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Vivian E-An Shih, M.D.

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Research
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  1. R01EY005633 (SHIH, VIVIAN E) Dec 1, 1984 - Nov 30, 1992
    NIH/NEI
    MOLECULAR DEFECT IN GYRATE ATROPHY OF CHOROID AND RETIN
    Role: Principal Investigator
  2. R01NS005096 (SHIH, VIVIAN E) Sep 1, 1977 - Nov 30, 2001
    NIH/NINDS
    NEW AMINO ACID DISORDERS IN CEREBRAL DISEASE
    Role: Principal Investigator

Bibliographic
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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PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
  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.
    Citations: 2     Fields:    Translation:Humans
  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.
    Citations: 11     Fields:    Translation:HumansPHPublic Health
  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.
    Citations: 12     Fields:    Translation:Humans
  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.
    Citations: 8     Fields:    Translation:HumansPHPublic Health
  5. Shih VE. Alternative-pathway therapy for hyperammonemia. N Engl J Med. 2007 May 31; 356(22):2321-2. PMID: 17538092.
    Citations: 2     Fields:    Translation:Humans
  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.
    Citations: 2     Fields:    Translation:Humans
  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.
    Citations:    Fields:    Translation:Humans
  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.
    Citations: 5     Fields:    Translation:HumansCells
  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.
    Citations: 16     Fields:    Translation:Humans
  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.
    Citations: 16     Fields:    Translation:HumansCells
  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.
    Citations: 7     Fields:    Translation:HumansCellsPHPublic Health
  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.
    Citations: 32     Fields:    Translation:HumansCTClinical Trials
  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.
    Citations: 25     Fields:    Translation:Humans
  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.
    Citations: 2     Fields:    Translation:HumansCTClinical Trials
  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.
    Citations: 1     Fields:    Translation:HumansCTClinical Trials
  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.
    Citations: 3     Fields:    Translation:HumansCells
  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.
    Citations: 11     Fields:    Translation:Humans
  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.
    Citations: 49     Fields:    Translation:HumansPHPublic Health
  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.
    Citations: 3     Fields:    Translation:HumansCells
  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.
    Citations: 24     Fields:    Translation:HumansAnimals
  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.
    Citations: 10     Fields:    Translation:Humans
  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.
    Citations: 9     Fields:    Translation:HumansCells
  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.
    Citations: 1     Fields:    Translation:HumansPHPublic Health
  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.
    Citations: 41     Fields:    Translation:Humans
  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.
    Citations: 4     Fields:    Translation:Humans
  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.
    Citations: 57     Fields:    Translation:HumansPHPublic Health
  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.
    Citations: 4     Fields:    Translation:Humans
  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.
    Citations: 3     Fields:    Translation:Humans
  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.
    Citations: 13     Fields:    Translation:HumansCells
  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.
    Citations:    Fields:    Translation:HumansPHPublic Health
  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.
    Citations: 1     Fields:    Translation:Humans
  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.
    Citations: 1     Fields:    Translation:HumansCells
  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.
    Citations:    Fields:    Translation:Humans
  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.
    Citations: 58     Fields:    Translation:HumansCells
  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.
    Citations: 18     Fields:    Translation:HumansAnimalsCells
  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.
    Citations: 1     Fields:    Translation:Humans
  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.
    Citations: 15     Fields:    Translation:Humans
  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.
    Citations: 2     Fields:    Translation:HumansCells
  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.
    Citations: 11     Fields:    Translation:HumansCells
  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.
    Citations: 4     Fields:    Translation:Humans
  41. Shih VE, Stewart B. Organ donation by a maple syrup urine disease patient. J Inherit Metab Dis. 1995; 18(3):367. PMID: 7474912.
    Citations:    Fields:    Translation:Humans
  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.
    Citations: 17     Fields:    Translation:HumansCells
  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.
    Citations: 2     Fields:    Translation:HumansCells
  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.
    Citations: 1     Fields:    Translation:HumansCells
  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.
    Citations: 4     Fields:    Translation:HumansCTClinical Trials
  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.
    Citations:    Fields:    Translation:HumansCells
  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.
    Citations: 1     Fields:    Translation:HumansCells
  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.
    Citations: 4     Fields:    Translation:AnimalsCells
  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.
    Citations:    Fields:    Translation:Humans
  50. Shih VE. Detection of hereditary metabolic disorders involving amino acids and organic acids. Clin Biochem. 1991 Aug; 24(4):301-9. PMID: 1959221.
    Citations:    Fields:    Translation:Humans
  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.
    Citations: 3     Fields:    Translation:Humans
  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.
    Citations: 4     Fields:    Translation:HumansCells
  53. Shih VE, Tenanbaum A. Aminoaciduria due to vinyl-GABA administration. N Engl J Med. 1990 Nov 08; 323(19):1353. PMID: 2215628.
    Citations:    Fields:    Translation:Humans
  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.
