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Modern Concepts in Gastroenterology pp 299–336Cite as

Gastrointestinal and Hepatobiliary Complications of Cystic Fibrosis

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Part of the book series: Topics in Gastroenterology ((TGEN))

Abstract

Cystic fibrosis remains the commonest semilethal inherited disease in the Caucasian population. The disease is characterized by dysfunction of exocrine glands with disturbances of electrolyte and mucus secretion, resulting in multiorgan involvement by predominantly obstructive lesions. The gene was localized to the long arm of chromosome 7 in September 1989 in a landmark discovery by the team of Lap-Chee Tsui, Jack Riordan, and Francis Collins at the Universities of Toronto and Michigan. The major mutation associated with the severe multi-organ form of the disease results in the deletion of a phenylalanine residue at position AF508 and is found in 70% of North American Caucasian CF patients.1 There are now upwards of 150 mutations of various kinds identified as causing CF, most occurring in the nucleotide binding domain of the cystic fibrosis transmembrane regulator (CFTR), the protein product of the gene. It is likely that the CFTR is either a chloride channel itself, or regulates another protein which transports chloride in response to intracellular stimuli such as raised cAMP levels and protein kinase activity. Thus, the defect appears to involve abnormal phosphorylation of a protein necessary in the gating mechanism of the chloride channel.2

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References

  1. Rommens JM, Iannuzzi MC, Kerem BS, et al. Identification of the cystic fibrosis gene: Chromosome walking and jumping. Science 1989; 245: 1059–1065.

    Article  PubMed  CAS  Google Scholar 

  2. Riordan JR, Rommens JM, Kerem BS, et al. Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA. Science 1989; 245: 1066–1072.

    Article  PubMed  CAS  Google Scholar 

  3. Zentler-Munro P. Cystic fibrosis—a gastroenterological cornucopia. Gut 1987; 28: 1531–1547.

    Article  PubMed  CAS  Google Scholar 

  4. Wood R, Boat T, Doershuk C. Cystic fibrosis. Am Rev Respir Dis 1976; 113: 833–877.

    PubMed  CAS  Google Scholar 

  5. Kulczycki L, Schauf V. Cystic fibrosis in blacks in Washington DC. Incidence and characteristics. Am J Dis Child 1974; 127: 64–67.

    PubMed  CAS  Google Scholar 

  6. Wright SW, Morton NE. Genetic studies on cystic fibrosis in Hawaii. Am J Hum Genetics 1968; 20: 157–169.

    CAS  Google Scholar 

  7. Corey M, Levison H, Crozier D. Five to seven years course of pulmonary function in cystic fibrosis. Am Rev Respir Dis 1976; 114: 1085–1092.

    PubMed  CAS  Google Scholar 

  8. Anderson D. Cystic fibrosis of the pancreas and its relation to celiac disease. Am J Dis Child 1938; 56: 344–349.

    Google Scholar 

  9. di Sant’Agnese P, Darling R, Perera G, Shea E. Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas. Pediatrics 1953; 12: 549–563.

    Google Scholar 

  10. Shwachman H, Kowalski M, Khaw K. Cystic fibrosis: a new outlook. 70 patients above 25 years of age. Medicine 1977; 56: 29–49.

    Article  Google Scholar 

  11. MacLusky I, McLaughlin FJ, Levison H. Cystic fibrosis. Curr ProblPediatr 1985; 15 (7):1–39.

    Google Scholar 

  12. Penketh ALH, Wise A, Mearns MB, et al. Cystic fibrosis in adolescents and adults. Thorax 1987; 42: 526–532.

    Article  PubMed  CAS  Google Scholar 

  13. Donnison A, Shwachmann H, Gross R. A review of 164 children in meconium ileus seen at the Children’s Hospital Medical Center, Boston. Pediatrics 1966; 37: 833–850.

    PubMed  CAS  Google Scholar 

  14. McPartlin J, Dickson J, Swain V. Meconium ileus, immediate and long term survival. Arch Dis Child 1972; 47: 207–210.

    Article  PubMed  CAS  Google Scholar 

  15. Denholm T, Crow H, Edward W, et al. Prenatal sonographic appearance of meconium ilus in twins. Am J Radiol 1984; 143: 371–372.

    CAS  Google Scholar 

  16. Antonowicz I, Shwachmann H. Meconium in health and disease. Adv Pediatr 1963; 63: 444–453.

    Google Scholar 

  17. Thomaidis T, Arey J. The intestinal lesions in cystic fibrosis of the pancreas. J Pediatr 1963; 63: 444–453.

    Article  PubMed  CAS  Google Scholar 

  18. Oppenheimer E, Esterly J. Cystic fibrosis of the pancreas, morphologic findings in infants with and without pancreatic lesions. Arch Pathol 1973; 96: 149–154.

    PubMed  CAS  Google Scholar 

  19. Hurwitt E, Arnheim E. Meconium ileus associated with stenosis of the pancreatic ducts. Am J Dis Child 142;64: 443–454.

    Google Scholar 

  20. Auburn R, Feldman S, Gadacz T, Towe M. Meconium ileus secondary to partial aplasia of the pancreas: report of a case. Surgery 1969; 65: 689–693.

    PubMed  CAS  Google Scholar 

  21. Dolan T, Touloukian R. Familial meconium ileus not associated with cystic fibrosis. J Pediatr Surg 1974; 9: 821–824.

    Article  PubMed  Google Scholar 

  22. Bernstein J, Vawter G, Harris G, et al. The occurrence of intestinal atresia in newborns with meconium ileus. Am J Dis Child 1960; 99: 804–818.

    CAS  Google Scholar 

  23. Park RW, Grand RJ. Gastrointestinal manifestations of cystic fibrosis: a review. Gastroenterology 1987; 81: 1143–1161.

    Google Scholar 

  24. Holsclaw D, Eckstein H, Nixon H. Meconium ileus, a 20 year review of 109 cases. Am J Dis Child 1965; 109: 101–113.

    PubMed  CAS  Google Scholar 

  25. White H. Meconium ileus; a new roentgen sign. Radiology 1956; 66: 567–571.

    PubMed  CAS  Google Scholar 

  26. Neuhauser EBD. Roentgen changes associated with pancreatic insufficiency in early life. Radiology 1946; 46: 319–328.

    PubMed  CAS  Google Scholar 

  27. Leonidas JC, Berdon WE, Baker DH, et al. Meconium ileus and its complications. A reappraisal of plain film diagnostic criteria. Am J Radiol 1970; 108: 598–609.

    CAS  Google Scholar 

  28. Neuhauser E. The roentgen diagnosis of fetal meconium peritonitis. Am J Roentgenol 1944; 51: 421–425.

    Google Scholar 

  29. Leonidas J, Berdon W, Baker D, Santulli T. Meconium ileus and its complications, a reappraisal of plain film roentgen diagnostic criteria. Am J Roentgenol 1970; 108: 598–608.

    CAS  Google Scholar 

  30. Zimmer J. Microcolon: with report of two cases. Radiology 1948; 29: 228–236.

    CAS  Google Scholar 

  31. Noblett H. Meconium ileus. In: Pediatric Surgery (Raviktch M, Welch K, Benson G, et al., eds). Chicago: Year Book Medical, 1979, pp. 943–952.

    Google Scholar 

  32. Rosenstein B, Langbaum T. Incidence of meconium abnormalities in newborn infants with cystic fibrosis. Am J Dis Child 1980; 134: 72–73.

