Function of the Kidney in Adult Patients with Cooley’s Disease

Mastrangelo, F; Lopez, Thais Torralbo; Rizzelli, S; Manisco, G; Corlianò, C; Alfonso, Libertad Martín

doi:10.1159/000180452

Cited by 30 publications

(34 citation statements)

References 5 publications

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“…In the study of Lapatsanis et al, increased hemoglobin level of patient group over 7.5 mg/dl was accompanied by increasing level of serum ALP; that increase was linked to improved osteoblastic dysfunction. Although mean hemoglobin level was 8.78 ± 0.71 mg/dl in our patient group, such an increase was not detected [9]. Phosphorus levels of thalassemia group were significantly higher in the control group (p <0.05).…”

Section: Discussioncontrasting

confidence: 66%

“…Many reasons are proposed, although mechanism of kidney dysfunction in thalassemia is not exactly known [9,10]. Increased [11,12].…”

Section: Discussionmentioning

confidence: 99%

“…Despite normal serum phosphorus levels, the table of increased phosphaturia was linked to hemolysis [18]. According to the literature [9,10], oxygen radicals, caused by non-hemoglobin iron (Fenton reaction), generate lysosomal damage by accumulation of iron in lysosomes of renal cells. MDA (Malondialdehyde), yielding as a result of lipid peroxidation of polyunsaturated fatty acids which are catalyzed by iron, covalently bonds to protein, phospholipids and DNA.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Levels of Beta-2 Microglobulin and Cystatin C in Beta Thalassemia Major Patients

Kacar¹,

Şehitoğlu²,

Kaçar³

et al. 2015

Ann Clin Anal Med

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ÖzetAmaç: Talaseminin dünyadaki en yaygın genetik hastalık olduğu düşünülmek-tedir. Gerek hastalık, gerekse tekrarlayan transfüzyonlar nedeniyle birçok sistemde komplikasyon ortaya çıkmaktadır. Bu çalışma; üzerinde daha az durulan böbrek glomerüler hasarının beta talasemi majorlü hastalarda mevcut olup olmadığının saptanması ve glomerüler hasarı saptarken üre, kreatinin, kreatinin klirensinin erken böbrek hasarı göstergeçlerinden olan sistatin c, B2 mikroglobulin ile kıyaslanması amacıyla yapıldı. Gereç ve Yöntem: Bu çalışma beta talasemi major tanısıyla çocuk hematoloji polikliniğimizce düzenli takip edilen hastalarda prospektif olarak yapılmıştır. Bulgular: Üre ile kreatinin klirensi ve sistain C arasında istatistiksel olarak anlamlı bir iliş-ki bulunmamaktadır(p>0.05). Kreatinin ile kreatinin klirensi arasında negatif yönde, %53.7 düzeyinde ve istatistiksel olarak ileri düzeyde anlamlı bir iliş-ki bulunmaktadır (p:0.002; p<0.01). Kreatinin ile sistain C ve Beta 2 mikroglobulin düzeyleri arasında istatistiksel olarak anlamlı bir ilişki bulunmamaktadır (p>0.05). Sistain C ile β-2 mikroglobulin arasında pozitif yönde, %86.1 düzeyinde ve istatistiksel olarak ileri düzeyde anlamlı bir ilişki bulunmaktadır (p:0.001; p<0.01). Tartışma: Literatürde GFR>40 ml/dk/1.73 m² olduğun-da kreatinin ile β-2 mikroglobulin ve sistatin c arası ilişkinin azaldığı belirtilmiştir. Bizim çalışmamız da bu literatür sonuçlarıyla benzerlik göstermekte-dir. Ancak renal parametrelerin birbiriyle korelasyonu hakkında kesin sonuç-lara varmak için daha geniş populasyonda yapılacak daha fazla sayıda çalış-malara ihtiyaç vardır. Anahtar KelimelerSistatin C; Böbrek; Talasemi; Beta 2-Mikroglobulin Abstract Aim: Thalassemia is accepted to be the most common genetic disease in the world. This study was performed to establish whether there was a glomerular renal damage, which was usually a less mentioned subject in patients with Beta Thalassemia Major, and to compare urea, creatinine and creatinine clearance with early indicators of kidney damage as Cystatin-C and β-2 microglobulin as on determining the glomerular damage. Material and Method:This study was prospectively performed in patients, who were regularly followed in the children hematology outpatient clinic with a diagnosis of Beta Thalassemia Major. Results: There was no statistically significant difference between urea and levels of creatinine clearance and Cystatin-C. There was a statistically negative relationship between creatinine and creatinine clearance at an advanced level as 53.7% (p: 0.002, p<0.01). There was not any significant relationship between Cystatin-C and levels of creatinine and B-2 microglobulin. There was a significant high positive relationship between Cystatin-C and B-2 microglobulin at level of 86.1% (p: 0.001; p<0.01). Discussion: The results of our study were also similar to the literature. However, new studies are required to carry out in wider populations in order to reach definite conclusions about correlation of renal parameters with each other.

