| Autor: |
Cruz, M W, Novis, S A P, Raia, S |
| Zdroj: |
Neuromuscular Disorders; 1996, Vol. 6 Issue: 1, Number 1 Supplement 1 pS82-S82, 1p |
| Abstrakt: |
The authors present a multidisciplinary protocol for the evaluation of FAP type I patients before and after liver transplantation (LT), including: clinical and ENMG examination; EKG and echocardiogram, tests of autonomic, renal, and digestive functions; ophtalmological examinations and TTR met-30 detection by ELISA (CEPP). The patients were selected by criteria dependent on: age; clinical stage; ENMG biopsy of peripheral nerve; TTR Met 30 presence, time of disease and associated diseases. Partial results from 3 men and 1 woman (mean age of 33 years) submitted to LT by the last author, will be presented. Between them a man (33) was sent to LT after 3 years of disease presenting 7 points at a specific developed clinical scale and ENMG disclosing a peripheral neuropathy of axonal type. Ten months after LT he was found to have 5 points at the disability scale mostly due to autonomical symptoms amellioraton. He had a weight gain of 4 kg. ENMG was worsened in every aspect. More time is needed for the correct access of the electrophysiological outcome of these patients but clinically we noted stabilization reinforcing the importance of performing LT early in the course of disease. |
| Databáze: |
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