    PubMed  CAS  Google Scholar 

  33. Sokal M, Koenigsberger M, Rose JS, et al. Neonatal hypermagnesaemia and the meconium plug syndrome. N Engl J Med 1972; 286: 823–825.

    Article  PubMed  CAS  Google Scholar 

  34. Hiatt R, Wilson P. Celiac syndrome VII. Therapy of meconium ileus, report of eight cases with a review of the literature. Surg Gynecol Obstet 1948; 87: 317–327.

    PubMed  CAS  Google Scholar 

  35. Noblett H. Treatment of uncomplicated meconium ileus by gastrografin enema; a preliminary report. J Pediatr Surg 1969; 4: 190–197.

    Article  PubMed  CAS  Google Scholar 

  36. Lutzger L, Factor SE. Effects of some water soluble contrast media on the colonic mucosa. Radiology 1976; 118: 545–548.

    PubMed  CAS  Google Scholar 

  37. Rowe M, Seagram G, Weinberger M. Gastrografm-induced hypertonicity. The pathogenesis of a neonatal hazard. Am J Surg 1973; 125: 185–188.

    Article  PubMed  CAS  Google Scholar 

  38. Shaw A. Safety of N acetylcysteine in treatment of meconium obstruction of the newborn. J Pediatr Surg 1969; 4: 119–125.

    Article  PubMed  CAS  Google Scholar 

  39. Hudson I, Phelan PD, Are sex, age at diagnosis or mode of presentation prognostic factors for cystic fibrosis? Pediatr Pulmonol 1987; 2: 288–297.

    Article  Google Scholar 

  40. Katz JN, Horwitz RI, Dolan TF, Shapiro ED. Clinical features as predictors of functional status in children with CF. J Pediatr 1986; 108: 352–358.

    Article  PubMed  CAS  Google Scholar 

  41. McPartlin J, Dickson J, Swain V. Meconium ileus: immediate and longterm survival. Arch Dis Child 1972; 47: 207–210.

    Article  PubMed  CAS  Google Scholar 

  42. Mcintosh R. Cystic fibrosis of the pancreas in patients over 10 years of age. Acta Paediatr Scand 1954; 100 (Suppl):471–480.

    Google Scholar 

  43. Kulczycki L, Shwachman H. Studies in cystic fibrosis of the pancreas occurrence of rectal prolapse. N Engl J Med 1958; 259: 409–412.

    Article  PubMed  CAS  Google Scholar 

  44. Shwachman H. Cystic fibrosis. Curr Probi Pediatr 1978; 811–872.

    Google Scholar 

  45. Stern RC, Izant RJ, Boat TE, et al. Treatment and prognosis of rectal prolapse in cystic fibrosis. Gastroenterology 1982; 82: 707–710.

    PubMed  CAS  Google Scholar 

  46. Dolan T, Meyers A. Mild cystic fibrosis presenting as an asymptomatic distended appendiceal mass—a case report. Clin Pediatr 1975; 14: 862–863.

    Article  Google Scholar 

  47. Matseshe J, Go V, DiMano E, et al. Meconium ileus equivalent complicating cystic fibrosis in postneonatal children and young adults. Gastroenterology 1911; 12: 132–136.

    Google Scholar 

  48. Rosenstein BJ, Langbaum TS. Incidence of distal intestinal obstruction syndrome in cystic fibrosis. J Pediatr Gastroenterol Nutr 1983; 2: 299–301.

    PubMed  CAS  Google Scholar 

  49. Holsclaw D, Rocmans C, Shwachman H. Abdominal complaints and appendiceal changes leading to the diagnosis of cystic fibrosis. J Pediatr Surg 1974; 9: 867–873.

    Article  PubMed  CAS  Google Scholar 

  50. Jaffe B, Graham W, Goldman L. Postinfancy intestinal obstruction in children with cystic fibrosis. Arch Surg 1966; 92: 337–343.

    Article  PubMed  CAS  Google Scholar 

  51. Anderson CM. Hypotinesis revisited: cystic fibrosis: a disturbance of water and electrolyte movement in exocrine secretory tissue associated with prostaglandin (PGE2) metabolism. J Pediatr Gastroenterol Nutr 1984; 3: 15–22.

    Article  PubMed  CAS  Google Scholar 

  52. Mullins F, Talamo R, di Sant’ Agnese P. Late intestinal complications of cystic fibrosis. JAMA 1965; 192: 741–746.

    Article  PubMed  CAS  Google Scholar 

  53. Berk R, Lee F. The late gastrointestinal manifestations of cystic fibrosis of the pancreas. Radiology 1973; 106: 377–381.

    PubMed  CAS  Google Scholar 

  54. McCarthy VP, Mischler EH, Hubbard VS, et al. Appendiceal abscess in cystic fibrosis—a diagnostic challenge. Gastroenterology 1984; 86: 564–568.

    PubMed  CAS  Google Scholar 

  55. Lillibridge C, Docter J, Eidelman S. Oral administration of n-acetyl cysteine in the prophylaxis of “meconium ileus equivalent.” J Pediatr 1967; 71: 887–889.

    Article  PubMed  CAS  Google Scholar 

  56. Gracey M, Burke V, Anderson C. Treatment of abdominal pain in cystic fibrosis by oral administration of n-acetyl cysteine. Arch Dis Child 1969; 44: 404–405.

    Article  PubMed  CAS  Google Scholar 

  57. O’Halloran SM, Gilbert J, McKendrick OM, et al. Gastrografin in acute meconium ileus equivalent. Arch Dis Child 1986; 61: 1128–1130.

    Article  PubMed  Google Scholar 

  58. Cleghorn GJ, Stringer DA, Forstner GG, Durie PR. Treatment of distal intestinal obstruction syndrome with a balanced intestinal lavage solution. Lancet 1986; 1: 8–11.

    Article  PubMed  CAS  Google Scholar 

  59. Holsclaw D, Rocmans C, Shwachman H. Intussusception in patients with cystic fibrosis. Pediatrics 1971; 48: 51–58.

    PubMed  CAS  Google Scholar 

  60. Brown M, Weaver D. Multiple intussusception. J Iowa State Med Soc 1950; 40: 57–59.

    PubMed  CAS  Google Scholar 

  61. Bendig DW, Seitheimer DK, Wagner ML, et al. Complications of gastroesophageal reflux in patients with cystic fibrosis. J Pediatr 1982; 100: 536–540.

    Article  PubMed  CAS  Google Scholar 

  62. Vinocur CD, Marmon L, Schidlow DV, Weintraub WH. Gastroesophageal reflux in the infant with cystic fibrosis. Am J Surg 1985; 149: 182–186.

    Article  PubMed  CAS  Google Scholar 

  63. Scott RB, O’Loughlin EV, Gall DG. Gastroesophageal reflux in patients with cystic fibrosis. J Pediatr 1985; 106: 223–227.

    Article  PubMed  CAS  Google Scholar 

  64. Dab I, Malfroot A. Gastro-oesophageal reflux: a primary defect in cystic fibrosis. Scand J Gastroenterol 1988; 23 (Suppl 143):125–131.

    Article  Google Scholar 

  65. Feigelson J, Girault F, Pecau Y. Gastro-oesophageal reflux and esophagitis in cystic fibrosis. Acta Paediatr Scand 1987; 76: 989–1070.

    Article  PubMed  CAS  Google Scholar 

  66. Stein MR, Towner TG, Weber PW, et al. The effect of theophylline on the lower esophageal sphincter pressure. Am Allergy 1980; 45: 238.

