J Lawrence Merritt II, MD

J Lawrence Merritt II, MD

Non-Malignant Transplant Program, Biochemical Genetics, Clinical Genetics

On staff since July 2007

Children's Title: Medical Director, Biochemical Genetics; Program Director, Medical Biochemical Genetics Fellowship

Academic Title: Professor

  • Biography

    Lawrence Merritt, II, MD, is attending physician at Seattle Childrens Hospital and associate professor in the Department of Pediatrics at the University of Washington School of Medicine. He joined Childrens after completing his pediatric residency, medical genetics residency and biochemical genetics fellowship at the Mayo Clinic in Rochester, MN.

     

    He provides inpatient and outpatient clinical services in biochemical genetics at Children's hospital campus in Seattle and Bellevue clinics and at the Spokane newborn screening outreach clinic.  His clinical interests include long-term follow up of infants with abnormal newborn screens, urea cycle disorders and fatty acid oxidation disorders.  His clinical work has led efforts to further understand how we can better promote educational efforts and improve the quality of our follow-up of these infants.  He is the site principle investigator for the Urea Cycle Disorders Consortium Longitudinal study and received a grant to study the effects of newborn screening in patients and families with urea cycle disorders.  He is involved with development of novel pharmaceutical therapies for treatment of urea cycle disorders, fatty acid oxidation disorders, organic acidemias, glycogen storage diseases and lysosomal storage diseases.  The expansion of newborn screening the the past two decades has driven much of his clinical work.  He serves as the Program Director for the Medical Biochemical Genetics Fellowship program here at the University of Washington.

