Download PDF
Zentralbl Chir 2017; 142(02): 169-179
DOI: 10.1055/s-0032-1328743
Übersicht
Georg Thieme Verlag KG Stuttgart · New York

Lebertransplantation als Therapie von seltenen Erkrankungen beim Erwachsenen

Liver Transplantation as Treatment for Rare Diseases in Adults
A. Bauschke
Klinik für Allgemein-, Viszeral-, Gefäßchirurgie, Universitätsklinikum Jena, Deutschland
,
C. Malessa
Klinik für Allgemein-, Viszeral-, Gefäßchirurgie, Universitätsklinikum Jena, Deutschland
,
F. Rauchfuss
Klinik für Allgemein-, Viszeral-, Gefäßchirurgie, Universitätsklinikum Jena, Deutschland
,
J. Zanow
Klinik für Allgemein-, Viszeral-, Gefäßchirurgie, Universitätsklinikum Jena, Deutschland
,
U. Settmacher
Klinik für Allgemein-, Viszeral-, Gefäßchirurgie, Universitätsklinikum Jena, Deutschland
› Author Affiliations
Further Information

Publication History

Publication Date:
15 November 2013 (online)

Also available at
    Permissions and Reprints

Zusammenfassung

Neben den Hauptindikationen, die 95 % aller Patienten haben, welche in Deutschland lebertransplantiert werden, gibt es eine Vielzahl von Erkrankungen, die auch im Erwachsenenalter klinisch manifest werden und zur Indikationsstellung führen. Es können dies Stoffwechselerkrankungen, deren Defekt hauptsächlich in der Leber lokalisiert ist, Missbildungen der lebereigenen Zellen, hepatische Gefäßerkrankungen und seltene Tumoren der Leber sein. Nur für einige Erkrankungen gelten „Standard Exceptions“ bei der Listung. Die exakte Diagnostik und der Zeitpunkt der Transplantation sind entscheidend für die Prognose.

Abstract

In addition to the main indications pertaining to 95 % of all patients receiving liver transplantation in Germany, there are numerous other diseases that may become clinically evident in the adult age and may lead to the decision for liver transplantation. These may be metabolic diseases with their main defect located in the liver, malformations of liver cells, hepatic vascular diseases and rare tumours of the liver. Standard exceptions for the listing are in place only for a limited number of diseases. Exact diagnostics and the point in time for transplantation are crucial for the prognosis.

