Assessment of Subclinical Renal Glomerular and Tubular Dysfunction in Children with Beta Thalassemia Major

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Abstract

Background: A good survival rate among patients with beta thalassemia major (beta-TM) has led to the appearance of an unrecognized renal disease. Therefore, we aimed to assess the role of serum cystatin-C as a promising marker for the detection of renal glomerular dysfunction and N-acetyl beta-D-glucosaminidase (NAG) and kidney injury molecule 1 (KIM-1) as potential markers for the detection of renal tubular injury in beta-TM children. Methods: This case-control study was implemented on 100 beta-TM children receiving regular blood transfusions and undergoing iron chelation therapy and 100 healthy children as a control group. Detailed histories of complete physical and clinical examinations were recorded. All subjected children underwent blood and urinary investigations. Results: There was a significant increase in serum cystatin-C ( p < 0.001) and a significant decrease in eGFR in patients with beta-TM compared with controls ( p = 0.01). There was a significant increase in urinary NAG, KIM-1, UNAG/Cr, and UKIM-1/Cr ( p < 0.001) among thalassemic children, with a significant positive correlation between serum cystatin-C, NAG and KIM-1 as regards serum ferritin, creatinine, and urea among thalassemic patients. A negative correlation between serum cystatin-C and urinary markers with eGFR was noted. Conclusion: Serum cystatin-C is a good marker for detection of glomerular dysfunction. NAG and KIM-1 may have a predictive role in the detection of kidney injury in beta-TM children.

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Most cited references 47

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New equations to estimate GFR in children with CKD.

George J Schwartz, Alvaro Muñoz, Michael F Schneider … (2009)

The Schwartz formula was devised in the mid-1970s to estimate GFR in children. Recent data suggest that this formula currently overestimates GFR as measured by plasma disappearance of iohexol, likely a result of a change in methods used to measure creatinine. Here, we developed equations to estimate GFR using data from the baseline visits of 349 children (aged 1 to 16 yr) in the Chronic Kidney Disease in Children (CKiD) cohort. Median iohexol-GFR (iGFR) was 41.3 ml/min per 1.73 m(2) (interquartile range 32.0 to 51.7), and median serum creatinine was 1.3 mg/dl. We performed linear regression analyses assessing precision, goodness of fit, and accuracy to develop improvements in the GFR estimating formula, which was based on height, serum creatinine, cystatin C, blood urea nitrogen, and gender. The best equation was: GFR(ml/min per 1.73 m(2))=39.1[height (m)/Scr (mg/dl)](0.516) x [1.8/cystatin C (mg/L)](0.294)[30/BUN (mg/dl)](0.169)[1.099](male)[height (m)/1.4](0.188). This formula yielded 87.7% of estimated GFR within 30% of the iGFR, and 45.6% within 10%. In a test set of 168 CKiD patients at 1 yr of follow-up, this formula compared favorably with previously published estimating equations for children. Furthermore, with height measured in cm, a bedside calculation of 0.413*(height/serum creatinine), provides a good approximation to the estimated GFR formula. Additional studies of children with higher GFR are needed to validate these formulas for use in screening all children for CKD.

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Chronic hypoxia and tubulointerstitial injury: a final common pathway to end-stage renal failure.

Masaomi Nangaku (2006)

Recent studies emphasize the role of chronic hypoxia in the tubulointerstitium as a final common pathway to end-stage renal failure. When advanced, tubulointerstitial damage is associated with the loss of peritubular capillaries. Associated interstitial fibrosis impairs oxygen diffusion and supply to tubular and interstitial cells. Hypoxia of tubular cells leads to apoptosis or epithelial-mesenchymal transdifferentiation. This in turn exacerbates fibrosis of the kidney and subsequent chronic hypoxia, setting in train a vicious cycle whose end point is ESRD. A number of mechanisms that induce tubulointerstitial hypoxia at an early stage have been identified. Glomerular injury and vasoconstriction of efferent arterioles as a result of imbalances in vasoactive substances decrease postglomerular peritubular capillary blood flow. Angiotensin II not only constricts efferent arterioles but, via its induction of oxidative stress, also hampers the efficient utilization of oxygen in tubular cells. Relative hypoxia in the kidney also results from increased metabolic demand in tubular cells. Furthermore, renal anemia hinders oxygen delivery. These factors can affect the kidney before the appearance of significant pathologic changes in the vasculature and predispose the kidney to tubulointerstitial injury. Therapeutic approaches that target the chronic hypoxia should prove effective against a broad range of renal diseases. Current modalities include the improvement of anemia with erythropoietin, the preservation of peritubular capillary blood flow by blockade of the renin-angiotensin system, and the use of antioxidants. Recent studies have elucidated the mechanism of hypoxia-induced transcription, namely that prolyl hydroxylase regulates hypoxia-inducible factor. This has given hope for the development of novel therapeutic approaches against this final common pathway.

