Zusammenfassung
Cholangiopathien sind akute oder chronische biliäre Erkrankungen mit erheblicher Morbidität
und Mortalität. Sie sind charakterisiert durch pathophysiologische Veränderungen an
den Gallengangsepithelien (Cholangiozyten). Die Entwicklung neuer experimenteller
Modelle hat in den letzten Jahren zu einem enormen Wissenszuwachs um diese Zellen
geführt. Heute lassen sich die Cholangiopathien in verschiedene Untergruppen unterteilen.
Dazu gehören hereditäre oder entwicklungsbedingte, immunologische, infektiöse, toxische,
ischämische, neoplastische und unklassifizierte Erkrankungen. Die Erkenntnisse der
letzten Jahre haben dazu beigetragen, dass wir die pathophysiologischen Zusammenhänge
besser verstehen können (z.B. cholangiozelluläres Karzinom, polyzystische Leber- und
Nierenerkrankung, AIDS-Cholangiopathie). Dies wiederum bietet die Möglichkeit, neue
therapeutische Strategien zu entwickeln.
Summary
Cholangiopathies are acute or chronic biliar diseases with substantial morbidity and
mortality. They are characterized by pathophysiological changes at the cholangiocellular
level. Due to the development of new experimental models our knowledge about physiology
and pathophysiology of the biliary epithelial cells has grown enormously. Today, cholangiopathies
are divided into several groups, for example hereditary, immunological, infectious,
toxic, ischemic, neoplastic and unclassified diseases. We begin to understand the
pathophysiology of several diseases (cholangiocellular carcinoma, polycystic kidney
and liver disease, AIDS-cholangiopathy) and new therapeutic strategies can be developed
from this knowledge.
Key Words
cholangiocytes - cholestasis - sklerosing cholangitis - cholangiocellular carcinoma
- polycystic liver disease
Literatur
- 1
Alpini G, McGill JM, LaRusso NF.
The pathobiology of biliary epithelia.
Hepatology.
2002;
35
1256-1268
- 2
Batts KP.
Ischemic cholangitis.
Mayo Clin Proc.
1988;
73
380-385
- 3
Chen XM, Keithly JS, Paya CV, LaRusso N.
Cryptosporidiosis.
N Engl J Med.
2002;
346
1723-1731
- 4
Degott C.
Drug-induced liver injury.
Pathology Oncology Research.
1997;
3
260-263
- 5
Elsing C, Schlenker T, Stremmel W.
Diagnostik der Cholangiopathien.
Dtsch Arztebl.
2003;
100
2955-2959
- 6
Engler S, Elsing C, Flechtenmacher C. et al. .
Progressive sclerosing cholangitis after septic shock: a new variant of vanishing
bile duct disorders.
Gut.
2003;
52
688-693
- 7
Gores GJ.
A spotlight on cholangiocarcinoma.
Gastroenterol.
2003;
125
1536-1537
- 8
Haruna Y, Kanda T, Honda M. et al. .
Detection of Hepatitis C in the bile and bile duct epithelial cells of Hepatitis C
virus-infected patients.
Hepatology.
2001;
33
977-980
- 9
Howell CD, Yoder T, Claman HN, Vierling JM.
Hepatic homing of mononuclear cells isolated during murine chronic graft-vs-host disease.
J Immunol.
1989;
143
476-483
- 10
Masyuk TV, Huang BQ, Ward CJ. et al. .
Defects in cholangiocyte fibrocystin expression and ciliary structure in the PCK rat.
Gastroenterol.
2003;
125
1303-1310
- 11
Reynoso-Paz S, Coppel RL, Mackay IR. et al. .
The immunobiology of bile and biliary epithelium.
Hepatology.
1999;
30
351-357
- 12
Schlenker T, Fitz JG.
Ca(2+) activated Cl-channels in a human biliary cell line: regulation by Ca2+/calmodulin-dependent
protein kinase.
Am J Physiol.
1996;
271
G304-G310
- 13
Spirli C, Nathanson MH, Fiorotto R. et al. .
Proinflammatory cytokines inhibit secretion in rat bile duct epithelium.
Gastroenterol.
2001;
121
156-169
- 14
Taniai M, Higuchi H, Burgart U, Gores GJ.
P16INK4a promotor mutations are frequent in primary sclerosing cholangitis (PSC) and
PSC-associated cholangiocarcinoma.
Gastroenterol.
2002;
123
1090-1098
- 15
Van de Water J, Ishibashi H, Coppel RL, Gershwin ME.
Molecular mimicry and primary biliary cirrhosis: premises not promises.
Hepatology.
2001;
33
771-775
Anschrift für die Verfasser
PD Dr. Chr. Elsing
Medizinische Klinik, St. Elisabeth-Krankenhaus
Pf.-W.-Schmitz-Str. 1
46282 Dorsten
