[Neurologic complications in uremia].
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Biomedical subjects
Publications and source records attributed to S Halvorsen.
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A new case of assumed beta-ketothiolase deficiency, excreting 2-methyl-3-hydroxybutyrate and tiglylglycine is described in a 15-year-old boy. The patient presented with episodes of metabolic acidosis following intercurrent infections in the early childhood. After the age of 7 years he has had periods of headache, but no acidotic episodes have occurred even during infections. Systematic dietary treatment has not been instituted, and the patient is physically and mentally normal. This indicates a mild variant of the beta-ketothiolase deficiency. Diagnosis of the condition may be obscured by large quantities of ordinary ketone bodies, and requires gas chromatographic and mass spectrometric techniques.
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A significant influence of matching both for the HLA-A and B and the D/DR antigens on graft survival in patients transplanted with kidneys from living related or cadaveric donors is demonstrated. A generally reduced survival of cadaveric grafts during the last few years may at least in part be explained by the use of more three and four antigen-mismatched donors. The beneficial effect of pretransplant blood transfusions on graft survival in our material is almost nulled when uremic patients, dying while waiting for a transplant, are also considered. In addition, significantly more high-risk patients are included in the nontransfused patient group.
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A low mixed-lymphocyte-culture response, indicating matching for HLA-D, was associated with a relatively good chance of graft survival in 62 recipients of renal transplants from living relatives mismatched for one or two HLA A and/or B antigens. In 96 cadaveric transplants prospectively typed for HLA-DR antigens, compatibility for these antigens improved the prognosis, irrespective of matching for HLA A and B antigens. In cadaveric transplants, a positive B-cell cross-match test before transplantation tended to predict inferior graft survival.
The erythropoietin (ESF) content of plasma and urine has been studied in children with protein-energy-malnutrition (PEM) living in the Kivu province at an altitude of 1467--2000 m. On admission, packed cell volume (PCV) was moderately reduced; after 2 months of refeeding PCV had increased but was still lower than in the controls. Plasma ESF was increased on admission and in patients refed for 2 months. The expected positive correlation between serum and urine ESF levels was found after refeeding but not on admission; the last finding could not be explained by the presence of erythropoiesis inhibiting factor(s) in the urine. In spite of the normal 2,3-DPG and P50 previously described in PEM in Kivu, the anaemia associated with this disease is not merely an adaptation to lowered oxygen consumption and there must be some disturbances in the responsiveness of bone marrow to ESF. The high ESF values observed after 2 months of refeeding confirm that the restoration of the red cell volume proceeds slowly.
A metabolic study with radioactively labelled C3 has been performed in a patient with angioimmunoblastic lymphadenopathy (AILD). The labelled protein was metabolized at a faster rate in the patient than in normal individuals. This indicates that the humoral immune system may be of pathogenetic significance in the AILD syndrome.
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A case of malacoplakia in a transplanted patient is reported. Several months after the operation typical lesions with numerous Michaelis-Gutmann bodies were found by biopsies from the urinary bladder and the renal transplant, the latter being ultimately destroyed by the process. Recurrent urinary infections with Escherichia coli might have contributed to the development of the process in combination with an altered macrophage function owing to the immunosuppressive regimen, which was considered mainly responsible for the progressive course of an otherwise benign inflammatory reaction. If malacoplakia occurs in a renal transplant and the renal function deteriorates despite adequate antibiotic therapy, transplantectomy should not be delayed.
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