FollowMyHealth Patient Portal

Faculty Physicians

Dorothy Katherine Grange, MD

Professor, Pediatrics
Division of Medical Genetics

Specialty Areas

Genetics - Pediatric
Medical Genetics - Pediatric

Board Certifications

Pediatrics
Medical Genetics - Clinical Genetics

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

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


Rare variants in FBN1 and FBN2 are associated with severe adolescent idiopathic scoliosis.
Buchan JG, Alvarado DM, Haller GE, Cruchaga C, Harms MB, Zhang T, Willing MC, Grange DK, Braverman AC, Miller NH, Morcuende JA, Tang NL, Lam TP, Ng BK, Cheng JC, Dobbs MB, Gurnett CA
Hum Mol Genet. 2014 Oct 1; 23(19)5271-82. doi: 10.1093/hmg/ddu224.

PMID:
    24833718
    [PubMed - indexed for MEDLINE]
Related citations


Pathogenic mutations in GLI2 cause a specific phenotype that is distinct from holoprosencephaly.
Bear KA, Solomon BD, Antonini S, Arnhold IJ, França MM, Gerkes EH, Grange DK, Hadley DW, Jääskeläinen J, Paulo SS, Rump P, Stratakis CA, Thompson EM, Willis M, Winder TL, Jorge AA, Roessler E, Muenke M
J Med Genet. 2014 Jun; 51(6)413-8. doi: 10.1136/jmedgenet-2013-102249.

PMID:
    24744436
    [PubMed - indexed for MEDLINE]
Related citations


Single-dose, subcutaneous recombinant phenylalanine ammonia lyase conjugated with polyethylene glycol in adult patients with phenylketonuria: an open-label, multicentre, phase 1 dose-escalation trial.
Longo N, Harding CO, Burton BK, Grange DK, Vockley J, Wasserstein M, Rice GM, Dorenbaum A, Neuenburg JK, Musson DG, Gu Z, Sile S
Lancet. 2014 Jul 5; 384(9937)37-44. doi: 10.1016/S0140-6736(13)61841-3.

PMID:
    24743000
    [PubMed - indexed for MEDLINE]
Related citations


Cantú syndrome resulting from activating mutation in the KCNJ8 gene.
Cooper PE, Reutter H, Woelfle J, Engels H, Grange DK, van Haaften G, van Bon BW, Hoischen A, Nichols CG
Hum Mutat. 2014 Jul; 35(7)809-13. doi: 10.1002/humu.22555.

PMID:
    24700710
    [PubMed - indexed for MEDLINE]
Related citations


Sapropterin dihydrochloride use in pregnant women with phenylketonuria: an interim report of the PKU MOMS sub-registry.
Grange DK, Hillman RE, Burton BK, Yano S, Vockley J, Fong CT, Hunt J, Mahoney JJ, Cohen-Pfeffer JL, Phenylketonuria Demographics Outcomes and Safety (PKUDOS) registry, Maternal Phenylketonuria Observational Program (PKU MOMS) sub-registry
Mol Genet Metab. 2014 May; 112(1)9-16. doi: 10.1016/j.ymgme.2014.02.016.

PMID:
    24667082
    [PubMed - indexed for MEDLINE]
Related citations


Phenylketonuria Scientific Review Conference: state of the science and future research needs.
Camp KM, Parisi MA, Acosta PB, Berry GT, Bilder DA, Blau N, Bodamer OA, Brosco JP, Brown CS, Burlina AB, Burton BK, Chang CS, Coates PM, Cunningham AC, Dobrowolski SF, Ferguson JH, Franklin TD, Frazier DM, Grange DK, Greene CL, Groft SC, Harding CO, Howell RR, Huntington KL, Hyatt-Knorr HD, Jevaji IP, Levy HL, Lichter-Konecki U, Lindegren ML, Lloyd-Puryear MA, Matalon K, MacDonald A, McPheeters ML, Mitchell JJ, Mofidi S, Moseley KD, Mueller CM, Mulberg AE, Nerurkar LS, Ogata BN, Pariser AR, Prasad S, Pridjian G, Rasmussen SA, Reddy UM, Rohr FJ, Singh RH, Sirrs SM, Stremer SE, Tagle DA, Thompson SM, Urv TK, Utz JR, van Spronsen F, Vockley J, Waisbren SE, Weglicki LS, White DA, Whitley CB, Wilfond BS, Yannicelli S, Young JM
Mol Genet Metab. 2014 Jun; 112(2)87-122. doi: 10.1016/j.ymgme.2014.02.013.

PMID:
    24667081
    [PubMed - indexed for MEDLINE]
Related citations


Expanding the clinical and mutational spectrum of Kaufman oculocerebrofacial syndrome with biallelic UBE3B mutations.
Basel-Vanagaite L, Yilmaz R, Tang S, Reuter MS, Rahner N, Grange DK, Mortenson M, Koty P, Feenstra H, Farwell Gonzalez KD, Sticht H, Boddaert N, Désir J, Anyane-Yeboa K, Zweier C, Reis A, Kubisch C, Jewett T, Zeng W, Borck G
Hum Genet. 2014 Jul; 133(7)939-49. doi: 10.1007/s00439-014-1436-2.

PMID:
    24615390
    [PubMed - indexed for MEDLINE]
Related citations


Variability in phenylalanine control predicts IQ and executive abilities in children with phenylketonuria.
Hood A, Grange DK, Christ SE, Steiner R, White DA
Mol Genet Metab. 2014 Apr; 111(4)445-51. doi: 10.1016/j.ymgme.2014.01.012.

