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Renal tubular dysfunction in children with sickle cell haemoglobinopathy
Faculty
Not Specified
Year:
2013
Type of Publication:
Article
Pages:
299-303
Authors:
Badr, Mohamed, El Koumi, Mohamed A, Ali, Yasser F, El-Morshedy, Salah, Abd Almonem, Nermin, Hassan, Tamer, Abd El Rahman, Rehab, Afify, Mona
DOI:
10.1111/nep.12040
Journal:
NEPHROLOGY WILEY-BLACKWELL
Volume:
18
Research Area:
Urology \& Nephrology
ISSN
ISI:000316916400008
Keywords :
proximal renal tubular dysfunction, retinol binding protein, sickle cell disease, 2-microglobulin
Abstract:
Aim Children with sickle cell disease (SCD) are remarkably more prone than others to renal dysfunction. The kidneys, as one of the systemic long-term hazards in SCD, may be affected by both the haemodynamic changes of chronic anaemia as well as by the consequences of vaso-occlusion. The aim of this study was to evaluate the proximal tubular function in a group of Saudi children with established SCD. Methods This study was conducted in Al-Khafji Joint Operations (KJO) Hospital, in Saudi Arabia during the period from June 2011 to August 2012. Thirty-four children: Group I (18 males and 16 females) with SCD (HBSS) and 27 children: Group II (17 males and 10 females) with sickle cell trait (HBAS) were evaluated for urinary excretion of retinol binding protein (RBP) and Beta 2 microglobulin (2 MG). Results Group I patients showed a significantly impaired urinary concentrating ability compared to that of Group II (417 +/- 94mOsm/kg vs 581 +/- 165mOsm/kg). The urinary excretions of RBP and 2-microglobulin were significantly higher in Group I than in Group II. The values were 762.01 +/- 124.20g/L and 841.84 +/- 389.02g/L versus 198.12 +/- 42.24g/L and 298.3 +/- 38.11g/L, respectively. Conclusion Significant proximal tubular dysfunction was a feature in the SCD group, indicated by high urinary RBP and 2-microglobulin excretion. Assessing the urinary excretion of these low molecular weight proteins in children with sickle cell disease at different points of diagnosis may add key clinical information to the follow up of renal tubular function in patients with SCD.
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