID-19 though vaccination should also be undertaken to reduce the impact of the pandemic. |
Asia; COVID-19; Dengue; Infection control |
communicable disease control; dengue; epidemic; human; pandemic; Communicable Disease Control; COVID-19; Dengue; Disease Outbreaks; Humans; Pandemics; SARS-CoV-2 |
NLM (Medline) |
20927193 |
|
34607402 |
Article |
Q1 |
1109 |
3739 |
|
|
805 |
Wahid M.H., Findyartini A., Soemantri D., Mustika R., Felaza E., Steinert Y., Samarasekera D.D., Greviana N., Hidayah R.N., Khoiriyah U., Soeselo D.A. |
57192887722;56543777300;36640659100;57220273147;57217487889;7003537985;6506358769;57197709749;57219977261;56803884800;57199998747; |
Professional identity formation of medical teachers in a non-Western setting |
2021 |
Medical Teacher |
43 |
8 |
|
868 |
873 |
|
1 |
https://www.scopus.com/inward/record.uri?eid=2-s2.0-85106243417&doi=10.1080%2f0142159X.2021.1922657&partnerID=40&md5=fbc77286f37d3aa77b3991698ebc01ec |
Department of Medical Education and Medical Education Center Indonesia Medical Education and Research Institute (IMERI), Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia; Institute of Health Sciences Education, Faculty of Medicine and Health Sciences, McGill University, Montreal, Canada; Center for Medical Education (CenMED), Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore; Centre for Development of Teaching and Learning, National University of Singapore, Singapore, Singapore; Department of Medical Education and Bioethics, Faculty of Medicine, Public Health, and Nursing, Universitas Gadjah Mada, Yogyakarta, Indonesia; Faculty of Medicine, Medical Education Unit, Universitas Islam Indonesia, Yogyakarta, Indonesia; Medical Education Unit, School of Medicine and Health Sciences, Universitas Katolik Atma Jaya, Jakarta, Indonesia |
Wahid, M.H., Department of Medical Education and Medical Education Center Indonesia Medical Education and Research Institute (IMERI), Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia; Findyartini, A., Department of Medical Education and Medical Education Center Indonesia Medical Education and Research Institute (IMERI), Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia; Soemantri, D., Department of Medical Education and Medical Education Center Indonesia Medical Education and Research Institute (IMERI), Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia; Mustika, R., Department of Medical Education and Medical Education Center Indonesia Medical Education and Research Institute (IMERI), Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia; Felaza, E., Department of Medical Education and Medical Education Center Indonesia Medical Education and Research Institute (IMERI), Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia; Steinert, Y., Institute of Health Sciences Education, Faculty of Medicine and Health Sciences, McGill University, Montreal, Canada; Samarasekera, D.D., Center for Medical Education (CenMED), Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore, Centre for Development of Teaching and Learning, National University of Singapore, Singapore, Singapore; Greviana, N., Department of Medical Education and Medical Education Center Indonesia Medical Education and Research Institute (IMERI), Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia; Hidayah, R.N., Department of Medical Education and Bioethics, Faculty of Medicine, Public Health, and Nursing, Universitas Gadjah Mada, Yogyakarta, Indonesia; Khoiriyah, U., Faculty of Medicine, Medical Education Unit, Universitas Islam Indonesia, Yogyakarta, Indonesia; Soeselo, D.A., Medical Education Unit, School of Medicine and Health Sciences, Universitas Katolik Atma Jaya, Jakarta, Indonesia |
