Erythrocyte Glutamine Level Relation to Pulmonary Hypertension Risk in Beta Thalassemia Major Children

Study Overview
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Brief Summary
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The study will investigate the relation between erythrocyte glutamine/glutamate ratio and pulmonary hypertension risk in Egyptian thalassemic children in Assiut University Children Hospital

Official Title
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Erythrocyte Glutamine Level Relation to Pulmonary Hypertension Risk in Beta Thalassemia Major Children in Assiut University Children Hospital

Conditions
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Thalassemia in Children, Pulmonary Hypertension, Hemolysis

Intervention / Treatment
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* Diagnostic Test: blood sample
* Diagnostic Test: Tricuspid regurge velocity


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Established diagnosis of Thalassemia. PH risk documented by doppler echocardiography, defined as tricuspid regurge velocity (TRV) equal to or greater than 2.5 m/s Exclusion Criteria: Acute crisis or hospitalization within 1 month of enrollment 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.

Ages Eligible for Study
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Minimum Age: 10 Years
Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| on chelation<br>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 | Diagnostic Test: blood sample<br>* blood sample for measuring liver and kidney function, CBC, ferritin level and erythrocyte glutamine level<br>Diagnostic Test: Tricuspid regurge velocity<br>* Tricuspid regurge velocity will be measured by doppler echocardiography denoting the pulmonary hypertension risk in children<br>|
| No chelation<br>group 2: cases without chelation Diagnostic Test: blood sample Diagnostic Test: Tricuspid regurge velocity | Diagnostic Test: blood sample<br>* blood sample for measuring liver and kidney function, CBC, ferritin level and erythrocyte glutamine level<br>Diagnostic Test: Tricuspid regurge velocity<br>* Tricuspid regurge velocity will be measured by doppler echocardiography denoting the pulmonary hypertension risk in children<br>|
| splenectomy<br>group 3: cases with splenectomy Diagnostic Test: blood sample Diagnostic Test: Tricuspid regurge velocity | Diagnostic Test: blood sample<br>* blood sample for measuring liver and kidney function, CBC, ferritin level and erythrocyte glutamine level<br>Diagnostic Test: Tricuspid regurge velocity<br>* Tricuspid regurge velocity will be measured by doppler echocardiography denoting the pulmonary hypertension risk in children<br>|
| no splenectomy<br>group 4: cases without splenectomy Diagnostic Test: blood sample Diagnostic Test: Tricuspid regurge velocity | Diagnostic Test: blood sample<br>* blood sample for measuring liver and kidney function, CBC, ferritin level and erythrocyte glutamine level<br>Diagnostic Test: Tricuspid regurge velocity<br>* Tricuspid regurge velocity will be measured by doppler echocardiography denoting the pulmonary hypertension risk in children<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Erythrocyte glutamine level | marker for oxidative stress | 2 months | 
| Tricuspid regurge velocity | Measures the risk of pulmonary hypertension | 2 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Plasma glutamine level | Assess the glutamine level at each visit | 2 months | 
| Liver function tests | Evaluates the state of liver | 2 months | 
| Renal function tests | Evaluates the state of kidney | 2 months | 
| Ferritin level | measuring the iron overload | 2 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Thalassemia, Glutamine/glutamate ratio, Pulmonary hypertension