• 1
    Walker-Smith J, Walker WA. The development of pediatric gastroenterology. Pediatr Res 2003;53:706-715.
  • 2
    Reye RDK, Morgan G, Baral J. Encephalopathy and fatty degeneration of the viscera: a disease entity in childhood. Lancet 1963;2:749-752.
  • 3
    Partin JC. Reye's syndrome (encephalopathy and fatty liver). Diagnosis and treatment. Gastroenterology 1975;69:511-518.
  • 4
    Bobo RC, Schubert WK, Partin JC, Partin JS. Reye syndrome: treatment by exchange transfusion with special reference to the 1974 epidemic in Cincinnati, Ohio. J Pediatr 1975;87:881-886.
  • 5
    Lichtenstein PK, Heubi JE, Daugherty CC, Farrell MK, Sokol RJ, Rothbaum RJ, et al. Grade I Reye's syndrome. A frequent cause of vomiting and liver dysfunction after varicella and upper-respiratory-tract infection. N Engl J Med 1983;309:133-139.
  • 6
    Heubi JE, Daugherty CC, Partin JS, Partin JC, Schubert WK. Grade I Reye's syndrome—outcome and predictors of progression to deeper coma grades. N Engl J Med 1984;311:1539-1542.
  • 7
    Balistreri WF. Idiopathic Reye's syndrome and its metabolic mimickers. Pediatr Hepatol 1990:183-202.
  • 8
    Partin JC, Schubert WK, Partin JS. Mitochondrial ultrastructure in Reye's syndrome (encephalopathy and fatty degeneration of the viscera). N Engl J Med 1971;285:1339-1343.
  • 9
    Bove KE, McAdams AJ, Partin JC, Partin JS, Hug G, Schubert WK. The hepatic lesion in Reye's syndrome. Gastroenterology 1975;69:685-689.
  • 10
    Rowe PC, Valle D, Brusilow SW. Inborn errors of metabolism in children referred with Reye's syndrome (a changing pattern). JAMA 1988;260:3167-3170.
  • 11
    Partin JS, Partin JC, Schubert WK, Hammond JG. Serum salicylate concentrations in Reye's disease. A study of 130 biopsy-proven cases. Lancet 1982;1:191-194.
  • 12
    Partin JS, Daugherty CC, McAdams AJ, Partin JC, Schubert WK. A comparison of liver ultrastructure in salicylate intoxication and Reye's syndrome. Hepatology 1984;4:687-690.
  • 13
    Lee WS and Sokol RJ. Liver disease in mitochondrial disorders. Semin Liver Dis 2007;27:259-273.
  • 14
    McKenzie R, Fried MW, Sallie R, Conjeevaram H, Di Bisceglie AM, Park Y, et al. Hepatic failure and lactic acidosis due to fialuridine (FIAU), an investigational nucleoside analogue for chronic hepatitis B. N Engl J Med 195;333:1099-1105.
  • 15
    Hofmann AF, Poley JR. Role of bile acid malabsorption in pathogenesis of diarrhea and steatorrhea in patients with ileal resection. Gastroenterology 1972;62:918-934.
  • 16
    Balistreri WF, Partin JC, Schubert WK. Bile acid malabsorption-a consequence of terminal ileal dysfunction in protracted diarrhea of infancy. J Pediatr 1977;89:21-28.
  • 17
    Heubi J, Balistreri WF, Partin JC, Schubert WK, McGraw CA. Refractory infantile diarrhea due to the primary bile acid malabsorption. J Pediatr 1979;94:546-551.
  • 18
    Heubi J, Balistreri WF, Fondacaro JD, Partin JC, Schubert WK. Primary bile acid malabsorption: defective in vitro ileal active bile acid transport. Gastroenterology 1982;83:804-811.
  • 19
    Hofmann AF. Bile acids: trying to understand their chemistry and biology with the hope of helping patients. Hepatology 2009;49:1403-1418.
  • 20
    Sharp HL, Peeler J, Carey JB, Crivitz W. Primary and secondary bile acids in meconium. Pediatr Res 1971;5:274-279.