Introduction: Understanding and supporting professional identity formation (PIF) among medical teachers has become increasingly important in faculty development programs. In this study, we explored medical teachers’ PIF in Indonesia. Methods: We conducted a qualitative descriptive study using focus group discussions (FGDs) at four medical schools in Indonesia. Basic science and clinical teachers from four different schools were selected to participate. Data were transcribed, coded, and analysed to develop themes and subthemes. Results: Seventeen FGDs were completed, involving 60 basic science and 59 clinical teachers. Four major themes regarding the formation of medical teachers’ professional identity emerged: an internal dialogue between intrinsic values and external influences, empowerment through early socialization, experiential workplace learning, and envisioning the future. The PIF process was similar for basic science and clinical teachers. Conclusion: Our findings suggested that PIF among medical teachers in a non-Western setting is a continuous and dynamic process that is shaped by key socialization factors (e.g. role models, workplace learning, peer support), with significant influences from religious beliefs, family values, and societal recognition. Faculty development programs should consider the dynamic and continuous nature of PIF among medical teachers and encourage clinicians and basic scientists to explore their values and beliefs, realize their goals, and envision their future. © 2021 Informa UK Limited, trading as Taylor & Francis Group. |
professionalism; roles of teacher; Staff development |
article; empowerment; genetic transcription; human; Indonesia; learning; medical school; peer group; personnel management; professionalism; religion; socialization; teacher; workplace; medical school; qualitative research; social behavior; university; Faculty; Humans; Learning; Qualitative Research; Schools, Medical; Social Identification |
Taylor and Francis Ltd. |
0142159X |
|
33989110 |
Article |
Q1 |
1355 |
2689 |
|
|
918 |
Ramani S., McKimm J., Findyartini A., Nadarajah V.D., Hays R., Chisolm M.S., Filipe H.P., Fornari A., Kachur E.K., Kusurkar R.A., Thampy H., Wilson K.W. |
56186462600;26433565200;56543777300;14048599600;7202509928;25957566100;36657108400;6701517660;6603938626;6603461994;55349958700;56926010000; |
Twelve tips for developing a global community of scholars in health professions education |
2021 |
Medical Teacher |
43 |
8 |
|
966 |
971 |
|
3 |
https://www.scopus.com/inward/record.uri?eid=2-s2.0-85094560355&doi=10.1080%2f0142159X.2020.1839034&partnerID=40&md5=5cfe14caf9b8e1e02d7e63fd250a2375 |
Brigham and Women’s Hospital and Harvard Medical School, Boston, MA, United States; Swansea University Medical School, United Kingdom; Faculty of Medicine Universitas Indonesia, Jakarta, Indonesia; International Medical University, Kuala Lumpur, Malaysia; James Cook University, Townesville, QLD, Australia; Johns Hopkins University School of Medicine, Baltimore, MD, United States; University of Lisbon, Lisbon, Portugal; Donald and Barbara Zucker SOM at Hofstra/Northwell, Hempstead, NY, United States; Medical Education Development, Global Consulting, New York, NY, United States; Faculty of Medicine, Vrije Universiteit Amsterdam, Netherlands; Faculty of Biology, Medicine and Health, University of Manchester, Manchester, United Kingdom; Dalhousie University, Halifax, NS, Canada |
Ramani, S., Brigham and Women’s Hospital and Harvard Medical School, Boston, MA, United States; McKimm, J., Swansea University Medical School, United Kingdom; Findyartini, A., Faculty of Medicine Universitas Indonesia, Jakarta, Indonesia; Nadarajah, V.D., International Medical University, Kuala Lumpur, Malaysia; Hays, R., James Cook University, Townesville, QLD, Australia; Chisolm, M.S., Johns Hopkins University School of Medicine, Baltimore, MD, United States; Filipe, H.P., University of Lisbon, Lisbon, Portugal; Fornari, A., Donald and Barbara Zucker SOM at Hofstra/Northwell, Hempstead, NY, United States; Kachur, E.K., Medical Education Development, Global Consulting, New York, NY, United States; Kusurkar, R.A., Faculty of Medicine, Vrije Universiteit Amsterdam, Netherlands; Thampy, H., Faculty of Biology, Medicine and Health, University of Manchester, Manchester, United Kingdom; Wilson, K.W., Dalhousie University, Halifax, NS, Canada |
