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profileVivian E-An Shih, M.D.

TitleProfessor of Neurology, Emerita
InstitutionMassachusetts General Hospital
DepartmentEmeritus
AddressMassachusetts General Hospital
55 Fruit St
Boston MA 02114
Phone617/726-3884
Fax617/726-5739
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R01EY005633     (SHIH, VIVIAN E)Dec 1, 1984 - Nov 30, 1992
NIH/NEI
MOLECULAR DEFECT IN GYRATE ATROPHY OF CHOROID AND RETIN
Role: Principal Investigator

R01NS005096     (SHIH, VIVIAN E)Sep 1, 1977 - Nov 30, 2001
NIH/NINDS
NEW AMINO ACID DISORDERS IN CEREBRAL DISEASE
Role: Principal Investigator

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Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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  1. Kim SZ, Song WJ, Nyhan WL, Ficicioglu C, Mandell R, Shih VE. Long-term follow-up of four patients affected by HHH syndrome. Clin Chim Acta. 2012 Jul 11; 413(13-14):1151-5. PMID: 22465082.
    View in: PubMed
  2. Ficicioglu C, Mandell R, Shih VE. Argininosuccinate lyase deficiency: longterm outcome of 13 patients detected by newborn screening. Mol Genet Metab. 2009 Nov; 98(3):273-7. PMID: 19635676; PMCID: PMC2773214.
  3. Shih V, Wan HS, Chan A. Clinical predictors of chemotherapy-induced nausea and vomiting in breast cancer patients receiving adjuvant doxorubicin and cyclophosphamide. Ann Pharmacother. 2009 Mar; 43(3):444-52. PMID: 19193584.
    View in: PubMed
  4. Hsu HW, Zytkovicz TH, Comeau AM, Strauss AW, Marsden D, Shih VE, Grady GF, Eaton RB. Spectrum of medium-chain acyl-CoA dehydrogenase deficiency detected by newborn screening. Pediatrics. 2008 May; 121(5):e1108-14. PMID: 18450854.
    View in: PubMed
  5. Shih VE. Alternative-pathway therapy for hyperammonemia. N Engl J Med. 2007 May 31; 356(22):2321-2. PMID: 17538092.
    View in: PubMed
  6. Eichler F, Tan WH, Shih VE, Grant PE, Krishnamoorthy K. Proton magnetic resonance spectroscopy and diffusion-weighted imaging in isolated sulfite oxidase deficiency. J Child Neurol. 2006 Sep; 21(9):801-5. PMID: 16970890.
    View in: PubMed
  7. Wattanasirichaigoon D, Khowsathit P, Visudtibhan A, Suthutvoravut U, Charoenpipop D, Kim SZ, Levy HL, Shih VE. Pericardial effusion in primary systemic carnitine deficiency. J Inherit Metab Dis. 2006 Aug; 29(4):589. PMID: 16830263.
    View in: PubMed
  8. Zeng WQ, Gao H, Brueton L, Hutchin T, Gray G, Chakrapani A, Olpin S, Shih VE. Fumarase deficiency caused by homozygous P131R mutation and paternal partial isodisomy of chromosome 1. Am J Med Genet A. 2006 May 01; 140(9):1004-9. PMID: 16575891.
    View in: PubMed
  9. Browning MF, Levy HL, Wilkins-Haug LE, Larson C, Shih VE. Fetal fatty acid oxidation defects and maternal liver disease in pregnancy. Obstet Gynecol. 2006 Jan; 107(1):115-20. PMID: 16394048.
    View in: PubMed
  10. Tan WH, Eichler FS, Hoda S, Lee MS, Baris H, Hanley CA, Grant PE, Krishnamoorthy KS, Shih VE. Isolated sulfite oxidase deficiency: a case report with a novel mutation and review of the literature. Pediatrics. 2005 Sep; 116(3):757-66. PMID: 16140720.
    View in: PubMed
  11. Cleary MA, Dorland L, de Koning TJ, Poll-The BT, Duran M, Mandell R, Shih VE, Berger R, Olpin SE, Besley GT. Ornithine aminotransferase deficiency: diagnostic difficulties in neonatal presentation. J Inherit Metab Dis. 2005; 28(5):673-9. PMID: 16151897.
    View in: PubMed
  12. Goff DC, Herz L, Posever T, Shih V, Tsai G, Henderson DC, Freudenreich O, Evins AE, Yovel I, Zhang H, Schoenfeld D. A six-month, placebo-controlled trial of D-cycloserine co-administered with conventional antipsychotics in schizophrenia patients. Psychopharmacology (Berl). 2005 Apr; 179(1):144-50. PMID: 15502972.
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  13. Goff DC, Bottiglieri T, Arning E, Shih V, Freudenreich O, Evins AE, Henderson DC, Baer L, Coyle J. Folate, homocysteine, and negative symptoms in schizophrenia. Am J Psychiatry. 2004 Sep; 161(9):1705-8. PMID: 15337665.
    View in: PubMed
  14. Wong W, Tolkoff-Rubin N, Delmonico FL, Cardarelli F, Saidman SL, Farrell ML, Shih V, Winkelmayer WC, Cosimi AB, Pascual M. Analysis of the cardiovascular risk profile in stable kidney transplant recipients after 50% cyclosporine reduction. Clin Transplant. 2004 Aug; 18(4):341-8. PMID: 15233807.
