Renal tubular dysfunction in children with sickle cell haemoglobinopathy

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...

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Bibliographic Details
Published in:	Nephrology (Carlton, Vic.) Vol. 18; no. 4; pp. 299 - 303
Main Authors:	Badr, Mohamed, El Koumi, Mohamed A, Ali, Yasser F, El-Morshedy, Salah, Almonem, Nermin Abd, Hassan, Tamer, El Rahman, Rehab Abd, Afify, Mona
Format:	Journal Article
Language:	English
Published:	Australia Blackwell Publishing Ltd 01-04-2013
Subjects:	Adolescent Anemia, Sickle Cell - blood Anemia, Sickle Cell - complications Anemia, Sickle Cell - physiopathology Anemia, Sickle Cell - urine beta 2-Microglobulin - urine Biomarkers - blood Biomarkers - urine Chi-Square Distribution Child Child, Preschool Creatinine - blood Female Humans Kidney Concentrating Ability Kidney Diseases - blood Kidney Diseases - etiology Kidney Diseases - physiopathology Kidney Diseases - urine Kidney Tubules, Proximal - metabolism Kidney Tubules, Proximal - physiopathology Linear Models Male proximal renal tubular dysfunction retinol binding protein Retinol-Binding Proteins - urine Saudi Arabia sickle cell disease Sickle Cell Trait - blood Sickle Cell Trait - complications Sickle Cell Trait - physiopathology Sickle Cell Trait - urine Time Factors β2-microglobulin Saudi Arabia
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Summary:	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 (β2 MG). Results Group I patients showed a significantly impaired urinary concentrating ability compared to that of Group II (417 ± 94 mOsm/kg vs 581 ± 165 mOsm/kg). The urinary excretions of RBP and β2‐microglobulin were significantly higher in Group I than in Group II. The values were 762.01 ± 124.20 μg/L and 841.84 ± 389.02 μg/L versus 198.12 ± 42.24 μg/L and 298.3 ± 38.11 μg/L, respectively. Conclusion Significant proximal tubular dysfunction was a feature in the SCD group, indicated by high urinary RBP and β2‐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. Summary at a Glance The authors studied proximal tubular function in 34 children with sickle cell disease by comparing urinary excretion of these low molecular weight proteins (LMWP) with a group of 27 children with sickle cell traits. They concluded that a significant high level of urinary LMWP excretion caused by proximal tubular function would provide important clinical information during the follow up of renal tubular function in these patients.
Bibliography:	ArticleID:NEP12040 ark:/67375/WNG-20KKWNKJ-7 istex:D1E4E844B13C8866B72DA4EF1D9B219BE8BB8718 ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	1320-5358 1440-1797
DOI:	10.1111/nep.12040