الفهرس 
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Abstract
recovery or removal of the toxin. Urinary NAG activity can be used in conjunction with other tests to assess disease activity and prognosis.
The present study aims to evaluate the prevalence of renal dysfunction in Patients with β-thalassemia (β-T) major.
The study included 84 transfusion dependent betathalassemia major patients (45 males and 39 females), aged 10 - 25 years (mean 18.38 ± 4.257 years), patients were followed up in the Pediatric Hematology-Oncology Clinic,Children’s Hospital, Ain Shams University :
The study also included 50 controls matching the
patients in age and sex .
All patients were subjected to:
Full history taking, through clinical examination and laboratory investigation including: Urinary N-acetyl beta-Dglucosaminidase
(NAG), Urinary β2 micro globulin, serum
Ferritin, serum Creatinine, Glomerular filtration rate, urinary protein,Creatinine clearance.
The Studied Patients were divided into 2 groups
according to chelators: First group it consisted of 51 patients on DFO chelation therapy.
Second group: It consisted of 33 patients on combined chelation therapy as DFO and ferriprox.
The study Shows highly significant difference
between patients chelated with DFO and patients chelated with combined chelating as regards laboratory parameters (serum ferritin, NAG, NAG Creat).
There is no difference between the two groups as regard the anthropometric measures also there is no difference between the two groups as regard the serum Creatinine,Creatinine clearance, serum protein.
Our study showed elevated UNAG and urinary β2MG in patients with β-thalassemia major compared to controls indicated evidence of renal dysfunction in this patients.
Our study revealed that, there was positive significant correlation between UNAG &anthropometric measures as (age,weight and hight) and laboratory of (S Ferritin,95
Beta thalassemia is the commonest type of thalassemia usually produce severe anemia in their homozygous and compound heterozygous form. The use of regular and frequent blood transfusion in thalassemia has improved life span and quality of life of the patients, but it lead to chronic iron overload.
Severely affected patients are treated by blood
transfusion every 3-4 weeks, which results in iron overload in various tissue including the liver heart and endocrine tissue.
The kidneys are another site of iron accumulation in thalassemia. Unlike in the other organs, it is unclear whether kidney affection results solely from intravascular hemolysis,chronic transfusion or as a complication of iron chelation therapy.
Beta2-Microglobulin (beta2MG), an interesting and underutilized metabolite, can be used in assessing renal function, particularly in patients suspected of having renal tubulointerstitial disease.
The assay of urinary N-acetyl-beta-D-glucosaminidase NAG) provides an early indication of tubular dysfunction resulting from renal disease or nephrotoxic damage. False
positives are rare and its activity remains high during active disease or a sustained toxic insult but falls to normal levels on95 Beta thalassemia is the commonest type of thalassemia usually produce severe anemia in their homozygous and
compound heterozygous form. The use of regular and frequent blood transfusion in thalassemia has improved life span and quality of life of the patients, but it lead to chronic iron overload.
Severely affected patients are treated by blood
transfusion every 3-4 weeks, which results in iron overload in various tissue including the liver heart and endocrine tissue.
The kidneys are another site of iron accumulation in thalassemia. Unlike in the other organs, it is unclear whether kidney affection results solely from intravascular hemolysis,chronic transfusion or as a complication of iron chelation therapy.
Beta2-Microglobulin (beta2MG), an interesting and underutilized metabolite, can be used in assessing renal function, particularly in patients suspected of having renal tubulointerstitial disease.
The assay of urinary N-acetyl-beta-D-glucosaminidase NAG) provides an early indication of tubular dysfunction resulting from renal disease or nephrotoxic damage. False
positives are rare and its activity remains high during active disease or a sustained toxic insult but falls to normal levels on recovery or removal of the toxin. Urinary NAG activity can be
used in conjunction with other tests to assess disease activity and prognosis.
The present study aims to evaluate the prevalence of renal dysfunction in Patients with β-thalassemia (β-T) major.
The study included 84 transfusion dependent betathalassemia major patients (45 males and 39 females), aged 10 - 25 years (mean 18.38 ± 4.257 years), patients were followed up in the Pediatric Hematology-Oncology Clinic,Children’s Hospital, Ain Shams University :
The study also included 50 controls matching the
patients in age and sex .
All patients were subjected to:
Full history taking, through clinical examination and laboratory investigation including: Urinary N-acetyl beta-Dglucosaminidase (NAG), Urinary β2 micro globulin, serum Ferritin, serum Creatinine, Glomerular filtration rate, urinary
protein,Creatinine clearance.
The Studied Patients were divided into 2 groups
according to chelators: First group it consisted of 51 patients on DFO chelation therapy.
Second group: It consisted of 33 patients on combined chelation therapy as DFO and ferriprox.
The study Shows highly significant difference
between patients chelated with DFO and patients chelated with combined chelating as regards laboratory parameters (serum ferritin, NAG, NAG Creat).
There is no difference between the two groups as regard the anthropometric measures also there is no difference between the two groups as regard the serum Creatinine,Creatinine clearance, serum protein.
Our study showed elevated UNAG and urinary β2MG in patients with β-thalassemia major compared to controls indicated evidence of renal dysfunction in this patients.
Our study revealed that, there was positive significant correlation between UNAG &anthropometric measures as (age,weight and hight) and laboratory of (S Ferritin,S
urea/Creat, NAG/Creat, and S Na).