    Google Scholar 

  67. Foster AC, Voyles JB, Murray BL, Murphy SA. Twenty four hour PH monitoring in children with cystic fibrosis: association of chest physical therapy to gastro-oesophageal reflux. Paediatr Res 1983; 17: 188.

    Google Scholar 

  68. Sultz HA, Schlesinger ER, Feldman JG, et al. The epidemiology of peptic ulcer in childhood. Am J Public Health 1970; 60: 492–498.

    Article  CAS  Google Scholar 

  69. Miranti JP. A review of experience with peptic ulcer in adolescents and young adults in a university health service. J Am Coll Health 1970; 18: 387.

    Google Scholar 

  70. Phelan MS, Fine DR, Zentler-Munro PH, et al. Radiographic abnormalities of the duodenum in cystic fibrosis. Clin Radiol 1983; 34: 573–577.

    Article  PubMed  CAS  Google Scholar 

  71. Markel IJ. Fibrocystic disease of the pancreas with an unusual associated lesion. J Indiana State MedAssoc 1944; 37: 674–678.

    Google Scholar 

  72. Lepare M. Cystic fibrosis of the pancreas in the adult. Gastroenterology 1963; 44: 696–699.

    Google Scholar 

  73. Koch E. Hereditary adult mucoviscidosis and its relationship to peptic ulcer. Ger Med Monogr 1960; 5: 40–46.

    Google Scholar 

  74. Fiedorek SC, Shulman RJ, Klish WJ. Endoscopic detection of peptic ulcer disease in cystic fibrosis. Clin Pediatr 1986; 25: 243–246.

    Article  CAS  Google Scholar 

  75. Rosenstein BJ, Perman JA, Kramer SS. Peptic ulcer disease in cystic fibrosis: an unusual occurrence in black adolescents. Am J Dis Child 1986; 140: 966–968.

    PubMed  CAS  Google Scholar 

  76. Cox KL, Isenberg JN, Ament ME. Gastric acid hypersecretion in cystic fibrosis. J Pediatr Gastroenterol Nutr 1982; 1: 559–565.

    Article  PubMed  CAS  Google Scholar 

  77. Youngberg CA, Berardi RR, Howat WF, et al. Comparison of gastrointestinal pH in cystic fibrosis and healthy subjects. Dig Dis Sci 1987; 32: 472–480.

    Article  PubMed  CAS  Google Scholar 

  78. Saunders JHB, Cargill JM, Wormsley KG. Gastric secretion of acid in patients with pancreatic disease. Digestion 1978; 17: 365–369.

    Article  PubMed  CAS  Google Scholar 

  79. Silen W. The prevention and management of stress ulcer. Hosp Pract 1980; 15: 93–100.

    PubMed  CAS  Google Scholar 

  80. Robert A, Nezamis JE. Histopathology of steroid-induced ulcers. An experimental study in the rat. Arch Pathol 1964; 77: 407–410.

    PubMed  CAS  Google Scholar 

  81. Ojeda VJ, Levitt S, Ryan G, Lawrence BH. Cystic fibrosis, Crohn’s disease, and adult meconium ileus equivalent. Dis Colon Rectum 1986; 29: 567–571.

    Article  PubMed  CAS  Google Scholar 

  82. Hernanz-Schulman M, Kirkpatrick J, Shwachman H, et al. Pneumatosis intestinalis in cystic fibrosis. Radiology 1986; 160: 497–499.

    PubMed  CAS  Google Scholar 

  83. Goodchild M, Nelson R, Anderson C. Cystic fibrosis and coeliac disease: report of 2 cases. Arch Dis Child 1973; 48: 684–691.

    Article  PubMed  CAS  Google Scholar 

  84. Stead RG, Redington AN, Hinks LM, et al. Selenium deficiency and possible increased risk of carcinoma in adults with cystic fibrosis. Lancet 1985; 2: 862–863.

    Article  PubMed  CAS  Google Scholar 

  85. Oppenheimer E, Esterly J. Pathology of cystic fibrosis, review of the literature and comparison with 146 autopsied cases. In: Perspectives in Pediatric Pathology, Vol. 2. Chicago: Year Book Medical Publishers, 1975, pp. 241–278.

    Google Scholar 

  86. Imrie J, Fagan D, Sturgess J. Quantitative evaluation of the development of the exocrine pancreas in cystic fibrosis and control infants. Am J Pathol 1979; 95: 697–708.

    PubMed  CAS  Google Scholar 

  87. Bodian M. Fibrocystic Disease of the Pancreas: A Congenital Disease of Mucus Production. New York: Grune and Stratton, 1953.

    Google Scholar 

  88. Walters M. The ductular cell in pancreatic cystic fibrosis. J Pathol 1965; 90: 45–52.

    Article  CAS  Google Scholar 

  89. Allen R, Baggenstoss A. The pathogenesis of fibrocystic disease of the pancreas, study of the ducts by serial section. Am J Pathol 1955; 31: 337–352.

    PubMed  CAS  Google Scholar 

  90. Porta E, Stein A, Patterson P. Ultrastructural changes of the pancreas and ulcer in cystic fibrosis. Am J Clin Pathol 1964; 42: 457–465.

    Google Scholar 

  91. Daneman A, Gaskin K, Martin DJ, et al. Pancreatic changes in cystic fibrosis: CT and sonographic appearances. Am J Radiol 1983; 141: 653–655.

    CAS  Google Scholar 

  92. Graham M, Manhire AR, Stead RJ, et al. Cystic fibrosis: ultrasonographic findings in the pancreas and hepatobiliary system correlated with clinical data and pathology. Clin Radiol 1985; 36: 199–203.

    Article  PubMed  CAS  Google Scholar 

  93. Cleghorn G, Benjamin L, Corey M, et al. Serum immunoreactive pancreatic lipase and cationic trypsinogen for the assessment of exocrine pancreatic function in older patients with cystic fibrosis. Pediatrics 1986; 77: 301–306.

    PubMed  CAS  Google Scholar 

  94. Shwachman H, Lebenthal E, Khaw KT. Recurrent acute pancreatitis in patients with cystic fibrosis with normal enzymes. Pediatrics 1975, 55: 86–95.

    PubMed  CAS  Google Scholar 

  95. Lippe B, Kaplan S, Neufeld N, et al. Insulin receptors in cystic fibrosis: increased receptor number and altered affinity. Pediatrics 1980; 65: 1018–1022.

    PubMed  CAS  Google Scholar 

  96. Gaskin K, Gurwitz D, Durie P, et al. Improved respiratory prognosis in patients with normal fat absorption. J Pediatr 1982; 100: 857–862.

    Article  PubMed  CAS  Google Scholar 

  97. Mastella G, Barbato G, Trabucchi C, et al. Exocrine pancreas in cystic fibrosis: function tests in 169 patients and 118 controls. Rendi Gastroenterol 1976; 8: 134.

    Google Scholar 

  98. Kraemer R, Rudeborg A, Hadorn B, Ross I. Relative underweight in cystic fibrosis and its prognostic value. Acta Paediatr Scand 1978; 67: 33–37.

    Article  PubMed  CAS  Google Scholar 

  99. Abman SH, Accurso FJ, Bowman CM. Persistent morbidity and mortality of protein-calorie malnutrition in young infants with cystic fibrosis. J Pediatr Gastroenterol Nutr 1986; 5: 393–396.

    Article  PubMed  CAS  Google Scholar 

  100. Roy CC, Weber AM. Nutrition of the child with cystic fibrosis. In: Nutrition in Pediatrics (Walker WA, Watkins JB, eds). Boston: Little Brown and Company, 1985, pp. 463–484.