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  15. Baid-Agrawal S, Delmonico FL, Tolkoff-Rubin NE, Farrell M, Williams WW, Shih V, Auchincloss H, Cosimi AB, Pascual M. Cardiovascular risk profile after conversion from cyclosporine A to tacrolimus in stable renal transplant recipients. Transplantation. 2004 Apr 27; 77(8):1199-202. PMID: 15114085.
    View in: PubMed
  16. Stoler JM, Sabry MA, Hanley C, Hoppel CL, Shih VE. Successful long-term treatment of hepatic carnitine palmitoyltransferase I deficiency and a novel mutation. J Inherit Metab Dis. 2004; 27(5):679-84. PMID: 15669684.
    View in: PubMed
  17. Kelly PJ, Kistler JP, Shih VE, Mandell R, Atassi N, Barron M, Lee H, Silveira S, Furie KL. Inflammation, homocysteine, and vitamin B6 status after ischemic stroke. Stroke. 2004 Jan; 35(1):12-5. PMID: 14657454.
    View in: PubMed
  18. Waisbren SE, Albers S, Amato S, Ampola M, Brewster TG, Demmer L, Eaton RB, Greenstein R, Korson M, Larson C, Marsden D, Msall M, Naylor EW, Pueschel S, Seashore M, Shih VE, Levy HL. Effect of expanded newborn screening for biochemical genetic disorders on child outcomes and parental stress. JAMA. 2003 Nov 19; 290(19):2564-72. PMID: 14625333.
    View in: PubMed
  19. Lathrop Stern L, Shane B, Bagley PJ, Nadeau M, Shih V, Selhub J. Combined marginal folate and riboflavin status affect homocysteine methylation in cultured immortalized lymphocytes from persons homozygous for the MTHFR C677T mutation. J Nutr. 2003 Sep; 133(9):2716-20. PMID: 12949355.
    View in: PubMed
  20. Kelly PJ, Shih VE, Kistler JP, Barron M, Lee H, Mandell R, Furie KL. Low vitamin B6 but not homocyst(e)ine is associated with increased risk of stroke and transient ischemic attack in the era of folic acid grain fortification. Stroke. 2003 Jun; 34(6):e51-4. PMID: 12738890.
    View in: PubMed
  21. Picker JD, Puga AC, Levy HL, Marsden D, Shih VE, Degirolami U, Ligon KL, Cederbaum SD, Kern RM, Cox GF. Arginase deficiency with lethal neonatal expression: evidence for the glutamine hypothesis of cerebral edema. J Pediatr. 2003 Mar; 142(3):349-52. PMID: 12640389.
    View in: PubMed
  22. Kelly PJ, Furie KL, Kistler JP, Barron M, Picard EH, Mandell R, Shih VE. Stroke in young patients with hyperhomocysteinemia due to cystathionine beta-synthase deficiency. Neurology. 2003 Jan 28; 60(2):275-9. PMID: 12552044.
    View in: PubMed
  23. Waisbren SE, Read CY, Ampola M, Brewster TG, Demmer L, Greenstein R, Ingham CL, Korson M, Msall M, Pueschel S, Seashore M, Shih VE, Levy HL. Newborn screening compared to clinical identification of biochemical genetic disorders. J Inherit Metab Dis. 2002 Nov; 25(7):599-600. PMID: 12638945.
    View in: PubMed
  24. Kelly PJ, Rosand J, Kistler JP, Shih VE, Silveira S, Plomaritoglou A, Furie KL. Homocysteine, MTHFR 677C-->T polymorphism, and risk of ischemic stroke: results of a meta-analysis. Neurology. 2002 Aug 27; 59(4):529-36. PMID: 12196644.
    View in: PubMed
  25. Levy HL, Vargas JE, Waisbren SE, Kurczynski TW, Roeder ER, Schwartz RS, Rosengren S, Prasad C, Greenberg CR, Gilfix BM, MacGregor D, Shih VE, Bao L, Kraus JP. Reproductive fitness in maternal homocystinuria due to cystathionine beta-synthase deficiency. J Inherit Metab Dis. 2002 Aug; 25(4):299-314. PMID: 12227460.
    View in: PubMed
  26. Zytkovicz TH, Fitzgerald EF, Marsden D, Larson CA, Shih VE, Johnson DM, Strauss AW, Comeau AM, Eaton RB, Grady GF. Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program. Clin Chem. 2001 Nov; 47(11):1945-55. PMID: 11673361.
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  27. Ovuworie CA, Fox ER, Chow CM, Pascual M, Shih VE, Picard MH, Tolkoff-Rubin NE. Vascular endothelial function in cyclosporine and tacrolimus treated renal transplant recipients. Transplantation. 2001 Oct 27; 72(8):1385-8. PMID: 11685108.
    View in: PubMed
  28. Palmer-Toy DE, Szczepiorkowski ZM, Shih V, Van Cott EM. Compatibility of the Abbott IMx homocysteine assay with citrate-anticoagulated plasma and stability of homocysteine in citrated whole blood. Clin Chem. 2001 Sep; 47(9):1704-7. PMID: 11514408.