    Citations: 6     Fields:    Translation:HumansCells
  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.
    Citations: 1     Fields:    Translation:HumansCells
  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.
    Citations: 7     Fields:    Translation:AnimalsCells
  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.
    Citations: 6     Fields:    Translation:HumansCells
  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.
    Citations: 8     Fields:    Translation:HumansAnimalsCells
  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.
    Citations:    Fields:    Translation:Humans
  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.
    Citations:    Fields:    Translation:HumansCells
  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.
    Citations:    Fields:    Translation:AnimalsCells
  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.
    Citations: 5     Fields:    Translation:HumansCells
  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.
    Citations: 10     Fields:    Translation:Humans
  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.
    Citations: 11     Fields:    Translation:HumansCells
  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.
    Citations: 18     Fields:    Translation:HumansAnimalsCells
  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.
    Citations: 28     Fields:    Translation:HumansCells
  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.
    Citations: 4     Fields:    Translation:Humans
  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.
    Citations: 19     Fields:    Translation:HumansCells
  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.
    Citations:    Fields:    Translation:AnimalsCells
  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.
    Citations:    Fields:    Translation:Humans
  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.
    Citations: 1     Fields:    Translation:Animals
  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.
    Citations: 10     Fields:    Translation:Humans
  73. Shih VE, Nikiforov V, Carney MM. Acetaminophen metabolite interferes in analysis for amino acids. Clin Chem. 1985 Jan; 31(1):148. PMID: 3965192.
    Citations:    Fields:    Translation:Humans
  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.
    Citations: 12     Fields:    Translation:Humans
  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.
    Citations:    Fields:    Translation:Humans
  76. Shih VE, Aubry RH, DeGrande G, Gursky SF, Tanaka K. Maternal isovaleric acidemia. J Pediatr. 1984 Jul; 105(1):77-8. PMID: 6547481.
    Citations: 3     Fields:    Translation:Humans
  77. Shih VE. Maple-syrup-urine disease. N Engl J Med. 1984 Mar 01; 310(9):596-7. PMID: 6694715.
    Citations: 1     Fields:    Translation:Humans
  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.
    Citations:    Fields:    Translation:Animals
  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.
    Citations: 4     Fields:    Translation:Humans
  80. Shih VE. Ampicillin interference with test for sulfite oxidase deficiency. Clin Chem. 1983 Jan; 29(1):211-2. PMID: 6848268.
    Citations:    Fields:    Translation:Humans
  81. Carr DB, Shih VE, Richter JM, Martin JB. Encephalopathy following jejunoileostomy. JAMA. 1982 Feb 26; 247(8):1127. PMID: 7057599.
    Citations:    Fields:    Translation:Humans
  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.
    Citations: 4     Fields:    Translation:HumansCells
  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.
    Citations: 1     Fields:    Translation:Humans
  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.
    Citations: 2     Fields:    Translation:HumansCTClinical Trials
  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.
    Citations: 1     Fields:    Translation:HumansCells
  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.
    Citations: 1     Fields:    Translation:HumansPHPublic Health
  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.
    Citations: 1     Fields:    Translation:Humans
  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.
    Citations: 2     Fields:    Translation:Humans
  89. Shih VE. Regulation of ornithine metabolism. Enzyme. 1981; 26(5):254-8. PMID: 7028478.
    Citations: 5     Fields:    Translation:HumansAnimalsCells
  90. Coulombe JT, Shih VE, Levy HL. Massachusetts Metabolic Disorders Screening Program. II. Methylmalonic aciduria. Pediatrics. 1981 Jan; 67(1):26-31. PMID: 7243433.
    Citations: 14     Fields:    Translation:Humans
  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.
    Citations:    Fields:    Translation:HumansCells
  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.
    Citations: 13     Fields:    Translation:Humans
  93. Geiser CF, Shih VE. Cystathioninuria and its origin in children with hepatoblastoma. J Pediatr. 1980 Jan; 96(1):72-5. PMID: 6243161.
    Citations: 3     Fields:    Translation:Humans
  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.
    Citations: 5     Fields:    Translation:Humans
  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.
    Citations: 2     Fields:    Translation:Humans
  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.
    Citations: 12     Fields:    Translation:HumansCells
  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.
    Citations: 2     Fields:    Translation:Animals
  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.
    Citations: 27     Fields:    Translation:HumansCells
  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.
    Citations: 6     Fields:    Translation:HumansCells
  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.
    Citations: 17     Fields:    Translation:Humans
  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.
    Citations: 9     Fields:    Translation:HumansCells
  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.
    Citations: 1     Fields:    Translation:Humans
  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.
    Citations: 11     Fields:    Translation:HumansCells
  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.
    Citations: 7     Fields:    Translation:HumansCells
  105. Shih VE. Congenital hyperammonemic syndromes. Clin Perinatol. 1976 Mar; 3(1):3-14. PMID: 954343.
    Citations: 3     Fields:    Translation:Humans
  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.