  • 21
    Watkins JB, Ingalls D, Szczepanik P, Klein PD, Lester R. Bile salt metabolism in the newborn: measurement of pool size and synthesis by stable isotope technique. N Engl J Med 1973;288:431-434.
  • 22
    Balistreri WF, Cowen AE, Hofmann AF, Szczepanik PA, Klein PD. Validation of use of 1 1,12-2H-labeled chenodeoxycholic acid in isotope dilution measurements of bile acid kinetics in man. Pediatr Res 1975;9:752-760.
  • 23
    Berry FB. The story of “the Berry Plan.” Bull N Y Acad Med 1976;52:278-282.
  • 24
    Balistreri WF, Soloway RD, Weiss SG, Widzer S, Silva de Barros SG, Miller P. Determination of urine bile acid concentration in children in health and disease by radioimmunoassay. Gastroenterology 1978;74:1115.
  • 25
    Balistreri WF, Shapiro MJ, Soloway RD. Serum sulfated and nonsulfated bile acid concentration via dualbeam spectrophotofluorometry. Pediatr Res 1978;12:429.
  • 26
    Silva de Barros SG, Balistreri WF, Soloway RD, Weiss SG, Miller P. Total serum bile acid and cholylglycine concentrations: response to different stimuli of the enterohepatic circulation in man. Gastroenterology 1978;74:1145.
  • 27
    Balistreri WF. Neonatal cholestasis: medical progress. J Pediatr 1985;106:171-184.
  • 28
    Balistreri WF, Bezerra JA, Jansen P, Karpen SJ, Shneider BL, Suchy FJ. Intrahepatic cholestasis: summary of an AASLD single topic conference. Hepatology 2005;42:222-235.
  • 29
    Balistreri WF, Grand R, Suchy FJ, Hoofnagle JH, Ryckman FC, Perlmutter DH, et al. Biliary atresia: current concepts and research directions. Hepatology 1996;23:1682-1692.
  • 30
    Ryckman FC, Fisher RA, Pederson SH, Dittrich V, Heubi JE, Farrell MK, et al. Improved survival in biliary atresia patients in the present era of liver transplantation. J Pediatr Surg 1993;28:382-386.
  • 31
    Bezerra JA, Balistreri WF. Progress in pediatric hepatology. Curr Opin Gastroenterol 1996;12:237-245.
  • 32
    Kasai M, Kimura S, Asakura Y. Surgical treatment of biliary atresia. J Pediatr Surg 1968;3:665-671.
  • 33
    Kasai M, Watanabe I, Ohi R. Follow-up studies of long-term survivors after hepatic portoenterostomy for “non-correctable” biliary atresia. J Pediatr Surg 1975;10:173-182.
  • 34
    Kasai M, Suzuki H, Ohashi E. Technique and results of operative management of biliary atresia. World J Surg 1978;2:571-580.
  • 35
    Mowat AP. Biliary atresia into the 21st century: a historical perspective. Hepatology 1996;23:1693-1695.
  • 36
    Laurent J, Gauthier F, Bernard O, Hadchouel M, Odievre M, Valayer J, et al. Long-term outcome after surgery for biliary atresia; study of 40 patients surviving for more than 10 years. Gastroenterology 1990;99:1793-1797.
  • 37
    Balistreri WF. Inborn errors of bile acid biosynthesis and transport: novel form of metabolic liver diseases. Gastroenterol Clin North Am 1999;28:145-172.
  • 38
    Mowat AP (ed.). Liver disorders in childhood. Oxford, UK: Butterworth-Heinemann; 1979.
  • 39
    Javitt NB (ed.). Neonatal hepatitis and biliary atresia. Washington, DC: U.S. Government Printing Office; 1977.
  • 40
    Hong R, Schubert WK. Menghini needle biopsy of the liver. Am J Dis Child 1960;100:42-46.
  • 41
    Walker WA, Krivit W, Sharp HL. Needle biopsy of the liver of infancy and childhood: a safe diagnostic aid in liver disease. Pediatrics 1967;40:946-950.
  • 42
    Sharp HL, Bridges RA, Krivit W, Freier EF. Cirrhosis associated with alpha-1-antitrypsin deficiency: a previously unrecognized inherited disorder. J Lab Clin Med 1969;73:934-939.
