Caso clínico: cuidados de enfermería a una paciente con enfermedad de Wilson / Clinical case: nursing care to a patient with Wilson's disease

La enfermedad de Wilson o degeneración hepatolenticular es un trastorno congénito de herencia autosómica recesiva del metabolismo del cobre (gen ATP7B) que produce una disminución de su excreción por vía biliar y, como consecuencia, un aumento de la concentración de cobre hepático y en tejidos extrahepáticos como cerebro, riñón y córnea, siendo este acúmulo el responsable de las manifestaciones clínicas. Al ser esta una enfermedad poco común y difícil de diagnosticar, dado su amplio espectro clínico, los facultativos han de sospechar su diagnóstico ante un paciente con hepatopatía de causa no clara. Cuando la detección de la enfermedad es precoz, el tratamiento es farmacológico, pero cuando la detección ocurre en fases avanzadas, o en individuos que no han respondido al tratamiento, la enfermedad puede evolucionar hacia un fallo hepático agudo grave, cuyo tratamiento definitivo es el trasplante hepático. En este artículo se presenta el caso clínico y el seguimiento de una paciente joven con hepatopatía no filiada y en la que se sospecha enfermedad de Wilson. Siguiendo el modelo conceptual de Virginia Henderson, se describen los problemas de colaboración y los diagnósticos de enfermería, presentando un plan de cuidados según la taxonomía NANDA (North American Nursing Asociación), NIC (Nursing Intervention Classification) y NOC (Nursing Outcomes Classification). En este caso, a la vez que se amplían conocimientos sobre la enfermedad, se establece un plan de cuidados que ayuda a la atención individualizada por parte de los profesionales a los pacientes afectos de dicha enfermedad (AU)

Wilson's disease or hepatolenticular degeneration is a congenital autosomal recessive disorder of cooper metabolism (ATP7B gene) which causes a decrease in biliary excretion and consequently, an increase in cooper concentration in hepatic and extrahepatic tissues such as brain, kidney and cornea. This accumulation is responsible for the clinical manifestations. Since this is a rare and difficult disease to diagnose given its wide clinical spectrum, doctors suspect this diagnosis on patients with liver disease of unclear cause. When detection of disease is early, treatment is pharmacological, but when detection occurs in advanced stages, or in individuals who have not responded to treatment, the disease can develop into an acute liver failure, whose definitive treatment is liver transplantation. In this article we present the clinical case and tracking of a young patient with liver disease of unknown origin, which is suspected of having Wilson's disease. Following the conceptual model of Virginia Henderson, collaboration problems and nursing diagnoses are described, presenting a care plan according to the NANDA taxonomy (North American Nursing Association), NIC (Nursing Intervention Classification), and NOC (Nursing Outcomes Classification). In this case, while disease knowledge expands, a care plan that helps individualized attention from professionals to patients affected by the disease is established (AU)

Host and environmental factors influencing respiratory secretion of pro-wheezing biomarkers in preterm children.

Cytokines are actively secreted by the respiratory mucosa of preterm children and participate in the pathogenesis of wheezing. This study aimed to identify the factors that could potentially influence respiratory secretion of cytokines in these children. A nasopharyngeal aspirate (NPA) was collected from 77 preterm children 1 yr after birth. NPAs from 14 healthy, 1-yr-old term children were collected in parallel. 27 cytokines were measured in the NPAs using a multiplex assay. Multivariate stepwise regression analysis with Bonferroni correction evidenced that the variable [daycare attendance] was associated with higher levels of [monocyte chemoattractant protein-1 (MCP-1), IL-6, vascular endothelial growth factor (VEGF), IL-1ß, IL-10, tumor necrosis factor (TNF)-α]; [male sex] with higher levels of (MCP-1, VEGF, and IL-1ß); [smokers at home] was associated with higher levels of MCP-1 (p < 0.0013). In turn, [prophylaxis with palivizumab] was associated with lower levels of (IL-6, IL-7) (p < 0.0013). All these mediators participate in the pathogenesis of asthma and recurrent wheezing. Preterm children secreted higher levels of chemokines (interferon-gamma inducible protein-10, macrophage inflammatory protein-1α, Eotaxin, MCP-1), growth factors (platelet-derived growth factor-bb, VEGF, fibroblast growth factor-basic, granulocyte macrophage colony-stimulating factor), Th1 (IL12, interferon-γ), Th2 (IL-9, IL-13), Th17 (IL-6, IL-17) cytokines, and immunomodulatory mediators (IL1RA and granulocyte colony-stimulating factor) than term children. In conclusion, we have identified for the first time a group of individual and environmental factors influencing respiratory secretion of cytokines in preterm children at the long term after birth. To know these factors could help to prevent the instauration of conditions linked to the appearance of chronic respiratory diseases such as wheezing or asthma.