Renal tubular dysfunction in children with sickle cell disease
Background: Children with sickle cell disease (SCD) are remarkably more prone than others to renal dysfunction. The kidneys, as a part of the systemic long term hazards in SCD, may be affected by both the hemodynamic changes of chronic anemia as well as by the consequences of vaso-occlusion. Obje...
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| Format: | Article |
| Language: | English |
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PAGEPress Publications
2014-08-01
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| Series: | Mediterranean Journal of Hematology and Infectious Diseases |
| Subjects: | |
| Online Access: | https://mjhid.org/index.php/mjhid/article/view/1813 |
| _version_ | 1826911420255567872 |
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| author | Mohamed El Koumi Yasser Fathy Ali Salah El Morshedy Asmaa Samir |
| author_facet | Mohamed El Koumi Yasser Fathy Ali Salah El Morshedy Asmaa Samir |
| author_sort | Mohamed El Koumi |
| collection | DOAJ |
| description | Background: Children with sickle cell disease (SCD) are remarkably more prone than others to renal dysfunction. The kidneys, as a part of the systemic long term hazards in SCD, may be affected by both the hemodynamic changes of chronic anemia as well as by the consequences of vaso-occlusion.
Objective: 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, between mid-2011 and 2012. A case-control study design was conducted including two groups. Urinary excretion of retinol binding protein (RBP) and ß2- microglobulin were checked in 34 children (18 males and 16 females) with SCD (Group I), and in 27 children with sickle cell trait matched as controls (Group II).
Results: Group I showed a significantly impaired urinary concentrating ability compared to the Group II (417 ± 94 mOsm/kg vs 581±165 mOsm/kg). The urinary excretion of RBP and ß2-microglobulin was significantly higher in Group I than in Group II. The values being 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 SCD group, indicated by high urinary RBP and ß2-microglobulin excretion. Evaluating the urinary excretion of these low molecular weight proteins, particularly RBP, may add a key clinical information to the follow up of renal tubular function in patients with SCD. |
| first_indexed | 2024-12-24T00:50:21Z |
| format | Article |
| id | doaj.art-c5bb0f65ef1349d9b04eaef206319982 |
| institution | Directory Open Access Journal |
| issn | 2035-3006 |
| language | English |
| last_indexed | 2025-02-17T10:14:20Z |
| publishDate | 2014-08-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Mediterranean Journal of Hematology and Infectious Diseases |
| spelling | doaj.art-c5bb0f65ef1349d9b04eaef2063199822025-01-02T00:30:30ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062014-08-011Renal tubular dysfunction in children with sickle cell diseaseMohamed El Koumi0Yasser Fathy Ali1Salah El Morshedy2Asmaa Samir3Lecturer pediatrics-zagazig university children hospitals Current consultant pediatrician-Al khafji joint operation hospital-KSAAssistant professor pediatrics-zagazig university children hospitals Current consultant pediatrician-Al khafji joint operation hospital-KSAAssistant professor pediatrics-zagazig university children hospitals Current consultant pediatrician-Al khafji joint operation hospital-KSALecturer clinical pathology-zagazig university children hospitals Current consultant clinical pathology-Al khafji joint operation hospital-KSABackground: Children with sickle cell disease (SCD) are remarkably more prone than others to renal dysfunction. The kidneys, as a part of the systemic long term hazards in SCD, may be affected by both the hemodynamic changes of chronic anemia as well as by the consequences of vaso-occlusion. Objective: 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, between mid-2011 and 2012. A case-control study design was conducted including two groups. Urinary excretion of retinol binding protein (RBP) and ß2- microglobulin were checked in 34 children (18 males and 16 females) with SCD (Group I), and in 27 children with sickle cell trait matched as controls (Group II). Results: Group I showed a significantly impaired urinary concentrating ability compared to the Group II (417 ± 94 mOsm/kg vs 581±165 mOsm/kg). The urinary excretion of RBP and ß2-microglobulin was significantly higher in Group I than in Group II. The values being 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 SCD group, indicated by high urinary RBP and ß2-microglobulin excretion. Evaluating the urinary excretion of these low molecular weight proteins, particularly RBP, may add a key clinical information to the follow up of renal tubular function in patients with SCD.https://mjhid.org/index.php/mjhid/article/view/1813ß2-microglobulinRetinol binding proteinSickle cell diseaseProximal renal tubular dysfunction |
| spellingShingle | Mohamed El Koumi Yasser Fathy Ali Salah El Morshedy Asmaa Samir Renal tubular dysfunction in children with sickle cell disease Mediterranean Journal of Hematology and Infectious Diseases ß2-microglobulin Retinol binding protein Sickle cell disease Proximal renal tubular dysfunction |
| title | Renal tubular dysfunction in children with sickle cell disease |
| title_full | Renal tubular dysfunction in children with sickle cell disease |
| title_fullStr | Renal tubular dysfunction in children with sickle cell disease |
| title_full_unstemmed | Renal tubular dysfunction in children with sickle cell disease |
| title_short | Renal tubular dysfunction in children with sickle cell disease |
| title_sort | renal tubular dysfunction in children with sickle cell disease |
| topic | ß2-microglobulin Retinol binding protein Sickle cell disease Proximal renal tubular dysfunction |
| url | https://mjhid.org/index.php/mjhid/article/view/1813 |
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