ABSTRACT
Este artigo propõe estudar os primeiros 12 anos de existência do Instituto de Radium de Minas Gerais, fundado em 1922. Sua atuação na luta contra o câncer no Brasil, ainda pouco conhecida, começa a ser esboçada pelo estudo de documentação institucional inédita. Através de um banco de dados elaborado com informações constantes em seu livro de registro de pacientes, foram feitos levantamentos estatísticos dos tipos de câncer e das formas de tratamento existentes entre 1923 e 1935. Esse livro faz parte de um conjunto de outros cinco recentemente descobertos no Centro de Memória da Medicina/UFMG. A documentação permite resgatar os primórdios das intervenções de radioterapia no país e acompanhar seu desenvolvimento e a influência exercida por esse hospital modelo.
This article proposes to study the first 12 years of the Minas Gerais Radium Institute, founded in 1922. Its work in the fight against cancer in Brazil, albeit still little known, is coming to light as its institutional documents are studied. A database has been prepared using information from its patient register, based on which statistical analyses have been done to identify the types of cancer and treatments available there between 1923 and 1935. This register is one of five recently unearthed at the Medicine Memory Center of the Universidade Federal de Minas Gerais. Through them, the earliest experiments in radiotherapy in Brazil can be reconstituted, and its development and the influence of this model hospital can be mapped out.
Subject(s)
Female , Humans , Male , Aspergillus nidulans/enzymology , Dioxygenases , Homogentisic Acid/analysis , Oxygenases/metabolism , Spectrophotometry/methods , Alkaptonuria/metabolism , Aspergillus nidulans/drug effects , Aspergillus nidulans/metabolism , Chromatography, High Pressure Liquid , Homogentisic Acid/metabolism , Homogentisic Acid/urine , Oxygenases/genetics , Phenylacetates/metabolism , Phenylacetates/pharmacology , Sensitivity and SpecificityABSTRACT
Alkaptonuria (AKU) is a very rare autosomal recessive disorder of tyrosine metabolism in the liver due to deficiency of homogentisate 1,2 dioxygenase (HGD) activity, resulting in the accumulation of homogentisic acid (HGA). Circulating HGA pass into various tissues through-out the body, mainly in cartilage and connective tissues, where its oxidation products polymerize and deposit as a melanin-like pigment. Gram quantities of HGA are excreted in the urine. AKU is a progressive disease and the three main features, according the chronology of appearance, are: darkening of the urine at birth, then ochronosis (blue-dark pigmentation of the connective tissue) clinically visible at around 30 yrs in the ear and eye, and finally a severe ochronotic arthropathy at around 50 yrs with spine and large joints involvements. Cardiovascular and renal complications have been described in numerous case report studies. A treatment now is available in the form of a drug nitisinone, which decreases the production of HGA. The enzymatic defect in AKU is caused by the homozygous or compound heterozygous mutations within the HGD gene. This disease has a very low prevalence (1:100,000-250,000) in most of the ethnic groups, except Slovakia and Dominican Republic, where the incidence has shown increase up to 1:19,000. This review highlights classical and recent findings on this very rare disease.
Subject(s)
Alkaptonuria/complications , Alkaptonuria/genetics , Alkaptonuria/metabolism , Alkaptonuria/therapy , Homogentisic Acid/metabolism , Humans , Melanins/biosynthesis , Ochronosis/complicationsABSTRACT
La alcaptonuria es una enfermedad rara, muy poco conocida en nuestro medio y que muchas veces se podria confundir con alguna enfermedad de tipo reumatico. En la literatura nacional no hay ningun informe sobre esta enfermedad, y ademas, no encontramos ninguna bibliografia sobre estudios gammagraficos en ocronosis, por ello queremos resaltar estas observaciones que creemos pertinentes por la forma completa como se estudio