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INTRODUCCIÓN. Las alteraciones hereditarias de la hemostasia son patologías raras, dentro de estas se encuentran: Hemofilia A, Hemofilia B y von Willebrand. La hemofilia es un trastorno hereditario, ligado al cromosoma X, causado por ausencia o actividad reducida del factor VIII o IX. La enfermedad de von Willebrand es causada por la deficiencia del factor VIII. OBJETIVO. Determinar el perfil demográfico y epidemiológico de pacientes con Hemofilia y von Willebrand. MATERIALES Y MÉTODOS. Estudio observacional, descriptivo, transversal. La población de estudio fueron 133719 con una muestra de 144 pacientes, los criterios de inclusión fueron: pacientes de ambos sexos entre 2 a 88 años de edad, con diagnóstico de Hemofilia A, B, von Willebrand. Atendidos en la consulta externa del Área de Estomatología del Hospital de Especialidades Carlos Andrade Marín, en el periodo 2015-2018. Datos obtenidos del sistema AS400, analizados en el programa International Business Machines Statistical Package for the Social Sciences, Versión 22.0. RESULTADOS. El 77,0% (111; 144) perteneció al género masculino. El rango de edad fue entre 23 y 33 años con 24,0% (34; 144). Tuvieron Hemofilia A 62,0% (93; 144); Hemofilia B 6,0% (9; 144); von Willerbrand 28,0% (42; 144). El 50,0% (77; 144) recibieron tratamientos odontológicos; preventivos 15,0% (21; 144) y curativos 13,0% (18; 144); siendo la mayor patología caries dental. CONCLUSIÓN. Se determinó el perfil demográfico y epidemiológico de los pacientes con Hemofilia y von Willebrand que permitió brindar un tratamiento integral, interdisciplinario y oportuno.
INTRODUCTION. Hereditary abnormalities of hemostasis are rare pathologies, within these are: Hemophilia A, Hemophilia B and von Willebrand. Hemophilia is an inherited disorder, linked to the X chromosome, caused by absence or reduced activity of factor VIII or IX. Von Willebrand's disease is caused by factor VIII deficiency. OBJECTIVE. Determine the demographic and epidemiological profile of patients with hemophilia and von Willebrand. MATERIALS AND METHODS. Observational, descriptive, cross-sectional study. The study population was 133719 with a sample of 144 patients, the inclusion criteria were: patients of both sexes between 2 and 88 years of age, with a diagnosis of Hemophilia A, B, von Willebrand. Attended in the external consultation of the Stomatology Area of the Carlos Andrade Marín Specialty Hospital, in the period 2015-2018. Data obtained from the AS400 system, analyzed in the International Business Machines Statistical Package for the Social Sciences program, Version 22.0. RESULTS 77,0% (111; 144) belonged to the male gender. The age range was between 23 and 33 years with 24,0% (34; 144). They had hemophilia at 62,0% (93; 144); Hemophilia B 6,0% (9; 144); von Willerbrand 28,0% (42; 144). 50,0% (77; 144) received dental treatments; preventive 15,0% (21; 144) and curative 13,0% (18; 144); being the biggest dental caries pathology. CONCLUSION. The demographic and epidemiological profile of patients with Hemophilia and von Willebrand was determined, which allowed to provide a comprehensive, interdisciplinary and timely treatment.
Subject(s)
Humans , Male , Female , von Willebrand Diseases , Preventive Dentistry , Hemophilia B , Dental Care for Chronically Ill , Factor XI Deficiency , Hemophilia A , X Chromosome , Platelet Adhesiveness , HemostasisABSTRACT
Resumen: Los pacientes crónicos críticamente enfermos mantienen un ambiente inflamatorio persistente, inmunidad reducida y consumo progresivo de reservas fisiológicas. Se caracterizan por ingresos hospitalarios con estadías más prolongadas, mayor mortalidad y costos. Objetivo general: Describir las características del síndrome de inflamación, inmunosupresión y catabolismo persistente en pacientes críticos crónicamente enfermos hospitalizados en la Unidad de Cuidados Intensivos del Hospital General La Villa. Con el fin de conocer los aspectos epidemiológicos. Material y métodos: Estudio clínico prospectivo, descriptivo y observacional, analítico. Se ingresaron 25 pacientes con criterios de PICS acorde a la escala NUTRIC y cuadro clínico de enero-abril de 2018. Se realizaron pruebas de estadística descriptiva para variables cualitativas y para cuantitativas se utilizaron medidas de tendencia central. Pruebas inferenciales para contrastar variables cualitativas y para cuantitativas tablas de 2 x 2 para el cálculo de la odds ratio. Resultados: 25 pacientes cumplieron criterios de PICS, incidencia de 37.9%, la distribución por sexos fue 60% hombres y 40% mujeres. Edad promedio fue 48.8. Los diagnósticos más comunes fueron choque hipovolémico (28%), choque séptico (16%), 68% de los pacientes tenían un riesgo nutricional bajo y 32% riesgo alto, 84% de los pacientes requirieron algún tipo de soporte, el más común fue ventilación mecánica, Las complicaciones más comunes fueron las infecciosas (80%) principalmente NAVM. La mortalidad fue de 20%. Conclusiones: Los resultados en este estudio no difieren de los reportados en la bibliografía a nivel internacional, el síndrome de PICS se presenta en pacientes con mayor edad, mayor estancia hospitalaria y carencias nutricionales superiores en quienes las reservas biológicas no son suficientes para evitar la inmunosupresión que conlleva y genera el círculo vicioso que finalmente conduce a la muerte no sólo dentro de la UCI.