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  • Publications

    Manuscripts in Refereed Journals

    • Golden-Grant K, Merritt JL 2nd, Scott CR
      Ethical considerations of population screening for late-onset genetic disease.
      25677830 Clinical genetics, 2015 Dec. : 88(6)589-92
    • Nagamani SC, Diaz GA, Rhead W, Berry SA, Le Mons C, Lichter-Konecki U, Bartley J, Feigenbaum A, Schulze A, Longo N, Berquist W, Gallagher R, Bartholomew D, Harding CO, Korson MS, McCandless SE, Smith W, Vockley J, Kronn D, Zori R, Cederbaum S, Merritt JL 2nd, Wong D, Coakley DF, Scharschmidt BF, Dickinson K, Marino M, Lee BH, Mokhtarani M
      Self-reported treatment-associated symptoms among patients with urea cycle disorders participating in glycerol phenylbutyrate clinical trials.
      26296711 Molecular genetics and metabolism, 2015 Sept. : 116(1-2)29-34
    • Lee B, Diaz GA, Rhead W, Lichter-Konecki U, Feigenbaum A, Berry SA, Le Mons C, Bartley JA, Longo N, Nagamani SC, Berquist W, Gallagher R, Bartholomew D, Harding CO, Korson MS, McCandless SE, Smith W, Cederbaum S, Wong D, Merritt JL 2nd, Schulze A, Vockley G, Kronn D, Zori R, Summar M, Milikien DA, Marino M, Coakley DF, Mokhtarani M, Scharschmidt BF
      Blood ammonia and glutamine as predictors of hyperammonemic crises in patients with urea cycle disorder.
      25503497 Genetics in medicine : official journal of the American College of Medical Genetics, 2014 Dec 11
    • Merritt JL 2nd, Berry S, Le Mons C, Teigen C, Matern D, Rinaldo P.
      Rationale For Newborn Screening For Proximal Urea Cycle Disorders.
      2014 May
    • Merritt JL 2nd, Vedal S, Abdenur JE, Au SM, Barshop BA, Feuchtbaum L, Harding CO, Hermerath C, Lorey F, Sesser DE, Thompson JD, Yu A
      Infants suspected to have very-long chain acyl-CoA dehydrogenase deficiency from newborn screening.
      24503138 Molecular genetics and metabolism, 2014 April : 111(4)484-92
    • J Lawrence Merritt II, MD, Underhill HR, Hahn SH, Hale S, Merritt JL 2nd.
      Asymptomatic methylmalonic acidemia in a homozygous MUT mutation (p.P86L).
      Pediatr Int., 2013 Dec. : 55(6)e156-8
    • Ruhoy IS, Merritt JL 2nd, Amlie-Lefond C
      Cystathionine Beta-synthase Deficiency Heralded by Cerebral Sinus Venous Thrombosis and Stroke.
      24138954 Pediatric neurology, 2013 Oct.
    • Ah Mew N, Krivitzky L, McCarter R, Batshaw M, Tuchman M, Urea Cycle Disorders Consortium of the Rare Diseases Clinical Research Network
      Clinical outcomes of neonatal onset proximal versus distal urea cycle disorders do not differ.
      22901741 The Journal of pediatrics, 2013 Feb. : 162(2)324-9.e1
    • Merritt JL 2nd, Askenazi D, Hahn SH
      Newborn screening and renal disease: where we have been; where we are now; where we are going.
      21947256 Pediatric nephrology (Berlin, Germany), 2011 Sep 27
    • Lichter-Konecki U, Diaz GA, Merritt JL 2nd, Feigenbaum A, Jomphe C, Marier JF, Beliveau M, Mauney J, Dickinson K, Martinez A, Mokhtarani M, Scharschmidt B, Rhead W
      Ammonia control in children with urea cycle disorders (UCDs); phase 2 comparison of sodium phenylbutyrate and glycerol phenylbutyrate.
      21612962 Molecular genetics and metabolism, 2011 Aug. : 103(4)323-9
    • Seminara J, Tuchman M, Krivitzky L, Krischer J, Lee HS, Lemons C, Baumgartner M, Cederbaum S, Diaz GA, Feigenbaum A, Gallagher RC, Harding CO, Kerr DS, Lanpher B, Lee B, Lichter-Konecki U, McCandless SE, Merritt JL, Oster-Granite ML, Seashore MR, Stricker T, Summar M, Waisbren S, Yudkoff M, Batshaw ML
      Establishing a consortium for the study of rare diseases: The Urea Cycle Disorders Consortium.
      20188616 Molecular genetics and metabolism, 2010 : S97-105
    • Bachmann-Gagescu R, Merritt JL 2nd, Hahn SH
      A cognitively normal PDH-deficient 18-year-old man carrying the R263G mutation in the PDHA1 gene.
      19639391 Journal of inherited metabolic disease, 2009 Jul 29
    • Merritt JL 2nd, Nguyen T, Daniels J, Matern D, Schowalter DB
      Biochemical correction of very long-chain acyl-CoA dehydrogenase deficiency following adeno-associated virus gene therapy.
      19156135 Molecular therapy : the journal of the American Society of Gene Therapy, 2009 March : 17(3)425-9 PMCID:PMC2835097
    • Merritt JL, Lindor NM
      Further clinical description of duplication of Williams-Beuren region presenting with congenital glaucoma and brachycephaly.
      18266245 American journal of medical genetics. Part A, 2008 Apr 15 : 146A(8)1055-8
    • Merritt JL 2nd, Zou Y, Jalal SM, Michels VV
      Delineation of the cryptic 1qter deletion phenotype.
      17304549 American journal of medical genetics. Part A, 2007 Mar 15 : 599-603
    • Hu WT, Kantarci OH, Merritt JL 2nd, McGrann P, Dyck PJ, Lucchinetti CF, Tippmann-Peikert M
      Ornithine transcarbamylase deficiency presenting as encephalopathy during adulthood following bariatric surgery.
      17210820 Archives of neurology, 2007 Jan. : 126-8
    • Merritt JL 2nd, Davis DM, Pittelkow MR, Babovic-Vuksanovic D
      Extensive acrochordons and pancreatic islet-cell tumors in tuberous sclerosis associated with TSC2 mutations.
      16835931 American journal of medical genetics. Part A, 2006 Aug 1 : 140(15)1669-72
    • Merritt JL 2nd, Matern D, Vockley J, Daniels J, Nguyen TV, Schowalter DB
      In vitro characterization and in vivo expression of human very-long chain acyl-CoA dehydrogenase.
      16621643 Molecular genetics and metabolism, 2006 Aug. : 88(4)351-8
    • Merritt JL 2nd, Jalal SM, Barbaresi WJ, Babovic-Vuksanovic D
      14q32.3 deletion syndrome with autism.
      15657934 American journal of medical genetics. Part A, 2005 Feb 15 : 99-100
    • Arakawa T, Chong DK, Merritt JL, Langridge WH
      Expression of cholera toxin B subunit oligomers in transgenic potato plants.
      9423288 Transgenic research, 1997 Nov. : 6(6)403-13