  • 43
    Sveger T, Eriksson S. The liver in adolescents with alpha-1-antitrypsin deficiency. Hepatology 1995;22:514-517.
  • 44
    Bove KE, Kosmetatos N, Wedig KE, Frank DJ, Whitlatch S, Saldivar V, et al. Vasculopathic hepatotoxicity associated with E-FerolR syndrome in low-birth-weight infants. JAMA 1989;254:2422-2430.
  • 45
    Balistreri WF, Suchy FJ, Farrell MK, Heubi JE. Pathologic versus physiologic cholestasis: elevated serum concentration of a secondary bile acid in the presence of hepatobiliary disease J Pediatr 1981;98:399-402.
  • 46
    Balistreri WF, Heubi JE, Suchy FJ. Immaturity of the enterohepatic circulation in early life: factors predisposing to “physiologic” maldigestion and cholestasis. J Pediatr Gastroenterol Nutr 1983;2:346-354.
  • 47
    Suchy FJ, Bucuvalas JC, Novak DA. Determinants of bile formation during development: ontogeny of hepatic bile acid metabolism and transport. Semin Liver Dis 1987;7:77-84.
  • 48
    Balistreri WF, Bove KE. Hepatobiliary consequences of parenteral alimentation. Prog Liver Dis 1990;9:567-601.
  • 49
    Balistreri WF, Farrell MK, Bove KE. Lessons from the E-Ferol tragedy. Pediatrics 1986;78:503-506.
  • 50
    Balistreri WF, Heubi JE, Suchy FJ. Immaturity of the enterohepatic circulation in early life: factors predisposing to “physiologic” maldigestion and cholestasis. J Pediatr Gastroenterol Nutr 1983;2:346-354.
  • 51
    Suchy FJ, Balistreri WF. Uptake of taurocholate by hepatocytes isolated from developing rats. Pediatr Res 1982:4282-4285.
  • 52
    Suchy FJ, Balistreri WF, Hung J, Miller P, Garfield SA. Intracellular bile acid transport in rat liver as visualized by electron microscope autoradiography using a bile acid analogue. Am J Physiol 1983;245:G681-G689.
  • 53
    Suchy FJ. Hepatocellular transport of bile acids. Semin Liver Dis 1993;13:235-247.
  • 54
    Suchy FJ, Sippel CJ, Ananthanarayanan M. Bile acid transport across the hepatocyte canalicular membrane. FASEB J 1997;11:199-205.
  • 55
    Suchy FJ, Ananthanarayanan M. Bile salt excretory pump: biology and pathobiology. J Pediatr Gastroenterol Nutr 2006;43:S10-S16.
  • 56
    Sokol RJ, Heubi JE, Iannaccone ST, Bove KE, Balistreri WF. Mechanism causing vitamin E deficiency during chronic childhood cholestasis. Gastroenterology 1983;85:1172-1182.
  • 57
    Sokol RJ, Heubi JE, Iannaccone ST, Bove KE, Balistreri WF. Vitamin E deficiency with normal serum vitamin E concentrations in children with chronic cholestasis. N Engl J Med 1984;310:1209-1212.
  • 58
    Sokol RJ, Heubi JE, McGraw C, Balistreri WF. Correction of vitamin E deficiency in children with chronic cholestasis. II. Effect on gastrointestinal and hepatic function. Hepatology 1986;6:1263-1269.
  • 59
    Setchell KDR, Lawson AM, Blackstock EJ, Murphy GM. Diurnal changes in serum unconjugated bile acids in normal man. Gut 1982;23:637-642.
  • 60
    Setchell KDR, Lawson AM, Tanida N, Sjövall J. General methods for the analysis of bile acids and related compounds in feces. J Lipid Res 1983;24:1085-1100.
  • 61
    Setchell KDR, Matsui A. Serum bile acid analysis. Clin Chim Acta 1983;127:1-17.
  • 62
    Setchell KDR. Gas chromatography and mass spectrometry of serum bile acids. In: Proceedings of 1st International Symposium on Bile Acids in Hepatobiliary Disease. Oxford, UK: IRL Press; 1984. p 63-78.