    Citations:    Fields:    Translation:Humans
  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.
    Citations: 5     Fields:    Translation:Humans
  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.
    Citations: 1     Fields:    Translation:HumansCells
  109. Shih VE, Mandell R. Letter: Metabolic defect in hyperornithinaemia. Lancet. 1974 Dec 21; 2(7895):1522-3. PMID: 4140439.
    Citations: 1     Fields:    Translation:HumansCells
  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.
    Citations: 9     Fields:    Translation:Humans
  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.
    Citations: 1     Fields:    Translation:HumansCells
  112. Shih VE, Glick TH, Bercu BB. Letter: Lysine metabolism in Reye's syndrome. Lancet. 1974 Jul 20; 2(7873):163-4. PMID: 4135529.
    Citations:    Fields:    Translation:Humans
  113. Shih VE, Brink EW, Peneva P, Brody JA. Letter: Aminoacidopathies in Guam. Lancet. 1974 Apr; 1(7859):681. PMID: 4132347.
    Citations:    Fields:    Translation:Humans
  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.
    Citations: 3     Fields:    Translation:HumansCells
  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.
    Citations: 4     Fields:    Translation:Animals
  116. Levy HL, Shih VE. Screening of galactosemia. N Engl J Med. 1972 Oct 05; 287(14):723. PMID: 5055427.
    Citations:    Fields:    Translation:HumansPHPublic Health
  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.
    Citations: 7     Fields:    Translation:AnimalsCells
  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.
    Citations: 10     Fields:    Translation:HumansPHPublic Health
  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.
    Citations: 5     Fields:    Translation:HumansCells
  120. Shih VE. Early dietary management in an infant with argininosuccinase deficiency: preliminary report. J Pediatr. 1972 Apr; 80(4):645-8. PMID: 5015075.
    Citations: 2     Fields:    Translation:Humans
  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.
    Citations: 23     Fields:    Translation:HumansCells
  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.
    Citations: 8     Fields:    Translation:Animals
  123. Matthysse S, Lipinski J, Shih V. L-dopa and S-adenosylmethionine. Clin Chim Acta. 1971 Nov; 35(1):253-4. PMID: 5127003.
    Citations:    Fields:    Translation:Humans
  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.
    Citations: 6     Fields:    Translation:Humans
  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.
    Citations: 8     Fields:    Translation:Humans
  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.
    Citations: 7     Fields:    Translation:HumansCellsPHPublic Health
  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.
    Citations:    Fields:    Translation:Animals
  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.
    Citations: 3     Fields:    Translation:HumansCells
  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.
    Citations: 7     Fields:    Translation:HumansCells
  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.
    Citations: 19     Fields:    Translation:HumansCells
  131. Shih VE. Soybean milk. J Am Diet Assoc. 1970 Dec; 57(6):520-2. PMID: 5536916.
    Citations:    Fields:    Translation:HumansAnimals
  132. Shih VE, Efron ML. Pyridoxine-unresponsive homocystinuria. N Engl J Med. 1970 Nov 26; 283(22):1206-8. PMID: 5472941.
    Citations: 2     Fields:    Translation:Humans
  133. Levy HL, Shih VE, Karolkewicz V, MacCready RA. Screening for phenylketonuria. Lancet. 1970 Sep 05; 2(7671):522-3. PMID: 4194965.
    Citations: 1     Fields:    Translation:HumansPHPublic Health
  134. Shih VE, Littlefield JW. Argininosuccinase activity in amniotic-fluid cells. Lancet. 1970 Jul 04; 2(7662):45. PMID: 4193779.
    Citations: 3     Fields:    Translation:HumansCells
  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.
    Citations: 5     Fields:    Translation:HumansCells
  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.
    Citations: 5     Fields:    Translation:HumansCells
  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.
    Citations: 2     Fields:    Translation:Humans
  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.
    Citations: 4     Fields:    Translation:HumansCells
  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.
    Citations: 2     Fields:    Translation:Humans
  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.
    Citations:    Fields:    
  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.
    Citations:    Fields:    Translation:HumansPHPublic Health
  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.
    Citations: 1     Fields:    Translation:HumansPHPublic Health
  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.
    Citations: 3     Fields:    Translation:HumansAnimalsPHPublic Health
  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.
    Citations: 12     Fields:    Translation:Humans
  145. Shih V. Encephalopathy and pyruvate metabolism. N Engl J Med. 1968 Aug 08; 279(6):324. PMID: 5660312.
    Citations:    Fields:    Translation:Humans
  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.
    Citations: 3     Fields:    Translation:Humans
  147. Kennedy C, Shih VE, Rowland LP. Homocystinuria: a report in two siblings. Pediatrics. 1965 Nov; 36(5):736-41. PMID: 5844007.
    Citations: 4     Fields:    Translation:Humans
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Funded by the NIH National Center for Advancing Translational Sciences through its Clinical and Translational Science Awards Program, grant number UL1TR002541.