강훈철

  #신경#뇌전증#발달#학습장애#소아수면질환 진단 및 치료

학력

연세대학교 의학과 학사(1992)
연세대학교 대학원 의학석사(2001)
연세대학교 대학원 의학박사(2006)

경력

1993년 연세대학교 세브란스병원 인턴 수료
2001년 연세대학교 세브란스병원 소아과 전공의 수료
2002년 연세대학교 세브란스병원 소아과 연구강사 수료
2002년-2008년 인제대학교 상계백병원 소아과, 뇌전증센터 조교수
2009년-2013년 연세대학교 세브란스병원 소아신경과 임상부교수
2013년-현재 연세대학교 세브란스병원 소아신경과 교수
2017.3.-현재 연세대학교 세브란스 어린이병원 소아신경과 과장

논문

Factors Influencing the Evolution of West Syndrome to Lennox-Gastaut Syndrome
Pediatric Neurology 41/2 :111-113,2009
Clinical characteristics of patients with non-specific and non-categorized mitochondrial diseases
Acta Paediatrica 98/11 :1825-1829,2009
Outcome of surgical treatment in non-lesional intractable childhood epilepsy
Seizure-European Journal Of Epilepsy 18/9 :625-629,2009
편두통의 전 단계인 소아기주기성증후군의 다기관 임상 연구 : 국제두통질환분류 제2판 제1차 수정판 적용
Korean Journal of Pediatrics 52/5 :557-566,2009
저산소 허혈 뇌 손상을 유발시킨 미성숙 흰쥐에서 마우스 골수 기원 중간엽 줄기 세포 이식 후 기능 회복
Korean Journal of Pediatrics 52/7 :824-831,2009
Ictal single-photon emission computed tomography with slow dye injection for determining primary epileptic foci in infantile spasms
Korean Journal of Pediatrics 52/7 :804-810,2009
Resective Pediatric Epilepsy Surgery in Lennox-Gastaut Syndrome
Pediatrics 125/1 :E58-E66,2010
Comparison of temporal lobectomies of children and adults with intractable temporal lobe epilepsy
CHILDS NERVOUS SYSTEM 26/ :177-183,2010
Efficacy and Safety of Adjunctive Levetiracetam Therapy in Pediatric Intractable Epilepsy
Pediatric Neurology 42/2 :86-92,2010
Efficacy and tolerability of adjunctive therapy with zonisamide in childhood intractable epilepsy
Brain & Development 32/2 :208-212,2010
Behavioral improvement after transplantation of neural precursors derived from embryonic stem cells into the globally ischemic brain of adolescent rats
Brain & Development 32/8 :658-668,2010
외측두엽 간질의 수술적 결과
대한소아신경외과학회지 7/1 :31-40,2010
Iron deficiency in children with mitochondrial disease
Metabolic Brain Disease 25/2 :185-189,2010
Electrocardiography as an early cardiac screening test in children with mitochondrial disease
Korean Journal of Pediatrics 54/5 :644-647,2010
Surgical Outcomes and Prognostic Factors after Epilepsy Surgery in Children with Extratemporal Lobe Epilepsy
Journal of the Korean Child Neurology Society(대한소아신경학회지) 18/1 :20-32,2010
소아청소년기 수면 장애의 임상적 연구
Journal of the Korean Child Neurology Society(대한소아신경학회지) 18/2 :187-196,2010
중심-측두엽 극파 동반 양성 간질에서의 인지 행동 관찰 및 topiramate 1일 1회 투약 효과
Journal of the Korean Child Neurology Society(대한소아신경학회지) 18/2 :206-213,2010
Usefulness of liquid ketogenic milk for intractable childhood epilepsy
e-SPEN, the European e-journal of Clinical Nutrition and Metabolism 5/5 :203-207,2010
Risk factors of ocular involvement in children with mitochondrial respiratory chain complex defect
Korean Journal of Pediatrics 53/12 :994-999,2010
Leigh 증후군 환아에서의 안과적 이상 소견
Korean Journal of Pediatrics 53/2 :163-166,2010
Comparison of short-versus long-term ketogenic diet for intractable infantile spasms
EPILEPSIA 52/4 :781-787,2011
Neuroimaging in identifying focal cortical dysplasia and prognostic factors in pediatric and adolescent epilepsy surgery
EPILEPSIA 52/4 :722-727,2011
Uncovered primary seizure foci in Lennox-Gastaut syndrome after corpus callosotomy
BRAIN & DEVELOPMENT 33/8 :672-677,2011
Comparative trial of low- and high-dose zonisamide as monotherapy for childhood epilepsy
