Renal Function in Adult Beta-Thalassemia/Hb E Disease
| Autor: | Sompong Ong-ajyooth, Somkiat Vasuvattakul, Leena Ong-Ajyooth, Nopadol Siritanaratkul, Prida Malasit, Nilwarangkur S, Suthat Fucharoen, Pensri Pootrakul |
|---|---|
| Rok vydání: | 1998 |
| Předmět: |
Adult
Male Hemolytic anemia medicine.medical_specialty Kidney Renal Agents Nephropathy Ammonia Furosemide Malondialdehyde Internal medicine Acetylglucosaminidase medicine Humans Urea Deamino Arginine Vasopressin Renal Aminoacidurias Diuretics Water Deprivation Red Cell business.industry Hemoglobin E Osmolar Concentration beta-Thalassemia Beta thalassemia Hydrogen-Ion Concentration Thailand medicine.disease Hemoglobinopathies Bicarbonates Proteinuria medicine.anatomical_structure Endocrinology Hemoglobinopathy Creatinine Ferritins Erythrocyte Count Splenectomy Female beta 2-Microglobulin business Kidney disease |
| Zdroj: | Nephron. 78:156-161 |
| ISSN: | 2235-3186 1660-8151 |
| DOI: | 10.1159/000044904 |
| Popis: | β-Thalassemia hemoglobin E (β-thal/Hb E) is the commonest form of hemoglobinopathy in Thailand. Shortened red cell life span, rapid iron turnover and tissue deposition of excess iron are major factors responsible for functional and physiological abnormalities found in various forms of thalassemia. Increased deposition of iron had been found in renal parenchyma of thalassemic patients, but no systematic study of the effect of the deposits on renal functions has been available. The purpose of this study is to describe the functional abnormalities of the kidney in patients with β-thal/Hb E and provide evidence that increased oxidative stress might be one of the factors responsible for the damage. Urine and serum samples from 95 patients with β-thal/Hb E were studied comparing with 27 age-matched healthy controls. No difference in the creatinine clearance was observed. β-thal/Hb E patients excreted significantly more urinary protein (0.8 ± 0.5 vs. 0.3±0.1 g/day, p < 0.001). Aminoaciduria was found in 16% of the patients. Analysis of urinary protein by SDS-PAGE electrophoresis and silver staining revealed abnormal pattern of protein with increased small molecular weight (D-glucosaminidase, 26.3 ± 41.3 vs. 8.4 ± 3.9 U/g Cr, p < 0.0001) and β2-microglobulin, 124.3 ± 167 vs. 71 ± 65.5 µg/g Cr, p = 0.001. Plasma and urine MDA (malonyldialdehyde) levels were both raised (p < 0.0001). Nine patients were selected for renal acidification study. All were found to be normal, but showed poor response to DDAVP challenge (urine osmolality 533 ± 71). This is the first report of renal tubular defects found associated with β-thal/Hb E disease. The mechanism leading to the damage is not known but it might be related to increased oxidative stress secondary to tissue deposition of iron, as indicated by the raised levels of serum and urine MDA. It is not known whether these functional defects would have any long-term effects on the patients. Further studies are warranted and means of prevention of these defects should urgently be sought. |
| Databáze: | OpenAIRE |
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