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Dorothy Katherine Grange, MD

Professor, Pediatrics
Division of Medical Genetics

Specialty Areas

Genetics - Pediatric
Medical Genetics - Pediatric

Board Certifications

Pediatrics
Medical Genetics - Clinical Genetics

Hospital Affiliations

St. Louis Children's Hospital
Barnes-Jewish Hospital

Areas of Clinical Interest

medical genetics, genetic disease, genetic counseling, inherited disorders, inborn errors of metabolism, phenylketonuria (PKU), birth defects, malformation syndromes, chromosomal disorders, metabolic disorders,Marfan syndrome, connective tissue disorders, overgrowth disorders, craniofacial disorders, pediatric pathology

  • Maps & Directions
  • Education
  • Publication & Research

Maps & Directions

Maps & Directions

St. Louis Children’s Hospital

1 Children’s Place
St. Louis, MO 63110

Suite: Medical Genetics
Office Phone: 314-454-6093
Fax: 314-454-2075

Education

Education

Fellowship: Interinstitute Fellowship, Medical Genetics,National Institutes of Health, Bethesda, Maryland 1989
Residency: Pathology, University of Wisconsin 1986
Residency: Pediatrics, University of Wisconsin 1985
Residency: Pathology, University of Florida, Gainesville, Florida, 1982
Medical Degree: University of Florida College of Medicine, Gainesville, Florida 1981
A.B. Biochemistry: Mount Holyoke College, South Hadley, Massachusetts 1976

Publication & Research

Publication & Research

A postnatal role for embryonic myosin revealed by MYH3 mutations that alter TGFβ signaling and cause autosomal dominant spondylocarpotarsal synostosis.
Zieba J, Zhang W, Chong JX, Forlenza KN, Martin JH, Heard K, Grange DK, Butler MG, Kleefstra T, Lachman RS, Nickerson D, Regnier M, Cohn DH, Bamshad M, Krakow D
Sci Rep. 2017 Feb 16; 741803. doi: 10.1038/srep41803.

PMID:
    28205584
    [PubMed - in process]
Related citations


Classification of Genes: Standardized Clinical Validity Assessment of Gene-Disease Associations Aids Diagnostic Exome Analysis and Reclassifications.
Smith ED, Radtke K, Rossi M, Shinde DN, Darabi S, El-Khechen D, Powis Z, Helbig K, Waller K, Grange DK, Tang S, Farwell Hagman KD
Hum Mutat. 2017 Jan 20; doi: 10.1002/humu.23183.

PMID:
    28106320
    [PubMed - as supplied by publisher]
Related citations


Clinical utility gene card for: Cantú syndrome.
Kirk EP, Scurr I, van Haaften G, van Haelst MM, Nichols CG, Williams M, Smithson SF, Grange DK
Eur J Hum Genet. 2017 Jan 4; doi: 10.1038/ejhg.2016.185.

PMID:
    28051078
    [PubMed - as supplied by publisher]
Related citations


Loss-of-Function Mutations in YY1AP1 Lead to Grange Syndrome and a Fibromuscular Dysplasia-Like Vascular Disease.
Guo DC, Duan XY, Regalado ES, Mellor-Crummey L, Kwartler CS, Kim D, Lieberman K, de Vries BB, Pfundt R, Schinzel A, Kotzot D, Shen X, Yang ML, University of Washington Center for Mendelian Genomics., Bamshad MJ, Nickerson DA, Gornik HL, Ganesh SK, Braverman AC, Grange DK, Milewicz DM
Am J Hum Genet. 2017 Jan 5; 100(1)21-30. doi: 10.1016/j.ajhg.2016.11.008.