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Section: Discussioncontrasting

confidence: 66%

“…Many reasons are proposed, although mechanism of kidney dysfunction in thalassemia is not exactly known [9,10]. Increased [11,12].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Levels of Beta-2 Microglobulin and Cystatin C in Beta Thalassemia Major Patients

Kacar¹,

Şehitoğlu²,

Kaçar³

et al. 2015

Ann Clin Anal Med

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“…The severity correlated with the degree of anemia is least severe in patients on hyper transfusion and iron chelation therapy, suggesting that the damage might be caused by the anemia and increased oxidation induced by excess iron deposits. [1][2][3][4][5][6][7][8][9] Although it was reported that urinary excretion of proximal renal tubular damage parameters increased in patients with TM and TI, renal functional parameters were not separately studied as glomerular and tubular markers in all types of the disease. The aims of the present study were to assess the glomerular and tubular renal functions in three different types of beta thalassemia, and to identify the similarities and differences between groups and to compare with healthy controls.…”

Section: Introductionmentioning

confidence: 99%

Glomerular and tubular functions in children with different forms of beta thalassemia

Uzun

Balcı²,

Yüksel

et al. 2015

Renal Failure

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Background: Although there are many available data about renal involvement in patients with beta thalassemia major (TM), the changes in renal functions of other types, such as thalassemia intermedia (TI) and thalassemia minor (TMin), were reported less. Therefore, we aimed to evaluate renal tubular and glomerular functions in patients with three types of beta thalassemia. Methods: This prospective case-control study was conducted on 118 betathalassemia patients (49 in TM, 18 in TI and 51 TMin) and 51 healthy controls. Glomerular functions [estimated glomerular filtration rate (GFR), serum cystatin C and urinary protein creatinine ratio] and tubular functions [fractioned sodium excretion (FENa), tubular reabsorption of phosphorus, urinary excretion of uric acid, levels of retinol-binding protein, alpha-1 macroglobulin (alpha-1M), and beta-2 microglobulin, calcium creatinine ratio] were assessed in all patients and controls. Results: The mean ages of the groups and controls at presentation were similar. Although GFR was similar in all patients and control groups, serum levels of cystatin C in patients with TM and TI were significantly higher compared to TMin and controls. Alpha-1M, FENa, urinary excretion of uric acid, and urine protein/creatinine ratio in TM and TI groups were significantly higher than the others. Mean cystatin C level was also higher in patients with TMin compared the controls. However, there were no significant differences according to all tubular and other glomerular functions between TMin and control groups. Conclusions: Although all types of beta thalassemia patients should be closely monitored to prevent further decrease in renal functions, the patients with TI should be considered to have a higher risk of glomerular and tubular deterioration as well as TM.

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“…Renal involvement has been extensively studied in both Cooley’s disease and sickle cell anemia [1, 2, 3, 4, 5, 6, 7, 8] but sparingly in β-thalassemia/Hb E disease [9, 10]. There are several factors that may adversely affect the kidney in thalassemia: chronic anemia, iron overload, hemosiderosis and oxidative stress.…”

Section: Introductionmentioning

confidence: 99%

Renal Function in Adult Beta-Thalassemia/Hb E Disease

Ong-Ajyooth¹,

Malasit²,

Ong-Ajyooth³

et al. 1998

Nephron

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β-Thalassemia hemoglobin E (β-thal/Hb E) is the commonest form of hemoglobinopathy in Thailand. Shortened red cell life span, rapid iron turnover and tissue deposition of excess iron are major factors responsible for functional and physiological abnormalities found in various forms of thalassemia. Increased deposition of iron had been found in renal parenchyma of thalassemic patients, but no systematic study of the effect of the deposits on renal functions has been available. The purpose of this study is to describe the functional abnormalities of the kidney in patients with β-thal/Hb E and provide evidence that increased oxidative stress might be one of the factors responsible for the damage. Urine and serum samples from 95 patients with β-thal/Hb E were studied comparing with 27 age-matched healthy controls. No difference in the creatinine clearance was observed. β-thal/Hb E patients excreted significantly more urinary protein (0.8 ± 0.5 vs. 0.3±0.1 g/day, p < 0.001). Aminoaciduria was found in 16% of the patients. Analysis of urinary protein by SDS-PAGE electrophoresis and silver staining revealed abnormal pattern of protein with increased small molecular weight (<45 kD) bands. Morning urine analysis showed significant lower urine osmolality (578.3 ± 164.6 vs. 762.4 ± 169.9 mosm/kg, p < 0.001) in patients. Patients excreted more NAG (N-acetyl beta-D-glucosaminidase, 26.3 ± 41.3 vs. 8.4 ± 3.9 U/g Cr, p < 0.0001) and β₂-microglobulin, 124.3 ± 167 vs. 71 ± 65.5 µg/g Cr, p = 0.001. Plasma and urine MDA (malonyldialdehyde) levels were both raised (p < 0.0001). Nine patients were selected for renal acidification study. All were found to be normal, but showed poor response to DDAVP challenge (urine osmolality 533 ± 71). This is the first report of renal tubular defects found associated with β-thal/Hb E disease. The mechanism leading to the damage is not known but it might be related to increased oxidative stress secondary to tissue deposition of iron, as indicated by the raised levels of serum and urine MDA. It is not known whether these functional defects would have any long-term effects on the patients. Further studies are warranted and means of prevention of these defects should urgently be sought.

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Function of the Kidney in Adult Patients with Cooley’s Disease

Cited by 30 publications

References 5 publications

Levels of Beta-2 Microglobulin and Cystatin C in Beta Thalassemia Major Patients

Levels of Beta-2 Microglobulin and Cystatin C in Beta Thalassemia Major Patients

Glomerular and tubular functions in children with different forms of beta thalassemia

Renal Function in Adult Beta-Thalassemia/Hb E Disease

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