Abstract: Critically ill chronic patients maintain a persistent inflammatory environment, reduced immunity and progressive consumption of physiological reserves. They are characterized by hospital admissions with longer stays, higher mortality and costs. General objective: To describe the characteristics of the syndrome of inflammation, immunosuppression and persistent catabolism in critically ill critically ill patients hospitalized in the Intensive Care Unit of the La Villa General Hospital. In order to know the epidemiological aspects. Material and methods: Prospective, descriptive and observational clinical, analytical study. Patients were admitted with PICS criteria according to the NUTRIC score and clinical features from January-April 2018. Descriptive statistics tests were performed for qualitative variables, quantitative measures were used for quantitative central. Inferential tests to compare qualitative variables and for quantitative 2 x 2 tables for calculating the odds ratio. Results: 25 patients met criteria of PICS, incidence of 37.9%, the distribution by sex was 60% men and 40% women. Average age was 48.8. The most common diagnoses hypovolemic shock (28%), septic shock (16%), 68% of patients had a low nutritional risk and 32% high risk, 84% of patients required some kind of support the most common was ventilation mechanical, the most common complications were infectious (80%) mainly VAP. The mortality was 20%. Conclusions: The results in this study do not differ from those reported in the literature at the international level, the syndrome of PICS is presented in patients with older age, longer hospital stay and higher nutritional deficiencies in which biological reserves are not sufficient to avoid immunosuppression that leads to and generates the vicious circle that ultimately leads to death not only within the ICU.
Resumo: Os pacientes crônicos em estado crítico mantêm um ambiente inflamatório persistente, imunidade reduzida e consumo progressivo de reservas fisiológicas. Eles são caracterizados por internações hospitalares com estadias prolongadas, maior mortalidade e custos. Objetivo geral: Descrever as características da síndrome de inflamação, imunossupressão e catabolismo persistente em pacientes críticos cônicos internados na Unidade de Terapia Intensiva do Hospital Geral La Villa, com a finalidade de conhecer os aspectos epidemiológicos. Métodos: Estudo clínico prospectivo, analítico, descritivo e observacional. Foram admitidos 25 pacientes com critérios da PICS segundo a escala NUTRIC e quadro clínico de janeiro a abril de 2018. Realizou-se testes estatísticos descritivos para variáveis qualitativas e medidas de tendência central foram utilizadas para variáveis quantitativas. Testes inferenciais para comparar variáveis qualitativas e para quantitativas tabelas 2 x 2 para calcular o Odds Ratio. Resultados: 25 pacientes preencheram os critérios da PICS, incidência de 37.9%. A distribuição por sexo foi de 60% homens e 40% mulheres sendo que a idade média foi de 48.8 anos. Os diagnósticos mais comuns foram: choque hipovolêmico (28%) e choque séptico (16%) sendo que 68% dos pacientes apresentaram baixo risco nutricional e 32% risco elevado. 84% dos pacientes necessitaram de algum tipo de suporte sendo o mais comum a ventilação mecânica. As complicações mais comuns foram infecciosas (80%) principalmente a PAVM. A mortalidade foi de 20%. Conclusões: Os resultados deste estudo não diferem dos relatados na literatura a nível internacional. A síndrome PICS ocorre em pacientes com idade avançada, maior tempo de internação e deficiências nutricionais graves em que as reservas biológicas não são suficientes para evitar imunossupressão. Isso implica e gera o círculo vicioso que finalmente leva à morte não apenas dentro da UTI.
ABSTRACT
A study of the attitudes of 507 patients with chronic illnesses and 961 personnel working with these patients towards a recreational activity service was performed using a questionnaire. Most of them agreed that the hospital should arrange recreational activity for patients with chronic illnesses in addition to physical treatment (95.6, 99.0 percent of patient and personnel groups respectively). Both groups evaluated the benefit of recreational activity with a score of 7.93 ฑ 2.09 and 8.71 ฑ 1.42. Activities ranked first were reading books, watching television or a video, gardening and singing/listening to music. Concerning health professionals willingness to join these activities, 80% of the nurse group (registered nurses and practical nurses) were willing to do so whereas only 51.4 percent of the doctor group were available due to different working styles. Most of the personnel accepted that the recreational therapist should be the one who arranges this service. Both groups agreed that a recreational activity service should be free of charge (45.2-51.4 percent). From the results of this study, we can conclude that patients and personnel appreciate a recreational activity service for chronically ill patients, so we should develop and distribute this service to patients in order to improve their quality of life and the quality of our service.