  • 63
    Clayton PT, Leonard JV, Lawson AM, Setchell KDR, Anderson S, Egestad B, et al. Familial giant cell hepatitis associated with synthesis of 3β,7α-dihydroxy- and 3β,7α,12α-trihydroxy-5-cholenoic acids. J Clin Investig 1987;79:1031-1038.
  • 64
    Setchell KDR, Street JM. Inborn errors of bile acid synthesis. Semin Liver Dis 1987;7:85-99.
  • 65
    Setchell KDR, Heubi JE. Defects in bile acid synthesis — diagnosis and treatment. J Pediatr Gastroenterol Nutr 2006;43:S17-S22.
  • 66
    Setchell KD, Suchy FJ, Welsh MB, Zimmer-Nechemia L, Heubi JE, Balistreri WF. Δ4−3-oxosteroid 5 β-reductase deficiency described in identical twins with neonatal hepatitis. A new inborn error in bile acid synthesis. J Clin Invest 1988;82:2148-2157.
  • 67
    Stieger B, Zhang J, O'Neill B, Sjovall J, Meier PJ. Differential interaction of bile acids from patients with inborn errors of bile acid synthesis with hepatocellular bile acid transporters. Eur J Biochem 1997;244:39-44.
  • 68
    Daugherty CC, Setchell KD, Heubi JE, Balistreri WF. Resolution of liver biopsy alterations in three siblings with bile acid treatment of an inborn error of bile acid metabolism (Δ4−3-oxosteroid 5 β-reductase deficiency). Hepatology 1993;18:1096-1101.
  • 69
    Fu D, Wakabayashi Y, Lippincott-Schwartz J, Arias IM. Bile acid stimulates hepatocyte polarization through a cAMP-Epac-MEK-LKB1-AMPK pathway. Proc Natl Acad Sci U S A 2011;108:1403-1408.
  • 70
    Hofmann AF, Hagey LR. Bile acids: chemistry, pathochemistry, biology, pathobiology, and therapeutics. Cell Mol Life Sci 2008;65:2461-2483.
  • 71
    Bove K, Daugherty CC, Tyson W, Heubi JE, Balistreri WF, Setchell KDR. Bile acid synthetic defects and liver disease. Pediatr Dev Pathol 2000;3:1-16.
  • 72
    Gonzales E, Gerhardt M F, Fabre M, Setchell KD, Davit-Spraul A, Vincent I, et al. Oral cholic acid for hereditary defects of primary bile acid synthesis: a safe and effective long-term therapy. Gastroenterology 2009;137:1310-1320.
  • 73
    Haas JE, Gan-Schreier H, Langhans CD, Rohrer T, Engelmann G, Heverin M, et al. Differential diagnosis in patients with suspected bile acid synthesis defects. World J Gastroenterol 2012;18:1067-1076.
  • 74
    Molho-Pessach V, Rios JJ, Xing C, Setchell KD, Cohen JC, Hobbs HH. Homozygosity mapping identifies a bile acid biosynthetic defect in an adult with cirrhosis of unknown etiology. Hepatology 2012;55:1139-1145.
  • 75
    Bull LN, van Eijk MJT, Pawlikowska L, DeYoung J A, Juijn JA, Liao M, et al. A gene encoding a P-type AT Pase mutated in two forms of hereditary cholestasis. Nat Genet 1998;18:219-224.
  • 76
    Alagille D, Odievre M, Gautier M, Dommergues JP. Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur. J Pediatr 1975;86:63.
  • 77
    Li L, Krantz ID, Deng Y, Genin A, Banta AB, Collins CC, et al. Alagille syndrome is caused by mutations in human jagged1, which encodes a ligand for notch1. Nat Genet 1997;16:243-251.
  • 78
    Oda T, Elkahloun AG, Pike BL, Okajima K, Krantz ID, Genin A, et al. Mutations in the human jagged1 gene are responsible for Alagille syndrome. Nat Genet 1997;16:235-242.
