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Hereditäre Pankreaserkrankungen

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Pathologie

Part of the book series: Pathologie ((PATHOL))

Zusammenfassung

Das Kapitel behandelt die bekannten erblichen Erkrankungen wie zystische Fibrose und Hämochromatose. Es werden jedoch auch die „neueren“ Erbkrankheiten wie die Pankreasagenesie und das Shwachman-Diamond-Syndrom zur Sprache gebracht. Auf das familiäre (hereditäre) Pankreaskarzinom, die hereditäre Pankreatitis sowie die hereditären zystischen Veränderungen wird nur kurz eingegangen, da diese Erkrankungen primär in den Kapiteln zu den Tumoren und zur Pankreatitis behandelt werden.

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Literatur

  1. Anderson CM, Goodchild MC (1976) Cystic fibrosis. Manual of diagnosis and management. Blackwell, Oxford

    Google Scholar 

  2. Azmy AF, Ziervogel MA (1983) Meconium ileus equivalent in children with cystic fibrosis. Z Kinderchir 38:253–255

    CAS  PubMed  Google Scholar 

  3. Bedrossian CW, Greenberg SD, Singer DB, Hansen JJ, Rosenberg HS (1976) The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups. Human Pathol 7:195–204

    Article  CAS  Google Scholar 

  4. di Sant’Agnese PA, Davis PB (1979) Cystic fibrosis in adults. 75 cases and a review of 232 cases in the literature. Am J Med 66:121–132

    Article  Google Scholar 

  5. Dolbey CH (2001) Hemochromatosis: a review. Clin J Oncol Nurs 5:257–260

    CAS  PubMed  Google Scholar 

  6. Elborn JS (2016) Cystic fibrosis. Lancet 388:2519–2531

    Article  CAS  Google Scholar 

  7. Farrelly G, Fitzgerald R, Mathews T (1983) Meconium thorax. J Pediatr Surg 18:623–624

    Article  CAS  Google Scholar 

  8. Gould NS, Paton JB, Bennett AR (1989) Johanson-Blizzard syndrome: clinical and pathological findings in 2 sibs. Am J Med Genet 33:194–199

    Article  CAS  Google Scholar 

  9. Gross V, Schoelmerich J, Denzel K, Gerok W (1989) Relapsing pancreatitis as initial manifestation of cystic fibrosis in a young man without pulmonary disease. Int J Pancreatol 4:221–228

    CAS  PubMed  Google Scholar 

  10. Iannucci A, Mukai K, Johnson D, Burke B (1984) Endocrine pancreas in cystic fibrosis: an immunohistochemical study. Human Pathol 15:278–284

    Article  CAS  Google Scholar 

  11. Isenberg JN, Heureux PR, Warwick WJ, Sharp HL (1976) Clinical observations on the biliary system in cystic fibrosis. Am J Gastroenterol 65:134–141

    CAS  PubMed  Google Scholar 

  12. Kulczycki LL, Kostuch M, Bellanti JA (2003) A clinical perspective of cystic fibrosis and new genetic findings: relationship of CFTR mutations to genotype-phenotype manifestations. Am J Med Genet 116A:262–267

    Article  Google Scholar 

  13. Lebenthal E, Lerner A, Rolston DDK, Go VLW, DiMagno EP, Gardner JD, Reber HA, Scheele GA (1993) The pancreas in cystic fibrosis. In: Go VLW, DiMagno EP, Gardner JD, Lebenthal E, Reber HA, Scheele GA (Hrsg) The pancreas: biology, pathobiology, and disease, chapter 54, 2. Aufl. Raven Press, New York, S 1041–1081

    Google Scholar 

  14. Lerner A, Lebenthal E (1993) Hereditary diseases of the pancreas. In: Go VLW, DiMagno EP, Gardner JD, Lebenthal E, Reber HA, Scheele GA (Hrsg) The pancreas, biology, pathobiology, and disease, chapter 55, 2. Aufl. Raven Press, New York, S 1083–1094

    Google Scholar 

  15. Löhr M, Goertchen P, Nizze H, Gould NS, Gould VE, Oberholzer M, Heitz PU, Klöppel G (1989) Islet changes associated with advanced cystic fibrosis. An immunocytochemical and morphometrical study. Virchows Arch A Pathol Anat Histopathol 414:179–185

    Article  Google Scholar 

  16. Masaryk TJ, Achkar E (1983) Pancreatitis as initial presentation of cystic fibrosis in young adults. A report of two cases. Dig Dis Sci 28:874–878

    Article  CAS  Google Scholar 

  17. McLaughlin FJ, Matthews WJ Jr., Strieder DJ, Khaw KT, Schuster S, Shwachman H (1982) Pneumothorax in cystic fibrosis: management and outcome. J Pediatr 100:863–869

