Use of CFTR Modulators for Cystic Fibrosis in a Patient with Liver Transplant and ESRD on Hemodialysis.

Cystic fibrosis is an autosomal recessive disorder caused by mutations of the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most recent therapeutic approach to cystic fibrosis aims to correct structural and functional abnormalities of CFTR protein. CFTR modulators including ivacaftor-tezacaftor-elexacaftor are used in patients with F508del mutation, with clinical improvement. To date, there are no experiences of CFTR modulator therapy in cystic fibrosis patients with organ transplantation and severe renal impairment. We report the case of a patient diagnosed with cystic fibrosis with F508del mutation, who underwent liver transplantation at the age of 19 and started hemodialysis at the age of 24 due to end-stage renal disease secondary to membranous glomerulonephritis. She was treated with Kaftrio (ivacaftor-tezacaftor-elexacaftor) with clinical benefits on appetite, improvement of body mass index, and reduction of pulmonary exacerbations. A reduction of dosage to 75% of the standard dose was required due to alterations of the liver function. Conclusions. Use of CFTR modulators in patient with cystic fibrosis, liver transplant and end-stage renal disease could be considered safe but a clinical and laboratoristic monitoring of hepatic function is needed.

[Acute kidney injury in severely burned patient: prevention and treatment].

Acute Kidney Injury (AKI) is associated with a great increase in morbidity and mortality in severely burned patients and occurs as a complication in more than 25% of these cases. The onset of ARF may be early or late. Early AKI depends mainly on reduced cardiac output resulting from fluid loss, rhabdomyolysis, or hemolysis. Late AKI, instead, is usually a consequence of sepsis and is often associated with multiorgan failure (MOF). The first sign of AKI is the contraction of diuresis despite adequate volemic filling, which is followed by elevation of serum urea and creatinine. Fluid therapy is the main treatment in the burned victim: in the first few hours after injury, it aims to avoid hypovolemic shock and the possible related MOF, while later it becomes the cornerstone of treatment, besides antibiotic therapy in the case of sepsis onset. Particular care must also be taken in the choice of administered drugs in order to avoid possible nephrotoxic damage in addition to burning injury. Hemodialytic renal replacement therapy is used both for water balance management in patients requiring massive fluid infusions and for blood purification purposes to control the metabolic state, acid-base balance, and electrolytes abnormality. Our team has been collaborating for over 25 years in the management of severely burned patients admitted to the Centro Grandi Ustionati at the Bufalini Hospital in Cesena.

Construção e implantação de diretrizes para os acidentes de trabalho com o material biológico / Construction and implementation of guidelines for work accidents with biological material

Os trabalhadores da área da saúde se expõem diariamente a vários riscos no seu ambiente de trabalho, podendo ocasionar os acidentes de trabalho. Nesta pesquisa foi enfocado o acidente de trabalho com material biológico por apresentar o risco em contrair doenças transmissíveis que acarretam vários danos ao trabalhador. Esta pesquisa teve como objetivo geral estabelecer diretrizes frente à exposição biológica nos acidentes de trabalho, como objetivos específicos determinar o perfil dos acidentes de trabalho com exposição biológica; diagnosticar os problemas relacionados à exposição biológica; construir diretrizes para o enfrentamento dos problemas levantados; e, implementar as diretrizes no programa informatizado do referido cenário. Trata-se de uma pesquisa-ação de abordagem qualitativa, composta por 4 fases, delineada pelo método proposto por Thiollent, composta por amostra de onze membros da Comissão Gestora Multidisciplinar de Prevenção de Acidentes com Perfurocortantes. A coleta de dados ocorreu nas fases I e II da pesquisa-ação. Na fase I, foi realizado o diagnóstico dos acidentes de trabalho com material biológico com dados secundários fornecidos pelo Serviço de Epidemiologia do Hospital, dados retirados do SINAN referentes ao período de setembro de 2010 a setembro de 2013, bem como a realização de um seminário para a apresentação da pesquisa aos membros da referida comissão. Na fase II foram realizados 3 seminários de planejamento para a construção coletiva das diretrizes. Na fase III, fase de ação, foi realizada a divulgação das diretrizes, na comunidade hospitalar, por meio de reuniões, pelo correio eletrônico, pelo boletim informativo mensal e pela página do hospital na intranet. Na fase IV foi realizado o seminário de avaliação de todo o processo proposto. Os dados foram analisados segundo a técnica de análise de conteúdo, na modalidade temática de Bardin. A partir das categorias foi possível discutir sobre os problemas relacionados à exposição biológica, estruturar diretrizes e avaliar sua forma de aplicação, coletivamente. A pesquisa-ação possibilitou a compreensão do acidente de trabalho com material biológico e suas implicações, permitiu a construção do consenso das diretrizes, contribuindo para a produção de conhecimentos, confirmando a necessidade e a importância de mudanças nos processos de trabalho e o comprometimento da instituição na promoção da saúde do trabalhador. Conclui-se que são necessárias novas pesquisas, principalmente, relacionadas à avaliação das diretrizes e do conhecimento do trabalhador acerca do acidente com materiais biológicos.