    Book Chapters

    • JL Merritt, II
      Patau Syndrome
      Brenner's Encyclopedia of Genetics, 2nd Edition, 2013 : Vol. 5375-376
    • J Lawrence Merritt II, MD, Sihoun Hahn, MD, PhD
      Chapter 26 Inherited Metabolic Disorders
      Oxford American Handbook of Pediatrics, 2009 : Oxford Uni

    Published Abstracts

    • Merritt JL 2nd.
      Ethical Rationale For Reconsidering Newborn Screening For Proximal Urea Cycle Disorders.
      2014 March
    • Merritt JL 2nd, Abdenur JE, Au SM, Barshop BA, Feuchtbaum L, Harding C, Hermerath C, Lorey FW, Sesser DE, Thompson JD, Vedal S, Yu A.
      Newborn Screening for Infants with Suspected Very Long-Chain Acyl-COA Dehydrogenase Deficiency in the Western United States.
      2010 May 5 - 2013 May 10

    Other Publications

    • JL Merritt, II
      Brachydactyly
      Brenner's Encyclopedia of Genetics, 2nd Edition, 2013 : Vol. 1369-370
    • JL Merritt, II
      Metabolic Disorders, Mutants
      Brenner's Encyclopedia of Genetics, 2nd Edition, 2013 : Vol. 4375-376
    • JL Merritt, II
      Gaucher's Disease
      Brenner's Encyclopedia of Genetics, 2013 : 3(2nd Ed.)162-163
    • Johannesen KM, Gardella E, Linnankivi T, Courage C, de Saint Martin A, Lehesjoki AE, Mignot C, Afenjar A, Lesca G, Abi-Warde MT, Chelly J, Piton A, Merritt JL 2nd, Rodan LH, Tan WH, Bird LM, Nespeca M, Gleeson JG, Yoo Y, Choi M, Chae JH, Czapansky-Beilman D, Reichert SC, Pendziwiat M, Verhoeven JS, Schelhaas HJ, Devinsky O, Christensen J, Specchio N, Trivisano M, Weber YG, Nava C, Keren B, Doummar D, Schaefer E, Hopkins S, Dubbs H, Shaw JE, Pisani L, Myers CT, Tang S, Tang S, Pal DK, Millichap JJ, Carvill GL, Helbig KL, Mecarelli O, Striano P, Helbig I, Rubboli G, Mefford HC, Møller RS
      Defining the phenotypic spectrum of SLC6A1 mutations.
      29315614 Epilepsia, 59(2)389-402 PMCID:PMC5912688
    • Tieder JS, Bonkowsky JL, Etzel RA, Franklin WH, Gremse DA, Herman B, Katz ES, Krilov LR, Merritt JL 2nd, Norlin C, Percelay J, Sapién RE, Shiffman RN, Smith MB, SUBCOMMITTEE ON APPARENT LIFE THREATENING EVENTS.
      Brief Resolved Unexplained Events (Formerly Apparent Life-Threatening Events) and Evaluation of Lower-Risk Infants: Executive Summary.
      27244836 Pediatrics, 137(5)
    • Johannesen KM, Gardella E, Linnankivi T, Courage C, de Saint Martin A, Lehesjoki AE, Mignot C, Afenjar A, Lesca G, Abi-Warde MT, Chelly J, Piton A, Merritt JL 2nd, Rodan LH, Tan WH, Bird LM, Nespeca M, Gleeson JG, Yoo Y, Choi M, Chae JH, Czapansky-Beilman D, Reichert SC, Pendziwiat M, Verhoeven JS, Schelhaas HJ, Devinsky O, Christensen J, Specchio N, Trivisano M, Weber YG, Nava C, Keren B, Doummar D, Schaefer E, Hopkins S, Dubbs H, Shaw JE, Pisani L, Myers CT, Tang S, Tang S, Pal DK, Millichap JJ, Carvill GL, Helbig KL, Mecarelli O, Striano P, Helbig I, Rubboli G, Mefford HC, Møller RS
      Defining the phenotypic spectrum of SLC6A1 mutations.
      29315614 Epilepsia, 59(2)389-402 PMCID:PMC5912688
    • Posset R, Garbade SF, Boy N, Burlina AB, Dionisi-Vici C, Dobbelaere D, Garcia-Cazorla A, de Lonlay P, Teles EL, Vara R, Ah Mew N, Batshaw ML, Baumgartner MR, McCandless S, Seminara J, Summar M, Hoffmann GF, Kölker S, Burgard P, Additional individual contributors of the UCDC and the E-IMD consortium.
      Transatlantic combined and comparative data analysis of 1095 patients with urea cycle disorders-a successful strategy for clinical research of rare diseases.
      29974348 Journal of inherited metabolic disease
    • Tieder JS, Bonkowsky JL, Etzel RA, Franklin WH, Gremse DA, Herman B, Katz ES, Krilov LR, Merritt JL 2nd, Norlin C, Percelay J, Sapién RE, Shiffman RN, Smith MB, SUBCOMMITTEE ON APPARENT LIFE THREATENING EVENTS.
      Brief Resolved Unexplained Events (Formerly Apparent Life-Threatening Events) and Evaluation of Lower-Risk Infants: Executive Summary.
      27244836 Pediatrics, 137(5)