    View in: PubMed
  29. Dipple KM, Zhang YH, Huang BL, McCabe LL, Dallongeville J, Inokuchi T, Kimura M, Marx HJ, Roederer GO, Shih V, Yamaguchi S, Yoshida I, McCabe ER. Glycerol kinase deficiency: evidence for complexity in a single gene disorder. Hum Genet. 2001 Jul; 109(1):55-62. PMID: 11479736.
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  30. Albers S, Waisbren SE, Ampola MG, Brewster TG, Burke LW, Demmer LA, Filiano J, Greenstein RM, Ingham CL, Korson MS, Marsden D, Schwartz RC, Seashore MR, Shih VE, Levy HL. New England Consortium: a model for medical evaluation of expanded newborn screening with tandem mass spectrometry. J Inherit Metab Dis. 2001 Apr; 24(2):303-4. PMID: 11405349.
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  31. Takeoka M, Soman TB, Shih VE, Caviness VS, Krishnamoorthy KS. Carbamyl phosphate synthetase 1 deficiency: a destructive encephalopathy. Pediatr Neurol. 2001 Mar; 24(3):193-9. PMID: 11301219.
    View in: PubMed
  32. Huang T, Yang W, Pereira AC, Craigen WJ, Shih VE. Cloning and characterization of a putative human d-2-hydroxyacid dehydrogenase in chromosome 9q. Biochem Biophys Res Commun. 2000 Feb 16; 268(2):298-301. PMID: 10679197.
    View in: PubMed
  33. Shih VE, Safran AP, Ropper AH, Tuchman M. Ornithine carbamoyltransferase deficiency: unusual clinical findings and novel mutation. J Inherit Metab Dis. 1999 Jun; 22(5):672-3. PMID: 10399103.
    View in: PubMed
  34. Kraus JP, Janosík M, Kozich V, Mandell R, Shih V, Sperandeo MP, Sebastio G, de Franchis R, Andria G, Kluijtmans LA, Blom H, Boers GH, Gordon RB, Kamoun P, Tsai MY, Kruger WD, Koch HG, Ohura T, Gaustadnes M. Cystathionine beta-synthase mutations in homocystinuria. Hum Mutat. 1999; 13(5):362-75. PMID: 10338090.
    View in: PubMed
  35. Coughlin EM, Christensen E, Kunz PL, Krishnamoorthy KS, Walker V, Dennis NR, Chalmers RA, Elpeleg ON, Whelan D, Pollitt RJ, Ramesh V, Mandell R, Shih VE. Molecular analysis and prenatal diagnosis of human fumarase deficiency. Mol Genet Metab. 1998 Apr; 63(4):254-62. PMID: 9635293.
    View in: PubMed
  36. Zammarchi E, Ciani F, Pasquini E, Buonocore G, Shih VE, Donati MA, Bonocore G. Neonatal onset of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome with favorable outcome. J Pediatr. 1997 Sep; 131(3):440-3. PMID: 9329423.
    View in: PubMed
  37. Evins AE, Amico ET, Shih V, Goff DC. Clozapine treatment increases serum glutamate and aspartate compared to conventional neuroleptics. J Neural Transm (Vienna). 1997; 104(6-7):761-6. PMID: 9444574.
    View in: PubMed
  38. Mandell R, Packman S, Laframboise R, Golbus MS, Schmidt K, Workman L, Saudubray JM, Shih VE. Use of amniotic fluid amino acids in prenatal testing for argininosuccinic aciduria and citrullinaemia. Prenat Diagn. 1996 May; 16(5):419-24. PMID: 8843999.
    View in: PubMed
  39. Shih VE, Fringer JM, Mandell R, Kraus JP, Berry GT, Heidenreich RA, Korson MS, Levy HL, Ramesh V. A missense mutation (I278T) in the cystathionine beta-synthase gene prevalent in pyridoxine-responsive homocystinuria and associated with mild clinical phenotype. Am J Hum Genet. 1995 Jul; 57(1):34-9. PMID: 7611293; PMCID: PMC1801250.
  40. Bostom AG, Shemin D, Nadeau MR, Shih V, Stabler SP, Allen RH, Selhub J. Short term betaine therapy fails to lower elevated fasting total plasma homocysteine concentrations in hemodialysis patients maintained on chronic folic acid supplementation. Atherosclerosis. 1995 Feb; 113(1):129-32. PMID: 7755649.
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  41. Shih VE, Stewart B. Organ donation by a maple syrup urine disease patient. J Inherit Metab Dis. 1995; 18(3):367. PMID: 7474912.
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  42. Hu FL, Gu Z, Kozich V, Kraus JP, Ramesh V, Shih VE. Molecular basis of cystathionine beta-synthase deficiency in pyridoxine responsive and nonresponsive homocystinuria. Hum Mol Genet. 1993 Nov; 2(11):1857-60. PMID: 7506602.
    View in: PubMed
  43. Park JK, Herron BJ, O'Donnell JJ, Shih VE, Ramesh V. Three novel mutations of the ornithine aminotransferase (OAT) gene in gyrate atrophy. Genomics. 1992 Oct; 14(2):553-4. PMID: 1427882.
