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Sihoun Hahn, MD, PhD

Sihoun Hahn, MD, PhD

Biochemical Genetics, Genetics, Mitochondrial Medicine

On staff since October 2006

Children's Title: Director, Biochemical Genetics

Academic Title: Professor

Research Center: Center for Developmental Therapeutics

Overview

Board Certification(s)
Clinical Biochemical Genetics
Clinical Genetics (MD)
Medical/Professional School
Korea University College of Medicine, Seoul
Korea University College of Medicine, Seoul
Residency
Korea University College of Medicine, Seoul, Pediatrics
Fellowship
National Institute of Health, Bethesda, Medical Genetics
Clinical Interests

Copper transport disorders Mitochondrial disorders

Research Description

My research has been focusing on copper metabolism, population screening for Wilson's disease and mitochondrial disease. The work focuses on developing a newborn screening using tandem mass spectrometry for Wilson's disease, a genetic disease in which the body cannot excrete copper properly leading to its accumulation in various organs including the liver and brain. I am developing an assay that quickly determines if the mitochondrial disease is present. I hope to improve clinical practice through integrated laboratory testing-true translational research.

Lab URL

http://www.seattlechildrens.org/geneticslab

Research Focus Area

Health Promotion and Disease Prevention, Genetics and Developmental Biology, Biochemical Genetics

Awards and Honors

Award NameAward DescriptionAwarded ByAward Date

Luminex Award Grant - $100,000

American College of Medical GeneticsJan. 1, 2009

Publications

Genotype-phenotype correlation in Wilson disease.
Journal of clinical gastroenterology , 2010 Jul: 387-8
Next generation sequence analysis for mitochondrial disorders.
Genome medicine , 2009 Oct 23: 100
Prevalence of ALDH7A1 mutations in 18 North American pyridoxine-dependent seizure (PDS) patients.
Epilepsia , 2009 May: 1167-75
Tryptic peptide analysis of ceruloplasmin in dried blood spots using liquid chromatography-tandem mass spectrometry: application to newborn screening.
Clinical chemistry , 2008 Dec: 1961-8
Nonspecific mitochondrial disease with epilepsy in children: diagnostic approaches and epileptic phenotypes.
Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery , 2007 Nov: 1301-7
Development of a newborn screening follow-up algorithm for the diagnosis of isobutyryl-CoA dehydrogenase deficiency.
Genetics in medicine : official journal of the American College of Medical Genetics , 2007 Feb: 108-16
Normal muscle respiratory chain enzymes can complicate mitochondrial disease diagnosis.
Pediatric neurology , 2006 Oct: 289-92
Retrospective determination of ceruloplasmin in newborn screening blood spots of patients with Wilson disease.
Molecular genetics and metabolism , 2006 Sep-Oct: 134-8
Quantitative determination of succinylacetone in dried blood spots for newborn screening of tyrosinemia type I.
Molecular genetics and metabolism , 2006 May: 16-21
Automated spectrophotometric analysis of mitochondrial respiratory chain complex enzyme activities in cultured skin fibroblasts.
Clinical chemistry , 2005 Nov: 2110-6
Wilson Disease--keeping the bar for diagnosis raised.
Hepatology (Baltimore, Md.) , 2005 Oct: 974
Genotypic differences of MCAD deficiency in the Asian population: novel genotype and clinical symptoms preceding newborn screening notification.
Genetics in medicine : official journal of the American College of Medical Genetics , 2005 May-Jun: 339-43
Screening of newborns and high-risk group of children for inborn metabolic disorders using tandem mass spectrometry in South Korea: a three-year report.
Clinica chimica acta; international journal of clinical chemistry , 2005 Apr: 167-80
A common mutation is associated with a mild, potentially asymptomatic phenotype in patients with isovaleric acidemia diagnosed by newborn screening.
American journal of human genetics , 2004 Dec: 1136-42
Steroid profiling by tandem mass spectrometry improves the positive predictive value of newborn screening for congenital adrenal hyperplasia.
The Journal of clinical endocrinology and metabolism , 2004 Aug: 3687-93
Improved specificity of newborn screening for congenital adrenal hyperplasia by second-tier steroid profiling using tandem mass spectrometry.
Clinical chemistry , 2004 Mar: 621-5
Ethylmalonic encephalopathy is caused by mutations in ETHE1, a gene encoding a mitochondrial matrix protein.
American journal of human genetics , 2004 Feb: 239-52
Molecular analysis of PCCB gene in Korean patients with propionic acidemia.
Molecular genetics and metabolism , 2002 Nov: 209-16
Pilot study of mass screening for Wilson's disease in Korea.
Molecular genetics and metabolism , 2002 Jun: 133-6
Nuclear proteins that bind to metal response element a (MREa) in the Wilson disease gene promoter are Ku autoantigens and the Ku-80 subunit is necessary for basal transcription of the WD gene.
European journal of biochemistry / FEBS , 2002 Apr: 2151-61

Primary Office

Seattle Children's
B-6594 - Biochemical Genetics
4800 Sand Point Way NE
Seattle, WA 98105
206-987-3012

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