    Google Scholar 

  101. Gaskin KJ, Durie PR, Corey M, et al. Evidence for a primary defect of pancreatic HC03 secretion in cystic fibrosis. Pediatr Res 1982; 16: 554–557.

    Article  PubMed  CAS  Google Scholar 

  102. Kopelman H, Durie P, Gaskin K, et al. Pancreatic fluid secretion and protein hyperconcentra-tion in cystic fibrosis. N Engl J Med 1985; 312: 329–334.

    Article  PubMed  CAS  Google Scholar 

  103. Nielson OH, Larsen BF. The incidence of anaemia, hypoproteinaemia, and oedema in infants as presenting symptoms of cystic fibrosis, a retrospective survey of the frequency of the symptom complex in 130 patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 1982; 1: 355–359.

    Article  Google Scholar 

  104. Strober NA, Peter G, Schwartz R. Albumin metabolism in cystic fibrosis. Am J Dis Child 1964; 108: 360–365.

    Google Scholar 

  105. Shmerling DH, Forrer JEW, Prader A. Fecal fat and nitrogen in healthy children and in children with malabsorption or maldigestion. Pediatrics 1970; 46: 690–695.

    PubMed  CAS  Google Scholar 

  106. Lapey A, Kirkpatrich J, di Sant’Agnese PA, Lester L. Steatorrhea and azotorrhea and their relation to growth and nutrition in adolescents and young adults with cystic fibrosis. J Pediatr 1974; 84: 328–334.

    Article  PubMed  CAS  Google Scholar 

  107. Shepherd RW, Holt TL, Thomas BJ, et al. Nutritional rehabilitation in cystic fibrosis: controlled studies of effect on nutritional growth retardation; body protein turnover and course of disease. J Pediatr 1986; 109: 788–794.

    Article  PubMed  CAS  Google Scholar 

  108. DiMaggio EP, Go VLW, Summerskill WHJ. Relations between enzyme outputs and malabsorption in severe pancreatic insufficiency. N Engl J Med 1973; 288: 813–815.

    Article  Google Scholar 

  109. Durie PR, Gaskin KJ, Corey M, et al. Pancreatic function testing in cystic fibrosis. J Pediatr Gastroenterol Nutr 1984; 3: 589–598.

    Article  Google Scholar 

  110. Jordan PH, Grossman MI. Pancreatico-duodenectomy for chronic relapsing pancreatitis. Arch Surg 1957; 75: 871–873.

    Article  Google Scholar 

  111. Muller DPR, McCollum JPR, Trompeter RS. Studies on the mechanism of fat absorption in congenital isolated lipase deficiency (abstract). Gut 1975; 16: 838.

    Google Scholar 

  112. Hamosh M, Scanlon JW, Ganot D, et al. Fat digestion in the newborn: characterization of lipase in gastric aspirates of premature and term infants. J Clin Invest 1981; 67: 838–846.

    Article  PubMed  CAS  Google Scholar 

  113. Levy E, Goldstein R, Freier S, Shafrir E. Characterization of gastric lipolytic activity. Biochem Biophys Acta 1981; 666: 316–326.

    Google Scholar 

  114. Roberts IM, Jaffe R. Lingual lipase, immunocytochemical localisation in the rat von Ebner gland. Gastroenterology 1986; 90: 1170–1175.

    PubMed  CAS  Google Scholar 

  115. Levy E, Goldstein R, Freier S, Shafrir E. Gastric lipase in the newborn rat. Pediatr Res 1982; 16: 69–74.

    Article  PubMed  CAS  Google Scholar 

  116. Roy CC, Roulet M, Lefebvre D, et al. The role of gastric lipolysis on fat absorption and bile acid malabsorption in the rat. Lipids 1979; 14: 811–815.

    Article  PubMed  CAS  Google Scholar 

  117. Dutta SK, Hamosh M, Abrams CK, Hamosh P. Quantitative estimation of lingual lipase activity in the upper small intestine in adult patients with pancreatic insufficiency. Gastroenterology 1982; 82: 1047.

    Google Scholar 

  118. Zentler-Munro PL, Fitzpatrick WJF, Batten JC, Northfield TC. Effect of intrajejunal acidity on aqueous phase bile acid and lipid concentrations in pancreatic steatorrhea due to cystic fibrosis. Gut 1984; 25: 500–507.

    Article  PubMed  CAS  Google Scholar 

  119. Regan PT, Malagelada JR, DiMagno EP, Go VLW. Reduced intraluminal bile acid concentrations and fat maldigestion in pancreatic insufficiency: correction by enzyme. Gastroenterology 1979; 77: 285–289.

    PubMed  CAS  Google Scholar 

  120. Borgstrom B, Erlanson C. Pancreatic juice colipase: physiological importance. Biochem Biophys Acta 1971; 242: 509–513.

    PubMed  CAS  Google Scholar 

  121. Gaskin KJ, Durie PR, Hill RE, et al. Colipase and maximally activated pancreatic lipase in normal subjects and in patients with steatorrhea. J Clin Invest 1982; 69: 427–434.

    Article  PubMed  CAS  Google Scholar 

  122. Weber AM, Roy CC. Intraduodenal events in cystic fibrosis. J Pediatr Gastroenterol Nutr 1984; 3 (Suppl 1):S113–S119.

    Article  PubMed  Google Scholar 

  123. Mansbach II CM, Pieroni G, Verger R. Intestinal phospholipase, a novel enzyme. J Clin Invest 1982; 69: 368–376.

    Article  PubMed  CAS  Google Scholar 

  124. Borgstrom B, Erlanson C. Interaction of serum albumin and other proteins with porcine pancreatic lipase. Gastroenterology 1978; 75: 382–386.

    CAS  Google Scholar 

  125. Weber AM, Roy CC. Bile acid metabolism in children with cystic fibrosis. Acta Paedriatr Scand 1985; 317 (Suppl):9–15.

    Article  CAS  Google Scholar 

  126. Thompson GN. Excessive fecal taurine loss predisposes to taurine deficiency in cystic fibrosis. J Pediatr Gastroenterol Nutr 1988; 7: 214–219.

    Article  PubMed  CAS  Google Scholar 

  127. Dutta SK, Armand K, Gadacz TR. Bile salt malabsorption in pancreatic insufficiency secondary to alcoholic pancreatitis. Gastroenterology 1986; 91: 1243–1249.

    PubMed  CAS  Google Scholar 

  128. Leroy C, Lepage G, Morin CL, Bertrand JM, Dufour-Larue O, Roy CC. Effect of dietary fat and residues on fecal loss of steroids and on their microbial degradation in cystic fibrosis. Dig DisSci 1986; 31: 911–918.

    Article  CAS  Google Scholar 

  129. Fondacaro JD, Heubi JE, Kellogg FW. Intestinal bile acid malabsorption in cystic fibrosis: a primary mucosal cell defect. Pediatr Res 1982; 16: 494–498.

    Article  PubMed  CAS  Google Scholar 

  130. Thompson GN, Davidson GP. In vitro bile acid uptake from terminal ileum in cystic fibrosis. Pediatr Res 1988; 23: 323–328.

    Article  PubMed  CAS  Google Scholar 

  131. Watkins JB, Tercyak AM, Szczepanic P, Klein PD. Bile salt kinetics in cystic fibrosis: influence of pancreatic enzyme replacement. Gastroenterology 1977; 73: 1023–1028.

