| Autor: |
Munar-Ques, M, Parilla, P, Acosta, F, Lo´pez-Andreu, F, Costa, PP, Escribano, JB, Mingo, P De, Sa´nchez-Bueno, F, Rami´ez, P, Robles, R, Miras, M, Pons, JA, Costa, PMP, Viader-Farre´, C, Forteza-Alberti´, JF, Cifuentes-Luna, C |
| Zdroj: |
Neuromuscular Disorders; 1996, Vol. 6 Issue: 1, Number 1 Supplement 1 pS79-S79, 1p |
| Abstrakt: |
Background The consensus of the 1st International Workshop (1993) was that liver transplantation (OLT) is a worthwhile procedure for the treatment of FAP. We studied the main data of our 17 patients who underwent transplantation between 13-9-1991 and 22-6-1995. Epidemiology Sex: 14 male. Age: 22-58; mean 42±12. Years FAP: 1-20. Heredity: 13 were familial cases, all with anticipation of onset age. Sibling from 2 families; 4 and 3 sibs respectively. TTR variant: 14 Met 30 and 4 Ala 71. Birthplace: 12 Majorca, 4 Spanish mainland and 2 Portugal Biochemical results Plasma levels of TTR variant immediately came down to negative values; later on levels remained negatives. Outcome 5 patients died (mortality 28%); 3 by sepsis (after 6, 1.5 and 8 months), 1 by complete atrioventricular block (after 1.5 months) and 1 in a very advanced FAP stage, by perioperatory multifactorial shock and secondary multiorgan failure (after 3 days). Interval after OLT in alive patients: between 8 and 44 months in 10, and less than 2 in the last 3 cases. Clinical results in 10 patients They were better in the youngest patients. 1) Disappearance of anxiety and depression in 100%. 2) Slow weight gain in 70%. 3) Peripheral neuropathy. No progression in any case; in 90% ability and stability improved slowly and we realized increase of muscular mass and strength; in 100% light recovery of termoalgesic sensation; electromyography only showed slight improvement after more than 12 months of OLT. 4) Oesophageal dysfunctions didn't recover, gastric and intestinal troubles improved in 60%. 5) Slow improvement of urinary disturbances in 87%. 6) In 89% significant improvement of orthostatic hypotension. 7) In 4 patients light and short erections reappeared. 8) Dry irritative cought disappeared in 4 patients. 9) Reappearance of transpiration on some anhidrotic zones in 2 patients. 10) Proteinuria disappeared in the only patient with it. Conclusions OLT results are encouraging. FAP patients in borderline conditions must be discarded. Immediate treatment of infections, and prevention of rejection and nephrotoxicity are mandatory. We advise periodic control of the cardiac conduction system in patients with important myocardial involvement specially during intercurrent processes. |
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