PMID:
    27939641
    [PubMed - in process]
Related citations


De novo and inherited mutations in the X-linked gene CLCN4 are associated with syndromic intellectual disability and behavior and seizure disorders in males and females.
Palmer EE, Stuhlmann T, Weinert S, Haan E, Van Esch H, Holvoet M, Boyle J, Leffler M, Raynaud M, Moraine C, van Bokhoven H, Kleefstra T, Kahrizi K, Najmabadi H, Ropers HH, Delgado MR, Sirsi D, Golla S, Sommer A, Pietryga MP, Chung WK, Wynn J, Rohena L, Bernardo E, Hamlin D, Faux BM, Grange DK, Manwaring L, Tolmie J, Joss S, DDD Study., Cobben JM, Duijkers FA, Goehringer JM, Challman TD, Hennig F, Fischer U, Grimme A, Suckow V, Musante L, Nicholl J, Shaw M, Lodh SP, Niu Z, Rosenfeld JA, Stankiewicz P, Jentsch TJ, Gecz J, Field M, Kalscheuer VM
Mol Psychiatry. 2016 Aug 23; doi: 10.1038/mp.2016.135.

PMID:
    27550844
    [PubMed - as supplied by publisher]
Related citations


Brain White Matter Integrity Mediates the Relationship Between Phenylalanine Control and Executive Abilities in Children with Phenylketonuria.
Hood A, Rutlin J, Shimony JS, Grange DK, White DA
JIMD Rep. 2016 Jul 22;

PMID:
    27450369
    [PubMed - as supplied by publisher]
Related citations


Status dystonicus in two patients with SOX2-anophthalmia syndrome and nonsense mutations.
Gorman KM, Lynch SA, Schneider A, Grange DK, Williamson KA, FitzPatrick DR, King MD
Am J Med Genet A. 2016 Nov; 170(11)3048-3050. doi: 10.1002/ajmg.a.37849.

PMID:
    27427475
    [PubMed - in process]
Related citations


Neurologic and neuroimaging manifestations of Cantú syndrome: A case series.
Leon Guerrero CR, Pathak S, Grange DK, Singh GK, Nichols CG, Lee JM, Vo KD
Neurology. 2016 Jul 19; 87(3)270-6. doi: 10.1212/WNL.0000000000002861.

PMID:
    27316244
    [PubMed - in process]
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Utility of clinical high-depth next generation sequencing for somatic variant detection in the PIK3CA-related overgrowth spectrum.
Hucthagowder V, Shenoy A, Corliss M, Vigh-Conrad KA, Storer C, Grange DK, Cottrell CE
Clin Genet. 2017 Jan; 91(1)79-85. doi: 10.1111/cge.12819.

PMID:
    27307077
    [PubMed - in process]
Related citations


K(ATP) channel gain-of-function leads to increased myocardial L-type Ca(2+) current and contractility in Cantu syndrome.
Levin MD, Singh GK, Zhang HX, Uchida K, Kozel BA, Stein PK, Kovacs A, Westenbroek RE, Catterall WA, Grange DK, Nichols CG
Proc Natl Acad Sci U S A. 2016 Jun 14; 113(24)6773-8. doi: 10.1073/pnas.1606465113.

PMID:
    27247394
    [PubMed - indexed for MEDLINE]
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Clinical and radiographic delineation of Bent Bone Dysplasia-FGFR2 type or Bent Bone Dysplasia with Distinctive Clavicles and Angel-shaped Phalanges.
Krakow D, Cohn DH, Wilcox WR, Noh GJ, Raffel LJ, Sarukhanov A, Ivanova MH, Danielpour M, Grange DK, Elliott AM, Bernstein JA, Rimoin DL, Merrill AE, Lachman RS
Am J Med Genet A. 2016 Oct; 170(10)2652-61. doi: 10.1002/ajmg.a.37772.

PMID:
    27240702
    [PubMed - in process]
Related citations


A Recurrent Mosaic Mutation in SMO, Encoding the Hedgehog Signal Transducer Smoothened, Is the Major Cause of Curry-Jones Syndrome.
Twigg SR, Hufnagel RB, Miller KA, Zhou Y, McGowan SJ, Taylor J, Craft J, Taylor JC, Santoro SL, Huang T, Hopkin RJ, Brady AF, Clayton-Smith J, Clericuzio CL, Grange DK, Groesser L, Hafner C, Horn D, Temple IK, Dobyns WB, Curry CJ, Jones MC, Wilkie AO
Am J Hum Genet. 2016 Jun 2; 98(6)1256-65. doi: 10.1016/j.ajhg.2016.04.007.