    PubMed  CAS  Google Scholar 

  132. Krag E, Phillips SF. Active and passive bile acid absorption in man. J Clin Invest 1974; 53: 1686–1694.

    Article  PubMed  CAS  Google Scholar 

  133. Darling P, Lepage G, Leroy C, et al. Effect of taurine supplements on fat absorption in cystic fibrosis. Pediatr Res 1985; 19: 578–582.

    Article  PubMed  CAS  Google Scholar 

  134. Belli DC, Levy E, Darling P, et al. Taurine improves the absorption of a fat meal in patients with cystic fibrosis. Pediatrics 1987; 80: 517–523.

    PubMed  CAS  Google Scholar 

  135. Colombo C, Arlati S, Curcio L, et al. Effect of taurine supplementation of fat and bile acid absorption in patients with cystic fibrosis. Acta Paediatr Scand 1988; Suppl 317: 16–21.

    Google Scholar 

  136. Morin C, Roy CC, Lasalle R, Bonin A. Small bowel mucosal dysfunction in patients with cystic fibrosis. J Pediatr 1976; 88: 213–216.

    Article  PubMed  CAS  Google Scholar 

  137. Frase LL, Strickland AD, Kachel FW, Krejs GJ. Enhanced glucose absorption in the jejunum of patients with cystic fibrosis. Gastroenterology 1985; 88: 478–484.

    PubMed  CAS  Google Scholar 

  138. Buts JP, Morin CL, Roy CC, et al. One hour blood xylose test: a reliable index of small bowel function. J Pediatr 1978; 92: 729–733.

    Article  PubMed  CAS  Google Scholar 

  139. Roy CC, Weber AM. Nutrition of the child with cystic fibrosis. In: Nutrition in Pediatrics (Walker WA, Watkins JB, eds). Boston: Little Brown and Company, 1985, pp. 463–484.

    Google Scholar 

  140. Milla PJ, Kilby A, Rassam UB, et al. Small intestinal absorption of amino acids and a dipeptide in pancreatic insufficiency. Gut 1983; 24: 878–924.

    Article  Google Scholar 

  141. Cox KL, Ament ME, Isenberg JN. Light microscopic abnormalities of proximal small intestinal biopsies of patients with cystic fibrosis. Pediatr Res 1978; 12: 472.

    Google Scholar 

  142. Taussig LM, Saldino RM, DiSant’Agnese PA. Radiographic abnormalities of the duodenum and small bowel in cystic fibrosis of the pancreas (mucoviscidosis). Radiology 1973; 106: 369–376.

    PubMed  CAS  Google Scholar 

  143. Lebenthal E, Lee PC. Effect of pancreozymin and secretin on intraluminal enterokinase, trypsin and chymotrypsin activities of cystic fibrosis and control children. Digestion 1982; 23: 39–47.

    Article  PubMed  CAS  Google Scholar 

  144. Eggermont E. The role of the small intestine in cystic fibrosis patients. Acta Paediatr Scand 1988; Suppl 317: 16–21.

    Google Scholar 

  145. Wesley A, Forstner J, Qureshi R, et al. Human intestinal mucin in cystic fibrosis. Pediatr Res 1983; 17: 65–69.

    Article  PubMed  CAS  Google Scholar 

  146. Forstner J, Wesley A, Mantle M, et al. Abnormal mucus: nominated but not yet elected. J Pediatr Gastroenterol Nutr 1984; Suppl:567–573.

    Google Scholar 

  147. Ross S, Morrison D, McArthur R. Hypersecretion of gastric inhibitory polypeptide in non-diabetic children with cystic fibrosis. Pediatrics 1981; 67: 252–254.

    PubMed  CAS  Google Scholar 

  148. Adrian T, McKiernan J, Johnstone D, et al. Hormonal abnormalities of the pancreas and gut in cystic fibrosis. Gastroenterology 1980; 79: 460–465.

    PubMed  CAS  Google Scholar 

  149. Tso P, Kendrick H, Balint JA, Simmonds WJ. Role of biliary phosphatidylcholine in the absorption and transport of dietary triolein in the rat. Gastroenterology 1987; 80: 60–65.

    Google Scholar 

  150. Brasitus TA, Davidson NO, Schachter D. Variations in dietary triacylglycerol saturation alter the lipid composition and fluidity of rat intestinal plasma membranes. Biochem Biophys Acta 1985; 872: 460–472.

    Google Scholar 

  151. Hadorn B, Zoppi G, Shmerling D, et al. Quantitative assessment of exocrine pancreatic function in infants and children. J Pediatr 1968; 73: 39–50.

    Article  PubMed  CAS  Google Scholar 

  152. Bonin A, Roy C, Lasalle R, et al. Fecal chymotrypsin: a reliable index of exocrine pancreatic function in children. J Pediatr 1973; 83: 594–600.

    Article  PubMed  CAS  Google Scholar 

  153. Hubbard VS, Wolf RO, Lester LA, Egge AC. Diagnostic and therapeutic applications of bentiromide screening test for exocrine pancreatic insufficiency in patients with cystic fibrosis. Dig Dis Sci 1984; 29: 881–889.

    Article  PubMed  CAS  Google Scholar 

  154. Cumming JGR, Forsyth JS, Boyd EJS, et al. Diagnosis of exocrine pancreatic insufficiency in cystic fibrosis by use of fluorescein dilaurate test. Arch Dis Child 1986; 61: 573–575.

    Article  PubMed  CAS  Google Scholar 

  155. Burnard E. Congenital pancreatic fibrosis, report of survival in a young adult. NZ Med J 1953; 52: 395–397.

    CAS  Google Scholar 

  156. Dutta SK, Rubin J, Harvey J. Comparative evaluation of the therapeutic efficacy of a pH-sensitive enteric-coated enzyme preparation with conventional pancreatic enzyme therapy in the treatment of pancreatic insufficiency. Gastroenterology 1983; 84: 476–482.

    PubMed  CAS  Google Scholar 

  157. Stead RJ, Skypala I, Hodson MC, Batten JC. Enteric coated microspheres of pancreatin in the treatment of cystic fibrosis: comparison with a standard enteric coated preparation. Thorax 1987; 42: 533–537.

    Article  PubMed  CAS  Google Scholar 

  158. Durie PR, Bell L, Linton W, et al. Effect of cimetidine and sodium bicarbonate on pancreatic replacement therapy in cystic fibrosis. Gut 1980; 21: 778–786.

    Article  PubMed  CAS  Google Scholar 

  159. Staub JL, Sarles H, Soule JC, et al. No effect of cimetidine on the therpeutic response to oral enzymes in severe pancreatic steatorrhea. N Engl J Med 1981; 304: 1364–1365.

    PubMed  CAS  Google Scholar 

  160. Sinaasappel M, Bouquet J, Neijers HJ. Problems in the treatment of malabsorption in CF. Acta Paediatr Scand 1988; Suppl 317: 22–27.

    Google Scholar 

  161. King RFGJ, Howdle PD, Kelleher J, Losowsky MS. Synthetic detergents in bile salt deficient steatorrhea. Clin Sci 1979; 56: 273–287.

    PubMed  CAS  Google Scholar 

  162. Robinson PJ, Sly PD, Smith AL. Effect of misoprostol on fat malabsorption in cystic fibrosis. Arch Dis Child 1988; 63: 1087–1092.

    Article  Google Scholar 

  163. Corey M, Gaskin K, Durie P, et al. Improved prognosis in CF patients with normal fat absorption. J Pediatr Gastroenterol Nutr 1984; Suppl 10: S99-S100.