Workers in the health field are exposed daily to various risks in their work environment, which may cause accidents at work, this research was focused on the accident involving biological material present risk contracting diseases that cause severe damage to the worker. This research aimed to establish opposite biological exposure guidelines in workplace accidents, specific objectives determine the profile of occupational accidents with biological exposure; diagnosing problems related to biological exposure; building guidelines for addressing the problems raised and implement guidelines on computerized program such a scenario. This is an action research qualitative approach, consisting of four phases outlined by the proposed Thiollent, the sample comprised of eleven members of the Management Committee Multidisciplinary Accident Prevention with Sharps method. Data collection occurred in phases, I and II of action research. In phase I, the diagnosis of occupational accidents with biological material with secondary data provided by the Department of Hospital Epidemiology, data from the Sinan for the period from September 2010 to September 2013 was conducted, as well as conducting a seminar for the presentation of research to members of the committee. In three Phase II planning seminars for the collective development of the guidelines were made. In phase III, the action phase release of the guidelines was held in the hospital community through meetings, by mail, the monthly newsletter and the hospital's intranet page. In phase IV the seminar evaluation of all proposed process was conducted. Data were analyzed using the technique of content analysis, the thematic Bardin. From the categories was possible to discuss the problems related to biological exposure and structure collectively, guidelines and assess your application form. The action research allowed the understanding of the accident with biological material and its implications, allowed the construction of consensus guidelines, contributing to the production of knowledge, confirming the need and the importance of changes in work processes and the commitment of the institution in promoting the health of workers. We conclude that the new research, especially related to the evaluation guidelines and the knowledge worker about the accident with biological materials is necessary.

Caracterização das vítimas e dos acidentes de trabalho com material biológico atendidas em um hospital público do Paraná, 2012 / Characterization of accidents at work with biological material and their victims treated at a public hospital in Paraná, Brazil, 2012

Objetivo: descrever as características dos acidentes de trabalho com material biológico atendidos em um hospital público do estado do Paraná, Brasil, no ano de 2012. Métodos: foi realizado estudo descritivo, utilizando-se registros das fichas de notificação de acidentes de trabalho com material biológico do Sistema de Informação de Agravos de Notificação (Sinan) relacionadas aos trabalhadores atendidos no hospital de referência em 2012. Resultados: dos 1.217 acidentes, 83,3 por cento ocorreram entre mulheres, 59,6 por cento na idade de 20 a 34 anos e 48,8 por cento entre profissionais de enfermagem; os tipos de exposição mais frequentes foram os percutâneos (65,7 por cento), em pele íntegra (20,5 por cento) e em mucosa (12,6 por cento); o material biológico mais frequentemente envolvido foi o sangue (78,9 por cento). Conclusão: as principais vítimas dos acidentes de trabalho com material biológico foram mulheres, adultos jovens e trabalhadores da equipe de enfermagem, destacando-se os acidentes envolvendo sangue.

Objective: to describe the characteristics of accidents with biological material and their victims treated at a public hospital in Paraná, Brazil, in 2012. Methods: a descriptive study was conducted using Notifiable Diseases Information System data on accidents at work with biological material and their victims treated at a referral hospital in the year 2012. Results: 1.217 accidents were identified. 83.3 per cent occurred among women, 59.6 per cent among those aged 20-34 years and 48.8 per cent among nursing professionals. The most frequent types of exposure were percutaneous (65.7 per cent) involving intact skin (20.5 per cent) and mucosa (12.6 per cent). Blood was the most frequently involved biological material (78.9 per cent). Conclusion: the main victims of accidents with biological material were women, young adults and nursing team workers. Most accidents involved blood.

Evidence of reverse mismatch with positron emission tomography imaging in a patient with reversible myocardial dysfunction.

We describe the case of a patient who came to our attention because of a reversible depression of myocardial contractility, probably due to myocarditis. A positron emission tomography study showed, in correspondence to the malfunctioning segments, a decreased F18-2-fluoro-2-deoxyglucose (F18-FDG) uptake in the presence of a normal perfusion as assessed by means of N13-labeled ammonia uptake. This phenomenon, called "reverse mismatch", shows that viability is not always dependent on FDG uptake and that it could be associated with the recovery of myocardial contractility. Some interpretations of the association between a reversible dysfunction and a reduced myocardial glucose metabolism are presented. The central role of nitric oxide and of cyclic guanosine monophosphate is hypothesized to explain both the mechanical and metabolic abnormalities.