Scholarship in Health Professions Education is not just original research, it also includes study of educational processes, and application of new knowledge to practice. The pathways to successful scholarship are not always clear to novice educators. In this article, we describe strategies to establish a Community of Scholars (CoS), where more experienced and senior members guide junior members in scholarship to advance the field. Drawing on Lave and Wenger’s concepts of Communities of Practice (CoP), we describe twelve practical tips, which include generation of a shared vision, formation of a global community of scholars, engagement in scholarly initiatives, and development of a professional identity, categorised under three major steps: establish, grow, and sustain the community. The tips embrace inclusivity for diverse cultural contexts which further provide opportunities for Health Professions Educators, interested in forming communities of practice, to work on scholarly outputs and add value to the professional arena. © 2020 Informa UK Limited, trading as Taylor & Francis Group. |
continuing; Leadership; mentoring; staff development |
article; drawing; human; human experiment; leadership; mentoring; occupation; personnel management; vision; medical education; medical profession; Fellowships and Scholarships; Health Occupations; Humans |
Taylor and Francis Ltd. |
0142159X |
|
33108740 |
Article |
Q1 |
1355 |
2689 |
|
|
No records
|
369 |
Yugo D., Lo L.-W., Wu Y.-H., Chung F.-P., Lin Y.-J., Chang S.-L., Hu Y.-F., Chao T.-F., Liao J.-N., Chang T.-Y., Lin C.-Y., Tuan T.-C., Kuo L., Wu C.-I., Liu C.-M., Liu S.-H., Cheng W.-H., Lugtu I.C., Jain A., Chen S.-A. |
57215499689;25224685100;57150405500;40560956700;56512618600;57154909400;57219519883;35335897300;55179923000;56763173800;56469967500;7005704090;57193883698;56320807100;57202736753;57191375309;26025932500;57212467736;57216788165;57211727582; |
Case series on stereotactic body radiation therapy in non-ischemic cardiomyopathy patients with recurrent ventricular tachycardia |
2021 |
PACE - Pacing and Clinical Electrophysiology |
44 |
6 |
|
1085 |
1093 |
|
|
https://www.scopus.com/inward/record.uri?eid=2-s2.0-85105961876&doi=10.1111%2fpace.14254&partnerID=40&md5=d3ccf6f5cafcbd0b6064e6a5a180d5ce |
Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan; Department of Cardiology and Vascular Medicine, Faculty of Medicine University of Indonesia, Jakarta, Indonesia; Arrhythmia Division, Pusat Jantung Nasional Harapan Kita, Jakarta, Indonesia; Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Division of Radiation Oncology, Department of Oncology, Taipei Veterans General Hospital, Taipei, Taiwan; Department of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Department of Biomedical Imaging and Radiological Sciences, National Yang Ming Chiao Tung University, Taipei, Taiwan; Heart Institute, Chinese General Hospital and Medical Center, Metro Manila, Philippines; Department of Cardiology, Vardhman Mahavir Medical College, New Delhi, India; Cardiovascular Center, Taichung Veterans General Hospital, Taichung, Taiwan |
Yugo, D., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Department of Cardiology and Vascular Medicine, Faculty of Medicine University of Indonesia, Jakarta, Indonesia, Arrhythmia Division, Pusat Jantung Nasional Harapan Kita, Jakarta, Indonesia; Lo, L.-W., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Wu, Y.-H., Division of Radiation Oncology, Department of Oncology, Taipei Veterans General Hospital, Taipei, Taiwan, Department of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan, Department of Biomedical Imaging and Radiological Sciences, National Yang Ming Chiao Tung University, Taipei, Taiwan; Chung, F.-P., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Lin, Y.-J., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Chang, S.-L., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Hu, Y.-F., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Chao, T.-F., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Liao, J.-N., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Chang, T.-Y., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Lin, C.-Y., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Tuan, T.-C., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Kuo, L., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Wu, C.-I., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Liu, C.-M., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Liu, S.-H., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Cheng, W.-H., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Lugtu, I.C., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Heart Institute, Chinese General Hospital and Medical Center, Metro Manila, Philippines; Jain, A., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Department of Cardiology, Vardhman Mahavir Medical College, New Delhi, India; Chen, S.-A., Heart Rhythm Center, Division of Cardiology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, Faculty of Medicine and Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan, Cardiovascular Center, Taichung Veterans General Hospital, Taichung, Taiwan |