    View in: PubMed
  44. Shih VE, Laframboise R, Mandell R, Pichette J. Neonatal form of the hyperornithinaemia, hyperammonaemia, and homocitrullinuria (HHH) syndrome and prenatal diagnosis. Prenat Diagn. 1992 Sep; 12(9):717-23. PMID: 1438066.
    View in: PubMed
  45. Hauser SL, Doolittle TH, Lopez-Bresnahan M, Shahani B, Schoenfeld D, Shih VE, Growdon J, Lehrich JR. An antispasticity effect of threonine in multiple sclerosis. Arch Neurol. 1992 Sep; 49(9):923-6. PMID: 1520082.
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  46. Wajner M, Sanseverino MT, Giugliani R, Sweetman L, Yamaguchi S, Fukao T, Shih VE. Biochemical investigation of a Brazilian patient with a defect in mitochondrial acetoacetylcoenzyme-A thiolase. Clin Genet. 1992 Apr; 41(4):202-5. PMID: 1349518.
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  47. Park JK, O'Donnell JJ, Shih VE, Gusella JF, Ramesh V. A 15-bp deletion in exon 5 of the ornithine aminotransferase (OAT) locus associated with gyrate atrophy. Hum Mutat. 1992; 1(4):293-7. PMID: 1301936.
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  48. Ramesh V, Cheng SV, Kozak CA, Herron BJ, Shih VE, Taylor BA, Gusella JF. Mapping of ornithine aminotransferase gene sequences to mouse chromosomes 7, X, and 3. Mamm Genome. 1992; 3(1):17-22. PMID: 1349842.
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  49. Mehta MC, Katsumi O, Shih VE, Hirose T. Gyrate atrophy of the choroid and retina in a 5-year-old girl. Acta Ophthalmol (Copenh). 1991 Dec; 69(6):810-4. PMID: 1789102.
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  50. Shih VE. Detection of hereditary metabolic disorders involving amino acids and organic acids. Clin Biochem. 1991 Aug; 24(4):301-9. PMID: 1959221.
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  51. Kvedar JC, Baden HP, Baden LA, Shih VE, Kolodny EH. Dietary management reverses grooving and abnormal polarization of hair shafts in argininosuccinase deficiency. Am J Med Genet. 1991 Aug 01; 40(2):211-3. PMID: 1897577.
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  52. Ramesh V, Gusella JF, Shih VE. Molecular pathology of gyrate atrophy of the choroid and retina due to ornithine aminotransferase deficiency. Mol Biol Med. 1991 Feb; 8(1):81-93. PMID: 1682785.
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  53. Shih VE, Tenanbaum A. Aminoaciduria due to vinyl-GABA administration. N Engl J Med. 1990 Nov 08; 323(19):1353. PMID: 2215628.
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  54. McClatchey AI, Kaufman DL, Berson EL, Tobin AJ, Shih VE, Gusella JF, Ramesh V. Splicing defect at the ornithine aminotransferase (OAT) locus in gyrate atrophy. Am J Hum Genet. 1990 Nov; 47(5):790-4. PMID: 2220818; PMCID: PMC1683684.
  55. Tuchman M, Knopman DS, Shih VE. Episodic hyperammonemia in adult siblings with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. Arch Neurol. 1990 Oct; 47(10):1134-7. PMID: 2222247.
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  56. Hoop B, Masjedi MR, Shih VE, Kazemi H. Brain glutamate metabolism during hypoxia and peripheral chemodenervation. J Appl Physiol (1985). 1990 Jul; 69(1):147-54. PMID: 1975573.
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  57. Shih VE, Axel SM, Tewksbury JC, Watkins D, Cooper BA, Rosenblatt DS. Defective lysosomal release of vitamin B12 (cb1F): a hereditary cobalamin metabolic disorder associated with sudden death. Am J Med Genet. 1989 Aug; 33(4):555-63. PMID: 2596518.
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  58. Johnson JL, Wuebbens MM, Mandell R, Shih VE. Molybdenum cofactor biosynthesis in humans. Identification of two complementation groups of cofactor-deficient patients and preliminary characterization of a diffusible molybdopterin precursor. J Clin Invest. 1989 Mar; 83(3):897-903. PMID: 2522104; PMCID: PMC303764.
  59. Chadefaux B, Bonnefont JP, Rabier D, Shih VE, Saudubray JM, Kamoun P. Potential for the prenatal diagnosis of hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. Am J Med Genet. 1989 Feb; 32(2):264. PMID: 2929667.
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  60. Shih VE, Mandell R, Berson EL. Pyridoxine effects on ornithine ketoacid transaminase activity in fibroblasts from carriers of two forms of gyrate atrophy of the choroid and retina. Am J Hum Genet. 1988 Dec; 43(6):929-33. PMID: 3195590; PMCID: PMC1715599.
  61. Hoop B, Systrom DM, Shih VE, Kazemi H. Central respiratory effects of glutamine synthesis inhibition in dogs. J Appl Physiol (1985). 1988 Sep; 65(3):1099-109. PMID: 2903134.
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  62. Johnson JL, Wuebbens MM, Mandell R, Shih VE. Molybdenum cofactor deficiency in a patient previously characterized as deficient in sulfite oxidase. Biochem Med Metab Biol. 1988 Aug; 40(1):86-93. PMID: 3219233.
