Muscle weakness: Understanding the principles of myopathy and neuropathy in the critically ill patient and the management options.

Myo-neuropathy of the critically ill patient is a difficult nosological entity to understand and manage. It appears soon after injury, and it is estimated that 20-30% of patients admitted to Intensive Care Units will develop it in some degree. Although muscular and nervous involvement are related, the former has a better prognosis.

[Seventh Jesús Culebras Lecture. Systemic inflammatory response and multi organic dysfunction/failure following aggression: metabolic implications].

Following any aggression, the body starts an infl ammatory response, mediated by humoral and cellular factors, intended to limit the process and eventually to heal. In some cases, either due to the intensity or the duration of the aggression or due to an inadequate response of the host, secondary to genetic polymorphisms, malnutrition or other causes, a state of hyper activation of infl ammatory cells is originated, with liberation of immature cells and activation of monocytes and macrophages, which liberate very powerful pro infl ammatory mediators that induce a state of generalized systemic infl ammation. Many processes may originate this infl ammatory response, (sepsis, trauma, burns, pancreatitis, etc.

[Results of a national survey on transfusion practice in Intensive Care Units].

Introduction: Blood derivatives are clinical products that are currently used, for which their lack of availability, clinical relevance and presence of associated side effect that make it necessary to known and evaluate their utility rigorously are characteristic.

Objective: To analyze knowledge of attitudes, knowledge and behavior on transfusional policy in the different Spanish Intensive Care Units (ICU).

Design: A mail-based survey (electronic and conventional) in the ICUs.

Protein energy malnutrition (PEM) in cancer patients.

Introduction: Cancer patients show protein energy malnutrition (PEM) throughout the evolution of the disease. The main objective of this work is to find out the prevalence of PEM in the studied sample, as well as how to assess the nutritional state of patients.

Material And Methods: Non-interventionist, longitudinal and prospective study.

[Recommendations for the nutritional assessment of critically ill patients].

Undernutrition affects 30 to 50% of hospitalized patients. The identification of patients with undernutrition or in risk to become undernourished is of utmost importance to prescribe a timely nutritional support. This article performs a critical review of anthropometrical, biochemical and functional parameters used for nutritional assessment.

[Fabry disease in Spain: first analysis of the response to enzyme replacement therapy].

Background And Objective: Fabry disease is a X-linked lysosomal disorder caused by a deficient activity of the enzyme alfa-galactosidase A. Lack of enzyme activity results in progressive accumulation of globotriaosylceramide (Gb3) leading to multiorgan dysfunction and early death. Enzyme replacement therapy (ERT) has recently become available and the database Fabry Outcome Survey (FOS) of Spain gives us the opportunity to asses the efficacy of this therapy.

[Fabry's disease in Spain. Study of 24 cases].

Background And Objective: Fabry's disease is a rare metabolic disorder inherited as an X-linked trait resulting from a decreased activity of the alpha-galactosidase A lysosomal enzyme. This defect leads to an accumulation of unmetabolized neutral glycosphingolipids in all cell types. This condition displays a high rate of early mortality and affects both hemizygous males and heterozygous females.