Department of Pediatrics

Clinical Interests

I take care of children, adolescents, and young adults with diabetes mellitus and endocrine disorders. I am the Medical Director of the Washington University Wolfram Syndrome Clinic and offer care for children, adolescents, and young adults with Wolfram Syndrome. The Washington University Wolfram Syndrome Study Group is conducting a longitudinal natural history study of Wolfram Syndrome, also known as DIDMOAD Syndrome. The clinic is funded by a grant from the National Institutes of Health (Tracking Neurodegeneration in Early Wolfram Syndrome (TRACK), Dr. Tamara Hershey, Principal Investigator). The TRACK team includes Dr. Hershey, Scientific Director along with members in endocrinology, ophthalmology, neurology, audiology, urology, neuroimaging, physical and occupational therapy, psychiatry, genetic counseling, and more. Most have more than 10 years of experience seeing and evaluating more than 40 children and young adults with Wolfram Syndrome. I am happy to provide information to physicians and patients regarding Wolfram Syndrome clinical care and recommendations. Clinical consultations can be arranged with me or any member of the Wolfram team by contacting me or Dr. Hershey. I also have a special interest in neonatal diabetes and provide care or consultation regarding infants, children, and adolescents with neonatal diabetes.

Education

• BS, Vanderbilt University College of Arts and Science, 1982
• MD, Vanderbilt University, 1986

Training

• Residency, University of Texas, Southwestern, Health Science Center at Dallas, 1987 - 1990
• Fellowship, Pediatric Endocrinology, Washington University School of Medicine, 1990 - 1993

Licensure and Board Certification

• 1990 - PresMO, State Medical License
• 1991 - PresAmerican Board of Pediatrics
• 1995 - PresAmerican Board of Pediatrics, Endocrinology

Honors and Awards

• National Merit Scholar
• Phi Beta Kappa
• Scholar of the Child Health Research Center of Excellence in Developmental Biology

Recent Publications view all (54)

Publication Co-Authors

Marwan Shinawi, M.D.

Jennifer A Wambach, M.D.

Linda Manwaring

Neil H. White, M.D.

Charles Canter, M.D.

Ryan P. Duncan, PT, DPT

Gammon M. Earhart, PhD, PT

Abby Hollander, M.D.

Francis Sessions Cole, III, M.D.

Washington University has a long tradition of collaboration amongst colleagues.The faculty above have co-authored publications with Bess A. Marshall, M.D.

