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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

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 Jul 18; doi: 10.1002/ajmg.a.37849.

PMID:
    27427475
    [PubMed - as supplied by publisher]
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]
Related citations


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. 2016 Jun 16; doi: 10.1111/cge.12819.

PMID:
    27307077
    [PubMed - as supplied by publisher]
Related citations


KATP channel gain-of-function leads to increased myocardial L-type Ca2+ 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 - in process]
Related citations


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 Jun; 745-9. doi: 10.1016/j.ymgmr.2016.03.003.

PMID:
    27114916
    [PubMed]
Related citations


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 - in process]
Related citations


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 - in process]
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]
Related citations


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]
Related citations


Overnight pulse oximetry for evaluation of sleep apnea among children with trisomy 21.
Coverstone AM, Bird M, Sicard M, Tao Y, Grange DK, Cleveland C, Molter D, Kemp JS
J Clin Sleep Med. 2014 Dec 15; 10(12)1309-15. doi: 10.5664/jcsm.4286.

PMID:
    25325597
    [PubMed - indexed for MEDLINE]
Related citations


Cantú Syndrome and Related Disorders
Grange DK, Nichols CG, Singh GK, Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Fong CT, Mefford HC, Smith RJH, Stephens K
. 1993;

PMID:
    25275207
    [PubMed]
Related citations


Fabry disease in infancy and early childhood: a systematic literature review.
Laney DA, Peck DS, Atherton AM, Manwaring LP, Christensen KM, Shankar SP, Grange DK, Wilcox WR, Hopkin RJ
Genet Med. 2015 May; 17(5)323-30. doi: 10.1038/gim.2014.120.

PMID:
    25232851
    [PubMed - indexed for MEDLINE]
Related citations


Fatal human herpesvirus 6-associated encephalitis in two boys with underlying POLG mitochondrial disorders.
Al-Zubeidi D, Thangarajh M, Pathak S, Cai C, Schlaggar BL, Storch GA, Grange DK, Watson ME Jr
Pediatr Neurol. 2014 Sep; 51(3)448-52. doi: 10.1016/j.pediatrneurol.2014.04.006.

PMID:
    25160553
    [PubMed - indexed for MEDLINE]
Related citations