Introduction: The efficacy of stereotactic body radiation therapy (SBRT) as an alternative treatment for recurrent ventricular tachycardia (VT) is still unclear. This study aimed to report the outcome of SBRT in VT patients with nonischemic cardiomyopathy (NICM). Methods: The determination of the target substrate for radiation was based on the combination of CMR results and electroanatomical mapping merged with the real-time CT scan image. Radiation therapy was performed by Flattening-filter-free (Truebeam) system, and afterward, patients were followed up for 13.5 ± 2.8 months. We analyzed the outcome of death, incidence of recurrent VT, ICD shocks, anti-tachycardia pacing (ATP) sequences, and possible irradiation side-effects. Results: A total of three cases of NICM patients with anteroseptal scar detected by CMR. SBRT was successfully performed in all patients. During the follow-up, we found that VT recurrences occurred in all patients. In one patient, it happened during a 6-week blanking period, while the others happened afterward. Re-hospitalization due to VT only appeared in one patient. Through ICD interrogation, we found that all patients have reduced VT burden and ATP therapies. All of the patients died during the follow-up period. Radiotherapy-related adverse events did not occur in all patients. Conclusions: SBRT therapy reduces the number of VT burden and ATP sequence therapy in NICM patients with VT, which had a failed previous catheter ablation. However, the efficacy and safety aspects, especially in NICM cases, remained unclear. © 2021 Wiley Periodicals LLC |
radio-ablation; stereotactic; ventricular tachycardia |
aged; Article; cardiovascular magnetic resonance; case report; clinical article; clinical effectiveness; clinical outcome; computer assisted tomography; death; disease burden; female; follow up; heart pacing; heart ventricle tachycardia; hospital readmission; human; incidence; male; nonischemic cardiomyopathy; recurrent disease; stereotactic body radiation therapy; very elderly; cardiomyopathy; diagnostic imaging; epicardium mapping; heart ventricle tachycardia; procedures; radiosurgery; radiotherapy dosage; scar; x-ray computed tomography; Aged; Aged, 80 and over; Cardiomyopathies; Cicatrix; Epicardial Mapping; Female; Humans; Male; Radiosurgery; Radiotherapy Dosage; Tachycardia, Ventricular; Tomography, X-Ray Computed |
John Wiley and Sons Inc |
01478389 |
|
33932305 |
Article |
Q2 |
686 |
7284 |
|
|
382 |
Vityadewi N., Bangun K., Budiman, Winarsih W., Fauzi A.R. |
57218771358;36902624600;57222390006;16053776500;57203133889; |
Correction to: Auricular cartilage regeneration on donor site defect with one-sided perichondrial cartilage graft in an experimental rabbit model (European Journal of Plastic Surgery, (2021), 44, 3, (307-314), 10.1007/s00238-020-01765-2) |
2021 |
European Journal of Plastic Surgery |
44 |
3 |
|
417 |
|
|
|
https://www.scopus.com/inward/record.uri?eid=2-s2.0-85102563175&doi=10.1007%2fs00238-021-01810-8&partnerID=40&md5=a522ad3fbe4e9f01f86ca4cc71a8915e |
Plastic, Reconstructive, and Aesthetic Surgery Division, Department of Surgery, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito Hospital, Yogyakarta, 55281, Indonesia; Plastic, Reconstructive, and Aesthetic Surgery Division, Department of Surgery, Faculty of Medicine, Universitas Indonesia/Dr. Cipto Mangunkusumo National Central General Hospital, Jakarta, 10430, Indonesia; Division of Plastic, Reconstructive, and Aesthetic Surgery, Department of Surgery, Gatot Subroto Army Hospital, Jakarta, Indonesia; Division of Pathology, Department of Veterinary Clinic Reproduction and Pathology, Bogor Agricultural University, Bogor, 16680, Indonesia |