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Influence of muscle mass and physical activity on serum and urinary creatinine and serum cystatin C.

Alessandra Baxmann, Marion Souza Ahmed, Natália Marques … (2008)

For addressing the influence of muscle mass on serum and urinary creatinine and serum cystatin C, body composition was assessed by skinfold thickness measurement and bioelectrical impedance analyses. A total of 170 healthy individuals (92 women, 78 men) were classified as sedentary or with mild or moderate/intense physical activity. Blood, 24-h urine samples, and 24-h food recall were obtained from all individuals. Serum and urinary creatinine correlated significantly with body weight, but the level of correlation with lean mass was even greater. There was no significant correlation between body weight and lean mass with cystatin C. Individuals with moderate/intense physical activity presented significantly lower mean body mass index (23.1 +/- 2.5 versus 25.7 +/- 3.9 kg/m(2)) and higher lean mass (55.3 +/- 10.0 versus 48.5 +/- 10.4%), serum creatinine (1.04 +/- 0.12 versus 0.95 +/- 0.17 mg/dl), urinary creatinine (1437 +/- 471 versus 1231 +/- 430 mg/24 h), protein intake (1.4 +/- 0.6 versus 1.1 +/- 0.6 g/kg per d), and meat intake (0.7 +/- 0.3 versus 0.5 +/- 0.4 g/kg per d) than the sedentary individuals. Conversely, mean serum cystatin did not differ between these two groups. A multivariate analysis of covariance showed that lean mass was significantly related to serum and urinary creatinine but not with cystatin, even after adjustment for protein/meat intake and physical activity. Cystatin C may represent a more adequate alternative to assess renal function in individuals with higher muscle mass when mild kidney impairment is suspected.

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Contributors

Marek Ussowicz: Role: Academic Editor

Journal

Journal ID (nlm-ta): Children (Basel)

Journal ID (iso-abbrev): Children (Basel)

Journal ID (publisher-id): children

Title: Children

Publisher: MDPI

ISSN (Electronic): 2227-9067

Publication date (Electronic): 03 February 2021

Publication date Collection: February 2021

Volume: 8

Issue: 2

Electronic Location Identifier: 100

Affiliations

[1 ]Department of Pediatrics, Faculty of Medicine, Menoufia University, Shebin Elkom 32511, Egypt; doaaelian@ 123456yahoo.com (D.M.E.); drnono23@ 123456yahoo.com (N.M.A.E.H.); samehabdallah75@ 123456yahoo.com (S.A.A.E.N.)

[2 ]Department of Pediatrics, College of Medicine, King Faisal University, Al-Ahsa 31982, Saudi Arabia

[3 ]Department of Clinical Pathology, National Liver Institute, Menoufia University, Shebin Elkom 32511, Egypt; dr.hebaabdallah2020@ 123456gmail.com

[4 ]Department of Clinical Biochemistry and Molecular Diagnostics, National Liver Institute, Menoufia University, Shebin Elkom 32511, Egypt; shimaa.abdelsattar@ 123456liver.menofia.edu.eg

[5 ]Department of Clinical and Molecular Microbiology and Immunology, National Liver Institute, Menoufia University, Shebin Elkom 32511, Egypt; fatma.khalil@ 123456liver.menofia.edu.eg

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[* ]Correspondence: asmaasoliman50@ 123456gmail.com ; Tel.: +2-0106-040-8035

Author information

Doaa M. Elian https://orcid.org/0000-0001-9596-6737

Shimaa Abdelsattar https://orcid.org/0000-0002-3993-5378

Fatma O. Khalil https://orcid.org/0000-0002-3157-5232

Article

Publisher ID: children-08-00100

DOI: 10.3390/children8020100

PMC ID: 7913373

PubMed ID: 33546213

SO-VID: 99499a9b-554c-47a9-84f9-5fdcf1663bf7

License:

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ( http://creativecommons.org/licenses/by/4.0/).

Assessment of Subclinical Renal Glomerular and Tubular Dysfunction in Children with Beta Thalassemia Major

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Most cited references 47

New equations to estimate GFR in children with CKD.

Chronic hypoxia and tubulointerstitial injury: a final common pathway to end-stage renal failure.

Influence of muscle mass and physical activity on serum and urinary creatinine and serum cystatin C.

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