  • 79
    Liu C, Aronow BJ, Jegga AG, Wang N, Miethke A, Mourya R, et al. Novel resequencing chip customized to diagnose mutations in patients with inherited syndromes of intrahepatic cholestasis. Gastroenterology 2007;132:119-126.
  • 80
    Balistreri WF, Hassan H A-K, Ryckman FC, Whitington PF, Heubi JE, Setchell KDR. Biochemical and clinical response to ursodeoxycholic acid administration in pediatric patients with chronic cholestasis. In: Paumgartner G, Stiehl A, Gerok W, eds. Bile Acids as Therapeutic Agents: From Basic Science to Clinical Practice. Dordrecht/Boston/London: Kluwer Academic Publishers; 1990. p 323-333.
  • 81
    Balistreri WF. Bile acid therapy in pediatric hepatobiliary disease: The role of ursodeoxycholic acid. J Pediatr Gastroenterol Nutr 1997;24:573-589.
  • 82
    Balistreri WF. Non-transplant options for the treatment of metabolic liver disease-saving livers while saving lives. Hepatology 1994;19:782-787.
  • 83
    Lindstedt S, Holme E, Lock EA, Hjalmarson O, Strandvik B. Treatment of hereditary tyrosinemia type I by inhibition of 4-hydroxyphenylpyruvate dioxygenase. Lancet 1992;340:813-817.
  • 84
    Stevens CE, Beasley, RP, Tsui J, Lee WC. Vertical transmission of hepatitis B antigen in Taiwan. N Engl J Med 1975;292:771-774.
  • 85
    Beasley RP, Hwang, LY, Stevens CE, Lin CC, Hsieh FJ, et al. Efficacy of hepatitis B immune globulin for prevention of perinatal transmission of hepatitis B virus carrier state: final report of a randomized double-blind placebo-controlled trial. Hepatology 1983;3:135-141.
  • 86
    Chang MH, Hsu HY, Hsu HC, Ni YH, Chen JS, Chen DS. The significance of spontaneous hepatitis B e antigen seroconversion in childhood: with special emphasis on the clearance of hepatitis B e antigen before 3 years of age. Hepatology 1995;22:1387-1392.
  • 87
    Chen HL, Chang MH, Ni YH, Hsu HY, Lee PI, Lee CY, et al. Seroepidemiology of hepatitis B infection in children: ten years of mass vaccination in Taiwan. JAMA 1996;276:906-908.
  • 88
    Chang MH, Chen CJ, Lai MS, Hsu HM, Wu TC, Kong MS, et al. Universal hepatitis B vaccination in Taiwan and the incidence of hepatocellular carcinoma in children. Taiwan Childhood Hepatoma Study Group. N Engl J Med 1997;336:1855-1859.
  • 89
    Starzl TE, Marchioro TL, Von Kaulla KN, Hermann G, Brittain RS, Waddell WR. Homotransplantation of the liver in humans. Surg Gynecol Obstet 193;117:659-676.
  • 90
    Starzl TE, Iwatsuki S, Van Thiel DH, Gartner JC, Zitelli BJ, Malatack JJ, et al. Evolution of liver transplantation. Hepatology 1982;2:614-636.
  • 91
    Gartner JC Jr, Zitelli BJ, Malatack JJ, Shaw BW, Iwatsuki S, Starzl TE. Orthotopic liver transplantation in children: two-year experience with 47 patients. Pediatrics 1984;74:140-145.
  • 92
    Malatack JJ, Schaid DJ, Urbach AH, Gartner JC Jr, Zitelli BJ, Rockette H, et al. Choosing a pediatric recipient for orthotopic liver transplantation. J Pediatr 1987;111:479-489.
  • 93
    National Institutes of Health. Consensus conference statement on liver transplantation. Hepatology 1984;4:107S-110S.
  • 94
    Whitington PF, Balistreri WF. Liver transplantation in pediatrics: indications, contraindications, and pre-transplant management. J Pediatr 1991;118:169-177.
  • 95
    Ryckman FC, Fisher RA, Pederson SH, Balistreri WF. Liver transplantation in children. Semin Pediatr Surg 1992;1:162-172.