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY 20/7 :558-563,2011
Induced Pluripotent Stem Cell Models from X-linked Adrenoleukodystrophy Patients
ANNALS OF NEUROLOGY 70/3 :402-409,2011
Evaluation of Algorithms for Intracranial EEG (iEEG) Source Imaging of Extended Sources: Feasibility of Using iEEG Source Imaging for Localizing Epileptogenic Zones in Secondary Generalized Epilepsy
BRAIN TOPOGRAPHY 24/ :91-104,2011
Localization of ictal onset zones in Lennox-Gastaut syndrome using directional connectivity analysis of intracranial electroencephalography
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY 20/6 :449-457,2011
A Case of Metachromatic Leukodystrophy Confirmed by Molecular Genetic Analysis
Journal of the Korean Child Neurology Society(대한소아신경학회지) 18/3 :272-276,2011
A Case of Early Onset MELAS Patient with Wolff-Parkinson-White Syndrome
Journal of the Korean Child Neurology Society(대한소아신경학회지) 18/3 :266-271,2011
A1 Segment Hypoplasia/aplasia Detected by Magnetic Resonance Angiography in Neuropediatric Patients
Journal of the Korean Child Neurology Society(대한소아신경학회지) 18/3 :231-239,2011
Various indications for a modified Atkins diet in intractable childhood epilepsy
BRAIN & DEVELOPMENT 34/ :570-575,2012
Highly pure and expandable PSA-NCAM-positive neural precursors from human ESC and iPSC-derived neural rosettes
PLOS ONE 7/7 :e39715-e39715,2012
Rufinamide as an adjuvant treatment in children with Lennox-Gastaut syndrome
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY 21/4 :288-291,2012
Genetic and Epileptic Features in Rett Syndrome
YONSEI MEDICAL JOURNAL 53/3 :495-500,2012
Localization of ictal onset zones in Lennox-Gastaut syndrome (LGS) based on information theoretical time delay analysis of intracranial electroencephalography (iEEG)
EPILEPSY RESEARCH 99/1-2 :78-86,2012
발작과 뇌전증에 대한 개정된 용어 및 개념: ILAE의 분류 및 용어 관련 보고(2005-2009) 한글판
Journal of Korean Epilepsy Society 16/1 :1-8,2012
Safety and role of ketogenic parenteral nutrition for intractable childhood epilepsy
BRAIN & DEVELOPMENT 34/8 :620-624,2012
Disease-specific induced pluripotent stem cells: a platform for human disease modeling and drug discovery.
EXPERIMENTAL AND MOLECULAR MEDICINE 44/3 :202-213,2012
Early cardiac evaluation in children with non-specific mitochondrial disease with isolated mitochondrial respiratory chain complex i defect
JOURNAL OF PAEDIATRICS AND CHILD HEALTH 48/11 :1016-1020,2012
Is the ketogenic diet effective in specific epilepsy syndromes?
EPILEPSY RESEARCH 100/3 :252-257,2012
A multicenter trial of oxcarbazepine oral suspension monotherapy in children newly diagnosed with partial seizures: A clinical and cognitive evaluation
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY 21/9 :679-684,2012
New Classification of Focal Cortical Dysplasia: Application to Practical Diagnosis
Journal of Epilepsy Research 2/2 :38-42,2012
Epilepsy in Korean patients with Angelman syndrome
Korean Journal of Pediatrics 55/5 :171-176,2012
Catch-up growth after long-term implementation and weaning from ketogenic diet in pediatric epileptic patients
CLINICAL NUTRITION 32/1 :98-103,2013
Mitochondrial disease and epilepsy
BRAIN & DEVELOPMENT 35/35 :757-761,2013
The Genetically Modified Polysialylated Form of Neural Cell Adhesion Molecule-Positive Cells for Potential Treatment of X-Linked Adrenoleukodystrophy
YONSEI MEDICAL JOURNAL 54/1 :246-252,2013
