Kidney Functions and Physiological Complications in Pediatric Thalassemia Patients of Indian Origin
DOI:
https://doi.org/10.61841/b1061q76Keywords:
renal function, microscopic investigations, malonaldehyde, hemolysis, fetal hemoglobinAbstract
Background: Thalassemia patients develop progressive anemia and require regular blood transfusions to sustain life, as adverse effects start with hemolysis of red blood cells (RBCs).
Objectives: The present study elucidates physiological complications and the effect of hypoxic stress on the physiology of kidney function in pediatric thalassemia.
Methods: The current study includes 45 thalassemia patients of age between 3 and 16 years. A sufficient number of urine and blood samples were obtained from all the participants. Samples were analyzed for the biochemical parameters such as pH, creatinine, protein, urea, sodium (Na+), potassium (K+), uric acid, ferritin, albumin, hemoglobin, fetal hemoglobin, and malonaldehyde (MDA). Physiological complications were also investigated with the help of patient data sheet, blood and urine biochemical investigations and microscopic examinations of urine.
Results: Most of the patients show severe physiological complications, including recurrent fever, joint and chest pain, shortness of breath, swelling of the abdomen, and an unusual headache. Serum creatinine level, serum uric acid, and hemoglobin levels in thalassemia are lower, while fetal hemoglobin levels are significantly elevated in thalassemia patients. Urine protein value, creatinine level, urea, and MDA were significantly high in thalassemia patients. High molecular weight proteins ranging from the molecular weight 10-260 kDa appeared in the urine of thalassemia patients.
Conclusion: Most of the urine and serum biochemical parameters are significantly altered, indicating glomerular dysfunction may be due to glomerular damage, tubular damage, and nephritis.
Downloads
References
[1] Aldudak B, Bayazit AK, Noyan A, Özel A, Anarat A, Sasmaz I, Kilinç Y, Gali E, Anarat R, Dikmen N.
Renal function in pediatric patients with β-thalassemia major. Pediatric nephrology. 2000 Oct 1; 15(1-
2):109-12. Doi: 10.1007/s004670000.
[2] Ali D, Mehran K, Moghaddam AG. Comparative evaluation of renal findings inBeta-thalassemia major and
intermedia. Saudi Journal of Kidney Diseases andTransplantation. 2008 Mar 1; 19(2):206.
[3] Balgir RS. The burden of haemoglobinopathies in India and the challenges ahead. Current Science. 2000
Dec 10:1536-47.
[4] Bekhit OE, El Dash HH, Ahmed MS. Early detection of kidney dysfunction inEgyptian patients with betathalassemia major. Egyptian Pediatric AssociationGazette. 2017 Sep 1; 65(3):85-9. Doi:
10.1016/j.epag.2017.02.002.
[5] Brittenham GM, Griffith PM, Nienhuis AW, McLaren CE, Young NS, Tucker EE,Allen CJ, Farrell DE,
Harris JW. Efficacy of deferoxamine in preventingcomplications of iron overload in patients with
thalassemia major. New EnglandJournal of Medicine. 1994 Sep 1; 331(9):567-73. Doi:
10.1056/NEJM199409013310902.
[6] Cianciulli P, Sorrentino F, Forte L, Palombi M, Papa G, Meloni C, Taccone MG,Casciani CU. Acute renal
failure occurring during intravenous desferrioxaminetherapy: recovery after haemodialysis. Haematologica.
1992; 77(6):514-5.
[7] Drabkin, D.L. Spectrophotometric studies: spectrophotometric constants for common haemoglobin
derivatives in human, dog and rabbit blood J.Biol.Chem (1932), 98:71.
[8] El-Nashar M, Mortagy AK, El-Beblawy NM, El-Gohary E, Kamel IM, Rashad M,Mouharam WA.
Parathyroid hormone in pediatric patients with β-thalassemiamajor and its relation to bone mineral density;
a case control study. EgyptianJournal of Medical Human Genetics. 2017; 18(1):75-8. Doi:
10.1016/j.ejmhg.2016.03.004.
[9] Joint WH. Joint WHO/TIF Meeting on the Prevention and Control of Haemoglobinopathies: 7th Meeting of
the WHO Working Group on theControl of Hereditary Anaemias. InJoint WHO/TIF Meeting on
thePrevention and Control of Haemoglobinopathies 1993. World Health Organization.
[10] Kassab-Chekir A, Laradi S, Ferchichi S, Khelil AH, Feki M, Amri F, Selmi H,Bejaoui M, Miled A.
Oxidant, antioxidant status and metabolic data in patients withbeta-thalassemia. Clinica Chimica Acta.