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  63. Ramesh V, McClatchey AI, Ramesh N, Benoit LA, Berson EL, Shih VE, Gusella JF. Molecular basis of ornithine aminotransferase deficiency in B-6-responsive and -nonresponsive forms of gyrate atrophy. Proc Natl Acad Sci U S A. 1988 Jun; 85(11):3777-80. PMID: 3375240; PMCID: PMC280301.
  64. Ramesh V, Benoit LA, Crawford P, Harvey PT, Shows TB, Shih VE, Gusella JF. The ornithine aminotransferase (OAT) locus: analysis of RFLPs in gyrate atrophy. Am J Hum Genet. 1988 Feb; 42(2):365-72. PMID: 2893548; PMCID: PMC1715265.
  65. Ramesh V, Eddy R, Bruns GA, Shih VE, Shows TB, Gusella JF. Localization of the ornithine aminotransferase gene and related sequences on two human chromosomes. Hum Genet. 1987 Jun; 76(2):121-6. PMID: 2886418.
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  66. Amendt BA, Greene C, Sweetman L, Cloherty J, Shih V, Moon A, Teel L, Rhead WJ. Short-chain acyl-coenzyme A dehydrogenase deficiency. Clinical and biochemical studies in two patients. J Clin Invest. 1987 May; 79(5):1303-9. PMID: 3571488; PMCID: PMC424368.
  67. Girgis N, McGravey V, Shah BL, Herrin J, Shih VE. Lethal ornithine transcarbamylase deficiency in a female neonate. J Inherit Metab Dis. 1987; 10(3):274-5. PMID: 3123791.
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  68. Ramesh V, Shaffer MM, Allaire JM, Shih VE, Gusella JF. Investigation of gyrate atrophy using a cDNA clone for human ornithine aminotransferase. DNA. 1986 Dec; 5(6):493-501. PMID: 3816496.
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  69. Kneussl M, Hitzig B, Hoop B, Pappagianopoulos P, Shih V, Kazemi H. [A model of the central control of respiration]. Wien Klin Wochenschr. 1986 Sep 12; 98(17):561-4. PMID: 3765644.
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  70. Applegarth DA, Levy HL, Shih VE, McGillivray B, Wong JT, Toone JR, Kirby LT. Prenatal diagnosis of non-ketotic hyperglycinemia. Prenat Diagn. 1986 Jul-Aug; 6(4):257-63. PMID: 3092206.
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  71. Hoop B, Shih VE, Kazemi H. Relationship between central nervous system hydrogen ion regulation and amino acid metabolism in hypercapnia, II. Am Rev Respir Dis. 1985 Aug; 132(2):248-53. PMID: 2862818.
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  72. Carpenter TO, Levy HL, Holtrop ME, Shih VE, Anast CS. Lysinuric protein intolerance presenting as childhood osteoporosis. Clinical and skeletal response to citrulline therapy. N Engl J Med. 1985 Jan 31; 312(5):290-4. PMID: 3917550.
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  73. Shih VE, Nikiforov V, Carney MM. Acetaminophen metabolite interferes in analysis for amino acids. Clin Chem. 1985 Jan; 31(1):148. PMID: 3965192.
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  74. Ledley FD, Levy HL, Shih VE, Benjamin R, Mahoney MJ. Benign methylmalonic aciduria. N Engl J Med. 1984 Oct 18; 311(16):1015-8. PMID: 6148691.
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  75. Shih VE, Coulombe JT, Wadman SK, Duran M, Waelkens JJ. Occurrences of methylmalonic aciduria and Hartnup disorder in the same family. Clin Genet. 1984 Sep; 26(3):216-20. PMID: 6478642.
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  76. Shih VE, Aubry RH, DeGrande G, Gursky SF, Tanaka K. Maternal isovaleric acidemia. J Pediatr. 1984 Jul; 105(1):77-8. PMID: 6547481.
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  77. Shih VE. Maple-syrup-urine disease. N Engl J Med. 1984 Mar 01; 310(9):596-7. PMID: 6694715.
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  78. Hoop B, Shih VE, Kazemi H. Relationship between central nervous system hydrogen ion regulation and amino acid metabolism in hypercapnia. Am Rev Respir Dis. 1983 Jul; 128(1):45-9. PMID: 6870069.
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  79. Dancis J, Hutzler J, Ampola MG, Shih VE, van Gelderen HH, Kirby LT, Woody NC. The prognosis of hyperlysinemia: an interim report. Am J Hum Genet. 1983 May; 35(3):438-42. PMID: 6407303; PMCID: PMC1685659.
  80. Shih VE. Ampicillin interference with test for sulfite oxidase deficiency. Clin Chem. 1983 Jan; 29(1):211-2. PMID: 6848268.
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  81. Carr DB, Shih VE, Richter JM, Martin JB. Encephalopathy following jejunoileostomy. JAMA. 1982 Feb 26; 247(8):1127. PMID: 7057599.
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  82. Shih VE, Mandell R, Herzfeld A. Defective ornithine metabolism in cultured skin fibroblasts from patients with the syndrome of hyperornithinemia, hyperammonemia and homocitrullinuria. Clin Chim Acta. 1982 Feb 05; 118(2-3):149-57. PMID: 7055977.