Vityadewi, N., Plastic, Reconstructive, and Aesthetic Surgery Division, Department of Surgery, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito Hospital, Yogyakarta, 55281, Indonesia; Bangun, K., Plastic, Reconstructive, and Aesthetic Surgery Division, Department of Surgery, Faculty of Medicine, Universitas Indonesia/Dr. Cipto Mangunkusumo National Central General Hospital, Jakarta, 10430, Indonesia; Budiman, Division of Plastic, Reconstructive, and Aesthetic Surgery, Department of Surgery, Gatot Subroto Army Hospital, Jakarta, Indonesia; Winarsih, W., Division of Pathology, Department of Veterinary Clinic Reproduction and Pathology, Bogor Agricultural University, Bogor, 16680, Indonesia; Fauzi, A.R., Plastic, Reconstructive, and Aesthetic Surgery Division, Department of Surgery, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito Hospital, Yogyakarta, 55281, Indonesia |
There is a leak in the name of Kristiania Bangun (The second author), and the correct name is “Kristaninta Bangun”. The original article has been corrected. © 2021, Springer-Verlag GmbH Germany, part of Springer Nature. |
|
erratum |
Springer Science and Business Media Deutschland GmbH |
0930343X |
|
|
Erratum |
Q3 |
219 |
18194 |
|
|
388 |
Vityadewi N., Bangun K., Budiman, Winarsih W., Fauzi A.R. |
57218771358;36902624600;57222390006;16053776500;57203133889; |
Auricular cartilage regeneration on donor site defect with one-sided perichondrial cartilage graft in an experimental rabbit model |
2021 |
European Journal of Plastic Surgery |
44 |
3 |
|
307 |
314 |
|
|
https://www.scopus.com/inward/record.uri?eid=2-s2.0-85099103344&doi=10.1007%2fs00238-020-01765-2&partnerID=40&md5=584c9d67f1904c8e4053cf3ba2855fbe |
Plastic, Reconstructive and Aesthetic Surgery Division, Department of Surgery, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito Hospital, Yogyakarta, 55281, Indonesia; Plastic Surgery Reconstruction and Aesthetics Division, Department of Surgery, Faculty of Medicine, Universitas Indonesia, Jakarta, 10430, Indonesia; Division of Plastic Reconstructive Aesthetic Surgery, Department of Surgery, Gatot Subroto Army Hospital, Jakarta, Indonesia; Division of Pathology, Department of Veterinary Clinic Reproduction and Pathology, Bogor Agricultural University, Bogor, 16680, Indonesia |
Vityadewi, N., Plastic, Reconstructive and Aesthetic Surgery Division, Department of Surgery, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito Hospital, Yogyakarta, 55281, Indonesia; Bangun, K., Plastic Surgery Reconstruction and Aesthetics Division, Department of Surgery, Faculty of Medicine, Universitas Indonesia, Jakarta, 10430, Indonesia; Budiman, Division of Plastic Reconstructive Aesthetic Surgery, Department of Surgery, Gatot Subroto Army Hospital, Jakarta, Indonesia; Winarsih, W., Division of Pathology, Department of Veterinary Clinic Reproduction and Pathology, Bogor Agricultural University, Bogor, 16680, Indonesia; Fauzi, A.R., Plastic, Reconstructive and Aesthetic Surgery Division, Department of Surgery, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito Hospital, Yogyakarta, 55281, Indonesia |
Background: Auricular cartilage is considered the source for cartilage grafts with good esthetic results and is often used in augmentation rhinoplasty. Most revision procedures require grafting, and the auricular cartilage can be the source for secondary rhinoplasty. Due to limited donor locations, donor site optimization of graft material is very important. We aimed to evaluate the healing process from the donor site, including the regeneration of cartilage from the donor site in an experimental animal model. Methods: Six New Zealand white rabbits were used for the investigation of cartilage regeneration from donor defects with one-sided perichondrium. Cartilage defect measuring 0.5 × 3 cm was made in rabbit ears. Two trial groups with 12 ears in each group were formed (one-sided perichondrium vs without perichondrium). Macroscopic and microscopic evaluation examinations were performed at week 4. The gap and thickness of the donor defect were measured with a centimeter grinder for macroscopic evaluation. Results: While macroscopic evaluation of wound thickness and the gap was not significant between groups (p = 1.00, p = 0.053, respectively), microscopic evaluation showed immature cartilage formation at week 4 of the donor defect with one-sided perichondrium (p = 0.004). Conclusions: The donor defect with one-sided perichondrium showed new cartilage formation within the area of the perichondrium. These findings showed the regeneration of cartilage was developed at the donor defects with one-sided perichondrium in the fourth week. Level of evidence: Not ratable. © 2021, Springer-Verlag GmbH Germany, part of Springer Nature. |
