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Selection criteria for liver transplantation in patients with familial amyloidotic polyneuropathy
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SELECTION CRITERIA FOR LIVER TRANSPLANTATION IN PATIENTS WITH FAMILIAL AMYLOIDOTIC POLYNEUROPATHY Munar-Qu6s M ~, Parrilla p2, Acosta F2, L6pez-Andreu F 3, S~inchez-Bueno F2, Ramfrez p2, Robles R2, Escribano JB 2, Miras M2, Pons JA2, Forteza-Albertt JF2, Costa PI~ ~Grupo de Estudio de la PAF, Hospital General Mallorca, Spain, 2Hospital Virgen de la Arrixaca, Murcia, Spain, 3Hospital General Universitario, Murcia, Spain, 4Centro de Estudos de Paramilodose, Porto, Portugal Background In Stockholm (1993) we presented the selection criteria for liver transplantation (OLT) in the first II FAP patients. Now we report the present approach after 18 transplants; the aims are to improve quality of life and diminish morbidity and mortality of an elective OLT. Methods We indicate OLT in FAP patients with objective troubles which reduce their quality of life; consequently we discard OLT in asymptomatic carriers with biopsy proof of amyloid deposition and in FAP patients with mild sensory and autonomic troubles which scarcely interfere with their activities; on another hand we refuse patients in very advanced stages because of high operatory risk and scarce possibilities of recovery. We selected 2 groups of candidates which FAP duration ranging from 1 to 20 years: a) 9 younger patients (3rd and 4th decade), 5 with rapid progress of symptoms (aggressive form) and 4 with slow progress, b) 9 elder patients (Sth and 6th decade), 4 with aggressive forms and 5 with slow progress. 7 selected patients were sibs from 2 families; 4 and 3 respectively. Basic conditions l) Cardiac assessment within normal limits. 2) Patients with severe renal failure are discarded for OLT not combined with renal transplantation; we accept those with renal amyloidosis (biopsy proven) with proteinura less than 3 gr. per 24 h. but normal renal function, and others who already have mild renal failure (creatinine less than 2 mg.). 3) During anhepatic stage, we advise the use of venovenous bypass, or much better, the piggyback procedure with preservation of the inferior vena cava, to avoid deleterous effects on cardiovascular and renal systems with the simple crossclamping technique, specially in patients with renal amyloidosis and amyloid myocardial involvement. Risk factors l) Atonic neurogenic bladder, syringomyelic anesthesia in the back, orthostatic hypotension and respiratory failure of muscular origin can originate complications in the postoperative period and later on. 2) Very remote possibility of inefective OLT if the donor was an assymptomatic carder without FAP antecedents, particularly in zones with high prevelance of carders (Portugal, Japan and Sweden). The FAP patient as a liver donor We insist on the possible transplantation of his removed liver, to another patient with serious liver disease.
SELECTION CRITERIA FOR LIVER TRANSPLANTATION IN PATIENTS WITH FAMILIAL AMYLOIDOTIC POLYNEUROPATHY Munar-Qu6s M ~, Parrilla p2, Acosta F2, L6pez-Andreu F 3, S~inchez-Bueno F2, Ramfrez p2, Robles R2, Escribano JB 2, Miras M2, Pons JA2, Forteza-Albertt JF2, Costa PI~ ~Grupo de Estudio de la PAF, Hospital General Mallorca, Spain, 2Hospital Virgen de la Arrixaca, Murcia, Spain, 3Hospital General Universitario, Murcia, Spain, 4Centro de Estudos de Paramilodose, Porto, Portugal Background In Stockholm (1993) we presented the selection criteria for liver transplantation (OLT) in the first II FAP patients. Now we report the present approach after 18 transplants; the aims are to improve quality of life and diminish morbidity and mortality of an elective OLT. Methods We indicate OLT in FAP patients with objective troubles which reduce their quality of life; consequently we discard OLT in asymptomatic carriers with biopsy proof of amyloid deposition and in FAP patients with mild sensory and autonomic troubles which scarcely interfere with their activities; on another hand we refuse patients in very advanced stages because of high operatory risk and scarce possibilities of recovery. We selected 2 groups of candidates which FAP duration ranging from 1 to 20 years: a) 9 younger patients (3rd and 4th decade), 5 with rapid progress of symptoms (aggressive form) and 4 with slow progress, b) 9 elder patients (Sth and 6th decade), 4 with aggressive forms and 5 with slow progress. 7 selected patients were sibs from 2 families; 4 and 3 respectively. Basic conditions l) Cardiac assessment within normal limits. 2) Patients with severe renal failure are discarded for OLT not combined with renal transplantation; we accept those with renal amyloidosis (biopsy proven) with proteinura less than 3 gr. per 24 h. but normal renal function, and others who already have mild renal failure (creatinine less than 2 mg.). 3) During anhepatic stage, we advise the use of venovenous bypass, or much better, the piggyback procedure with preservation of the inferior vena cava, to avoid deleterous effects on cardiovascular and renal systems with the simple crossclamping technique, specially in patients with renal amyloidosis and amyloid myocardial involvement. Risk factors l) Atonic neurogenic bladder, syringomyelic anesthesia in the back, orthostatic hypotension and respiratory failure of muscular origin can originate complications in the postoperative period and later on. 2) Very remote possibility of inefective OLT if the donor was an assymptomatic carder without FAP antecedents, particularly in zones with high prevelance of carders (Portugal, Japan and Sweden). The FAP patient as a liver donor We insist on the possible transplantation of his removed liver, to another patient with serious liver disease.