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  83. García-Castro JM, Isales-Forsythe CM, Levy HL, Shih VE, Laó-Vélez CR, González-Ríos MD, Reyes de Torres LC. Prenatal diagnosis of nonketotic hyperglycinemia. N Engl J Med. 1982 Jan 14; 306(2):79-81. PMID: 6796891.
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  84. Berson EL, Hanson AH, Rosner B, Shih VE. A two year trial of low protein, low arginine diets or vitamin B6 for patients with gyrate atrophy. Birth Defects Orig Artic Ser. 1982; 18(6):209-18. PMID: 6756498.
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  85. Jacoby LB, Shih VE, Struckmeyer C, Niermeijer MF, Boué J. Variation in argininosuccinate synthetase activity in amniotic fluid cell cultures: implications for prenatal diagnosis of citrullinemia. Clin Chim Acta. 1981 Oct 08; 116(1):1-7. PMID: 7318168.
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  86. Maties M, Shih VE, Evans J, Levy HL. Measurement of methylmalonic acid in urine filter paper specimens by gas chromatography. Clin Chim Acta. 1981 Aug 10; 114(2-3):303-8. PMID: 7285356.
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  87. Shih VE, Berson EL, Gargiulo M. Reduction of hyperornithinemia with a low protein, low arginine diet and pyridoxine in patients with a deficiency of ornithine-ketoacid transaminase (OKT) activity and gyrate atrophy of the choroid and retina. Clin Chim Acta. 1981 Jul 01; 113(3):243-51. PMID: 7261397.
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  88. Berson EL, Shih VE, Sullivan PL. Ocular findings in patients with gyrate atrophy on pyridoxine and low-protein, low-arginine diets. Ophthalmology. 1981 Apr; 88(4):311-5. PMID: 7254777.
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  89. Shih VE. Regulation of ornithine metabolism. Enzyme. 1981; 26(5):254-8. PMID: 7028478.
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  90. Coulombe JT, Shih VE, Levy HL. Massachusetts Metabolic Disorders Screening Program. II. Methylmalonic aciduria. Pediatrics. 1981 Jan; 67(1):26-31. PMID: 7243433.
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  91. Shih VE, Mandell R, Herzfeld A. Defective ornithine metabolism in the syndrome of hyperornithinaemia, hyperammonaemia and homocitrullinuria. J Inherit Metab Dis. 1981; 4(2):95-6. PMID: 6790865.
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  92. Leibel RL, Shih VE, Goodman SI, Bauman ML, McCabe ER, Zwerdling RG, Bergman I, Costello C. Glutaric acidemia: a metabolic disorder causing progressive choreoathetosis. Neurology. 1980 Nov; 30(11):1163-8. PMID: 6775244.
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  93. Geiser CF, Shih VE. Cystathioninuria and its origin in children with hepatoblastoma. J Pediatr. 1980 Jan; 96(1):72-5. PMID: 6243161.
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  94. Shih VE, Carney MM, Mandell R. A simple screening test for sulfite oxidase deficiency: detection of urinary thiosulfate by a modification of Sörbo's method. Clin Chim Acta. 1979 Jul 02; 95(1):143-5. PMID: 509724.
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  95. Pueschel SM, Bresnan MJ, Shih VE, Levy HL. Thiamine-responsive intermittent branched-chain ketoaciduria. J Pediatr. 1979 Apr; 94(4):628-31. PMID: 430308.
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  96. Berson EL, Schmidt SY, Shih VE. Ocular and biochemical abnormalities in gyrate atrophy of the choroid and retina. Ophthalmology. 1978 Oct; 85(10):1018-27. PMID: 740364.
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  97. Shih VE, Tanaka K. Plasma and urine amino acid changes in rats treated with hypoglycin. Clin Chim Acta. 1978 Sep 15; 88(3):539-43. PMID: 699343.
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  98. Shih VE, Berson EL, Mandell R, Schmidt SY. Ornithine ketoacid transaminase deficiency in gyrate atrophy of the choroid and retina. Am J Hum Genet. 1978 Mar; 30(2):174-9. PMID: 655164; PMCID: PMC1685558.
  99. Levy HL, Sepe SJ, Walton DS, Shih VE, Hammersen G, Houghton S, Beutler E. Galactose-1-phosphate uridyl transferase deficiency due to Duarte/galactosemia combined variation: clinical and biochemical studies. J Pediatr. 1978 Mar; 92(3):390-3. PMID: 632977.
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  100. Shih VE, Abroms IF, Johnson JL, Carney M, Mandell R, Robb RM, Cloherty JP, Rajagopalan KV. Sulfite oxidase deficiency. Biochemical and clinical investigations of a hereditary metabolic disorder in sulfur metabolism. N Engl J Med. 1977 Nov 10; 297(19):1022-8. PMID: 302914.
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  101. Levy HL, Sepe SJ, Shih VE, Vawter GF, Klein JO. Sepsis due to Escherichia coli in neonates with galactosemia. N Engl J Med. 1977 Oct 13; 297(15):823-5. PMID: 331112.
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  102. Shih VE, Coulombe JT, Maties M, Levy HL. Methylmalonic aciduria in the newborn. N Engl J Med. 1976 Dec 02; 295(23):1320-1. PMID: 980070.
