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ABSTRACT Objective: To describe causes of death and mortality data related to cystic fibrosis (CF) using a multiple-cause-of-death methodology. Methods: Annual mortality data for the 1999-2017 period were extracted from the Brazilian National Ministry of Health Mortality Database. All death certificates in which category E84 (CF) of the ICD-10, was listed as an underlying or associated cause of death were selected. Epidemiological and clinical data were described, and standardized mortality rates were calculated per year and for the 2000-2017 period. A joinpoint regression analysis was performed to detect changes in the mortality rates during the study period. Results: Overall, 2,854 CF-related deaths were identified during the study period, ranging from 68 in 1999 to 289 in 2017. CF was the underlying cause of death in 83.5% of the death certificates. A continuous upward trend in the death rates was observed, with a significant annual percent change of 6.84% (5.3-8.4%) among males and 7.50% (6.6-8.4%) among females. The median age at death increased from 7.5 years in 1999 to 56.5 years in 2017. Diseases of the respiratory system accounted for 77% of the associated causes in the death certificates that reported CF as the underlying cause of death. Conclusions: A significant and continuous increase in CF-related death rates was found in Brazil in the last years, as well as a concurrent increase in the median age at death.

RESUMO Objetivo: Descrever as causas de morte e dados sobre mortalidade relacionada à fibrose cística (FC) por meio da metodologia de causas múltiplas de morte. Métodos: Dados sobre a mortalidade anual no período de 1999 a 2017 foram extraídos do Sistema de Informações sobre Mortalidade do Ministério da Saúde do Brasil. Foram selecionadas todas as declarações de óbito em que a categoria E84 (FC) da CID-10 foi citada como causa básica ou associada de morte. Foram descritos os dados epidemiológicos e clínicos e calculadas as taxas padronizadas de mortalidade por ano e para o período de 2000 a 2017. Foi realizada a análise de regressão por pontos de inflexão para detectar mudanças nas taxas de mortalidade durante o período estudado. Resultados: No total, foram identificadas 2.854 mortes relacionadas à FC durante o período de estudo: de 68 em 1999 a 289 em 2017. A FC foi a causa básica de morte em 83,5% das declarações de óbito. Observou-se uma tendência contínua de aumento das taxas de mortalidade, com variação percentual anual significativa de 6,84% (5,3-8,4%) nos homens e de 7,50% (6,6-8,4%) nas mulheres. A mediana da idade de óbito aumentou de 7,5 anos em 1999 para 56,5 anos em 2017. As doenças do aparelho respiratório representaram 77% das causas associadas nas declarações de óbito em que a FC foi a causa básica de morte. Conclusões: Observou-se no Brasil um aumento significativo e contínuo das taxas de mortalidade relacionada à FC nos últimos anos, bem como um aumento concomitante da mediana da idade de óbito.

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RESUMEN Fundamento: aunque la enfermedad hepática crónica como complicación de la fibrosis quística ha sido eclipsada por otros signos y síntomas más obvios a nivel respiratorio y pancreático, constituye la segunda causa de muerte en los pacientes con fibrosis quística. Objetivo: presentar el caso de una transicional de tres años con hepatomegalia y pruebas analíticas hepáticas alteradas como manifestaciones iniciales de la fibrosis quística. Caso clínico: paciente de tres años, femenina, con historia de distensión abdominal, hepatomegalia y desnutrición. Por esta sintomatología es remitida a la consulta de Gastroenterología. Conclusiones: se debe sospechar la fibrosis quística en pacientes pediátricos con hepatopatía crónica. Se observó mejoría clínica de las manifestaciones respiratorias y del estado nutricional, sin embargo, la hepatopatía evolucionó a la cirrosis hepática establecida.

ABSTRACT Background: although the chronic hepatic illness as complication of the cystic fibrosis has been eclipsed by other signs and more obvious symptoms at breathing and pancreatic level, is the second cause of death in the patients with cystic fibrosis. Objective: to present the case of a three year-old transitional one with hepatomegaly and hepatic analytic tests altered as initial manifestations of the cystic fibrosis. Clinical case: a three-year old, female patient with history of abdominal distension, hepatomegaly, and malnutrition. For this symptoms, she is remitted to the Gastroenterology consultation. Conclusions: the cystic fibrosis should be suspected in pediatric patient with chronic hepatic diseases. Clinical improvement of the breathing manifestations was observed and of the nutritional state, however the chronic hepatic disease evolved to the established hepatic cirrhosis.

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INTRODUCTION: Cystic fibrosis (CF) is a life-limiting multisystemic genetic disease. Patients with CF have a high rate of hospitalization. We attempt to ascertain national trends of inpatient stays, prevalence of various co-morbidities during hospitalizations, outcomes and discharge disposition among CF patients. MATERIAL AND METHODS: Data from the National Inpatient Sample (NIS) was used to identify all hospitalizations of patients with CF and their demographic characteristics from 2003 to 2013. Prevalence and effects of various co-morbidities like acute kidney injury (AKI) were determined. Detailed sub-group analysis was performed for individuals with lung transplant. RESULTS: The annual rate of hospitalization per 1,000 CF patients in the U.S. increased from 994 in 2003 to 1,072 in 2013. The overall in-hospital mortality was 1.5%; median age at death was 27 years. In-hospital mortality trended down from 1.9% to 1.2% from 2003 to 2013 (p-value for trend: 0.002). The median length of stay was 7 days. The prevalence of chronic liver disease and AKI was 3.7% and 3.8% respectively. Multivariate adjusted odds of mortality for AKI was 1.74 (95% CI 1.57-1.93, p < 0.001). Patients with prior lung transplantation accounted for 6.5% of hospitalizations. These patients had a significantly higher prevalence of AKI. CONCLUSIONS: The annual hospitalization rates of CF patients is increasing over the years. Females with CF constitute a higher proportion of hospitalized patients despite a higher male preponderance of males with CF in the community. AKI is associated with a significantly higher in-hospital mortality. Lung transplant recipients have a higher prevalence of AKI and mortality.