title: [[35 Years of effort to improve the diagnosis of porphyria].
abstract: World history of porphyria is given in brief. The activities of Porphyria Center during period of 25 years have shown how difficult it was to introduce diagnostic measures for this rare and very little known group of diseases. The obstacles encountered during the popularization of the knowledge of porphyria were: minimal information on porphyria, very seldom seen in Poland, therefore rather negative approach of the doctors to these patients,--almost never porphyria was seen in differential diagnostics of abdominal pains or polyneuropathies, herefore diagnosis, if made, was very late, often just before death. From the other side insufficient support for doctors was given by laboratories because of the lack of simple diagnostic tests. Along the 35 years of the work in the Institute of Haematology and Transfusiology the situation was steadily improving but slowly due to technical and economical difficulties. The Center was organized, now well equipped and able to diagnose and differentiate all types of porphyrias. A computerised basis of the collected material consist of 383 families (6000 persons registered). Among them there are nearly 600 acute (who have passed one or more attacks of porphyria) and about 1000 latent cases of porphyria. The mortality which was 52% in the first collected group of patients (1960-1970) has fallen to less than 10% concerning very severe and late diagnosed attacks. In 1996-1998 there were only two deaths during attacks of porphyria in Poland.
authors.affiliation: Poradni i Pracowni Badań nad Porfiria Instytutu Hematologii i Transfuzjologii.
authors.forename: E
authors.initials: E
authors.lastname: Kostrzewska
authors.forename: A
authors.initials: A
authors.lastname: Gregor
mesh_terms: D006405:Hematology / Q000266:history; D049673:History, 20th Century; D006801:Humans; D011044:Poland / Q000453:epidemiology; D011164:Porphyrias / Q000175:diagnosis* / Q000266:history / Q000401:mortality; D015996:Survival Rate