Erythrocyte Glutamine Level Relation to Pulmonary Hypertension Risk in Beta Thalassemia Major Children
NCT ID: NCT03133169
Last Updated: 2019-08-28
Study Results
The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.
Basic Information
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COMPLETED
80 participants
OBSERVATIONAL
2017-06-01
2019-08-26
Brief Summary
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Detailed Description
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Conditions
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Study Design
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COHORT
CROSS_SECTIONAL
Study Groups
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on chelation
patients with B-thalassemia major samples of which will be examined for renal and liver function, Erythrocyte Glutamine level, ferritin level and complete blood picture. Also Echo will be done for measuring the Tricuspid regurge velocity.
group 1: cases on chelation: deferasirox 500mg oral tablet with initial dose 20 mg/kg guided by ferritin level
Diagnostic Test: blood sample
Diagnostic Test: Tricuspid regurge velocity
blood sample
blood sample for measuring liver and kidney function, CBC, ferritin level and erythrocyte glutamine level
Tricuspid regurge velocity
Tricuspid regurge velocity will be measured by doppler echocardiography denoting the pulmonary hypertension risk in children
No chelation
group 2: cases without chelation
Diagnostic Test: blood sample
Diagnostic Test: Tricuspid regurge velocity
blood sample
blood sample for measuring liver and kidney function, CBC, ferritin level and erythrocyte glutamine level
Tricuspid regurge velocity
Tricuspid regurge velocity will be measured by doppler echocardiography denoting the pulmonary hypertension risk in children
splenectomy
group 3: cases with splenectomy
Diagnostic Test: blood sample
Diagnostic Test: Tricuspid regurge velocity
blood sample
blood sample for measuring liver and kidney function, CBC, ferritin level and erythrocyte glutamine level
Tricuspid regurge velocity
Tricuspid regurge velocity will be measured by doppler echocardiography denoting the pulmonary hypertension risk in children
no splenectomy
group 4: cases without splenectomy Diagnostic Test: blood sample Diagnostic Test: Tricuspid regurge velocity
blood sample
blood sample for measuring liver and kidney function, CBC, ferritin level and erythrocyte glutamine level
Tricuspid regurge velocity
Tricuspid regurge velocity will be measured by doppler echocardiography denoting the pulmonary hypertension risk in children
Interventions
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blood sample
blood sample for measuring liver and kidney function, CBC, ferritin level and erythrocyte glutamine level
Tricuspid regurge velocity
Tricuspid regurge velocity will be measured by doppler echocardiography denoting the pulmonary hypertension risk in children
Eligibility Criteria
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Inclusion Criteria
* PH risk documented by doppler echocardiography, defined as tricuspid regurge velocity (TRV) equal to or greater than 2.5 m/s
Exclusion Criteria
* Hepatic dysfunction (SGPT greater than 3X normal)
* Renal dysfunction (Creatinine greater than 2X normal)
* Patients on sildenafil (Viagra), calcium channel blockers or other drugs for the control of PH.
10 Years
18 Years
ALL
Yes
Sponsors
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Assiut University
OTHER
Responsible Party
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Fatma Sami
Pediatric Resident
Principal Investigators
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Fahim M Fahim, PhD
Role: PRINCIPAL_INVESTIGATOR
Children Hospital, Assiut University
Fatma S AbdElshafi, bachelor's
Role: PRINCIPAL_INVESTIGATOR
Children Hospital, Assiut University
Eman F. Mohamed, PhD
Role: PRINCIPAL_INVESTIGATOR
Children Hospital, Assiut University
Locations
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Assiut University
Asyut, , Egypt
Countries
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References
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Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, Brown B, Coles WA, Nichols JS, Ernst I, Hunter LA, Blackwelder WC, Schechter AN, Rodgers GP, Castro O, Ognibene FP. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med. 2004 Feb 26;350(9):886-95. doi: 10.1056/NEJMoa035477.
Morris CR, Kuypers FA, Kato GJ, Lavrisha L, Larkin S, Singer T, Vichinsky EP. Hemolysis-associated pulmonary hypertension in thalassemia. Ann N Y Acad Sci. 2005;1054:481-5. doi: 10.1196/annals.1345.058.
Morris CR, Vichinsky EP. Pulmonary hypertension in thalassemia. Ann N Y Acad Sci. 2010 Aug;1202:205-13. doi: 10.1111/j.1749-6632.2010.05580.x.