    Article  CAS  Google Scholar 

  18. Myers KC, Davies SM, Shimamura A (2013) Clinical and molecular pathophysiology of Shwachman-Diamond syndrome: an update. Hematol Oncol Clin North Am 27:117–128

    Article  Google Scholar 

  19. Nousia-Arvanitakis S (1999) Cystic fibrosis and the pancreas: recent scientific advances. J Clin Gastroenterol 29:138–142

    Article  CAS  Google Scholar 

  20. Ode KL, Moran A (2013) New insights into cystic fibrosis-related diabetes in children. Lancet Diabetes Endocrinol 1:52–58

    Article  CAS  Google Scholar 

  21. Oppenheimer E, Esterly J (1973) Cystic fibrosis of the pancreas. Morphologic findings in infants with and without diagnostic pancreatic lesions. Arch Pathol Lab Med 96:149–154

    CAS  Google Scholar 

  22. Porter DK, Van Every MJ, Anthracite RF, Mack JW Jr (1983) Massive hemoptysis in cystic fibrosis. Arch Intern Med 143:287–290

    Article  CAS  Google Scholar 

  23. Rosenberg E, Shmerling DH (1983) Das Meconium-Ilieus-Äquivalent bei Mucoviscidose. Klin Pädiatr 195:323–329

    Article  CAS  Google Scholar 

  24. Röttger P (1976) Die cystische Fibrose, pathologische Anatomie. In: Forell MM (Hrsg) Pankreas. Handbuch der Inneren Medizin, Bd. 3/6. Springer, Berlin, S 890–894

    Google Scholar 

  25. Rüschoff J, Lippmann A, Leititis J (1986) Zur pathomorphologischen Frühdiagnose der Mukoviszidose. Kasuistik und Differentialdiagnose. Pathologe 7:227–232

    Google Scholar 

  26. Schidlow DV, Goldsmith DP, Palmer J, Huang NN (1984) Arthritis in cystic fibrosis. Arch Dis Child 59:377–379

    Article  CAS  Google Scholar 

  27. Seifert G (1984) Cystic fibrosis and haemochromatosis. In: Klöppel G, Heitz PU (Hrsg) Pancreatic pathology, chapter 4. Churchill Livingstone, Edinburgh, S 32–43

    Google Scholar 

  28. Seifert G, Klöppel G, Heitz PU (1984) Lipomatous atrophy and other forms. In: Klöppel G, Heitz PU (Hrsg) Pancreatic pathology, chapter 3. Churchill Livingstone, Edinburgh, S 27–31

    Google Scholar 

  29. Sosnay PR, Raraigh KS, Gibson RL (2016) Molecular genetics of cystic fibrosis transmembrane conductance regulator: genotype and phenotype. Pediatr Clin North Am 63:585–598

    Article  Google Scholar 

  30. Stern RC (1986) Cystic fibrosis: recent developments in diagnosis and treatment. Pediatr Rev 7:276–286

    Article  CAS  Google Scholar 

  31. Stern RC, Boat TF, Wood RE, Matthews LW, Doershuk CF (1982) Treatment and prognosis of nasal polyps in cystic fibrosis. Am J Dis Child 136:1067–1070

    CAS  PubMed  Google Scholar 

  32. Stern RC, Izant RJ Jr., Boat TF, Wood RE, Matthews LW, Doershuk CF (1982) Treatment and prognosis of rectal prolapse in cystic fibrosis. Gastroenterology 82:707–710

    Article  CAS  Google Scholar 

  33. Wood RE (1979) Cystic fibrosis: diagnosis, treatment, and prognosis. South Med J 72:189–202

    Article  CAS  Google Scholar 

  34. Zenker M, Mayerle J, Reis A, Lerch MM (2006) Genetic basis and pancreatic biology of Johanson-Blizzard syndrome. Endocrinol Metab Clin North Am 35:243–253

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Institut für Pathologie, Konsultationszentrum für Pankreas- und endokrine Tumoren, Technische Universität München, München, Deutschland

    Prof. Dr. Günter Klöppel

Authors
  1. Prof. Dr. Günter Klöppel
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Correspondence to Günter Klöppel .

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Editors and Affiliations

  1. Institut für Pathologie, Ruhr-Universität Bochem, Bochum, Nordrhein-Westfalen, Germany

    Andrea Tannapfel

  2. Konsultationszentrum für Pankreas- und Endokrine Tumore, Technische Universität München, München, Germany

    Günter Klöppel

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Klöppel, G. (2020). Hereditäre Pankreaserkrankungen. In: Tannapfel, A., Klöppel, G. (eds) Pathologie. Pathologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-04557-8_21

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  • DOI: https://doi.org/10.1007/978-3-642-04557-8_21

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-04556-1

  • Online ISBN: 978-3-642-04557-8

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