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  103. Valle D, Goodman SI, Applegarth DA, Shih VE, Phang JM. Type II hyperprolinemia. Delta1-pyrroline-5-carboxylic acid dehydrogenase deficiency in cultured skin fibroblasts and circulating lymphocytes. J Clin Invest. 1976 Sep; 58(3):598-603. PMID: 956388; PMCID: PMC333218.
  104. Tanaka K, Mandell R, Shih VE. Metabolism of [1-(14)C] and [2-(14)C] leucine in cultured skin fibroblasts from patients with isovaleric acidemia. Characterization of metabolic defects. J Clin Invest. 1976 Jul; 58(1):164-72. PMID: 932204; PMCID: PMC333167.
  105. Shih VE. Congenital hyperammonemic syndromes. Clin Perinatol. 1976 Mar; 3(1):3-14. PMID: 954343.
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  106. Shih VE, Hyslop NE. Letter: Chromatographic artifact in urine after normal human serum albumin. N Engl J Med. 1975 Aug 21; 293(8):408-9. PMID: 1152947.
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  107. Riccardi VM, Shih VE, Holmes LB, Nardi GL. Hereditary pancreatitis. Nonspecificity of aminoaciduria and diagnosis of occult disease. Arch Intern Med. 1975 Jun; 135(6):822-5. PMID: 1130928.
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  108. Shih VE, Mandell R, Levy HL, Littlefield JW. Free amino acids in extracts of cultured skin fibroblasts from patients with various amino acid metabolic disorders. Clin Genet. 1975 May-Jun; 7(5):421-5. PMID: 1149313.
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  109. Shih VE, Mandell R. Letter: Metabolic defect in hyperornithinaemia. Lancet. 1974 Dec 21; 2(7895):1522-3. PMID: 4140439.
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  110. Levy HL, Shih VE, Madigan PM. Routine newborn screening for histidinemia. Clinical and biochemical results. N Engl J Med. 1974 Dec 05; 291(23):1214-9. PMID: 4421298.
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  111. Shih VE, Mandell R, Scholl ML. Letter: Historical observation in maple syrup urine disease. J Pediatr. 1974 Dec; 85(6):868-9. PMID: 4419958.
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  112. Shih VE, Glick TH, Bercu BB. Letter: Lysine metabolism in Reye's syndrome. Lancet. 1974 Jul 20; 2(7873):163-4. PMID: 4135529.
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  113. Shih VE, Brink EW, Peneva P, Brody JA. Letter: Aminoacidopathies in Guam. Lancet. 1974 Apr; 1(7859):681. PMID: 4132347.
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  114. Shih VE, Mandell R, Tanaka K. Diagnosis of isovaleric acidemia in cultured fibroblasts. Clin Chim Acta. 1973 Nov 15; 48(4):437-9. PMID: 4761592.
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  115. Kazemi H, Shore NS, Shih VE, Shannon DC. Brain organic buffers in respiratory acidosis and alkalosis. J Appl Physiol. 1973 Apr; 34(4):478-82. PMID: 4698604.
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  116. Levy HL, Shih VE. Screening of galactosemia. N Engl J Med. 1972 Oct 05; 287(14):723. PMID: 5055427.
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  117. Shih VE, Jones TC, Levy HL, Madigan PM. Arginase deficiency in Macaca fascicularis. I. Arginase activity and arginine concentration in erythrocytes and liver. Pediatr Res. 1972 Jun; 6(6):548-51. PMID: 4625814.
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  118. Levy HL, Madigan PM, Shih VE. Massachusetts metabolic disorders screening program. I. Technics and results of urine screening. Pediatrics. 1972 Jun; 49(6):825-36. PMID: 5041315.
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  119. Jacoby LB, Littlefield JW, Milunsky A, Shih VE, Wilroy RS. A microassay for argininosuccinase in cultured cells. Am J Hum Genet. 1972 May; 24(3):321-4. PMID: 5028970; PMCID: PMC1762267.
  120. Shih VE. Early dietary management in an infant with argininosuccinase deficiency: preliminary report. J Pediatr. 1972 Apr; 80(4):645-8. PMID: 5015075.
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  121. Mudd SH, Uhlendorf BW, Freeman JM, Finkelstein JD, Shih VE. Homocystinuria associated with decreased methylenetetrahydrofolate reductase activity. Biochem Biophys Res Commun. 1972 Jan 31; 46(2):905-12. PMID: 5057914.
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  122. Tanaka K, Isselbacher KJ, Shih V. Isovaleric and -methylbutyric acidemias induced by hypoglycin A: mechanism of Jamaican vomiting sickness. Science. 1972 Jan 07; 175(4017):69-71. PMID: 5008580.
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  123. Matthysse S, Lipinski J, Shih V. L-dopa and S-adenosylmethionine. Clin Chim Acta. 1971 Nov; 35(1):253-4. PMID: 5127003.
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  124. Shih VE, Bixby EM, Alpers DH, Bartoscas CS, Thier SO. Studies of intestinal transport defect in Hartnup disease. Gastroenterology. 1971 Oct; 61(4):445-53. PMID: 5157127.
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  125. Levy HL, Shih VE, Karolkewicz V, French WA, Carr JR, Cass V, Kennedy JL, MacCready RA. Persistent mild hyperphenylalaninemia in the untreated state. A prospective study. N Engl J Med. 1971 Aug 19; 285(8):424-9. PMID: 5557279.