Schlüsselwörter

Lebertransplantation - seltene Indikation - Allokation

Key words

liver transplantation - rare indications - allocation
 

  • Literatur

  • 1 Bundesärztekammer. Richtlinien für die Wartelistenführung und die Organvermittlung gem. § 16 Abs. 1 S. 1 Nrn. 2 u. 5 TPG. Im Internet: http://www.bundesaerztekammer.de/page.asp?his=7.45.8858.8870 Stand:12. März 2012
    PubMedGoogle Scholar
  • 2 van Keimpema L, Nevens F, Adam R. et al. Excellent survival after liver transplantation for isolated polycystic liver disease: an European Liver Transplant Registry study. Transpl Int 2011; 24: 1239-1245
    CrossrefPubMedGoogle Scholar
  • 3 Chandok N, Uhanova J, Marotta P. Clinical outcomes of liver transplantation for polycystic liver disease: a single center experience. Ann Hepatol 2010; 9: 278-281
    PubMedGoogle Scholar
  • 4 Taner B, Willingham DL, Hewitt WR. et al. Polycystic liver disease and liver transplantation: single-institution experience. Transplant Proc 2009; 41: 3769-3771
    CrossrefPubMedGoogle Scholar
  • 5 Krohn PS, Hillingso JG, Kirkegaard P. Liver transplantation in polycystic liver disease: a relevant treatment modality for adults?. Scand J Gastroenterol 2008; 43: 89-94
    CrossrefPubMedGoogle Scholar
  • 6 Harring TR, Nguyen NTT, Liu H. et al. Caroli disease patients have excellent survival after liver transplant. J Surg Res 2012; 177: 365-372
    CrossrefPubMedGoogle Scholar
  • 7 Jacquemin E. Progressive familial intrahepatic cholestasis. Clin Res Hepatol Gastroenterol 2012; 36 (Suppl. 01) S26-S35
    CrossrefPubMedGoogle Scholar
  • 8 Davit-Spraul A, Gonzales E, Baussan C. et al. Progressive familial intrahepatic cholestasis. Orphanet J Rare Dis 2009; 4: 1
    CrossrefPubMedGoogle Scholar
  • 9 Hori T, Egawa H, Takada Y. et al. Progressive familial intrahepatic cholestasis: a single-center experience of living-donor liver transplantation during two decades in Japan. Clin Transplant 2011; 25: 776-785
    CrossrefPubMedGoogle Scholar
  • 10 Hori T, Egawa H, Miyagawa-Hayashino A. et al. Living-donor liver transplantation for progressive familial intrahepatic cholestasis. World J Surg 2011; 35: 393-402
    CrossrefPubMedGoogle Scholar
  • 11 Alagille D, Odievre M, Gautier M. et al. Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur. J Pediatr 1975; 86: 63-71
    CrossrefPubMedGoogle Scholar
  • 12 Turnpenny PD, Ellard S. Alagille syndrome: pathogenesis, diagnosis and management. Eur J Hum Genet 2012; 20: 251-257
    CrossrefPubMedGoogle Scholar
  • 13 Shneider BL. Liver transplantation for Alagille syndrome: the jagged edge. Liver Transpl 2012; 18: 878-880
    CrossrefPubMedGoogle Scholar
  • 14 Catana AM, Medici V. Liver transplantation for Wilson disease. World J Hepatol 2012; 4: 5-10
    CrossrefPubMedGoogle Scholar
  • 15 Arnon R, Kerkar N, Davis MK. et al. Liver transplantation in children with metabolic diseases: the studies of pediatric liver transplantation experience. Pediatr Transplant 2010; 14: 796-805
    CrossrefPubMedGoogle Scholar
  • 16 Nazer H, Ede RJ, Mowat AP. et al. Wilsonʼs disease: clinical presentation and use of prognostic index. Gut 1986; 27: 1377-1381
    CrossrefPubMedGoogle Scholar
  • 17 Dhawan A, Taylor RM, Cheeseman P. et al. Wilsonʼs disease in children: 37-year experience and revised Kingʼs score for liver transplantation. Liver Transpl 2005; 11: 441-448
    CrossrefPubMedGoogle Scholar
  • 18 Cheng F, Li SQ, Zhang F. et al. Outcomes of living-related liver transplantation for Wilsonʼs disease: a Single-center experience in China. Transplantation 2009; 87: 751-757
    CrossrefPubMedGoogle Scholar
  • 19 Park YK, Kim BW, Wang HJ. et al. Auxiliary partial orthotopic living donor liver transplantation in a patient with Wilsonʼs disease: a case report. Transplant Proc 2008; 40: 3808-3809
    CrossrefPubMedGoogle Scholar
  • 20 Dar FS, Faraj W, Zaman MB. et al. Outcome of liver transplantation in hereditary hemochromatosis. Transpl Int 2009; 22: 717-724
    CrossrefPubMedGoogle Scholar
  • 21 Raichlin E, Daly RC, Rosen CB. et al. Combined heart and liver transplantation: a single-center experience. Transplantation 2009; 88: 219-225