Auricular cartilage graft; Cartilage healing; Cartilage regeneration; Donor defect |
|
Springer Science and Business Media Deutschland GmbH |
0930343X |
|
|
Article |
Q3 |
219 |
18194 |
|
|
916 |
Coughlin C.R., II, Tseng L.A., Abdenur J.E., Ashmore C., Boemer F., Bok L.A., Boyer M., Buhas D., Clayton P.T., Das A., Dekker H., Evangeliou A., Feillet F., Footitt E.J., Gospe S.M., Jr., Hartmann H., Kara M., Kristensen E., Lee J., Lilje R., Longo N., Lunsing R.J., Mills P., Papadopoulou M.T., Pearl P.L., Piazzon F., Plecko B., Saini A.G., Santra S., Sjarif D.R., Stockler-Ipsiroglu S., Striano P., Van Hove J.L.K., Verhoeven-Duif N.M., Wijburg F.A., Zuberi S.M., van Karnebeek C.D.M. |
57203153211;57193113158;6602690660;55329181300;22133268100;18433647100;56442108100;54415352400;26643542700;7403597535;57072925500;6601972696;6701669931;24066333000;7004867097;19234327400;55964701800;57218823969;57219907275;6507651491;7004663930;7801612204;57202556029;57213632266;7003948257;54950108600;55990557800;37087697200;24172569400;6506242684;55930268500;6701766775;7005706056;35747586800;7003454408;7005936517;6506453512; |
Consensus guidelines for the diagnosis and management of pyridoxine-dependent epilepsy due to α-aminoadipic semialdehyde dehydrogenase deficiency |
2021 |
Journal of Inherited Metabolic Disease |
44 |
1 |
|
178 |
192 |
|
9 |
https://www.scopus.com/inward/record.uri?eid=2-s2.0-85096935873&doi=10.1002%2fjimd.12332&partnerID=40&md5=519c5479b1e22e3b77f1890b9b382e4d |
Section of Clinical Genetics and Metabolism, Department of Pediatrics, University of Colorado Anschutz Medical Campus, Aurora, CO, United States; Department of Pediatrics Emma Children's Hospital, Amsterdam University Medical Centre, Amsterdam, Netherlands; Division of Metabolic Disorders, CHOC Children's Hospital, Orange, CA, United States; Birmingham Women's and Children's NHS Foundation Trust, Birmingham, United Kingdom; Department of Human Genetics, Centre Hospitalier Universitaire Sart-Tilman, Liège, Belgium; Department of Pediatrics and Neonatology, Máxima Medical Center, Veldhoven, Netherlands; Division of Medical Genetics, Department of Specialized Medicine, Montreal Children's Hospital, McGill University Health Centre, Québec, Canada; Genetics and Genomic Medicine, UCL Great Ormond Street Institute of Child Health, London, United Kingdom; Clinic for Paediatric Kidney, Liver, and Metabolic Diseases, Hannover Medical School, Hannover, Germany; VKS: Dutch Patient Organization for Metabolic Diseases, Zwolle, Netherlands; Division of Child Neurology and Inherited Metabolic Disorders, 4th Department of Pediatrics, Aristotle University of Thessaloniki, General Hospital Papageorgiou, Thessaloniki, Greece; Reference Center for Inborn Errors of Metabolism, Pediatric Unit, University Hospital of Nancy, Nancy, France; INSERM UMR S 1256, Nutrition, Genetics, and Environmental Risk Exposure (NGERE), Faculty of Medicine of Nancy, University of Lorraine, Nancy, France; Department of Metabolic Paediatrics, Great Ormond Street Hospital, London, United Kingdom; Division of Pediatric Neurology, Departments of Neurology and Pediatrics, University of Washington, Seattle, WA, United States; Department of Pediatrics, Duke University, Durham, NC, United States; Department of Pediatrics, University of Tripoli, Tripoli, Libyan Arab Jamahiriya; National Management of Newborn Screening and Advanced Laboratory Diagnostics in Inborn Errors of Metabolism, Department of Children and Adolescent Medicine, Oslo University Hospital, Oslo, Norway; Department of Metabolic Medicine, The Royal Children's Hospital, Melbourne, VIC, Australia; Department of Children and Adolescent Medicine, Oslo University Hospital, Oslo, Norway; Division of Medical Genetics, Department of Pediatrics, University of Utah, Salt Lake City, UT, United