2003 Dec 1; 338(1-2):79-86. Doi: 10.1016/j.cccn.2003.07.010.
[11] Lowry OH, Rosebrough NJ, Farr AL, Randall RJ. Protein measurement with theFolin phenol reagent.
Journal of biological chemistry. 1951 Nov 1; 193(1):265-75.
[12] Mukherji M. Cooley’s anaemia (erythroblastic or mediterranean anaemia). The Indian Journal of Pediatrics.
1938 Jan 1; 5(1):1.
[13] Mula-Abed WA, Al-Hashmi HS, Al-Muslahi MN. Indicators of renal glomerular andtubular functions in
patients with beta-thalassaemia major: A cross sectional studyat the Royal Hospital, Oman. Sultan Qaboos
University medical journal. 2011 Feb;11(1):69.
[14] Ponticelli C, Musallam KM, Cianciulli P, Cappellini MD. Renal complications intransfusion-dependent
beta thalassaemia. Blood reviews. 2010 Nov 1; 24(6):239-44. Doi: 10.1016/j.blre.2010.08.004.
[15] Prasannan L, Flynn JT, Levine JE. Acute renal failure followingdeferoxamine overdose. Pediatric
nephrology. 2003 Mar 1; 18(3):283-5. Doi: 10.1007/s00467-002-1051-7.
[16] Quinn CT, Johnson VL, Kim HY, Trachtenberg F, Vogiatzi MG, Kwiatkowski JL,Neufeld EJ, Fung E,
Oliveri N, Kirby M, Giardina PJ. Renal dysfunction in patientswith thalassaemia. British journal of
haematology. 2011 Apr; 153(1):111-7. Doi: 10.1111/j.1365-2141.2010.08477.x.
[17] Şen V, Ece A, Uluca Ü, Söker M, Güneş A, Kaplan İ, Tan İ, Yel S, Mete N, Sahin C. Urinary early kidney
injury molecules in children with beta-thalassemia major. Renal failure. 2015 Apr 21; 37(4):607-13.
Doi:10.3109/0886022X.2015.1007871.
[18] Smolkin V, Halevy R, Levin C, Mines M, Sakran W, Ilia K, Koren A. Renal function in children with β-
thalassemia major and thalassemia intermedia. Pediatric nephrology. 2008 Oct 1; 23(10):1847. Doi:
10.1007/ s00467-008-0897-8.
[19] Sood R, Text Book of Hematology, 3rd ed. 1992; 280-282.
[20] Stevens LA, Levey AS. Measurement of kidney function. Medical Clinics. 2005May 1; 89(3):457-73. Doi:
10.1016/j.mcna.2004.11.009.
[21] Sukumaran PK. Abnormal haemoglobins in India. InTrends in haematology1975 (pp. 226-261). JB
Chatterjea Memorial Committee, Calcutta.
[22] Sumboonnanonda A, Malasit P, Tanphaichitr VS, Ong–ajyooth S, Petrarat S,Vongjirad A. Renal tubular
dysfunction in α-thalassemia. Pediatric nephrology. 2003 Mar 1; 18(3):257-60. Doi:
10.1007/s004670050453.
[23] Sumboonnanonda A, Malasit P, Tanphaichitr VS, Ong–ajyooth S, Petrarat S,VongjiradA. Renal tubular
dysfunction in α-thalassemia. Pediatric nephrology. 2003 Mar 1; 18(3):257-60. Doi: 10.1007/s00467-003-
1067-7.
[24] Uzun E, Balcı YI, Yüksel S, Aral YZ, Aybek H, Akdağ B. Glomerular and tubular functions in
children with different forms of beta thalassemia. Renal failure. 2015 Oct 21; 37(9):1414-8.
Doi:10.3109/0886022X.2015.1077314.
[25] Weatherall DJ, Clegg JB. Inherited haemoglobin disorders: an increasingglobal health problem. Bulletin of
the World Health Organization. 2001;79:704-12.
Downloads
Published
Issue
Section
License
This work is licensed under a Creative Commons Attribution 4.0 International License.
You are free to:
- Share — copy and redistribute the material in any medium or format for any purpose, even commercially.
- Adapt — remix, transform, and build upon the material for any purpose, even commercially.
- The licensor cannot revoke these freedoms as long as you follow the license terms.
Under the following terms:
- Attribution — You must give appropriate credit , provide a link to the license, and indicate if changes were made . You may do so in any reasonable manner, but not in any way that suggests the licensor endorses you or your use.
- No additional restrictions — You may not apply legal terms or technological measures that legally restrict others from doing anything the license permits.
Notices:
You do not have to comply with the license for elements of the material in the public domain or where your use is permitted by an applicable exception or limitation .
No warranties are given. The license may not give you all of the permissions necessary for your intended use. For example, other rights such as publicity, privacy, or moral rights may limit how you use the material.