Seizure outcome of infantile spasms with focal cortical dysplasia
BRAIN & DEVELOPMENT 35/35 :816-820,2013
Clinical analysis of catastrophic epilepsy in infancy and early childhood: Results of the Far-East Asia Catastrophic Epilepsy (FACE) study group
BRAIN & DEVELOPMENT 35/1 :786-792,2013
Localization of epileptogenic zones in Lennox-Gastaut syndrome using frequency domain source imaging of intracranial electroencephalography: a preliminary investigation
PHYSIOLOGICAL MEASUREMENT 34/1 :247-263,2013
Lower fat and better quality diet therapy for children with pharmacoresistant epilepsy
Korean Journal of Pediatrics 56/8 :327-331,2013
Interferon beta-1b Treatment in a Korean Girl with Multiple Sclerosis
Journal of the Korean Child Neurology Society(대한소아신경학회지) 21/1 :28-32,2013
Clinical Presentations and the Bacterial Meningitis Score in Children with Cerebrospinal Fluid (CSF) Pleocytosis
Journal of the Korean Child Neurology Society(대한소아신경학회지) 21/2 :46-52,2013
Schilder's disease in a young child with tumefactive demyelinating brain lesion
NEUROLOGY ASIA 18/4 :419-421,2013
The Significance of Insular Hypometabolism in Temporal Lobe Epilepsy in Children
Journal of Epilepsy Research 3/2 :54-62,2013
Epilepsy surgery in pediatric intractable epilepsy with destructive encephalopathy.
Journal of Epilepsy Research 3/2 :48-53,2013
Polyunsaturated fatty acid-enriched diet therapy for a child with epilepsy
BRAIN & DEVELOPMENT 36/Issue2 :163-166,2014
Changing name of epilepsy in Korea; cerebroelectric disorder(noi-jeon-jeung,뇌전증,): my epilepsy story.
EPILEPSIA 55/3 :384-386,2014
Clinical and Genetic Aspects in Twelve Korean Patients with Adrenomyeloneuropathy
YONSEI MEDICAL JOURNAL 55/3 :676-682,2014
Outcomes of epilepsy surgery in childhood-onset epileptic encephalopathy
BRAIN & DEVELOPMENT 36/6 :496-504,2014
Primary central nervous system anaplastic large cell lymphoma presenting initially with meningitis
NEUROLOGY ASIA 19/ :231-234,2014
Combined Use of Multiple Computational Intracranial EEG Analysis Techniques for the Localization of Epileptogenic Zones in Lennox-Gastaut Syndrome
CLINICAL EEG AND NEUROSCIENCE 45/3 :169-178,2014
The impact of an exercise therapy on psychosocial health of children with benign epilepsy: A pilot study
EPILEPSY & BEHAVIOR 37/ :151-156,2014
Epilepsy-related clinical factors and psychosocial functions in pediatric epilepsy
EPILEPSY & BEHAVIOR 37c/- :43-48,2014
Adjunctive levetiracetam treatment in pediatric lennox-gastaut syndrome
PEDIATRIC NEUROLOGY 51/4 :527-531,2014
A modified Atkin's diet for an infant with pyruvate dehydrogenase complex deficiency confirmed by PDHA1 gene mutation
NEUROLOGY ASIA 19/3 :327-329,2014
Congenital muscular dystrophy type 1A with residual merosin expression
Korean Journal of Pediatrics 57/3 :149-152,2014
Induced pluripotent stem cells for modeling of pediatric neurological disorders
BIOTECHNOLOGY JOURNAL 9/7 :871-881,2014
Psychological Problems and Clinical Outcomes of Children with Psychogenic Non-Epileptic Seizures
YONSEI MEDICAL JOURNAL 55/6 :1556-1561,2014
Isolated Cerebellar Variant of Adrenoleukodystrophy with a de novo Adenosine Triphosphate-Binding Cassette D1 (ABCD1) Gene Mutation
YONSEI MEDICAL JOURNAL 55/4 :1157-1160,2014
Wernicke’s encephalopathy in a child with high dose thiamine therapy.
Korean Journal of Pediatrics 57/11 :496-499,2014
