[The assessment of the nutritional status of patients with familial amyloid neuropathy type I (Corino Andrade's disease) who are candidates for orthotopic liver transplantation]. / Valoración del estado nutricional de los pacientes con polineuropatía amiloidótica familiar tipo I (enfermedad de Corino Andrade) candidatos a trasplante ortotópico de hígado.

Corino Andrade's disease (Type I Family amyloidotic polyneuropathy) (FAP I) is a slow-evolving hereditary amyloidosis affecting, among other things, the digestive system, with the appearance of an amyloid deposit which produces a malabsorptive syndrome with diarrhea in those affected. At present, the only effective therapeutic option is orthotopic liver transplant (OLT): clearance from the hospital's transplant commission as a candidate requires, among other things, a prior nutritional study. We therefore proposed to carry out a nutritional assessment of these patients, comparing them with a group of terminal liver patients who are OLT candidates. The PAF I group showed a high level of calorie energy malnutrition (86%) and, to a lesser extent, visceral and protein malnutrition. The liver patient group showed 67% of visceral malnutrition and lower levels of calorific energy and protein malnutrition. All the OLT candidates showed a high rate of nutritional deterioration. The greater presence of calorific energy malnutrition in the PAF I patients might be due to the neuropathic gastro-intestinal condition. The high level of malnutrition encountered suggest the need for pre-transplant nutritional intervention designed to minimise post-surgical risk.