Molybdenum cofactor deficiency associated with Dandy-Walker malformation.
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Biomedical subjects
Publications and source records attributed to M A Naranjo.
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We present a 13-year-old boy with a rapidly progressive glomerulonephritis and pulmonary haemorrhage with perinuclear anti-neutrophil cytoplasmic auto-antibodies (pANCA) corresponding to anti-myeloperoxidase antibodies. The diagnosis of microscopic polyarteritis was made on the basis of the clinical features, the positivity of pANCA, and the histological finding of a pauci-immune crescentic glomerulonephritis. He responded excellently to corticosteroids and cyclophosphamide therapy and complete clinical remission persists 1 year after withdrawal of treatment. We emphasize the usefulness of ANCA antibody assays to establish a prompt diagnosis and adequate treatment in systemic vasculitis in children.
A serological survey, involving indirect immunofluorescence testing of blood sera samples, was carried out on the residents of one in every five dwellings in the town of Barcelos (in the northern part of the State of Amazonas, on the right bank of the Rio Negro, 490 Km from Manaus by river) and on the rural populations of the villages of Piloto and Marará (also on the right bank of the Rio Negro, 30 minutes by boat from Barcelos). A total of 710 sera samples were tested, 628 from the resident population in the town of Barcelos, 35 from Piloto and 47 from Marará. The tests were carried out using human anti-gammaglobulin type IgG (Biolab) and antigen from formolized culture of T. cruzi Y strain. The sera were serially diluted from 1:40 to 1:320 in PBS 7.2. Of the 710 samples examined 89 (12.5%) were positive for anti-T.cruzi antibodies: 2 of these (2.2%) at a dilution of 1:320; 12 (13.4%) at 1:160; 38 (42.6%) at 1:80; and the remainder at 1:40, giving a median serological dilution of 1:80. The following questions are discussed: the high serological prevalence for Chagas' infection found in our survey; the possibility of serological cross-reactions; the need for confirmatory tests for the positives reactions; and the strong correlation between our results and preliminary epidemiological data (such as the level of human contact with wild triatominae, know locally as "Piaçava's lice". We draw attention to the isolation by xenodiagnosis of one strain of T.cruzi from a patient with positive serology for Chagas' infection.
An "attack" of wild triatomines (Rhodnius brethesi) to piaçaca workers (Leopoldinia piaçaba) is confirmed in the locality of Acuquaia, at Padauari river, affluent of Rio Negro in the municipality of Barcelos, State of Amazonas, Brazil. A serological prevalence of 12.5% for T. cruzi antibodies in human population, in the city of Barcelos, has already been described in a previous paper. A strong association between the serological positivity and the population contact with wild triatomines, known in the area as "piaçava's lice", was verified.