    Google Scholar 

  164. Sproul A, Huang N. Growth patterns in children with cystic fibrosis. J Pediatr 1964; 65: 664–676.

    Article  PubMed  CAS  Google Scholar 

  165. Soutter VL, Kristides P, Gruca MA, Gaskin KJ. Chronic undernutrition/growth retardation in cystic fibrosis. Clin Gastroenterol 1986; 15: 137–155.

    PubMed  CAS  Google Scholar 

  166. Bell L, Durie P, Forstner GG. What do cystic fibrosis patients eat? J Pediatr Gastroenterol Nutr 1984; 3 (Suppl 1):S137–S146.

    Article  PubMed  Google Scholar 

  167. Hubbard VS, Mangrum PJ. Energy intake and nutrition counselling in cystic fibrosis. J Am Diet Assoc 1984; 84: 664–669.

    Google Scholar 

  168. Kollberg J, Eriksson GB. What do CF patients eat? Monogr Paediatr 1981; 14: 202–210.

    Google Scholar 

  169. Hodges P, Sauriol D, Man SFP, et al. Nutrient intake of patients with cystic fibrosis. J Am Diet Assoc 1984; 84: 664–669.

    PubMed  CAS  Google Scholar 

  170. Parsons H, Dumas A, Beaudry P, Pencharz P. Dietary counselling and nutritional supplementation in the treatment of cystic fibrosis and its effect on growth. In: Perspectives in Cystic Fibrosis (Sturgess JM, ed). Toronto: Canadian Cystic Fibrosis Foundation, 1980, p 161.

    Google Scholar 

  171. Kinney JM. Energy expenditure and nutritional assessment. In: Nutritional Assessment: Present Status, Future Directions and Prospects 1981. (Report of the Second Ross Conference on Medical Research.) Columbus, OH: Ross Laboratories.

    Google Scholar 

  172. Cropp GJA, Rosenberg PN. Energy costs of breathing in cystic fibrosis: relation between severity of pulmonary disease and basal oxygen consumption. Cystic Fibrosis Club Abstracts. Atlanta, GA: Cystic Fibrosis Foundation, 1982, p. 15.

    Google Scholar 

  173. Shepherd RW, Holt TL, Vasques-Velasquez L, et al. Increased energy expenditure in young children with cystic fibrosis. Lancet 1988; 1: 1300–1303.

    Article  PubMed  CAS  Google Scholar 

  174. Corey ML. Longitudinal studies in cystic fibrosis. In: Perspectives in Cystic Fibrosis (Sturgess JM, ed). Toronto: Canadian Cystic Fibrosis Foundation, The Imperial Press Ltd., 1980, pp. 246–255.

    Google Scholar 

  175. Keens TG, Krastins IRB, Wannamaker EM, et al. Ventilatory muscle endurance training in normal subjects and patients with cystic fibrosis. Am Rev Respir Dis 1977; 116: 853–860.

    PubMed  CAS  Google Scholar 

  176. Rochester DF. Malnutrition and the respiratory muscles. Clin Chest Med 1986; 7: 91–99.

    PubMed  CAS  Google Scholar 

  177. Rochester DF, Esau SA. Malnutrition and the respiratory system. Chest 1984; 85: 411–414.

    Article  PubMed  CAS  Google Scholar 

  178. Kelsen SG. The effects of undernutrition on the respiratory muscles. Clin Chest Med 1986; 7: 107–110.

    Google Scholar 

  179. Lepage G, Levy E, Ronco N, et al. Direct transesterification of plasma fatty acids for the diagnosis of essential fatty acid deficiency in cystic fibrosis. J Lipid Res 1989; 30: 1483–1490.

    PubMed  CAS  Google Scholar 

  180. Levy E, Lepage G, Bendayan M, et al. Lipoprotein metabolism in cystic fibrosis: role of essential fatty acid deficiency. J Lipid Res 1989; 30: 1197–1209.

    PubMed  CAS  Google Scholar 

  181. Hopkins DT, Witter RL, Nesheim MC. A respiratory disease syndrome in children fed essential fatty acid deficient diets. Proc Soc Exp Biol Med 1963; 114: 82–86.

    PubMed  CAS  Google Scholar 

  182. Sevanian A, Hochstein P. Mechanisms and consequences of lipid peroxidation in biological systems. Ann Rev Nutr 1985; 5: 365–390.

    Article  CAS  Google Scholar 

  183. Lefkowith JB, Evers AS, Elliott WJ, Needleman P. Essential fatty acid deficiency: a new look at an old problem. Prostaglandins Leukotrienes Med 1986; 23: 123–127.

    Article  CAS  Google Scholar 

  184. Chase HP, Dupont J. Abnormal levels of prostaglandins and fatty acids in blood of children with cystic fibrosis. Lancet 1978; 2: 236–238.

    Article  PubMed  CAS  Google Scholar 

  185. Bach AC, Babayan VK. Medium chain triglycerides: an update. Am J Clin Nutr 1982; 36: 950.

    Google Scholar 

  186. Abman SH, Reardon MC, Accurso FJ, et al. Hypoalbuminemia at diagnosis as a marker for severe respiratory course in infants with cystic fibrosis identified by newborn screening. J Pediatr 1985; 107: 933–935.

    Article  PubMed  CAS  Google Scholar 

  187. Peterson R, Peterson V, Robb R. Vitamin A deficiency with xerophthalmia and night blindness in cystic fibrosis. Am J Dis Child 1968; 116: 662–665.

    Google Scholar 

  188. Abernathy R. Bulging fontanelle as presenting sign in cystic fibrosis; vitamin A metabolism and effect on cerebrospinal fluid pressure. Am J Dis Child 1976; 130: 1360–1362.

    PubMed  CAS  Google Scholar 

  189. Hubbard VS. Nutritional considerations in cystic fibrosis. Semin Resp Med 1985; 6: 308–313.

    Article  Google Scholar 

  190. Congden PJ, Bruce G, Rothburn MM, et al. Vitamin status in treated patients with cystic fibrosis. Arch Dis Child 1987; 56: 708–714.

    Article  Google Scholar 

  191. Smith F, Underwood B, Denning C, et al. Depressed plasma retinol binding protein levels in cystic fibrosis. J Lab Clin Med 1972; 80: 423–433.

    PubMed  CAS  Google Scholar 

  192. Guggenheim MA, Ringel SP, Silverman A, Grabert BE. Progressive neuromuscular disease in children with chronic cholestasis and vitamin E deficiency: diagnosis and treatment with a- tocopherol. J Pediatr 1982; 100: 51–58.

    Article  PubMed  CAS  Google Scholar 

  193. Sitrin MD, Lieberman F, Jensen WE, et al. Vitamin E deficiency and neurological disease in adults with cystic fibrosis. Ann Intern Med 1987; 107: 51–54.

    PubMed  CAS  Google Scholar 

  194. Willison HJ, Muller DPR, Matthews S, et al. A study of the relationship between neurological function and serum vitamin E concentrations in patients with cystic fibrosis. J Neurol Neu-rosurg Psych 1985; 48: 1097–1102.

    Article  CAS  Google Scholar 

  195. Stead PG, Muller DPR, Matthews S, et al. Effect of abnormal liver function on vitamin E status and supplementation in adults with cystic fibrosis. Gut 1986; 27: 714–718.

    Article  PubMed  CAS  Google Scholar 

  196. Cynamon HA, Milov DE, Valenstein E, Wagner M. Effect of vitamin E deficiency on neurologic function in patients with cystic fibrosis. J Pediatr 1988; 113: 637–640.