PMID:
    24568837
    [PubMed - indexed for MEDLINE]
Related citations


Scoliosis and vertebral anomalies: additional abnormal phenotypes associated with chromosome 16p11.2 rearrangement.
Al-Kateb H, Khanna G, Filges I, Hauser N, Grange DK, Shen J, Smyser CD, Kulkarni S, Shinawi M
Am J Med Genet A. 2014 May; 164A(5)1118-26. doi: 10.1002/ajmg.a.36401.

PMID:
    24458548
    [PubMed - indexed for MEDLINE]
Related citations


Expanding the genotype-phenotype correlation in subtelomeric 19p13.3 microdeletions using high resolution clinical chromosomal microarray analysis.
Peddibhotla S, Khalifa M, Probst FJ, Stein J, Harris LL, Kearney DL, Vance GH, Bull MJ, Grange DK, Scharer GH, Kang SH, Stankiewicz P, Bacino CA, Cheung SW, Patel A
Am J Med Genet A. 2013 Dec; 161A(12)2953-63. doi: 10.1002/ajmg.a.35886.

PMID:
    24123848
    [PubMed - indexed for MEDLINE]
Related citations


White matter integrity and executive abilities following treatment with tetrahydrobiopterin (BH4) in individuals with phenylketonuria.
White DA, Antenor-Dorsey JA, Grange DK, Hershey T, Rutlin J, Shimony JS, McKinstry RC, Christ SE
Mol Genet Metab. 2013 Nov; 110(3)213-7. doi: 10.1016/j.ymgme.2013.07.010.

PMID:
    23928118
    [PubMed - indexed for MEDLINE]
Related citations


White matter integrity and executive abilities in individuals with phenylketonuria.
Antenor-Dorsey JA, Hershey T, Rutlin J, Shimony JS, McKinstry RC, Grange DK, Christ SE, White DA
Mol Genet Metab. 2013 Jun; 109(2)125-31. doi: 10.1016/j.ymgme.2013.03.020.

PMID:
    23608077
    [PubMed - indexed for MEDLINE]
Related citations


Executive response monitoring and inhibitory control in children with phenylketonuria: effects of expectancy.
Araujo GC, Christ SE, Grange DK, Steiner RD, Coleman C, Timmerman E, White DA
Dev Neuropsychol. 2013; 38(3)139-52. doi: 10.1080/87565641.2012.718816.

PMID:
    23573793
    [PubMed - indexed for MEDLINE]
Related citations


KATP channels and cardiovascular disease: suddenly a syndrome.
Nichols CG, Singh GK, Grange DK
Circ Res. 2013 Mar 29; 112(7)1059-72. doi: 10.1161/CIRCRESAHA.112.300514.

PMID:
    23538276
    [PubMed - indexed for MEDLINE]
Related citations


Mutations in ECEL1 cause distal arthrogryposis type 5D.
McMillin MJ, Below JE, Shively KM, Beck AE, Gildersleeve HI, Pinner J, Gogola GR, Hecht JT, Grange DK, Harris DJ, Earl DL, Jagadeesh S, Mehta SG, Robertson SP, Swanson JM, Faustman EM, Mefford HC, Shendure J, Nickerson DA, Bamshad MJ, University of Washington Center for Mendelian Genomics
Am J Hum Genet. 2013 Jan 10; 92(1)150-6. doi: 10.1016/j.ajhg.2012.11.014.

PMID:
    23261301
    [PubMed - indexed for MEDLINE]
Related citations


Processing speed and executive abilities in children with phenylketonuria.
Janos AL, Grange DK, Steiner RD, White DA
Neuropsychology. 2012 Nov; 26(6)735-43. doi: 10.1037/a0029419.

PMID:
    22866986
    [PubMed - indexed for MEDLINE]
Related citations


Cantú syndrome is caused by mutations in ABCC9.
van Bon BW, Gilissen C, Grange DK, Hennekam RC, Kayserili H, Engels H, Reutter H, Ostergaard JR, Morava E, Tsiakas K, Isidor B, Le Merrer M, Eser M, Wieskamp N, de Vries P, Steehouwer M, Veltman JA, Robertson SP, Brunner HG, de Vries BB, Hoischen A
Am J Hum Genet. 2012 Jun 8; 90(6)1094-101. doi: 10.1016/j.ajhg.2012.04.014.

PMID:
    22608503
    [PubMed - indexed for MEDLINE]
Related citations


Mild fumarase deficiency and a trial of low protein diet.
Kimonis VE, Steller J, Sahai I, Grange DK, Shoemaker J, Zelaya BM, Mandell R, Shih K, Shih V
Mol Genet Metab. 2012 Sep; 107(1-2)241-2. doi: 10.1016/j.ymgme.2012.04.010.

PMID:
    22595425
    [PubMed - indexed for MEDLINE]
Related citations


Chromosome 2p15p16.1 microdeletion syndrome: 2.5 Mb deletion in a patient with renal anomalies, intractable seizures and a choledochal cyst.
Hucthagowder V, Liu TC, Paciorkowski AR, Thio LL, Keller MS, Anderson CD, Herman T, Dehner LP, Grange DK, Kulkarni S
Eur J Med Genet. 2012 Aug-Sep; 55(8-9)485-9. doi: 10.1016/j.ejmg.2012.04.003.

PMID:
    22579565
    [PubMed - indexed for MEDLINE]
Related citations