39 research outputs found

    The epidemiology of kuru: monitoring the epidemic from its peak to its end

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    Kuru is a fatal transmissible spongiform encephalopathy restricted to the Fore people and their neighbours in a remote region of the Eastern Highlands of Papua New Guinea. When first investigated in 1957 it was found to be present in epidemic proportions, with approximately 1000 deaths in the first 5 years, 1957–1961. The changing epidemiological patterns and other significant findings such as the transmissibility of kuru are described in their historical progression. Monitoring the progress of the epidemic has been carried out by epidemiological surveillance in the field for 50 years. From its peak, the number of deaths from kuru declined to 2 in the last 5 years, indicating that the epidemic is approaching its end. The mode of transmission of the prion agent of kuru was the local mortuary practice of transumption. The prohibition of this practice in the 1950s led to the decline in the epidemic, which has been prolonged into the present century by incubation periods that may exceed 50 years. Currently, the epidemiological surveillance is being maintained and further studies on human genetics and the past mortuary practices are being conducted in the kuru-affected region and in communities beyond it

    Thiamin mono- and pyrophosphatase activities from brain homogenate of Guamanian amyotrophic lateral sclerosis and parkinsonism-dementia patients.

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    Thiamin-pyrophosphatase (TPPase) and thiamin-monophosphatase (TMPase) were determined using a spectrophotometric method at various pH values (5.5, 7.5, and 9.0) in brain tissue obtained at autopsy from amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD) patients from Guam and from Guamanian patients who died from other diseases (controls). TPPase separation by thin-layer polyacrylamide gel isoelectric focusing (IEF) was also performed using both gray and white matter. TPPase content, chemically determined at pH 9.0, was found to be significantly reduced in the frontal cortex of ALS and PD patients compared to controls. TMPase content, on the contrary, was unchanged. IEF analysis showed 9 clear-cut bands with TPPase activity in the pH range 5.4-7.2 and a broad band at pH 4.7-5.2. The enzymatic activity was higher in gray than in white matter. In one patient the pattern was clearly different, with two additional bands observed at pH 7.1 and 6.7, and thought to be due to genetic microheterogeneity

    Epidemic hemorrhagic fever with renal syndrome in Yugoslavia, 1986

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    An epidemic of hemorrhagic fever with renal syndrome (HFRS) occurred in Yugoslavia May-November 1986; all Republics and Provinces were involved. Serum samples were received from 260 of 267 persons with symptoms clinically compatible with a diagnosis of HFRS. Presumptive infection with a hantavirus was determined serologically for 161 of these. Many patients with serious clinical pictures, including severe renal insufficiency and shock, were hospitalized; 11 died. Indirect fluorescent antibody tests with antigens of 4 hantaviruses (Hantaan, Fojnica, Puumala, and the Vranica strain of Puumala virus) showed that gt 1 serotype was circulating during this epidemic. Hantavirus antigens were detected in the lungs of 86 of 302 (28.5%) wild-caught small mammals
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