States; Department of Neurology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands; Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States; Neurometabolic Clinic, Children's Institute, University of Sao Paulo, Brazil; Division of General Pediatrics, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria; Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India; Department of Child Health, Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia; Division of Biochemical Genetics, BC Children's Hospital, University of British Columbia, Vancouver, BC, Canada; Pediatric Neurology and Muscular Diseases Unit, IRCCS “G. Gaslini” Institute, Genoa, Italy; Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genova, Genoa, Italy; Department of Genetics, University Medical Center Utrecht, Utrecht, Netherlands; Paediatric Neurosciences Research Group, Royal Hospital for Children & School of Medicine, University of Glasgow, Glasgow, United Kingdom; Department of Pediatrics, Amalia Children's Hospital, Radboud Centre for Mitochondrial Medicine, Radboud University Medical Center, Nijmegen, Netherlands |
Coughlin, C.R., II, Section of Clinical Genetics and Metabolism, Department of Pediatrics, University of Colorado Anschutz Medical Campus, Aurora, CO, United States; Tseng, L.A., Department of Pediatrics Emma Children's Hospital, Amsterdam University Medical Centre, Amsterdam, Netherlands; Abdenur, J.E., Division of Metabolic Disorders, CHOC Children's Hospital, Orange, CA, United States; Ashmore, C., Birmingham Women's and Children's NHS Foundation Trust, Birmingham, United Kingdom; Boemer, F., Department of Human Genetics, Centre Hospitalier Universitaire Sart-Tilman, Liège, Belgium; Bok, L.A., Department of Pediatrics and Neonatology, Máxima Medical Center, Veldhoven, Netherlands; Boyer, M., Division of Metabolic Disorders, CHOC Children's Hospital, Orange, CA, United States; Buhas, D., Division of Medical Genetics, Department of Specialized Medicine, Montreal Children's Hospital, McGill University Health Centre, Québec, Canada; Clayton, P.T., Genetics and Genomic Medicine, UCL Great Ormond Street Institute of Child Health, London, United Kingdom; Das, A., Clinic for Paediatric Kidney, Liver, and Metabolic Diseases, Hannover Medical School, Hannover, Germany; Dekker, H., VKS: Dutch Patient Organization for Metabolic Diseases, Zwolle, Netherlands; Evangeliou, A., Division of Child Neurology and Inherited Metabolic Disorders, 4th Department of Pediatrics, Aristotle University of Thessaloniki, General Hospital Papageorgiou, Thessaloniki, Greece; Feillet, F., Reference Center for Inborn Errors of Metabolism, Pediatric Unit, University Hospital of Nancy, Nancy, France, INSERM UMR S 1256, Nutrition, Genetics, and Environmental Risk Exposure (NGERE), Faculty of Medicine of Nancy, University of Lorraine, Nancy, France; Footitt, E.J., Department of Metabolic Paediatrics, Great Ormond Street Hospital, London, United Kingdom; Gospe, S.M., Jr., Division of Pediatric Neurology, Departments of Neurology and Pediatrics, University of Washington, Seattle, WA, United States, Department of Pediatrics, Duke University, Durham, NC, United States; Hartmann, H., Clinic for Paediatric Kidney, Liver, and Metabolic Diseases, Hannover Medical School, Hannover, Germany; Kara, M., Department of Pediatrics, University of Tripoli, Tripoli, Libyan Arab Jamahiriya; Kristensen, E., National Management of Newborn Screening and Advanced Laboratory Diagnostics in Inborn Errors of Metabolism, Department of Children and Adolescent Medicine, Oslo University Hospital, Oslo, Norway; Lee, J., Department of Metabolic Medicine, The Royal Children's Hospital, Melbourne, VIC, Australia; Lilje, R., Department of Children and Adolescent Medicine, Oslo University Hospital, Oslo, Norway; Longo, N., Division of Medical Genetics, Department of Pediatrics, University of Utah, Salt Lake City, UT, United States; Lunsing, R.J., Department of Neurology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands; Mills, P., Genetics and Genomic Medicine, UCL Great Ormond Street Institute of Child Health, London, United Kingdom; Papadopoulou, M.T., Division of Child Neurology and Inherited Metabolic Disorders, 4th Department of Pediatrics, Aristotle