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. Myo-neuropathy associates to more morbidity, longer stay in Intensive Care Unit and in hospital, and also to higher costs and mortality. It is considered part of the main determinants of the new entities: the Chronic Critical Patient and the Post Intensive Care Syndrome. This update focuses on aetiology, pathophysiology, diagnosis and strategies that can prevent, alleviate and/or improve muscle (or muscle-nerve) weakness.

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. Cancer patients from 103 researchers (6 specialties) from 65 hospitals of 15 Spanish Autonomous Communities. Results 561 patients were included in the study. The mean age was 62.6 years and 68.8% were men. The average basal body mass index (BMI) was 22.1 kg/m2. In 18.2% the BMI was low; 21.6% were overweight, pre-obese or obese; 22.9% had a localised tumour and 77.1% had an advanced one, first and foremost located in the head and neck, digestive system and lungs. In 72.7% of cases, it was treated with chemotherapy (CT) and in 38.3% with CT and radiotherapy (RT). 96.4% had nutrition problems: 70.9% (398/561) had anorexia, 34.8% (195/561) gastrointestinal pro blems, 32.6% (183/561) dysgeusia, 40.5% (227/561) dysphagia and others 8.6% (48/561). Weight loss occurred in 90.7% (an average of 4.2 months). CONCLUSION: If we analyse the BMI of patients, it can be seen that 60.2% have an adequate weight according to their size against 17.3% of patients who are overweight or preobese, and the remaining 4.3% are obese. Only 18.2% of patients are underweight. Over 90% have suffered recent weight loss.

Seventh Jesús Culebras Lecture. Systemic inflammatory response and multi organic dysfunction/failure following aggression: metabolic implications / Séptima Lección Jesús Culebras. Respuesta inflamatoria sistémica y disfunción/ fracaso multiorgánico tras una agresión: implicaciones metabólicas

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.) with activation of leukocyte, endothelial, coagulation and neuroendocrine response systems, generating a complex of mediators (cytokines, adhesion molecules, growth factors, etc.). Clinically, the response is characterized by infl ammation, anorexia, stillness, increase of vascular permeability, factors that originate edema and vasodilatation, which is followed by hypotension, tachycardia and increased cardiac output. On the other hand, the metabolic response to stress is part of the mechanism of adaptation, generated by the organism in order to survive the acute disease by means of increasing energetic substrates to vital tissues. As a result of this complex metabolic response, the control of substrate utilization is only partially regulated because, being impaired the mechanisms of energy supply, the organism seeks alternative substrates

Ante cualquier agresión, el organismo pone en marcha una respuesta inflamatoria, mediada por factores humorales y celulares, que tiende a limitar el proceso y a conseguir la curación. En algunos casos, ya sea por la intensidad o duración de la noxa, ya por una inadecuada respuesta del huésped secundaria a polimorfismos genéticos, malnutrición u otras causas, se produce un estado de hiperactivación de las células inflamatorias, con liberación de células inmaduras y activación de monocitos y macrófagos, que liberan poderosos mediadores proinflamatorios e inducen un estado de inflamación sistémica generalizada. Numerosos procesos pueden dar lugar a esta respuesta inflamatoria (sepsis, traumatismos, quemaduras, pancreatitis, etc.) con activación de los sistemas leucocitarios, endoteliales, de la coagulación y de la respuesta neuroendocrina, lo que genera un complejo entramado de mediadores (citocinas, moléculas de adhesión y factores de crecimiento, entre otros). Clínicamente, la respuesta se caracteriza por inflamación, anorexia, inmovilidad, aumento en la permeabilidad vascular que condiciona la aparición de edema, vasodilatación que se acompaña de hipotensión, taquicardia e incremento del gasto cardiaco. Por otro lado, la respuesta metabólica al estrés forma parte de ese mecanismo de adaptación, que el organismo genera para sobrevivir a la enfermedad aguda aumentando el aporte de sustratos energéticos a los tejidos vitales. Como resultado de esa compleja respuesta metabólica, el control de la utilización de los sustratos energéticos está solo parcialmente regulado por su disponibilidad, debido a que al estar alterados los mecanismos de producción energética, el organismo busca sustratos alternativos.

[Fabry disease in Spain: first analysis of the response to enzyme replacement therapy]. / Enfermedad de Fabry en España: primer análisis de la respuesta al tratamiento de sustitución enzimática.