    Article  PubMed  CAS  Google Scholar 

  197. Steiner M, Mower R. Mechanism of action of vitamin E on platelet function. Ann NY Acad Sci 1982; 393: 289–299.

    Article  PubMed  CAS  Google Scholar 

  198. Torterson O, Humphrey G, Edson J, Warwick W. Cystic fibrosis presenting with severe haemorrhage due to vitamin K malabsorption: a report of 3 cases. Pediatrics 1970; 45: 857–861.

    Google Scholar 

  199. Hubbard V, Farrell P, di Sant’Agnese P. 25-hydroxycholecalciferol levels in patients with cystic fibrosis. J Pediatr 1979; 94: 84–86.

    Article  PubMed  CAS  Google Scholar 

  200. Hahn T, Squires A, Halstead L, Strominger D. Reduced serum 25-hydroxy vitamin D concentration and disordered mineral metabolism in patients with cystic fibrosis. J Pediatr 1979; 94: 38–42.

    Article  PubMed  CAS  Google Scholar 

  201. Rucher RW, Harrison GM. Vitamin B12 deficiency in cystic fibrosis. N Engl J Med 1973; 289: 329.

    Google Scholar 

  202. Veeger W, Abeis J, Hellemans N, et al. Effect of sodium bicarbonate and pancreatic on the absorption of vitamin B12 and fat in pancreatic insufficiency. N Engl J Med 1962; 267: 1341–1345.

    Article  PubMed  CAS  Google Scholar 

  203. Jacob RA, Sandstead HH, Solomons NW, et al. Zinc status and vitamin A transport in cystic fibrosis. Am J Clin Nutr 1978; 31: 368.

    Google Scholar 

  204. Hambidge KM. The role of zinc and other trace metals in pediatric nutrition and health. Pediatr Clin North Am 1977; 24: 95–121.

    PubMed  CAS  Google Scholar 

  205. Chase HP, Long MA, Lavin MH. Cystic fibrosis and malnutrition. J Pediatr 1979; 95: 337–347.

    Article  PubMed  CAS  Google Scholar 

  206. Roy CC, Weber AM. Nutrition of the child with cystic fibrosis. In: Nutrition in Pediatrics (Walker WA, Watson JB, eds). Boston: Little Brown and Company, 1984.

    Google Scholar 

  207. Shepherd R, Cooksley WGE, Cooke WDD. Improved growth and clinical nutritional and respiratory changes in response to nutritional therapy in cystic fibrosis. J Pediatr 1980; 97: 351–357.

    Article  PubMed  CAS  Google Scholar 

  208. Levy L, Durie P, Pencharz P, Corey M. Prognostic factors associated with patient survival during nutritional rehabilitation in malnourished children and adolescents with cystic fibrosis. J Pediatr Gastroenterol Nutr 1986; 5: 97–102.

    Article  PubMed  CAS  Google Scholar 

  209. Yassa YG, Prosser R, Dodge JA. Effects of an artificial diet on growth of patients with cystic fibrosis. Arch Dis Child 1978; 53: 777–783.

    Article  PubMed  CAS  Google Scholar 

  210. Shepherd RW, Holt TL, Thomas BJ, et al. Nutritional rehabilitation in cystic fibrosis controlled studies of effects on nutritional growth retardation, body protein turnover, and course of pulmonary disease. J Pediatr 1986; 209: 788–794.

    Google Scholar 

  211. O’Loughlin E, Forbes D, Parsons H, et al. Nutritional rehabilitation of malnourished patients with cystic fibrosis. Am J Clin Nutr 1986; 43: 732–737.

    PubMed  Google Scholar 

  212. Boland MP, Stoski DS, McDonald NE, et al. Chronic jejunostomy feeding with a non-elemental formula in undernourished patients with cystic fibrosis. Lancet 1986; 1: 232–234.

    Article  PubMed  CAS  Google Scholar 

  213. Roy CC. Gastrointestinal and hepatobiliary complications: changing pattern with age. In: Perspectives in Cystic Fibrosis (Sturgess JM, ed). Toronto: Canadian Cystic Fibrosis Foundation, 1980, pp. 190–197.

    Google Scholar 

  214. Graham N, Manhire AR, Stead RJ, et al. Cystic fibrosis: ultrasonographic findings in the pancreas and hepatobiliary system controlled with clinical data and pathology. Clin Radiol 1985; 36: 199–203.

    Article  PubMed  CAS  Google Scholar 

  215. Boat TF, Doershuk CF, Stern RC, Mathews LW. Serum alkaline phosphatase in cystic fibrosis. Clin Pediatr (Phila) 1974; 13: 505–512.

    CAS  Google Scholar 

  216. Isenberg JN, L’Heureux P, Warwick WJ, Sharp HL. Detection of cirrhosis in cystic fibrosis: lack of correlation with gammaglutamyl transpeptidase and biliary tract roentgenograms (abstract). Gastroenterology 1974; 66: 887.

    Google Scholar 

  217. Shwachman H, Kowalski M, Klaw KT. Cystic fibrosis: a new outlook: 70 patients above 25 years of age. Medicine (Baltimore) 1977; 56: 129–149.

    CAS  Google Scholar 

  218. DiSant’Agnese PA, Davis PB. Cystic fibrosis in adults: 75 cases and a review of 232 cases in the literature. Am J Med 1979; 66: 121–132.

    Article  Google Scholar 

  219. Stern RC, Stevens DP, Boat TF, et al. Symptomatic hepatic disease in cystic fibrosis: incidence, course and outcome of portal systemic shunting. Gastroenterology 1976; 70: 645–649.

    PubMed  CAS  Google Scholar 

  220. Hultcrantz R, Mengarelli S, Strandvik B. Morphological findings in the liver of children with cystic fibrosis: a light and electron microscopical study. Hepatology 1986; 6: 881–889.

    Article  PubMed  CAS  Google Scholar 

  221. Abramson SJ, Baker DH, Amodia JB, Berdon WE. Gastrointestinal manifestations of cystic fibrosis. Semin Roentgenol 1987; 22: 97–113.

    Article  PubMed  CAS  Google Scholar 

  222. Cunningham DG, Churchill RJ, Reynes CJ. Computed tomography in the evaluation of liver disease in cystic fibrosis patients. J Comput Assist Tomogr 1980; 4: 151–154.

    Article  PubMed  CAS  Google Scholar 

  223. Oppenheimer E, Esterly J. Hepatic changes in young infants with cystic fibrosis: possible relation to focal biliary cirrhosis. J Pediatr 1975; 86: 683–689.

    Article  PubMed  CAS  Google Scholar 

  224. Oppenheimer EH, Esterly JR. Pathology of cystic fibrosis: review of the literature and comparison with 146 autopsied cases. Perspect Pediatr Pathol 1975; 2: 241–278.

    PubMed  CAS  Google Scholar 

  225. Maurage C, Lenaerts C, Weber AM, et al. Meconium ileus and its equivalent as a risk factor for the development of cirrhosis. An autopsy study in cystic fibrosis. J Pediatr Gastroenterol Nutr 1989; 9: 17–20.

    PubMed  CAS  Google Scholar 

  226. Sellinger M, Henderson RM, Zweifach A, et al. Identification and characterization of a chloride channel in canalicular rat liver plasma membranes. Presented at the convention of the American Association for the Study of Liver Diseases 1988 Nov 5-8, Chicago, Illinois.

    Google Scholar 

  227. Oppenheimer EH, Esterly JR. Congenital cytomegalovirus infection and bile duct destruction in newborn infant with cystic fibrosis of the pancreas. Lancet 1973; 2: 1031–1032.

