Mula-Abed Waad-Allah S, Al-Hashmi Huda S, Al-Muslahi Muhanna N
Departments of Chemical Pathology and.
Sultan Qaboos Univ Med J. 2011 Feb;11(1):69-76. Epub 2011 Feb 12.
There are limited data concerning the assessment of renal function in beta-thalassaemia major, with no study of such involvement in Omani patients. The objective of this study was to establish the pattern of renal glomerular and tubular function using traditional and specific laboratory tests in patients with beta-thalassaemia major.
This cross-sectional study, from January-July 2008, included 30 patients of the Thalassaemia Clinic at the Royal Hospital, Oman, with transfusion-dependent homozygous beta-thalassaemia major. They included 15 males and 15 females, aged 16-32 years with mean ± standard deviation of 21.23 ± 3.42 years. The medical records were reviewed and renal function states assessed as follows: serum creatinine, estimated glomerular filtration rate (eGFR); urea; phosphate, fractional excretion of filtered sodium (FENa); urine albumin: creatinine index; urine ß2-microglobulin:creatinine index; tubular reabsorption of phosphate (TRP), and tubular maximum phosphate reabsorption (TmP)/GFR.
All patients had eGFR >90 ml/min/1.73m(2); serum creatinine <90 μmol/L; serum urea <6.0 mmol/L, and urine albumin:creatinine <2.5 mg/mmol. Only 2 (6.7%) patients had FENa >1% and 3 (10.0%) patients had urine ß2-microglobulin: creatinine >22 μg/mmol. All patients had TRP >0.85, of whom seven (23.3%) patients had values within the range of 0.85-0.95 and 23 (76.7%) had values >0.95. Also, all patients had TmP/GFR >1.0 mmol/L, of whom only one (3.3%) patient had TmP/GFR of 1.0-1.5, and 29 (96.7%) patients had TmP/GFR >1.5 mmol/L. Finally, 24 (80%) patients had serum phosphate >1.4 mmol/L. Linear regression revealed a highly significant correlation between serum phosphate and TmP/GFR (r = 0.904, P < 0.001).
Renal function, glomerular and tubular, appears to be well preserved in beta-thalassaemia major. Almost all renal function indicators were within the recommended ranges. Raised TmP/GFR and TRP were noted in the majority of patients, reflecting an up-trend in serum phosphate and therefore increasing renal phosphate reabsorption.
关于重型β地中海贫血患者肾功能评估的数据有限,且尚无针对阿曼患者此类情况的研究。本研究的目的是通过传统和特定实验室检查确定重型β地中海贫血患者的肾小球和肾小管功能模式。
这项横断面研究于2008年1月至7月进行,纳入了阿曼皇家医院地中海贫血诊所的30例依赖输血的纯合子重型β地中海贫血患者。其中包括15名男性和15名女性,年龄在16 - 32岁之间,平均年龄±标准差为21.23±3.42岁。回顾病历并按以下方式评估肾功能状态:血清肌酐、估计肾小球滤过率(eGFR);尿素;磷酸盐、滤过钠分数排泄(FENa);尿白蛋白:肌酐指数;尿β2微球蛋白:肌酐指数;肾小管磷酸盐重吸收(TRP)以及肾小管最大磷酸盐重吸收(TmP)/GFR。
所有患者的eGFR>90 ml/min/1.73m²;血清肌酐<90μmol/L;血清尿素<6.0 mmol/L,且尿白蛋白:肌酐<2.5 mg/mmol。仅有2例(6.7%)患者的FENa>1%,3例(10.0%)患者的尿β2微球蛋白:肌酐>22μg/mmol。所有患者的TRP>0.85,其中7例(23.3%)患者的值在0.85 - 0.95范围内,23例(76.7%)患者的值>0.95。此外,所有患者的TmP/GFR>1.0 mmol/L,其中仅有1例(3.3%)患者的TmP/GFR为1.0 - 1.5,29例(96.7%)患者的TmP/GFR>1.5 mmol/L。最后,24例(80%)患者的血清磷酸盐>1.4 mmol/L。线性回归显示血清磷酸盐与TmP/GFR之间存在高度显著相关性(r = 0.904,P < 0.001)。
重型β地中海贫血患者的肾小球和肾小管功能似乎保存良好。几乎所有肾功能指标都在推荐范围内。大多数患者的TmP/GFR和TRP升高,反映出血清磷酸盐呈上升趋势,因此肾小管对磷酸盐的重吸收增加。