Decreased Urinary Excretion of β-Glucuronidase in Sickle Cell Anemia in Nigeria
العنوان: | Decreased Urinary Excretion of β-Glucuronidase in Sickle Cell Anemia in Nigeria |
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المؤلفون: | Romualda Szlachetka, Delvin Yazzie, Robert H. Glew, Donald E. Fry, Angela Okolo, Godwin I. Adoga |
المصدر: | Renal Failure. 17:57-64 |
بيانات النشر: | Informa UK Limited, 1995. |
سنة النشر: | 1995 |
مصطلحات موضوعية: | Male, Hemolytic anemia, medicine.medical_specialty, Adolescent, Hydrolases, Nigeria, Renal function, Anemia, Sickle Cell, Urine, Critical Care and Intensive Care Medicine, Excretion, chemistry.chemical_compound, Internal medicine, Humans, Medicine, Child, Glucuronidase, Creatinine, Red Cell, business.industry, General Medicine, Clinical Enzyme Tests, medicine.disease, Sickle cell anemia, Endocrinology, chemistry, Nephrology, Female, Hemoglobin, Lysosomes, business, Biomarkers |
الوصف: | The activities of five lysosomal hydrolases--namely beta-glucuronidase, beta-hexosaminidase, beta-galactosidase, alpha-galactosidase, and alpha-mannosidase--were measured in the plasma and urine of children (ages, 7 to 15 years) with sickle cell anemia (n = 11) and controls (n = 11) from Jos, Nigeria. The presence of SS hemoglobin was confirmed by electrophoresis of red cell hemolysates. Albuminuria was absent in all of the patients with sickle cell anemia. The creatinine-indexed urinary activity level (units of enzyme activity/milligrams creatinine) and the fractional enzyme excretion (FEE) value, which is defined as the ratio of enzyme clearance to creatinine clearance, were determined for each of the five lysosomal enzymes and compared between the two groups. The mean FEE values for beta-glucuronidase and alpha-galactosidase in the sickle cell patients were 10- and 3.5-fold lower, respectively, than the corresponding control values, and these differences were statistically significant (p.03) for both enzymes; however, beta-hexosaminidase, beta-galactosidase, and alpha-mannosidase levels in urine were not different between the two groups. When indexed to creatinine, a comparison of the urinary enzyme levels of control and sickle cell patients showed significant differences for beta-glucuronidase (p.01) and alpha-galactosidase (p.05) but not for the other three enzymes. Differences in level of plasma enzyme activity between control and sickle cell patients were not significant, except for alpha-galactosidase (p.05), which was increased slightly (25%) in the sickle cell group. These data indicate that there may be abnormalities in the metabolism of lysosomal enzymes in the kidneys of patients with sickle cell anemia.(ABSTRACT TRUNCATED AT 250 WORDS) |
تدمد: | 1525-6049 0886-022X |
URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::11ffab264184946f9c6db61845351e65 https://doi.org/10.3109/08860229509036376 |
حقوق: | OPEN |
رقم الأكسشن: | edsair.doi.dedup.....11ffab264184946f9c6db61845351e65 |
قاعدة البيانات: | OpenAIRE |
تدمد: | 15256049 0886022X |
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