  • Presentations

    Presentation Title Event Location Date
    Neurology Grand Rounds. University of Washington Medical Center Seattle, WA Apr 18, 2013
    A Clinical Approach to Inborn Errors of Metabolism. Excellence in Pediatrics 2012. Madrid, Spain. Nov 29, 2012
    The Diagnosis of Genetic Disease. 3) Northwest Medical Laboratory Symposium. American Society for Clinical Laboratory Science, Region IX. Seattle, WA Oct 23, 2009
    Newborn Screening: An Update on the Expansion. Pediatric Grand Rounds, Seattle Children's Hospital Seattle, WA Jan 29, 2009
    Metabolic Disorders for all ages. WWAMI Visiting Professor Grand Rounds, 2 lectures. St. Lukes Regional Medical Center, Boise, ID and Mercy Medical Center, Nampa, ID Sep 24, 2008

  • Research Funding

    Grant Title Grantor Amount Award Date
    An Open Label Study of the Safety, Efficacy and Pharmacokinetics of Glycerol Phenylbutyrate (GPB; RAVICTI®) in Pediatric Subjects under Two Years of Age with Urea Cycle Disorders (UCDs) (HPN-100-009) Horizon Therapeutics, Inc. Apr 22, 2015 - Jan 27, 2017
    Development of a Long-term Outcome Study of Newborn Screening for Urea Cycle Disorders (NBS+UCD) Horizon Therapeutics, Inc. Investigator Initiated Study Feb 5, 2015 - Mar 31, 2015
    Longitudinal Study of Urea Cycle Disorders, O’Malley Family Foundation O’Malley Family Foundation June 2008 - Dec. 2018

Overview

Board Certification(s)

Pediatrics

Medical/Professional School

Loma Linda University School Of Medicine, Loma Linda, CA

Residency

Mayo Clinic College of Medicine, Rochester, MN

Fellowship

Mayo Clinic College of Medicine, Rochester, MN

Clinical Interests

Treatment and management of individuals with inborn errors of metabolism, with an emphasis on fatty acid oxidation disorders and infants with abnormal newborn screening results.

Research Description

Areas of focus include newborn screening, urea cycle disorders, and fatty acid oxidation disorders

Research Focus Area

Biochemical Genetics, Mitochondrial disease, Translational Research