Reseña histórica de la fibrosis quística y su estudio y tratamiento en Cuba / Historical review of cystic fibrosis and study and treatment in Cuba

Se revisan los aspectos históricos, la fisiopatología, diagnóstico y tratamiento actualizado de la fibrosis quística, así como su magnitud en el mundo y en Cuba. Se plantean los aspectos relacionados con la creación en 1974 de la Comisión Cubana de Fibrosis Quística por el profesor Manuel Rojo Concepción y colaboradores, el trabajo de esta en estos 40 años y los retos actuales y futuros.

The historical aspects, the physiopathology, diagnosis and treatment of cystic fibrosis up to the present as well as its scope in Cuba and worldwide was reviewed in this article. The creation of the Cuban Commission for Cystic Fibrosis in 1974 by Professor Manuel Rojo Concepción and colleagues, the work of this group during 40 years and the present and future challenges were also presented.

Federico Chopin (1810-1849) y su enfermedad / Frederic Chopin (1810-1849), and his disease

Frédéric Chopin - a great Polish composer and pianist-suffered from a chronic disease. Both during his life and after his death, physicians disagreed on Chopin's diagnosis. His contemporaries accepted the diagnosis of tuberculosis, a common disease in the 18th century. Description of new clinical entities provoked new dilemmas in the 21th century. Although other alternative diagnoses to tuberculosis have emerged, such as cystic fibrosis or alpha-1 antitrypsin deficiency, we still sustain that the first diagnosis is the most probable. In this paper we report F. Chopin's case history and discuss cons and pros for different diseases as the cause of F. Chopin's suffering and death.

A fibrose cística, o sistema de escores e a espirometria / Cystic fibrosis, scoring systems and spirometry

A fibrose cística, doença subdiagnosticada em nosso meio, tem despertado progressivo interesse entre os pediatras. Níveis crescentes de sobrevivência requerem agora a integração dos clínicos e pneumologistas de adultos. Escores clínico e radiológicos demonstram sua utilidade e reprodutibilidade no tratamento e avaliação do paciente. Adicionalmente, muitos autores demonstraram correlações fortes entre esses escores e alguns parâmetros espiro- métricos. O coeficiente de correlação de Pearson, p.ex., pode alcançar até 0,86 quando a capacidade vital (% do previsto) é correlacionada com o escore de Shwachman-Kulczycki (SK). Isso significa que em Serviços de menor porte, onde o espirômetro não é disponível e a critério clínico, avaliações seriadas do escore de SK reduziriam a necessidade de exames espirométricos. Entretanto, estudos clínico-radiológicos e espirometria são complementares. Esta atualização apresenta e discute as correlações entre os escores clínico-radiológicos mais usados e a espirometria na fibrose cística.

Cystic fibrosis, an underdiagnosed disease in Brazil, has nowadays caused progressive interest among pediatricians. Increasing survival rates require the copartnership of the general practicians and adult pulmonologists from now on. Clinical and radiological scores have frequently been used to assess the severity of this disease and have demonstrated their reliability and usefulness in patient care. In addition to this, many authors demonstrated dose relationship between these scores and selected spirometric parameters. Pearson's correlation coeficient, for example, can reach.values up to 0.86 for the predicted value of vital capacity when correlated with Shwachman¬Kulczycki (SK) score. This means that walk-in clinics where spirometer is not available and under judicious clinical evaluation, serial measurements of SK score could reduce the number of spirometric assessments leading to a reduction of health costs. However, both methods are complementary. This update aims on presenting and discussing the correlations between the most used clinical scores and spirometry in cystic fibrosis.

Fibrosis quística / Quística Fibrosis

La Fibrosis Quística (FQ) es una enfermedad hereditaria letal con baja incidencia a nivel mundial; caracterizada por una alteración en la conducción del cloro, trayendo dentro de sus principales consecuencias infecciones respiratorias graves e insufiencia pancreática entre las principales. Esta rara patología debe ser diagnosticada de manera temprana y así instaurar un tratamiento antibiótico agresivo mas medidas de soporte, con la finalidad de mejorar la calidad de vida del paciente. A continuación presentamos el caso de una mujer de 25 años portadora de FQ.

Atuaçäo primária da fisioterapia na fibrose cística infantil / Primary actuation of physioterapy on cystic fibrosis infantile

O seguinte tema foi escolhido para pesquisa, uma vez que, de acordo com o desenvolvimento da doença em questäo, inúmeras säo as complicaçöes. Entre as mais evidentes, a pulmonar. A partir daí vimos a importância e a necessidade de um Fisioterapeuta como membro indispensável na reabilitaçäo desses pacientes