[III Working Meeting SENPE-Baxter: complementary parenteral nutrition in the critically ill patient]. / III Mesa de Trabajo SENPE-Baxter: nutrición parenteral complementaria en el paciente crítico.

In the setting of a multidisciplinary debate, and after reviewing the available evidence as well as the experience from experts, the indications and management guidelines for Complementary Parenteral Nutrition (CPN) in the critically ill patient are established. The conclusion refers to the importance of its indication in all the cases where enteral nutrition (EN) is insufficient to cover at least 60% of the caloric-protein target. At least 80% of the patient's caloric requirements should be covered with EN and CPN, with the recommendation of targeting 100% of the demands.

Fabry disease: overall effects of agalsidase alfa treatment.

BACKGROUND: Fabry disease is a rare X-linked disorder caused by deficient activity of the lysosomal enzyme alpha-galactosidase A. Progressive accumulation of the substrate globotriaosylceramide in cells throughout the body leads to major organ failure and premature death. The Fabry Outcome Survey (FOS) is a European outcomes database which was established to collect data on the natural history of this little-known disease and to monitor the long-term efficacy and safety of enzyme replacement therapy (ERT) with agalsidase alfa. This paper presents the first analysis of the FOS database on the effects of ERT on renal function, heart size, pain and quality of life. DESIGN: The effects of 1 and 2 years of ERT with agalsidase alfa on renal function (assessed by estimated glomerular filtration rate), heart size (assessed by echocardiography), pain (assessed by the Brief Pain Inventory) and quality of life (assessed by the European Quality of Life Questionnaire EQ-5D) were analyzed in a cohort of 545 patients, 314 of whom were receiving treatment (188 for at least 12 months and 92 for at least 24 months; mean duration of treatment, 17 months; maximum duration, 56 months). RESULTS: Treatment with agalsidase alfa stabilized renal function in patients with a mild or moderate deterioration in renal function at baseline, reduced left ventricular size in patients who had an enlarged heart at baseline, and improved pain scores and quality of life. These improvements were similar in hemizygous men and heterozygous women with Fabry disease. CONCLUSIONS: Enzyme replacement therapy with agalsidase alfa leads to significant clinical benefits in patients with Fabry disease, and treatment is likely to alter the natural history of this disorder.