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  126. Shih VE, Levy HI, Karolkewicz V, Houghton S, Efron ML, Isselbacher KJ, Beutler E, MacCready RA. Galactosemia screening of newborns in Massachusetts. N Engl J Med. 1971 Apr 08; 284(14):753-7. PMID: 4926707.
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  127. Jones TC, Levy HL, MacCeady RA, Shih VE, Garcia FG. Phenylalanine tolerance tests in simian primates. Proc Soc Exp Biol Med. 1971 Apr; 136(4):1087-90. PMID: 4995137.
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  128. Milunsky A, Littlefield JW, Kanvfer JN, Kolodny EH, Shih VE, Atkins L. Prenatal genetic diagnosis. 3. N Engl J Med. 1970 Dec 31; 283(27):1498-504. PMID: 4992307.
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  129. Milunsky A, Littlefield JW, Kanfer JN, Kolodny EH, Shih VE, Atkins L. Prenatal genetic diagnosis (second of three parts). N Engl J Med. 1970 Dec 24; 283(26):1441-7. PMID: 4098222.
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  130. Milunsky A, Littlefield JW, Kanfer JN, Kolodny EH, Shih VE, Atkins L. Prenatal genetic diagnosis. I. N Engl J Med. 1970 Dec 17; 283(25):1370-81. PMID: 4921307.
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  131. Shih VE. Soybean milk. J Am Diet Assoc. 1970 Dec; 57(6):520-2. PMID: 5536916.
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  132. Shih VE, Efron ML. Pyridoxine-unresponsive homocystinuria. N Engl J Med. 1970 Nov 26; 283(22):1206-8. PMID: 5472941.
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  133. Levy HL, Shih VE, Karolkewicz V, MacCready RA. Screening for phenylketonuria. Lancet. 1970 Sep 05; 2(7671):522-3. PMID: 4194965.
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  134. Shih VE, Littlefield JW. Argininosuccinase activity in amniotic-fluid cells. Lancet. 1970 Jul 04; 2(7662):45. PMID: 4193779.
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  135. Geiser CF, Baez A, Schindler AM, Shih VE. Epithelial hepatoblastoma associated with congenital hemihypertrophy and cystathioninuria: presentation of a case. Pediatrics. 1970 Jul; 46(1):66-73. PMID: 4316375.
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  136. Shih VE, Schulman JD. Ornithine-ketoacid transaminase activity in human skin and amniotic fluid cell culture. Clin Chim Acta. 1970 Jan; 27(1):73-5. PMID: 5412614.
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  137. Levy HL, Baden HP, Shih VE. A simple indirect method of detecting the enzyme defect in histidinemia. J Pediatr. 1969 Dec; 75(6):1056-8. PMID: 5360505.
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  138. Solitare GB, Shih VE, Nelligan DJ, Dolan TF. Argininosuccinic aciduria: clinical, biochemical, anatomical and neuropathological observations. J Ment Defic Res. 1969 Sep; 13(3):153-70. PMID: 5823673.
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  139. Levy HL, Shih VE, Madigan PM, MacCready RA. Transient tyrosinemia in full-term infants. JAMA. 1969 Jul 14; 209(2):249-50. PMID: 5819230.
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  140. Shih VE, Madigan PM. Improved paper-chromatographic method for imino acids. Incorporation of isatin, a color reagent, into developing solvents. Clin Chim Acta. 1969 Jun; 24(3):481-2. PMID: 5790335.
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  141. Efron ML, McPherson TC, Shih VE, Welsh CF, MacCready RA. D-methioninuria due to DL-methionine ingestion. An artefact detected by a mass screening program for errors of amino acid metabolism. Am J Dis Child. 1969 Jan; 117(1):104-7. PMID: 4235732.
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  142. Shih VE, Schulman JD. N-acetylcysteine-cysteine disulfide excretion in the urine following N-acetylcysteine administration. J Pediatr. 1969 Jan; 74(1):129-31. PMID: 5782819.
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  143. Levy HL, Shih VE, Madigan PM, Karolkewicz V, Carr JR, Lum A, Richards AA, Crawford JD, MacCready RA. Hypermethioninemia with other hyperaminoacidemias. Studies in infants on high-protein diets. Am J Dis Child. 1969 Jan; 117(1):96-103. PMID: 5812761.
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  144. Shih VE, Efron ML, Moser HW. Hyperornithinemia, hyperammonemia, and homocitrullinuria. A new disorder of amino acid metabolism associated with myoclonic seizures and mental retardation. Am J Dis Child. 1969 Jan; 117(1):83-92. PMID: 5782534.
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  145. Shih V. Encephalopathy and pyruvate metabolism. N Engl J Med. 1968 Aug 08; 279(6):324. PMID: 5660312.
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  146. Shih VE, Efron ML, Mechanic GL. Rapid short-column chromatography of amino acids. A method for blood and urine specimens in the diagnosis and treatment of metabolic disease. Anal Biochem. 1967 Aug; 20(2):299-311. PMID: 6048171.
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  147. Kennedy C, Shih VE, Rowland LP. Homocystinuria: a report in two siblings. Pediatrics. 1965 Nov; 36(5):736-41. PMID: 5844007.
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