1. Abreu D, Stone SI, Pearson TS, Bucelli RC, Simpson AN, Hurst S, Brown CM, Kries K, Onwumere C, Gu H, Hoekel J, Tychsen L, Van Stavern GP, White NH, Marshall BA, Hershey T, Urano F.. A phase Ib/IIa clinical trial of dantrolene sodium in patients with Wolfram syndrome. JCII. 2021;6(15):e145188. doi:10.1172/jci.insight.145188  PMCID:PMC8410026  PMID:43185708 
2. Preneet Cheema Brar, MD, MSCIRyan HekschKristina CossenDiva D De LeonManmohan K KambojSeth D MarksBess A MarshallRyan MillerLaura PageTakara StanleyDeborah MitchellPaul Thornton. Management and appropriate use of diazoxide in infants and children withhyperinsulinism JCEM. 2020. PMID:32810255 
3. Samara, Amjad; Rahn, Rachel; Neyman, Olga; Park, Ki Yun; Samara, Ahmad; Marshall, Bess; Dougherty, Joseph; Hershey, Tamara.. Developmental hypomyelination in Wolfram syndrome: new insights from neuroimaging and gene expression analyses. OJRD. 2019;14(1). PMCID:PMC6889680  PMID:PMC6889680 
4. Sandler-Wilson C; Wambach JA; Marshall BA; Wegner DJ; McAlister W; Cole FS; Shinawi M. . Phenotype and response to growth hormone therapy in siblings with B4GALT7 deficiency. Bone. 2019;124:14-21. doi:10.1016/j.bone.2019.03.029  PMCID:PMC6551519  PMID:30914273 
5. Stone, Stephen; Abreu, Damien; Mahadevan, Jana; Asada, Rie; Kries, Kelly; Graf, Rolf; Marshall, Bess A; Hershey, Tamara; Urano, Fumihiko.. Pancreatic stone protein/regenerating protein is a potential biomarker for endoplasmic reticulum stress in beta cells. Sci. Rep.. 2019;9(1):5199. doi:10.1038/s41598-019-41604-4   
6. Rove KO, Vricella GJ, Hershey T, Thu MH, Lugar HM, Vetter J, Marshall BA, Austin PF. Lower Urinary Tract Dysfunction and Associated Pons Volume in Patients with Wolfram Syndrome. J Urol. 2018. PMID:29883657 
7. Barton, Kevin; Davis, T Keefe; Marshall, Bess; Elward, Alexis; White, Neil H.. Posaconazole-induced hypertension and hypokalemia due to inhibition of the 11beta-hydroxylase enzyme. Clin Kidney J. 2018;11(5):691-693. doi:10.1093/ckj/sfx156   
8. Astuti D, Sabir A, Fulton P, Zatyka M, Williams D, Hardy C, Milan G, Favaretto F, Yu-Wai-Man P, Rohayem J, López de Heredia M, Hershey T, Tranebjaerg L, Chen JH, Chaussenot A, Nunes V, Marshall B, McAfferty S, Tillmann V, Maffei P, Paquis-Flucklinger V, Geberhiwot T, Mlynarski W, Parkinson K, Picard V, Bueno GE, Dias R, Arnold A, Richens C, Paisey R, Urano F, Semple R, Sinnott R, Barrett TG. Monogenic diabetes syndromes: Locus-specific databases for Alström, Wolfram, and Thiamine-responsive megaloblastic anemia. Hum Mutat. 2017;38(7):764-777. PMCID:PMC5535005  PMID:28432734 
9. Remedi MS, Thomas M, Nichols CG, Marshall BA. Sulfonylurea challenge test in subjects diagnosed with type 1 diabetes mellitus. Pediatr Diabetes. 2017. PMCID:PMC5522783  PMID:28111849 
10. Lugar HM, Koller JM, Rutlin J, Marshall BA, Kanekura K, Urano F, Bischoff AN, Shimony JS, Hershey T, Washington University Wolfram Syndrome Research Study Group. Neuroimaging evidence of deficient axon myelination in Wolfram syndrome. Sci Rep. 2016;6:21167. PMCID:PMC4758056  PMID:26888576 
11. Bischoff AN, Reiersen AM, Buttlaire A, Al-Lozi A, Doty T, Marshall BA, Hershey T, Washington University Wolfram Syndrome Research Group. Selective cognitive and psychiatric manifestations in Wolfram Syndrome. Orphanet J Rare Dis. 2015;10(1):66. doi:10.1186/s13023-015-0282-1  PMCID:PMC4450481  PMID:26025012 
12. Marshall BA, Green RP, Wambach J, White NH, Remedi MS, Nichols CG. Remission of severe neonatal diabetes with very early sulfonylurea treatment. Diabetes Care. 2015;38(3):e38-9. doi:10.2337/dc14-2124  PMID:25715421 
