Reporting centers and number of transplants performed
 (Dec 31, 2016)

COUNTRY CITY HOSPITAL No of TX No of TX/Country
Portugal Lisbon Hospital Curry Cabral 384 978
Porto Hospital Sto Antonio 327
Coimbra Coimbra University Hospital 260
  Porto Hospital S João 7  
France Villejuif Hospital Paul Brousse/Kremlin Bicêtre 232 277
  Strasbourg Hospital Hautpierre 13  
  Marseille Hospital de la Conception 10  
Lyon Hospital Edouard Herriot 8
  Clichy Hospital Beaujon 7  
  Bordeaux Pellegrin Hospital 7  
        
Sweden Stockholm Karolinska University Hospital Huddinge 134 160
  Gothenburg Sahlgrenska University Hospital 26  
 USA Rochester Mayo Clinic 47 128
Burlington Lahey Clinic Medical Center 21
Boston NEDH/Harvard Medical School 14
San Francisco UCSF Medical Center 11
  Miami Jackson Memorial 8  
Phoenix Mayo Clinic 6
  Pittsburgh Thomas E Starzl Transpl. Institute 4  
  Philadelphia The Penn Transplant Center 3  
  Baltimore University of Maryland 3  
  Cleveland Cleveland Clinic 2  
  Charleston Medical University of South Carolina 2  
  Chicago Northwestern Memorial Hospital 2  
Chapel Hill UNC Comprehensive Transplant Center 2
Cleveland University Hospitals of Cleveland 1
  Denver University Hospital 1  
  Durham Duke University Medical Center 1  
Spain Barcelona Hospital de Bellvitge 42 112
Murcia Hospital Virgen de la Arrixaca 27
  Seville University Hospital Virgen del Rocio 17  
Barcelona Hospital Clinic Provincial 12
Vizcaya Hospital de Cruces Barakaldo 5
  Pamplona Clinica Universitaria 4  
  Barcelona Hospital Vall D'Hebron 3  
  Madrid Hospital Ramón y Cajal 1
  Valencia La Fe Hospital 1
   
Brazil São Paulo Hospital Albert Einstein 66 110
Rio de Janeiro Federal University of Rio de Janeiro 29
  Santo Amaro Oswaldo Cruz University Hospital 14  
Paraná Federal University of Paraná 1
   
UK London King´s College Hospital 74 78
  London Royal Postgraduate Medical School 2  
  London Royal Free Hospital 1  
  Birmingham Queen Elizabeth Hospital 1  
Japan Matsumoto Shinshu University Hospital 40 76
Kumamoto Kumamoto University Hospital 23
Nagoya Nagoya University Hospital 4
Fukuoka Kyushu University Hospital 2
  Tokyo Tokyo University Hospital 2  
Sapporo Hokkaido University Hospital 2
Tokyo Keio University School of Medicine 1
Kyoto Kyoto University Hospital 1
Niigata Niigata University Hospital 1
Germany Mainz Der Johannes Gutenberg Universität 23 68
Heidelberg Klinikum der Universität 21
  Hannover Medizinische Hochschule 21  
Freiburg Chirurgische Universitätsklinik 1
Münster Universitätsklinikum 1
Tübingen Klinikum der Universität 1
Italy Bologna Hospital Bellaria 27 29
Milan National Cancer Institute 2
Belgium Brussels UCL St Luc 18 26
  Liège CHU Sart Tilman 5  
  Gent University Hospital 3  
         
Australia Brisbane Queensland LTS 19 25
Sydney Royal Prince Alfred Hospital 4
Bedford Park South Australia Liver Transplant Unit 2
Netherlands Groningen University Hospital 24 24
Switzerland Geneva Hôpital Cantonal 17 22
Bern Inselspital 5
Canada London London Health Science Center 9 19
Toronto Toronto General Hospital 8
Montreal Hospital Saint-Luc 2
Argentina Buenos Aires Hospital C Argerich 14 17
Buenos Aires Hospital Italiano 3
Denmark Copenhagen Copenhagen University Hospital 14 14
China Hong Kong Queen Mary Hospital 5 5
         
India Coimbatore Kovai Medical Center and Hospital 1 1
         
Singapore Singapore Singapore General Hospital 1 1
         
Taiwan Taipei Taipei Veterans General Hospital 1 1

 

Patient charachteristics:

Sex distribution: Male 57% / Female 43%

Age at time of transplantation: Mean 41.3±11.3 years, median 38.2 years, range 21 – 73 years.

Duration of disease before transplantation: Mean 3.8±2.8 years, median 3 years and ranging between 0 – 30 years.

 

Multiple Organ Transplant (Dec 31, 2015) OLT + Kidney:54
  OLT + Heart:49
OLT + Previous Heart:16
  OLT + Kidney + Heart: 4
  OLT + Sequential Heart: 1
OLT + Sequential Kidney: 1

 

Main causes of death:

Cardiac related deaths: 21%

Septicemia: 21%

Liver related complications: 14%

Peroperative deaths: 3%

 

Conclusions

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Liver transplantation for familial amyloidotic polyneuropathy is performed on a world wide basis

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An average of approximately 120 patients worldwide were transplanted each year for FAP. However, during the last 5 years there has been a decline in reported patients, probably because of the introduction of new medication for FAP. Wether this decline is stable remains to see.

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The modified body mass index (mBMI), disease duration, type of mutation and degree of autonomic involvment are important prognostic factors

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Sepsis and cardiac related deaths account for approximately 42% of the deaths post transplant