    CrossrefPubMedGoogle Scholar
  • 22 Moini M, Mistry P, Schilsky ML. Liver transplantation for inherited metabolic disorders of the liver. Curr Opin Organ Transplant 2010; 15: 269-276
    CrossrefPubMedGoogle Scholar
  • 23 Sperl J, Prochazkova J, Martasek P. et al. N-acetyl cysteine averted liver transplantation in a patient with liver failure caused by erythropoietic protoporphyria. Liver Transpl 2009; 15: 352-354
    CrossrefPubMedGoogle Scholar
  • 24 Reddy SK, Austin SL, Spencer-Manzon M. et al. Liver transplantation for glycogen storage disease type Ia. J Hepatol 2009; 51: 483-490
    CrossrefPubMedGoogle Scholar
  • 25 Chryssostalis A, Hubert D, Coste J. et al. Liver disease in adult patients with cystic fibrosis: a frequent and independent prognostic factor associated with death or lung transplantation. J Hepatol 2011; 55: 1377-1382
    CrossrefPubMedGoogle Scholar
  • 26 Jamieson NV, Jamieson KA. Primary hyperoxaluria type 1: gene therapy by liver transplantation. Transplantation 2009; 87: 1273-1274
    CrossrefPubMedGoogle Scholar
  • 27 Cochat P, Hulton SA, Acquaviva C. et al. Primary hyperoxaluria Type 1: indications for screening and guidance for diagnosis and treatment. Nephrol Dial Transplant 2012; 27: 1729-1736
    CrossrefPubMedGoogle Scholar
  • 28 Popescu I, Dima SO. Domino liver transplantation: how far can we push the paradigm?. Liver Transpl 2012; 18: 22-28
    CrossrefPubMedGoogle Scholar
  • 29 Saner FH, Treckmann J, Pratschke J. et al. Early renal failure after domino liver transplantation using organs from donors with primary hyperoxaluria type 1. Transplantation 2010; 90: 782-785
    PubMedGoogle Scholar
  • 30 Jamieson NV. A 20-year experience of combined liver/kidney transplantation for primary hyperoxaluria (PH1): the European PH1 transplant registry experience 1984–2004. Am J Nephrol 2005; 25: 282-289
    CrossrefPubMedGoogle Scholar
  • 31 Malde DJ, Pararajasingam R, Tavakoli A. et al. Transplantation in adults with primary hyperoxaluria: single unit experience and treatment algorithm. Ann Transplant 2011; 16: 111-117
    CrossrefPubMedGoogle Scholar
  • 32 Mazzaferro V, Pulvirenti A, Coppa J. Neuroendocrine tumors metastatic to the liver: How to select patients for liver transplantation?. J Hepatol 2007; 47: 460-466
    CrossrefPubMedGoogle Scholar
  • 33 Foss A, Adam R, Dueland S. Liver transplantation for colorectal liver metastases: revisiting the concept. Transpl Int 2010; 23: 679-685
    CrossrefPubMedGoogle Scholar
  • 34 Chan G, Kocha W, Reid R. et al. Liver transplantation for symptomatic liver metastases of neuroendocrine tumours. Curr Oncol 2012; 19: 217-221
    PubMedGoogle Scholar
  • 35 Gedaly R, Daily MF, Davenport D. et al. Liver transplantation for the treatment of liver metastases from neuroendocrine tumors: an analysis of the UNOS database. Arch Surg 2011; 146: 953-958
    CrossrefPubMedGoogle Scholar
  • 36 Bioulac-Sage P, Laumonier H, Laurent C. et al. Benign and Malignant Vascular Tumors of the Liver in Adults. Semin Liver Dis 2008; 28: 302-314
    Article in Thieme ConnectPubMedGoogle Scholar
  • 37 Mehrabi A, Kashfi A, Fonouni H. et al. Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. Cancer 2006; 107: 2108-2121
    CrossrefPubMedGoogle Scholar
  • 38 Lerut JP, Weber M, Orlando G. et al. Vascular and rare liver tumors: a good indication for liver transplantation?. J Hepatol 2007; 47: 466-475
    CrossrefPubMedGoogle Scholar
  • 39 Lerut JP, Orlando G, Adam R. et al. The place of liver transplantation in the treatment of hepatic epitheloid hemangioendothelioma: report of the European liver transplant registry. Ann Surg 2007; 246: 949-957 discussion 957
    CrossrefPubMedGoogle Scholar
  • 40 Grotz TE, Nagorney D, Donohue J. et al. Hepatic epithelioid haemangioendothelioma: is transplantation the only treatment option?. HPB 2010; 12: 546-553
    CrossrefPubMedGoogle Scholar
  • 41 Orlando G, Adam R, Mirza D. et al. Hepatic hemangiosarcoma: an absolute contraindication to liver transplantation–the European Liver Transplant Registry experience. Transplantation 2013; 95: 872-877