13. Lu S, Kanekura K, Hara T, Mahadevan J, Spears LD, Oslowski CM, Martinez R, Yamazaki-Inoue M, Toyoda M, Neilson A, Blanner P, Brown CM, Semenkovich CF, Marshall BA, Hershey T, Umezawa A, Greer PA, Urano F. A calcium-dependent protease as a potential therapeutic target for Wolfram syndrome. Proc Natl Acad Sci U S A. 2014;111(49):E5292-301. doi:10.1073/pnas.1421055111  PMCID:PMC4267371  PMID:25422446 
14. Hoekel J, Chisholm SA, Al-Lozi A, Hershey T, Tychsen L, Washington University Wolfram Study Group. Ophthalmologic correlates of disease severity in children and adolescents with Wolfram syndrome. J AAPOS. 2014;18(5):461-465.e1. doi:10.1016/j.jaapos.2014.07.162  PMID:25439303 
15. Marshall BA, Permutt MA, Paciorkowski AR, Hoekel J, Karzon R, Wasson J, Viehover A, White NH, Shimony JS, Manwaring L, Austin P, Hullar TE, Hershey T, Washington University Wolfram Study Group. Phenotypic characteristics of early Wolfram syndrome. Orphanet J Rare Dis. 2013;8:64. doi:10.1186/1750-1172-8-64  PMCID:PMC3651298  PMID:23981289 
16. Pickett KA, Duncan RP, Hoekel J, Marshall B, Hershey T, Earhart GM, Washington University Wolfram Study Group. Early presentation of gait impairment in Wolfram Syndrome. Orphanet J Rare Dis. 2012;7:92. doi:10.1186/1750-1172-7-92  PMCID:PMC3551701  PMID:23217193 
17. Nguyen C, Foster ER, Paciorkowski AR, Viehoever A, Considine C, Bondurant A, Marshall BA, Hershey T, Washington University Wolfram Study Group. Reliability and validity of the Wolfram Unified Rating Scale (WURS). Orphanet J Rare Dis. 2012;7:89. doi:10.1186/1750-1172-7-89  PMCID:PMC3552944  PMID:23148655 
18. Pickett KA, Duncan RP, Paciorkowski AR, Permutt MA, Marshall B, Hershey T, Earhart GM, Washington University Wolfram Study Group. Balance impairment in individuals with Wolfram syndrome. Gait Posture. 2012;36(3):619-24. doi:10.1016/j.gaitpost.2012.06.008  PMCID:PMC3417287  PMID:22771154 
19. Hershey T, Lugar HM, Shimony JS, Rutlin J, Koller JM, Perantie DC, Paciorkowski AR, Eisenstein SA, Permutt MA, Washington University Wolfram Study Group. Early brain vulnerability in Wolfram syndrome. PLoS One. 2012;7(7):e40604. doi:10.1371/journal.pone.0040604  PMCID:PMC3394712  PMID:22792385 
20. Wambach JA, Marshall BA, Koster JC, White NH, Nichols CG. Successful sulfonylurea treatment of an insulin-naïve neonate with diabetes mellitus due to a KCNJ11 mutation. Pediatr Diabetes. 2010;11(4):286-8. doi:10.1111/j.1399-5448.2009.00557.x  PMCID:PMC3245710  PMID:19656320 
21. Boushey RP, Abadir A, Flamez D, Baggio LL, Li Y, Berger V, Marshall BA, Finegood D, Wang TC, Schuit F, Drucker DJ. Hypoglycemia, defective islet glucagon secretion, but normal islet mass in mice with a disruption of the gastrin gene. Gastroenterology. 2003;125(4):1164-74. PMID:14517799 
22. Hollander AS, Olney RC, Blackett PR, Marshall BA. Fatal malignant hyperthermia-like syndrome with rhabdomyolysis complicating the presentation of diabetes mellitus in adolescent males. Pediatrics. 2003;111(6 Pt 1):1447-52. PMID:12777570 
23. Koster JC, Remedi MS, Flagg TP, Johnson JD, Markova KP, Marshall BA, Nichols CG. Hyperinsulinism induced by targeted suppression of beta cell KATP channels. Proc Natl Acad Sci U S A. 2002;99(26):16992-7. doi:10.1073/pnas.012479199  PMCID:PMC139257  PMID:12486236 
24. Buse MG, Robinson KA, Marshall BA, Hresko RC, Mueckler MM. Enhanced O-GlcNAc protein modification is associated with insulin resistance in GLUT1-overexpressing muscles. Am J Physiol Endocrinol Metab. 2002;283(2):E241-50. doi:10.1152/ajpendo.00060.2002  PMID:12110528 
