ABSTRACT
Wilson's disease (WD), also known as hepatolenticular degeneration, is a rare autosomal recessive disorder that results from abnormal ceruloplasmin metabolism, with copper deposition affecting multiple systems. Osmotic demyelination syndrome (ODS) refers to acute demyelination seen in the setting of osmotic changes, typically with the rapid correction of electrolyte disturbance. We present a 29-year-old male patient diagnosed with WD 1 year after the onset of extrapyramidal symptoms. Magnetic resonance imaging performed during hospitalization showed two patterns of pons involvement, which allowed the diagnosis of ODS in addition to WD. Classic imaging findings were observed and illustrate perfectly these two conditions.
Subject(s)
Demyelinating Diseases , Hepatolenticular Degeneration , Adult , Ceruloplasmin/metabolism , Copper/metabolism , Demyelinating Diseases/diagnostic imaging , Hepatolenticular Degeneration/complications , Hepatolenticular Degeneration/diagnostic imaging , Humans , Magnetic Resonance Imaging , MaleSubject(s)
Anus Neoplasms/pathology , Hemorrhage/diagnostic imaging , Lung Neoplasms/secondary , Melanoma, Amelanotic/pathology , Rectal Neoplasms/pathology , Anus Neoplasms/diagnosis , Drug Therapy , Female , Hemorrhage/etiology , Humans , Lung Neoplasms/pathology , Melanoma, Amelanotic/diagnosis , Middle Aged , Rectal Neoplasms/diagnosis , Tomography, X-Ray ComputedABSTRACT
BACKGROUND AND OBJECTIVES: The procedure used for screening Trypanosoma cruzi-infected blood donors by using two serological techniques has frequently led to discordant results. The TESA-blot, a confirmatory test for Chagas' disease, was applied in a survey of inconclusive sera from a Brazilian blood bank. MATERIALS AND METHODS: Four hundred and forty-eight sera, obtained from blood donors at the HRU-Fundação Hemominas, were tested by using the TESA-blot assay, a Western blotting method. Of these 448 sera, 348 had previously been determined as inconclusive for Chagas' disease owing to discordance between the indirect immunofluorescence assay (IFA) and the enzyme-linked immunosorbent assay (ELISA). RESULTS: The TESA-blot was positive for 2.87% (10/348) of the inconclusive sera, and 100% positive and negative for the sera from chagasic (n=50) and non-chagasic (n=50) donors, respectively. CONCLUSIONS: Our results clearly indicate the need to improve the diagnosis of Chagas' disease in blood banks by using new confirmatory diagnostic test(s). The TESA-blot, a new test with trypomastigote fractions of the T. cruzi Y strain, has made new approaches to the confirmation of Chagas' disease possible.
Subject(s)
Blotting, Western/methods , Chagas Disease/diagnosis , Adolescent , Adult , Animals , Antibodies, Protozoan/blood , Blood Banks , Blood Donors , Brazil/epidemiology , Chagas Disease/epidemiology , Chagas Disease/immunology , Diagnostic Errors , Enzyme-Linked Immunosorbent Assay , Female , Fluorescent Antibody Technique, Indirect , Humans , Male , Mass Screening , Middle Aged , Serologic Tests/methods , Trypanosoma cruzi/immunologyABSTRACT
The authors report the case of a patient with atrial septal defect who was submitted to surgical treatment when an intrapericardial hernia was found. The septal defect was closed with a bovine pericardial patch and the hernia with a pediculated autogenous pericardial graft. They discuss several aspects of the classification of the hernia, its association with other anomalies, embryogenesis, pathophysiology, prognosis and surgical correction.