    Article  Google Scholar 

  228. Yousef IM, Tuchweber B, Vonk RJ, et al. Lithocholate cholestasis: sulfated glycolithocholate induced intrahepatic cholestasis in rats. Gastroenterology 1987; 80: 233–234.

    Google Scholar 

  229. Shattuck KE, Little JM, Padominska A, et al. Lithocholate glucuronide and cholestasis: a possible vicious cycle. Presented at the convention of the American Association for the Study of Liver Diseases 1988 Nov 5-8, Chicago, Illinois.

    Google Scholar 

  230. Roy CC, Dorvil N, Tuchweber B, et al. Taurine prevents cholestasis induced by lithocholic acid sulfate in guinea pigs (abstract). Pediatr Res 1982; 16: 175.

    Google Scholar 

  231. Mieli-Vergani G, Psacharopoulos HT, Nicholson AM, et al. Immune responses to liver membrane antigens in patients with cystic fibrosis and liver disease. Arch Dis Child 1980; 55: 696–701.

    Article  PubMed  CAS  Google Scholar 

  232. Di Sant’Agnese P, Blanc W. A distinctive type of biliary cirrhosis of the liver associated with cystic fibrosis of the pancreas. Pediatrics 1956; 18: 387–409.

    Google Scholar 

  233. Schuster S, Shwachman H, Toyama W, et al. The management of portal hypertension in cystic fibrosis. J Pediatr Surg 1977; 12: 201–206.

    Article  PubMed  CAS  Google Scholar 

  234. Tyson K, Schuster S, Shwachman H. Portal hypertension in cystic fibrosis. J Pediatr Surg 1968; 3: 271–277.

    Article  PubMed  CAS  Google Scholar 

  235. Sty JR, Werlin SL, Bruns WT. Gray-scale ultrasonography, the liver and biliary tract in cystic fibrosis. Wisconsin Med J 1980; 79: 23–26.

    PubMed  CAS  Google Scholar 

  236. McHugo JM, McKeown C, Brown MT, et al. Ultrasound findings in children with cystic fibrosis. Br J Radiol 1987; 60: 137–141.

    Article  PubMed  CAS  Google Scholar 

  237. Tanner MS. Current clinical management of hepatic problems in cystic fibrosis. J R Soc Med 1986; Suppl 12(79):38–43.

    Google Scholar 

  238. Cox KL, Ward RE, Furgiele TL, et al. Orthotopic liver transplantation in patients with cystic fibrosis. Pediatrics 1987; 80: 571–574.

    PubMed  CAS  Google Scholar 

  239. Warwick WJ, L’Heureux PR, Sharp HL, Isenberg JN. Gallstones and cystic fibrosis. In: Proceedings of the Seventh International Cystic Fibrosis Congress, Paris, 1976, pp. 484–491.

    Google Scholar 

  240. Esterly J, Oppenheimer E. Observations in cystic fibrosis I. The gallbladder. Johns Hopkins Med J 1962; 110: 247–254.

    CAS  Google Scholar 

  241. Isenberg J, L’Heureux P, Warwick W, Sharp H. Clinical observations on the biliary system in cystic fibrosis. Am J Gastroenterol 1976; 65: 134–141.

    PubMed  CAS  Google Scholar 

  242. Roy C, Weber A, Morin C, et al. Abnormal biliary lipid composition in cystic fibrosis, effect of pancreatic enzymes. N Engl J Med 1977; 297: 1301–1305.

    Article  PubMed  CAS  Google Scholar 

  243. Vawter GF, Shwachman H. Cystic fibrosis in adults: an autopsy study. Pathol Annu 1979; 14: 357–382.

    PubMed  Google Scholar 

  244. Stern RC, Rothstein FC, Doershuk CF. Treatment and prognosis of symptomatic gallbladder disease in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 1986; S:35–40.

    Google Scholar 

  245. Bennian LJ, Ginsberg RL, Garnick MB, Bennett PH. Effects of oral contraceptives on the gallbladder bile of normal women. N Engl J Med 1974; 29: 189–192.

    Google Scholar 

  246. Bass S, Connon JJ, Ho CS. Biliary tree in cystic fibrosis: biliary tract abnormalities in cystic fibrosis demonstrated by endoscopic retrograde cholangiography. Gastroenterology 1983; 84: 1592–1596.

    PubMed  CAS  Google Scholar 

  247. Gaskin KJ, Waters DLM, Howman-Giles, R, et al. Liver disease and common bile duct stenosis in cystic fibrosis. AT Engl J Med 1988; 318: 340–346.

    Article  CAS  Google Scholar 

  248. Roy CC, Lenaerts C, Garel L, et al. Liver disease and common bile duct stenosis in cystic fibrosis (letter). N Engl J Med 1988; 318: 312.

    Google Scholar 

  249. Strandvik B, Hjelte L, Gabrielsson N, Glauman H. Sclerosing cholangitis in cystic fibrosis. Scand J. Gastroenterol 1988; 23 (Suppl 143): 121–124.

    Article  Google Scholar 

  250. Quinton PM. Chloride impermeability in cystic fibrosis. Nature 1983; 301: 421–422.

    Article  PubMed  CAS  Google Scholar 

  251. Peterson OH, Findlay I, Iwatsula N, et al. Human pancreatic acinar cells: studies on stimulus-secretion coupling. Gastroenterology 1985; 89: 109–117.

    Google Scholar 

  252. Frizzell RA, Reckheimer G, Shoemaker RL. Altered regulation of airway epithelial cell chloride channels in cystic fibrosis. Science 1986; 233: 558–560.

    Article  PubMed  CAS  Google Scholar 

  253. Sato K, Sato F. Defective p-adrenergic response of cystic fibrosis sweat glands in vivo and in vitro. J Clin Invest 1984; 73: 1763–1771.

    Article  PubMed  CAS  Google Scholar 

  254. Cotton CU, Stutts MJ, Knowles MR, et al. Abnormal apical cell membrane in cystic fibrosis respiratory epithelium. J Clin Invest 1987; 79: 80–85.

    Article  PubMed  CAS  Google Scholar 

  255. Li M, McCann JD, Liedtke CM, et al. Cyclic AMP-dependent protein kinase opens chloride channels in normal but not cystic fibrosis airway epithelium. Nature 1988; 331: 1–3.

    Article  Google Scholar 

  256. Berschneider HM, Knowles MR, Azizkhan RG, et al. Altered intestinal chloride transport in cystic fibrosis. FASEB J 1988; 2: 2625–2629.

    PubMed  CAS  Google Scholar 

  257. Pedersen PS, Larsen EH, Brandt MJ. Restitution of chloride permeability in cystic fibrosis. MedSciRes 1987; 15: 151–152.

    CAS  Google Scholar 

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  1. Department of Pediatrics, Research Unit, Gastroenterology-Nutrition Service, University of Montreal, Hôpital Ste-Justine, Montreal, Quebec, Canada, H3T 1C5

    Lesley Smith, Andrée Weber & Claude C. Roy

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  1. Lesley Smith
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  1. The University of Alberta, Edmonton, Alberta, Canada

    Alan B. R. Thomson M.D., Ph.D.

  2. University of Calgary, Calgary, Alberta, Canada

    Eldon Shaffer M. D.

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Smith, L., Weber, A., Roy, C.C. (1992). Gastrointestinal and Hepatobiliary Complications of Cystic Fibrosis. In: Thomson, A.B.R., Shaffer, E. (eds) Modern Concepts in Gastroenterology. Topics in Gastroenterology. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-3314-6_16

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