Diapositiva 1

PREVALENCE OF “RARE DISORDERS (RD)” IN THE ORGAN DONORS
Marina Alvarez Miranda, Ana María Garcia Pozo, María Sanchez Frías, Blanca Miranda Serrano
Introduction: The shortage of organs for transplants is the main problem we face have to. Although organ donation rate has increased
by 200% in the last 10 years and consecuently transplant activity has doubled (kidneys) and – by five (livers). The waiting list and the
mortality while waiting is increasing every year.The increase of the criteria for donor approval is a constant objective in our daily
practice.
Patients awaiting for a TX (31st Dec.)
% Patients dead while on the WL
(2003)
700
631
12
600
Patients awaiting for a Kidney TX (31st Dec.)
10
500
99
00
01
02
03
8
400
3922
3986
4014
3933
4026
6
300
4
200
112
128
100
1999
2000
2001
2002
2003
2
0
0
Liver
Liver
Heart
Heart
Lung
Lung
Objectives: Study the prevalence of “unknown diseases” in the organ donors, and the efficiency of these donated organs.
Methodology: The ONT donors´ data base, from January 2001 to December 2003, has been revised, including both months.
Results:
A total of 19 RD have been registered among-----valid donors (prevalence 0.4%). Another 29 potential donors considered as “non valid”
were also registered.
A total of 17 Kidneys, 12 livers, 12 hearts and 2 lungs were satisfactory grafted.
INVALID DONORS (RD)
VALID DONORS (RD)
Vascular Affectation
(7 cases)
Neurologic Affectation/
Muscular A. (6 cases)
•Dermatomiositis
•Displasia Fibromuscular
•Enfermedad de Kawasaky
•Síndrome de Rendu-OslerWeber
•Angiopatia Mieloide
Cerebral
•Moya-Moya
•Síndrome de Banti
•Sindrome de Arnold- Chiari
•Polineuropatía Amiloidótica
Familiar
•Síndrome de Sturge –
Weber
•Distrofia Miotónica de
Steiner (3)
Deposit/ Autoimmune
(3 cases)
Genetic Defect
(3 cases)
•Nefrocalcinosis
•Hemocromatosis Familiar
•Enfermedad de GravesBasedow
•Síndrome de Turner
•Síndrome de Wolf
Parkinson White
•Anemia Hemolítica
Esferocítica
Vascular Affectation
(12 cases)
•Buerguer
•Arteritis de Takayasu
•Enfermedad de Fabry
•Esclerodermia
•Síndrome Antifosfolípido
•Encéfalo-malacia Multiquística de
Origen Isquémico
•Fiebre Botonosa del Mediterráneo
•Púrpura Trombocitopénica
Idiopática
•Púrpura Trombótica Idiopática
•Púrpura Trombocitopénica
•Trombocitemia Esencial
•Tromboastemia de Glanzmann
Infections (2 cases)
•Mielistis Transversa
•Leptospirosis
Neurologic Affectation/
Muscular A. ( 10 cases)
•Guillain-Barre
•Enfermedad de Kuf
•Leucodistrofia Metacromática
Juvenile
•Síndrome de Arnold-Chiari
•Síndrome de Leigh
•Síndrome de Batten
•Síndrome de Paton
•Siringomielia-Malformación de
Chiari
•Distrofia Muscular de Becker
•Corea de Hungtinton
Genetic Defect/ Other (5 cases)
•Síndrome de WisKott-Aldrich
•Síndrome del Cromosoma Fácil
•Esferocitosis Hereditaria
•Intoxicación por Paraquat
•Tricoleucemia
Differences in the diagnosis of “unknown diseases” have been identified. The greater numbers of invalid donors are in the
thrombocytopenia and coagulation alterations group, while the most valid donors belong to diseases related to muscular irregularities.
Conclusions: In an effort to tackle a long waiting list, makes the criteria of donor approval should be amplified. True that the group of
“unknown diseases” constitutes a short percentage of donors, but it isn’t less true this short percentage of donors had not been
considered some years ago. However, these donors have increased, by little, the number of organs available for transplant. A general –
consulation pool would be useful to check wheather or not a given potential organ donor can be safety grafted.