Localization of epileptogenic zones in Lennox-Gastaut syndrome (LGS) using graph theoretical analysis of ictal intracranial EEG: A preliminary investigation
BRAIN & DEVELOPMENT 37/1 :29-36,2015
Neurocognitive function in children after anterior temporal lobectomy with amygdalohippocampectomy
PEDIATRIC NEUROLOGY 52/1 :88-93,2015
Long-term prognosis of patients with Lennox-Gastaut syndrome in recent decades
EPILEPSY RESEARCH 110/- :10-19,2015
Screening for depression and anxiety disorder in children with headache
Korean Journal of Pediatrics 58/2 :64-68,2015
Brain somatic mutations in MTOR cause focal cortical dysplasia type II leading to intractable epilepsy
NATURE MEDICINE 21/4 :395-400,2015
Neuropsychological effects of levetiracetam and carbamazepine in children with focal epilepsy
NEUROLOGY 84/23 :2312-2319,2015
결절성 경화증 (Tuberous sclerosis) 환자에서 뇌실막밑 거대세포 별아교세포종의 (Subependymal giant cell astrocytoma) 치료
Journal of the Korean Child Neurology Society(대한소아신경학회지) 23/1 :1-6,2015
Applying the bacterial meningitis score in children with cerebrospinal fluid pleocytosis: a single center’s experience
Korean Journal of Pediatrics 58/7 :251-255,2015
Chronic inflammatory demyelinating polyneuropathy in children: a report of four patients with variable relapsing courses
Korean Journal of Pediatrics 58/5 :194-198,2015
부신백질형성장애증 섬유모세포에서 발프로산의 항산화능
Journal of the Korean Child Neurology Society(대한소아신경학회지) 23/2 :45-50,2015
Clinical and Genetic Aspects of the Segmental Overgrowth Spectrum Due to Somatic Mutations in PIK3CA
JOURNAL OF PEDIATRICS 167/5 :957-962,2015
An AKT3-FOXG1-reelin network underlies defective migration in human focal malformations of cortical development
NATURE MEDICINE 21/12 :1445-1456,2015
Spinal Cord Tumor Presenting as Secondary Torticollis: A Case Report
Journal of the Korean Child Neurology Society(대한소아신경학회지) 23/3 :135-137,2015
Parry-Romberg syndrome with ipsilateral hemipons involvement presenting as monoplegic ataxia
Korean Journal of Pediatrics 58/9 :354-357,2015
Efficacy of the classic ketogenic and the modified Atkins diets in refractory childhood epilepsy
EPILEPSIA 57/1 :51-58,2016
The Impact of a 35-Week Long-Term Exercise Therapy on Psychosocial Health of Children With Benign Epilepsy
JOURNAL OF CHILD NEUROLOGY 31/8 :985-990,2016
Surgical treatment of pediatric focal cortical dysplasia: Clinical spectrum and surgical outcome
NEUROLOGY 30;87/9 :945-951,2016
미토콘드리아 호흡연쇄 복합체 결함이 동반된 난치성 소아 뇌전증에서의 Zonisamide 부가 요법
Journal of the Korean Child Neurology Society(대한소아신경학회지) 24/3 :89-94,2016
25-hydroxycholesterol contributes to cerebral inflammation of X-linked adrenoleukodystrophy through activation of the NLRP3 inflammasome
NATURE COMMUNICATIONS 7/ :13,129-,2016
X연관 부신백질이영양증의 분류, 진단 및 치료의 최신 지견
Journal of the Korean Child Neurology Society(대한소아신경학회지) 24/3 :71-84,2016
Juvenile Myasthenia Gravis in Korea: Subgroup Analysis According to Sex and Onset Age
JOURNAL OF CHILD NEUROLOGY 31/14 :1561-4568,2016
Epilepsy Characteristics and Clinical Outcome in Patients With Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes (MELAS)
PEDIATRIC NEUROLOGY 64/- :59-65,2016
Ketogenic Diet for Children with Epilepsy : A Practical Meal Plan in a Hospital
Clinical nutrition research 5/1 :60-63,2016
Automatic Identification of Interictal Epileptiform Discharges in Secondary Generalized Epilepsy.
COMPUTATIONAL AND MATHEMATICAL METHODS IN MEDICINE 2016/Arti :1-10,2016