    CrossrefPubMedGoogle Scholar
  • 42 Maluf D, Cotterell A, Clark B. et al. Hepatic angiosarcoma and liver transplantation: case report and literature review. Transplant Proc 2005; 37: 2195-2199
    CrossrefPubMedGoogle Scholar
  • 43 Zheng MH, Zhang L, Gu DN. et al. Hepatoblastoma in adult: review of the literature. J Clin Med Res 2009; 1: 13-16
    PubMedGoogle Scholar
  • 44 Rougemont AL, McLin VA, Toso C. et al. Adult hepatoblastoma: learning from children. J Hepatol 2012; 56: 1392-1403
    CrossrefPubMedGoogle Scholar
  • 45 Ishak KG, Goodman ZD, Stocker JT. Atlas of Tumor Pathology. Tumors of the liver and intrahepatic bile ducts. Hepatoblastoma. 2nd ed.. Washington, DC: American Registry of Pathology; 2001
    Google Scholar
  • 46 Di Sandro S, Slim AO, Lauterio A. et al. Liver adenomatosis: a rare indication for living donor liver transplantation. Transplant Proc 2009; 41: 1375-1377
    PubMedGoogle Scholar
  • 47 Hajdu CH, Murakami T, Diflo T. et al. Intrahepatic portal cavernoma as an indication for liver transplantation. Liver Transpl 2007; 13: 1312-1316
    CrossrefPubMedGoogle Scholar
  • 48 Shamseddine A, Faraj W, Mukherji D. et al. Unusually young age distribution of primary hepatic leiomyosarcoma: case series and review of the adult literature. World J Surg Oncol 2010; 8: 56
    CrossrefPubMedGoogle Scholar
  • 49 Darwish Murad S, Plessier A, Hernandez-Guerra M. et al. Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med 2009; 151: 167-175
    CrossrefPubMedGoogle Scholar
  • 50 Mancuso A. Budd-Chiari syndrome management: Lights and shadows. World J Hepatol 2011; 3: 262-264
    CrossrefPubMedGoogle Scholar
  • 51 Campos-Varela I, Castells L, Dopazo C. et al. Transjugular intrahepatic portosystemic shunt for the treatment of sinusoidal obstruction syndrome in a liver transplant recipient and review of the literature. Liver Transpl 2012; 18: 201-205
    CrossrefPubMedGoogle Scholar
  • 52 Plessier A, Rautou PE, Valla DC. Management of hepatic vascular diseases. J Hepatol 2012; 56 (Suppl. 01) S25-S38
    PubMedGoogle Scholar
  • 53 Shovlin CL, Guttmacher AE, Buscarini E. et al. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet 2000; 91: 66-67
    CrossrefPubMedGoogle Scholar
  • 54 Dupuis-Girod S, Chesnais AL, Ginon I. et al. Long-term outcome of patients with hereditary hemorrhagic telangiectasia and severe hepatic involvement after orthotopic liver transplantation: a single-center study. Liver Transpl 2010; 16: 340-347
    PubMedGoogle Scholar
  • 55 Lerut J, Orlando G, Adam R. et al. Liver transplantation for hereditary hemorrhagic telangiectasia: Report of the European liver transplant registry. Ann Surg 2006; 244: 854-862 discussion 862–864
    CrossrefPubMedGoogle Scholar
  • 56 Moore EE, Cogbill TH, Jurkovich GJ. et al. Organ injury scaling: spleen and liver (1994 revision). J Trauma 1995; 38: 323-324
    CrossrefPubMedGoogle Scholar
  • 57 Heuer M, Kaiser GM, Lendemans S. et al. Transplantation after blunt trauma to the liver: a valuable option or just a “waste of organs”?. Eur J Med Res 2010; 15: 169-173
    CrossrefPubMedGoogle Scholar
  • 58 Voigtlander T, Negm AA, Schneider AS. et al. Secondary sclerosing cholangitis in critically ill patients: model of end-stage liver disease score and renal function predict outcome. Endoscopy 2012; 44: 1055-1058
    Article in Thieme ConnectPubMedGoogle Scholar
  • 59 Vanatta JM, Modanlou KA, Dean AG. et al. Outcomes of orthotopic liver transplantation for hepatic sarcoidosis: an analysis of the United Network for Organ Sharing/Organ Procurement and Transplantation Network data files for a comparative study with cholestatic liver diseases. Liver Transpl 2011; 17: 1027-1034
    PubMedGoogle Scholar
  • 60 Schoenlank F. Ein Fall von Peliosis hepatis. Virchows Arch [Path Anat] 1916; 222: 358
    CrossrefPubMedGoogle Scholar
  • 61 Bexten T, Burck I, Bechstein WO. et al. [Hepatic peliosis – a rare liver tumor and challenge for diagnostic investigation plus therapy]. Dtsch Med Wochenschr 2012; 137: 1505-1509
    Article in Thieme ConnectPubMedGoogle Scholar