PMID:
    27236920
    [PubMed - in process]
Related citations


A Syndromic Intellectual Disability Disorder Caused by Variants in TELO2, a Gene Encoding a Component of the TTT Complex.
You J, Sobreira NL, Gable DL, Jurgens J, Grange DK, Belnap N, Siniard A, Szelinger S, Schrauwen I, Richholt RF, Vallee SE, Dinulos MB, Valle D, Armanios M, Hoover-Fong J
Am J Hum Genet. 2016 May 5; 98(5)909-18. doi: 10.1016/j.ajhg.2016.03.014.

PMID:
    27132593
    [PubMed - in process]
Related citations


Relationship between age and white matter integrity in children with phenylketonuria.
Wesonga E, Shimony JS, Rutlin J, Grange DK, White DA
Mol Genet Metab Rep. 2016 Apr 8; 745-9. doi: 10.1016/j.ymgmr.2016.03.003.

PMID:
    27114916
    [PubMed]
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Multigenerational autosomal dominant inheritance of 5p chromosomal deletions.
Zhang B, Willing M, Grange DK, Shinawi M, Manwaring L, Vineyard M, Kulkarni S, Cottrell CE
Am J Med Genet A. 2016 Mar; 170(3)583-93. doi: 10.1002/ajmg.a.37445.

PMID:
    26601658
    [PubMed - indexed for MEDLINE]
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Myhre syndrome: Clinical features and restrictive cardiopulmonary complications.
Starr LJ, Grange DK, Delaney JW, Yetman AT, Hammel JM, Sanmann JN, Perry DA, Schaefer GB, Olney AH
Am J Med Genet A. 2015 Dec; 167A(12)2893-901. doi: 10.1002/ajmg.a.37273.

PMID:
    26420300
    [PubMed - indexed for MEDLINE]
Related citations


Electrophysiologic consequences of KATP gain of function in the heart: Conduction abnormalities in Cantu syndrome.
Levin MD, Zhang H, Uchida K, Grange DK, Singh GK, Nichols CG
Heart Rhythm. 2015 Nov; 12(11)2316-24. doi: 10.1016/j.hrthm.2015.06.042.

PMID:
    26142302
    [PubMed - indexed for MEDLINE]
Related citations


Brainstem disconnection: two additional patients and expansion of the phenotype.
Poretti A, Denecke J, Miller DC, Schiffmann H, Buhk JH, Grange DK, Doherty D, Boltshauser E
Neuropediatrics. 2015 Apr; 46(2)139-44. doi: 10.1055/s-0034-1544127.

PMID:
    25671339
    [PubMed - indexed for MEDLINE]
Related citations


Prolonged exposure to high and variable phenylalanine levels over the lifetime predicts brain white matter integrity in children with phenylketonuria.
Hood A, Antenor-Dorsey JA, Rutlin J, Hershey T, Shimony JS, McKinstry RC, Grange DK, Christ SE, Steiner R, White DA
Mol Genet Metab. 2015 Jan; 114(1)19-24. doi: 10.1016/j.ymgme.2014.11.007.

PMID:
    25481106
    [PubMed - indexed for MEDLINE]
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Craniofacial morphometric analysis of individuals with X-linked hypohidrotic ectodermal dysplasia.
Goodwin AF, Larson JR, Jones KB, Liberton DK, Landan M, Wang Z, Boekelheide A, Langham M, Mushegyan V, Oberoi S, Brao R, Wen T, Johnson R, Huttner K, Grange DK, Spritz RA, Hallgrímsson B, Jheon AH, Klein OD
Mol Genet Genomic Med. 2014 Sep; 2(5)422-9. doi: 10.1002/mgg3.84.

PMID:
    25333067
    [PubMed]
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