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

Assiut University

Status

Completed

Conditions

Hemolysis

Thalassemia in Children

Pulmonary Hypertension

Treatments

Diagnostic Test: Tricuspid regurge velocity

Diagnostic Test: blood sample

Study type

Observational

Funder types

Other

Identifiers

NCT03133169

GlnThalassemia

Details and patient eligibility

About

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

Enrollment

80 patients

Sex

All

Ages

10 to 18 years old

Volunteers

Accepts Healthy Volunteers

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.

Trial design

80 participants in 4 patient groups

on chelation

Description:

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

Treatment:

Diagnostic Test: Tricuspid regurge velocity

Diagnostic Test: blood sample

No chelation

Description:

group 2: cases without chelation Diagnostic Test: blood sample Diagnostic Test: Tricuspid regurge velocity

Treatment:

Diagnostic Test: Tricuspid regurge velocity

Diagnostic Test: blood sample

splenectomy

Description:

group 3: cases with splenectomy Diagnostic Test: blood sample Diagnostic Test: Tricuspid regurge velocity