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. Our objective is to describe the safety and the effects on renal, cardiac and neurological (pain) aspects of ERT with agalsidase alfa. PATIENTS AND METHOD: The effects of 1, 2, 3 and 4 years of ERT with agalsidase alfa on renal function (assessed by estimated glomerular filtration rate), proteinuria, heart size (assessed by echocardiography), arrhythmias, cardiac valvular anomalies and pain (assessed by the need of concomitant pain therapy) were analyzed in 33 patients under treatment. Safety of ERT was assessed by the reported infusion-related reactions in FOS. RESULTS: Overall, treatment with agalsidase alfa stabilized renal function, but the final result depends on the onset of ERT: there is a tendency to stabilization of renal function in those patients with mild deterioration of renal function, a tendency to improve in those patients with moderate deterioration and to worse in those with severe deterioration of renal function. Proteinuria and left ventricular heart size also estabilized under ERT, and pain improved. TSE infusion-related reactions occurred with an incidence of 0.7%. CONCLUSIONS: ERT with agalsidase alfa is safe and stabilized the abnormal clinical parameters observed in patients with Fabry disease.

[Fabry's disease in Spain. Study of 24 cases]. / Enfermedad de Fabry en España. Análisis de 24 casos.

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. We present data from Spanish patients admitted to the Fabry Outcome Survey (FOS), a multicenter European registry for patients suffering from Fabry's disease, at the time of their inclusion. PATIENTS AND METHOD: The study presents baseline data from 24 Spanish patients upon inclusion in the registry, with an analysis of their overall clinical characteristics and relationship to gender. RESULTS: The median age of onset of symptoms was 13 years, while it was 25.5 years for diagnosis. In 7 patients, the manifestations of the disease had been attributed to other processes. Predominant manifestations in males were angiokeratomas (86%), pain (71%), cardiovascular involvement (71%), kidney involvement (50%), digestive symptoms (43%) and ophthalmologic complications (43%). Among females, ophthalmologic complications were present in 50%, followed by cardiovascular involvement (40%); 30% of females had a painful clinical condition which is characteristic of the process. CONCLUSIONS: We present the clinical characteristics of the largest Spanish cohort of Fabry's disease sufferers reported so far. Due to the wide spectrum of clinical manifestations, their awareness allows an early diagnosis as well as the possibility of starting the specific therapy currently available.

[Results of a national survey on transfusion practice in Intensive Care Units]. / Resultados de una encuesta nacional sobre hábito transfusional en unidades de cuidados intensivos.

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. DURATION: The study was planned in 2005 and conducted during the year 2006. SETTING: Spanish ICUs. PATIENTS AND METHOD: A 27-question questionnaire. RESULTS: Most are middle-sized ICUs (10-20 beds), with predominantly medical activities. The staff member, alone or with the resident, generally decides the transfusion based on his/her experience, although with a tendency to follow the scientific guidelines. Generally, there is no transfusional committee. When red blood packs are transfused, generally between 2 to 4 units are used. The hemoglobin value is orientative, although the decision is clear if < 7 g/dl (10 g/dl if there is heart disease). Drug alternatives to transfusion are not generally used due to lack of evidence and price. In 50% of cases, the association between transfusion and increase in mortality is considered to be certain. CONCLUSIONS: Strong consideration must be made about transfusion and its over use should be avoided. For this purpose, educational guidelines and consensus meetings are necessary to establish recommendations on the use of blood products and their pharmacological alternatives.

[Recommendations for the nutritional assessment of critically ill patients]. / Recomendaciones para la valoración nutricional del paciente crítico.

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. Prognostic indexes and other parameters used for the assessment of nutritional risk are also discussed. Some tools are proposed for the diagnosis of hospital malnutrition.

Recomendaciones para la valoración nutricional del paciente crítico / Recommendations for the nutritional assessment of critically ill patients

Undernutrition affects 30 to 50 percent 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. Prognostic indexes and other parameters used for the assessment of nutritional risk are also discussed. Some tools are proposed for the diagnosis of hospital malnutrition.

El paciente anciano en situación crítica

Contenido: Características especiales que condicionan al anciano críticamente enfermo. Evolución y pronóstico del paciente anciano ingresado en la UCI. Traumatismos y quemaduras en la tercera y cuarta edad. Sedación, analgesia y delirio en el paciente anciano. Particularidades de los índices pronósticos y de isogravedad en los ancianos. Infección y sepsis en el anciano críticamente enfermo. Soporte nutricional especializado en los ancianos críticos