Walter PB, Fung EB, Killilea DW, Jiang Q, Hudes M, Madden J, Porter J, Evans P, Vichinsky E, Harmatz P. Oxidative stress and inflammation in iron-overloaded patients with beta-thalassaemia or sickle cell disease. Br J Haematol. 2006 Oct;135(2):254-63. doi: 10.1111/j.1365-2141.2006.06277.x.
Morris CR. Mechanisms of vasculopathy in sickle cell disease and thalassemia. Hematology Am Soc Hematol Educ Program. 2008:177-85. doi: 10.1182/asheducation-2008.1.177.
Kuypers FA. Membrane lipid alterations in hemoglobinopathies. Hematology Am Soc Hematol Educ Program. 2007:68-73. doi: 10.1182/asheducation-2007.1.68.
Fung EB, Harmatz P, Milet M, Ballas SK, De Castro L, Hagar W, Owen W, Olivieri N, Smith-Whitley K, Darbari D, Wang W, Vichinsky E; Multi-Center Study of Iron Overload Research Group. Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: A report from the multi-center study of iron overload. Am J Hematol. 2007 Apr;82(4):255-65. doi: 10.1002/ajh.20809.
Machado RF, Gladwin MT. Pulmonary hypertension in hemolytic disorders: pulmonary vascular disease: the global perspective. Chest. 2010 Jun;137(6 Suppl):30S-38S. doi: 10.1378/chest.09-3057.
Janda S, Shahidi N, Gin K, Swiston J. Diagnostic accuracy of echocardiography for pulmonary hypertension: a systematic review and meta-analysis. Heart. 2011 Apr;97(8):612-22. doi: 10.1136/hrt.2010.212084. Epub 2011 Feb 25.
De Castro LM, Jonassaint JC, Graham FL, Ashley-Koch A, Telen MJ. Pulmonary hypertension associated with sickle cell disease: clinical and laboratory endpoints and disease outcomes. Am J Hematol. 2008 Jan;83(1):19-25. doi: 10.1002/ajh.21058.
Anthi A, Machado RF, Jison ML, Taveira-Dasilva AM, Rubin LJ, Hunter L, Hunter CJ, Coles W, Nichols J, Avila NA, Sachdev V, Chen CC, Gladwin MT. Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension. Am J Respir Crit Care Med. 2007 Jun 15;175(12):1272-9. doi: 10.1164/rccm.200610-1498OC. Epub 2007 Mar 22.
Hebbel RP. Reconstructing sickle cell disease: a data-based analysis of the "hyperhemolysis paradigm" for pulmonary hypertension from the perspective of evidence-based medicine. Am J Hematol. 2011 Feb;86(2):123-54. doi: 10.1002/ajh.21952.
Morris CR. Vascular risk assessment in patients with sickle cell disease. Haematologica. 2011 Jan;96(1):1-5. doi: 10.3324/haematol.2010.035097. No abstract available.
Mok E, Hankard R. Glutamine supplementation in sick children: is it beneficial? J Nutr Metab. 2011;2011:617597. doi: 10.1155/2011/617597. Epub 2011 Nov 14.
Xu H, Pearl RG. Effect of l-glutamine on pulmonary hypertension in the perfused rabbit lung. Pharmacology. 1994 Apr;48(4):260-4. doi: 10.1159/000139188.
Morris CR, Suh JH, Hagar W, Larkin S, Bland DA, Steinberg MH, Vichinsky EP, Shigenaga M, Ames B, Kuypers FA, Klings ES. Erythrocyte glutamine depletion, altered redox environment, and pulmonary hypertension in sickle cell disease. Blood. 2008 Jan 1;111(1):402-10. doi: 10.1182/blood-2007-04-081703. Epub 2007 Sep 11.
Niihara Y, Matsui NM, Shen YM, Akiyama DA, Johnson CS, Sunga MA, Magpayo J, Embury SH, Kalra VK, Cho SH, Tanaka KR. L-glutamine therapy reduces endothelial adhesion of sickle red blood cells to human umbilical vein endothelial cells. BMC Blood Disord. 2005 Jul 25;5:4. doi: 10.1186/1471-2326-5-4.
Machado RF, Farber HW. Pulmonary hypertension associated with chronic hemolytic anemia and other blood disorders. Clin Chest Med. 2013 Dec;34(4):739-52. doi: 10.1016/j.ccm.2013.08.006. Epub 2013 Oct 17.
Other Identifiers
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GlnThalassemia
Identifier Type: -
Identifier Source: org_study_id
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