Estimating the Prevalence of Treated Epilepsy Using Administrative Health Data and Its Validity: ESSENCE Study
JOURNAL OF CLINICAL NEUROLOGY 12/4 :434-440,2016
Cause of Death in Children With Mitochondrial Diseases
PEDIATRIC NEUROLOGY 66/ :82-88,2017
Somatic Mutations in TSC1 and TSC2 Cause Focal Cortical Dysplasia
AMERICAN JOURNAL OF HUMAN GENETICS 100/3 :454-472,2017
Ketogenic Diet for the Management of Epilepsy Associated with Tuberous Sclerosis Complex in Children
Journal of Epilepsy Research 7/1 :45-49,2017
Could Cannabidiol be a Treatment Option for Intractable Childhood and Adolescent Epilepsy?
Journal of Epilepsy Research 7/1 :16-20,2017
Long-term epilepsy-associated tumor in the amygdala of a 16-year-old boy: report of a rare case having intranuclear filaments
BRAIN TUMOR PATHOLOGY 34/4 :172-178,2017
드라베 증후군의 SCN1A 유전자 변이 양상
Journal of the Korean Child Neurology Society(대한소아신경학회지) 25/1 :9-12,2017
A Patient Diagnosed with Spinocerebellar Ataxia Type 5 associated with SPTBN2: Case Repor
Journal of the Korean Child Neurology Society(대한소아신경학회지) 25/3 :200-203,2017
Quinidine Trial in a Patient with Epilepsy of Infancy with Migrating Focal Seizure and KCNT1 Mutation
Journal of the Korean Child Neurology Society(대한소아신경학회지) 25/3 :169-173,2017
Discontinuing Antiepileptic Drugs after Pediatric Epilepsy Surgery for Focal Cortical Dysplasia
Journal of the Korean Child Neurology Society(대한소아신경학회지) 25/3 :127-132,2017
Low glycemic index treatment in patients with drug-resistant epilepsy
BRAIN & DEVELOPMENT 39/8 :687-692,2017
Mowat-Wilson syndrome presenting with fever-associated seizures.
EPILEPTIC DISORDERS 19/4 :481-485,2017
Generation of two induced pluripotent stem cell (iPSC) lines from X-linked adrenoleukodystrophy (X-ALD) patients with adrenomyeloneuropathy (AMN)
STEM CELL RESEARCH 25/ :46-49,2017
Alpers-Huttenlocher Syndrome First Presented with Hepatic Failure: Can Liver Transplantation Be Considered as Treatment Option?
Pediatric Gastroenterology, Hepatology & Nutrition 20/4 :259-262,2017
Efficacy of Stiripentol in Dravet Syndrome with or without SCN1A Mutations
JOURNAL OF CLINICAL NEUROLOGY 14/1 :22-28,2018
Efficient strategy for the molecular diagnosis of intractable early-onset epilepsy using targeted gene sequencing
BMC MEDICAL GENOMICS 11/1 :6-,2018
Targeted gene panel and genotype-phenotype correlation in children with developmental and epileptic encephalopathy
EPILEPSY RESEARCH 141/ :48-55,2018
Differential effects on sodium current impairments by distinct SCN1A mutations in GABAergic neurons derived from Dravet syndrome patients
BRAIN & DEVELOPMENT 40/4 :287-298,2018
Vigabatrin and high-dose prednisolone therapy for patients with West syndrome
EPILEPSY RESEARCH 145/1 :127-133,2018
Adverse Events During Perampanel Adjunctive Therapy in Intractable Epilepsy
JOURNAL OF CLINICAL NEUROLOGY 14/3 :296-302,2018
Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group
EPILEPSIA 3/2 :175-192,2018
The efficacy of ketogenic diet for specific genetic mutation in developmental and epileptic encephalopathy
FRONTIERS IN NEUROLOGY 9/JUL :530-538,2018
Three-dimensional brain-like microenvironments facilitate the direct reprogramming of fibroblasts into therapeutic neurons
Nature biomedical engineering 2/7 :522-539,2018
Brain Somatic Mutations in MTOR Disrupt Neuronal Ciliogenesis, Leading to Focal Cortical Dyslamination