University of Thessaloniki, General Hospital Papageorgiou, Thessaloniki, Greece; Pearl, P.L., Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States; Piazzon, F., Neurometabolic Clinic, Children's Institute, University of Sao Paulo, Brazil; Plecko, B., Division of General Pediatrics, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria; Saini, A.G., Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India; Santra, S., Birmingham Women's and Children's NHS Foundation Trust, Birmingham, United Kingdom; Sjarif, D.R., Department of Child Health, Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia; Stockler-Ipsiroglu, S., Division of Biochemical Genetics, BC Children's Hospital, University of British Columbia, Vancouver, BC, Canada; Striano, P., Pediatric Neurology and Muscular Diseases Unit, IRCCS “G. Gaslini” Institute, Genoa, Italy, Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genova, Genoa, Italy; Van Hove, J.L.K., Section of Clinical Genetics and Metabolism, Department of Pediatrics, University of Colorado Anschutz Medical Campus, Aurora, CO, United States; Verhoeven-Duif, N.M., Department of Genetics, University Medical Center Utrecht, Utrecht, Netherlands; Wijburg, F.A., Department of Pediatrics Emma Children's Hospital, Amsterdam University Medical Centre, Amsterdam, Netherlands; Zuberi, S.M., Paediatric Neurosciences Research Group, Royal Hospital for Children & School of Medicine, University of Glasgow, Glasgow, United Kingdom; van Karnebeek, C.D.M., Department of Pediatrics Emma Children's Hospital, Amsterdam University Medical Centre, Amsterdam, Netherlands, Department of Pediatrics, Amalia Children's Hospital, Radboud Centre for Mitochondrial Medicine, Radboud University Medical Center, Nijmegen, Netherlands |
Pyridoxine-dependent epilepsy (PDE-ALDH7A1) is an autosomal recessive condition due to a deficiency of α-aminoadipic semialdehyde dehydrogenase, which is a key enzyme in lysine oxidation. PDE-ALDH7A1 is a developmental and epileptic encephalopathy that was historically and empirically treated with pharmacologic doses of pyridoxine. Despite adequate seizure control, most patients with PDE-ALDH7A1 were reported to have developmental delay and intellectual disability. To improve outcome, a lysine-restricted diet and competitive inhibition of lysine transport through the use of pharmacologic doses of arginine have been recommended as an adjunct therapy. These lysine-reduction therapies have resulted in improved biochemical parameters and cognitive development in many but not all patients. The goal of these consensus guidelines is to re-evaluate and update the two previously published recommendations for diagnosis, treatment, and follow-up of patients with PDE-ALDH7A1. Members of the International PDE Consortium initiated evidence and consensus-based process to review previous recommendations, new research findings, and relevant clinical aspects of PDE-ALDH7A1. The guideline development group included pediatric neurologists, biochemical geneticists, clinical geneticists, laboratory scientists, and metabolic dieticians representing 29 institutions from 16 countries. Consensus guidelines for the diagnosis and management of patients with PDE-ALDH7A1 are provided. © 2020 SSIEM |
ALDH7A1; alpha aminoadipic semialdehyde; consensus guidelines; pyridoxine-dependent epilepsy; pyridoxine-responsive seizures |
aminoadipate semialdehyde dehydrogenase; arginine; pipecolic acid; pyridoxine; aldehyde dehydrogenase; arginine; lysine; pyridoxine; ALDH7A1 gene; apnea; Article; autosomal recessive disorder; coma; developmental delay; developmental screening; diet restriction; diet supplementation; epilepsy; gene; gene mutation; genetic screening; heterozygote detection; homocystinuria; human; hyperargininemia; incidence; infantile spasm; intellectual impairment; lumbar puncture; lysine restricted diet; MELAS syndrome; peripheral neuropathy; prenatal diagnosis; protein restriction; pyridoxine dependent epilepsy; recommended drug dose; sensory neuropathy; urea cycle disorder; consensus; dietary supplement; epilepsy; international cooperation; practice guideline; Aldehyde Dehydrogenase; Arginine; Consensus |
John Wiley and Sons Inc |
01418955 |
|
33200442 |
Article |
Q1 |
1462 |
2367 |
|
|