25. Hansen PA, Marshall BA, Chen M, Holloszy JO, Mueckler M. Transgenic overexpression of hexokinase II in skeletal muscle does not increase glucose disposal in wild-type or Glut1-overexpressing mice. J Biol Chem. 2000;275(29):22381-6. doi:10.1074/jbc.M001946200  PMID:10764781 
26. Davis A, Yarasheski KE, White NH, Canter C, Marshall BA. Defective insulin receptors in Rabson-Mendenhall syndrome cause complete peripheral insulin resistance but minimal hepatic insulin response remains. Pediatr Diabetes. 2000;1(2):66-73. doi:10.1034/j.1399-5448.2000.010203.x  PMID:15016231 
27. Ren JM, Barucci N, Marshall BA, Hansen P, Mueckler MM, Shulman GI. Transgenic mice overexpressing GLUT-1 protein in muscle exhibit increased muscle glycogenesis after exercise. Am J Physiol Endocrinol Metab. 2000;278(4):E588-92. PMID:10751190 
28. Koster JC, Marshall BA, Ensor N, Corbett JA, Nichols CG. Targeted overactivity of beta cell K(ATP) channels induces profound neonatal diabetes. Cell. 2000;100(6):645-54. PMID:10761930 
29. Hsieh PS, Moore MC, Marshall B, Pagliassotti MJ, Shay B, Szurkus D, Neal DW, Cherrington AD. The head arterial glucose level is not the reference site for generation of the portal signal in conscious dogs. Am J Physiol. 1999;277(4 Pt 1):E678-84. PMID:10516127 
30. Marshall BA, Tordjman K, Host HH, Ensor NJ, Kwon G, Marshall CA, Coleman T, McDaniel ML, Semenkovich CF. Relative hypoglycemia and hyperinsulinemia in mice with heterozygous lipoprotein lipase (LPL) deficiency. Islet LPL regulates insulin secretion. J Biol Chem. 1999;274(39):27426-32. PMID:10488074 
31. Cheverud JM, Pletscher LS, Vaughn TT, Marshall B. Differential response to dietary fat in large (LG/J) and small (SM/J) inbred mouse strains. Physiol Genomics. 1999;1(1):33-9. PMID:11015559 
32. Marshall BA, Hansen PA, Ensor NJ, Ogden MA, Mueckler M. GLUT-1 or GLUT-4 transgenes in obese mice improve glucose tolerance but do not prevent insulin resistance. Am J Physiol. 1999;276(2 Pt 1):E390-400. PMID:9950801 
33. Hansen PA, Han DH, Marshall BA, Nolte LA, Chen MM, Mueckler M, Holloszy JO. A high fat diet impairs stimulation of glucose transport in muscle. Functional evaluation of potential mechanisms. J Biol Chem. 1998;273(40):26157-63. PMID:9748297 
34. Hansen PA, Wang W, Marshall BA, Holloszy JO, Mueckler M. Dissociation of GLUT4 translocation and insulin-stimulated glucose transport in transgenic mice overexpressing GLUT1 in skeletal muscle. J Biol Chem. 1998;273(29):18173-9. PMID:9660777 
35. Scrocchi LA, Marshall BA, Cook SM, Brubaker PL, Drucker DJ. Identification of glucagon-like peptide 1 (GLP-1) actions essential for glucose homeostasis in mice with disruption of GLP-1 receptor signaling. Diabetes. 1998;47(4):632-9. PMID:9568697 
36. Wang W, Hansen PA, Marshall BA, Holloszy JO, Mueckler M. Insulin unmasks a COOH-terminal Glut4 epitope and increases glucose transport across T-tubules in skeletal muscle. J Cell Biol. 1996;135(2):415-30. PMCID:PMC2121045  PMID:8896598 
37. Buse MG, Robinson KA, Marshall BA, Mueckler M. Differential effects of GLUT1 or GLUT4 overexpression on hexosamine biosynthesis by muscles of transgenic mice. J Biol Chem. 1996;271(38):23197-202. PMID:8798515 
38. Connolly CC, Ivy RE, Adkins-Marshall BA, Dobbins RL, Neal DW, Williams PE, Cherrington AD. Counterregulation by epinephrine and glucagon during insulin-induced hypoglycemia in the conscious dog. Diabetes Res Clin Pract. 1996;31(1-3):45-56. PMID:8792101 
39. Hansen PA, Gulve EA, Marshall BA, Gao J, Pessin JE, Holloszy JO, Mueckler M. Skeletal muscle glucose transport and metabolism are enhanced in transgenic mice overexpressing the Glut4 glucose transporter. J Biol Chem. 1995;270(4):1679-84. PMID:7829503 