NEURON 99/1 :83-97,2018
Interregional metabolic connectivity of 2-deoxy-2[18 F]fluoro-D-glucose positron emission tomography in vagus nerve stimulation for pediatric patients with epilepsy: A retrospective cross-sectional study.
EPILEPSIA 59/12 :2,249-2,259,2018
Early Diagnosis of KBG Syndrome Using Diagnostic Exome Sequencing
Journal of the Korean Child Neurology Society(대한소아신경학회지) 26/4 :272-275,2018
Brain somatic mutations in SLC35A2 cause intractable epilepsy with aberrant N-glycosylation
Neurology: Genetics 4/6 :e294-e302,2018
Long-term Outcome of Resective Epilepsy Surgery in Patients With Lennox-Gastaut Syndrome
PEDIATRICS 142/4 :e20180449-e20180459,2018
Age-Based Characteristics of West Syndrome in Patients with Mitochondrial Disease
Journal of the Korean Child Neurology Society(대한소아신경학회지) 26/4 :197-204,2018
Rufinamide efficacy and safety in children aged 1-4?years with Lennox-Gastaut syndrome
BRAIN & DEVELOPMENT 40/10 :897-903,2018
BRAF somatic mutation contributes to intrinsic epileptogenicity in pediatric brain tumors
NATURE MEDICINE 24/11 :1,662-1,668,2018
Epilepsy Surgery for Children With Low-Grade Epilepsy-Associated Tumors: Factors Associated With Seizure Recurrence and Cognitive Function
PEDIATRIC NEUROLOGY 91/ :50-56,2019
Medication self-management and the quality of discharge education among parents of children with epilepsy
EPILEPSY & BEHAVIOR 94/5 :14-19,2019
Aligned Brain Extracellular Matrix Promotes Differentiation and Myelination of Human-Induced Pluripotent Stem Cell-Derived Oligodendrocytes
ACS APPLIED MATERIALS & INTERFACES 11/17 :15,344-15,353,2019
The use of technical replication for detection of low-level somatic mutations in next-generation sequencing
NATURE COMMUNICATIONS 10/1 :1047-,2019
A Child With Lymphangioma Due to Somatic Mutation in PIK3CA Successfully Treated With Everolimus
PEDIATRIC NEUROLOGY 91/ :65-67,2019
Clobazam as an adjunctive treatment for infantile spasms
EPILEPSY & BEHAVIOR 95/ :161-165,2019
Short- and long-term seizure-free outcomes of dietary treatment in infants according to etiology
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY 71/ :100-104,2019
Optimized Treatment for Infantile Spasms: Vigabatrin versus Prednisolone versus Combination Therapy
Journal of Clinical Medicine 8/10 :1591-1-1591-10,2019
Proband-Only Clinical Exome Sequencing for Neurodevelopmental Disabilities
PEDIATRIC NEUROLOGY 99/ :47-54,2019
Clinical Implementation of Targeted Gene Sequencing for Malformation of Cortical Development
PEDIATRIC NEUROLOGY 99/ :47-54,2019
Genetic and clinical features of SCN8A developmental and epileptic encephalopathy
EPILEPSY RESEARCH 158/1 :e106222-,2019
Precise detection of low-level somatic mutation in resected epilepsy brain tissue.
ACTA NEUROPATHOLOGICA 138/6 :901-912,2019
Brain somatic mutations in MTOR reveal translational dysregulations underlying intractable focal epilepsy
JOURNAL OF CLINICAL INVESTIGATION 129/10 :4,207-4,223,2019

저서

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보도자료

KAIST, 뇌전증 동반 소아 뇌종양 근본원인 밝혀
https://news.naver.com/main/read.nhn?mode=LSD&mid=sec&sid1=102&oid=030&aid=0002742050

KAIST, 난치성 뇌전증의 새로운 유전자 진단법 개발
https://www.sedaily.com/NewsView/1VMYDCMOVX

'난치성 뇌전증 원인 돌연변이' 더 정확히 분석한다
https://www.yna.co.kr/view/AKR20190813039600063?input=1195m

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