Renal Involvement in Sickle Cell-Beta Thalassemia

Kontessis, Panayotis; Mayopoulou-Symvoulidis, D.; Symvoulidis, A; Kontopoulou-Griva, I.

doi:10.1159/000186827

Cited by 27 publications

(9 citation statements)

References 21 publications

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“…Low levels of hemoglobin raised beta 2-microglobulin, NAG and Cystatin-C. This was the evidence of that chronic hypoxia due to anemia disrupted kidney function [15,16]. In our study, urea levels of thalassemia group were found significantly high in the control group (p<0.05).…”

Section: Discussionsupporting

confidence: 60%

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: Discussionsupporting

confidence: 60%

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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“…This urinary concentrating defect becomes apparent at an early age in SS individuals. The renal concentrating defect in sickle ␤-thalassemia may be as prominent as in sickle cell anemia, although in this and in the other SCDs, as well as in sickle cell trait, the concentrating defect becomes manifest much later in life [8,9]. After an 8-10 hr overnight water deprivation, the urine osmolality in SS patients was 414 ± 10 mmol/kg (mean ± SE), considerably lower than the values in normal subjects (911 ± 39 mmol/kg) [10].…”

Section: Abnormalities Of Distal Nephron Functionmentioning

confidence: 99%

Renal abnormalities in sickle cell disease

Ataga,

Orringer

2000

Am. J. Hematol.

107

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Sickle cell anemia and the related hemoglobinopathies are associated with a large spectrum of renal abnormalities. The patients have impaired urinary concentrating ability, defects in urinary acidification and potassium excretion, and supranormal proximal tubular function. The latter is manifest by increased secretion of creatinine and by reabsorption of phosphorus and β2‐microglobulin. Young patients with sickle cell disease (SCD) have supranormal renal hemodynamics with elevations in both effective renal plasma flow (ERPF) and glomerular filtration rate (GFR). These parameters decrease with age as well as following the administration of prostaglandin inhibitors. Proteinuria, a common finding in adults with sickle cell disease, may progress to the nephrotic syndrome. Proteinuria, hypertension, and increasing anemia predict end‐stage renal disease (ESRD). While ESRD can be managed by dialysis and/or renal transplantation, there may be an increased rate of complications in renal transplant recipients with SCD. Hematuria is seen in individuals with all of the SCDs as well as with sickle cell trait. In most cases the etiology of the hematuria turns out to be benign. However, there does appear to be an increased association between SCD and renal medullary carcinoma. Therefore, those SCD patients who present with hematuria should initially undergo a thorough evaluation in order to exclude this aggressive neoplasm. Papillary necrosis may occur due to medullary ischemia and infarction. Erythropoietin levels are usually lower than expected for their degree of anemia and decrease further as renal function deteriorates. An abnormal balance of renal prostaglandins may be responsible for some of the changes in sickle cell nephropathy. Acute renal failure is a component of the acute multiorgan failure syndrome (MOFS). Finally, progression of sickle cell nephropathy to ESRD may be slowed by adequate control of hypertension and proteinuria. However, the prevention of the renal complications of SCD will require a cure for this genetic disorder. Am. J. Hematol. 63:205–211, 2000. © 2000 Wiley‐Liss, Inc.

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“…Thus, although they had similar water intakes after dehydration, the SCD patients lost more fluid as urine than the normal subjects. These suggest that the SCD patients tend to remain in a state of water deficit, and may explain the most common defect in patients with SCD which is impaired urine concentrating ability or hyposthenuria (Kontessis et al, 1992) which is suggested in this study by the lower urine concentration index in these patients.…”

Section: Discussionmentioning

confidence: 54%

Thirst perception in dehydrated sickle cell disease patients in steady state

Ozoene¹,

Enosolease²,

Ajayi³

et al. 2010

Nig. J. Phys Sci

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Summary:Liberal fluid intake is one of the key management strategies in sickle cell anaemia (SCA) patients in steady state, but less work has been done on the desire of patients to drink water. Using the Visual Analogue Scale we studied thirst perception (TP) in 20 euhydrated SCA patients and 28 control (HbA) subjects, as well as during dehydration in 13 SCA patients and 9 HbA subjects. Serum and urine samples were collected and analyzed for Na, K ions, creatinine concentrations and haematocrit and specific gravity of urine were determined. During euhydration, TP was significantly [P<0.05] higher in male SCA patients compared to the HbA subjects. In females, TP in SCA patient was not statistically significant compared with HbA subjects. After 13 hours of dehydration, TP was significantly [P<0.05] reduced in female. While dehydration increased TP in HbA subjects, it reduced TP in SCA patients. Fluid intakes after dehydration in SCA patients were not significantly different from the control HbA subjects in both male and female. It can be concluded that female SCA patients do not have normal response to dehydration with regards to TP after a period of dehydration. Since dehydration stimulates the release of vasoactive hormones like vasopressin, this may explain why female patients are less prone to crisis than their male counterparts.

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Renal Involvement in Sickle Cell-Beta Thalassemia

Cited by 27 publications

References 21 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

Renal abnormalities in sickle cell disease

Thirst perception in dehydrated sickle cell disease patients in steady state

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