  • 96
    Ryckman FC, Flake AW, Fisher RA, Tchervenkov JI, Pedersen SH, Balistreri WF. Segmental orthotopic hepatic transplantation as a means to improve patient survival and diminish waiting-list mortality. J Pediatr Surg 1991;26:422-428.
  • 97
    Broelsch CE, Emond JC, Thistlewaite JR, Whitington PF, Zucker AR, Baker AL, et al. Liver transplantation, including the concept of reduced- size liver transplants in children. Ann Surg 1988;208:410-420.
  • 98
    Tanaka K, Uemoto S, Tokunaga Y, Sano K, Yamamoto E, Fujita S, et al. Living related liver transplantation in children. Am J Surg 1994;168:41-48.
  • 99
    Balistreri WF, Bucuvalas JC, Ryckman FC. The effect of immunosuppression on growth and development. Liver Transpl Surg 1995;1:64-73.
  • 100
    UNOS (United Network for Organ Sharing Transplant Registry). Accessed February 2013.
  • 101
    Kim WR, Stock PG, Smith JM, Heimbach JK, Skeans MA, Edwards EB, et al. OPTN/SRTR 2011 Annual Data Report: liver. Am J Transplant 2013;1:73-102.
  • 102
    Walker WA, Sherman P, Cohen M, Barnard J. State of pediatric gastroenterology, hepatology, and nutrition: 2006 and beyond. Gastroenterology 2007;132:434-436.
  • 103
    Balistreri WF, Schubert WK. Liver disease in infancy and childhood. In: Schiff L, Schiff ER (eds.). Diseases of the liver. 7th ed. Philadelphia: JB Lippincott; 1993. p 1099-1203.
  • 104
    Balistreri WF, Fjellstedt TA, Go VLW, Grand R, Javitt NB, Kalser SC, et al. Mechanisms and management of pediatric hepatobiliary disease. A report of a conference. J Pediatr Gastroenterol Nutr 1990;10:138-147.
  • 105
    Hoofnagle JH. Biliary Atresia Research Consortium (BARC). J Hepatol 2004;39:891.
  • 106
    Sokol RJ. New North American research network focuses on biliary atresia and neonatal liver disease [News and Views]. J Pediatr Gastroenterol Nutr 2003;36:1.
  • 107
  • 108
    Bacon BR. Certificate of added qualification in transplant hepatology. Liver Transplant 2005;1:4-6.
  • 109
    Sherman PM, Buller H, Rosenthal P. Is it time for separate pediatric hepatology and liver transplant training programs and special certification and qualification. J Pediatr Gastroenterol Nutr 2000;30:111.
  • 110
    Rosen HR, Fontana RJ, Brown RS, Wiesner RH, Schiano TD, Bass NM, et al. Curricular guidelines for training in transplant hepatology. Liver Transplant 2002;8:85-87.
  • 111
    Friedman LS, Brandt LJ, Elta GH, Fitz JG, Gores GJ, Katz PO, et al. Report of the multisociety task force on GI training. Gastroenterology 2009;137:1839-1843.
  • 112
  • 113
    Elta GH. GI training: where are we headed. Am J Gastroenterol 2011;106:395-397.
  • 114
    McGuinness GA. The transformation of pediatric education with a focus on the subspecialists. Pediatrics 2013;131:767.
  • 115
    Leichtner AM, Gillis LA, Gupta S, Heubi J, Kay M, Narkewicz MR, et al., and the NASPGHAN Training Committee. NASPGHAN guidelines for training in pediatric gastroenterology. J Pediatr Gastroenterol Nutr 2013;56:S1-S8.
  • 116
    Nasca TJ, Philibert I, Brigham T, Flynn TC. The next GME accreditation system — rationale and benefits. N Engl J Med 2012;366:1051.
  • 117
    Caverzagie KJ, Iobst WF, Aagaard EM, Hood S, Chick DA, Kane GC, et al. The internal medicine reporting milestones and the next accreditation system. Ann Int Med 2013;158:557-559.
  • 118
    Englander R, Hicks P, Benson B. Pediatric milestones: a developmental approach to the competencies. J Pediatr 2010;157:521-522.