40. Ren JM, Marshall BA, Mueckler MM, McCaleb M, Amatruda JM, Shulman GI. Overexpression of Glut4 protein in muscle increases basal and insulin-stimulated whole body glucose disposal in conscious mice. J Clin Invest. 1995;95(1):429-32. doi:10.1172/JCI117673  PMCID:PMC295454  PMID:7814644 
41. Hresko RC, Murata H, Marshall BA, Mueckler M. Discrete structural domains determine differential endoplasmic reticulum to Golgi transit times for glucose transporter isoforms. J Biol Chem. 1994;269(51):32110-9. PMID:7798206 
42. Marshall BA, Mueckler MM. Differential effects of GLUT-1 or GLUT-4 overexpression on insulin responsiveness in transgenic mice. Am J Physiol. 1994;267(5 Pt 1):E738-44. PMID:7977725 
43. Gulve EA, Ren JM, Marshall BA, Gao J, Hansen PA, Holloszy JO, Mueckler M. Glucose transport activity in skeletal muscles from transgenic mice overexpressing GLUT1. Increased basal transport is associated with a defective response to diverse stimuli that activate GLUT4. J Biol Chem. 1994;269(28):18366-70. PMID:8034582 
44. Marshall BA, Murata H, Hresko RC, Mueckler M. Domains that confer intracellular sequestration of the Glut4 glucose transporter in Xenopus oocytes. J Biol Chem. 1993;268(35):26193-9. PMID:8253739 
45. Marshall BA, Ren JM, Johnson DW, Gibbs EM, Lillquist JS, Soeller WC, Holloszy JO, Mueckler M. Germline manipulation of glucose homeostasis via alteration of glucose transporter levels in skeletal muscle. J Biol Chem. 1993;268(25):18442-5. PMID:8360145 
46. Ren JM, Marshall BA, Gulve EA, Gao J, Johnson DW, Holloszy JO, Mueckler M. Evidence from transgenic mice that glucose transport is rate-limiting for glycogen deposition and glycolysis in skeletal muscle. J Biol Chem. 1993;268(22):16113-5. PMID:8344895 
47. Connolly CC, Adkins-Marshall BA, Neal DW, Pugh W, Jaspan JB, Cherrington AD. Relationship between decrements in glucose level and metabolic response to hypoglycemia in absence of counterregulatory hormones in the conscious dog. Diabetes. 1992;41(10):1308-19. PMID:1397705 
48. Adkins-Marshall B, Pagliassotti MJ, Asher JR, Connolly CC, Neal DW, Williams PE, Myers SR, Hendrick GK, Adkins RB Jr, Cherrington AD. Role of hepatic nerves in response of liver to intraportal glucose delivery in dogs. Am J Physiol. 1992;262(5 Pt 1):E679-86. PMID:1590377 
49. Steiner KE, Stevenson RW, Adkins-Marshall BA, Cherrington AD. The effects of epinephrine on ketogenesis in the dog after a prolonged fast. Metabolism. 1991;40(10):1057-62. PMID:1943732 
50. Cherrington AD, Pagliassotti MJ, Myers SR, Adkins-Marshall B, McGuinness OP. Factors which regulate net hepatic glucose uptake in vivo. JPEN J Parenter Enteral Nutr. 1991;15(3):71S-73S. PMID:1865561 
51. Myers SR, Diamond MP, Adkins-Marshall BA, Williams PE, Stinsen R, Cherrington AD. Effects of small changes in glucagon on glucose production during a euglycemic, hyperinsulinemic clamp. Metabolism. 1991;40(1):66-71. PMID:1984573 
52. Adkins-Marshall BA, Myers SR, Hendrick GK, Williams PE, Triebwasser K, Floyd B, Cherrington AD. Interaction between insulin and glucose-delivery route in regulation of net hepatic glucose uptake in conscious dogs. Diabetes. 1990;39(1):87-95. PMID:2210065 
53. Adkins BA, Myers SR, Hendrick GK, Stevenson RW, Williams PE, Cherrington AD. Importance of the route of intravenous glucose delivery to hepatic glucose balance in the conscious dog. J Clin Invest. 1987;79(2):557-65. doi:10.1172/JCI112847  PMCID:PMC424126  PMID:2879854 
54. Cherrington AD, Stevenson RW, Steiner KE, Davis MA, Myers SR, Adkins BA, Abumrad NN, Williams PE. Insulin, glucagon, and glucose as regulators of hepatic glucose uptake and production in vivo. Diabetes Metab Rev. 1987;3(1):307-32. PMID:3552525