Ignite Creation Date:
2024-05-06 @ 7:36 PM
Last Modification Date:
2024-10-26 @ 3:10 PM
Study NCT ID:
NCT06069089
Status:
NOT_YET_RECRUITING
Last Update Posted:
2023-10-05
First Post:
2023-09-29
Brief Title:
Bone Denisty Change in Children With Beta Thalassemia Major
Sponsor:
Marwa Hassan Abdelhamed Hassan
Organization:
Assiut University
Study Overview
Official Title:
Assiut University Pediatrician Department
Status:
NOT_YET_RECRUITING
Status Verified Date:
2023-09
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
Brief Summary:
Bone denisty changes in children with beta thalassemia major
Detailed Description:
Beta Thalassemia major TM is a hereditary disease caused by defective Beta globin chain synthesis resulting in abnormal as well as a decreased quantity of globin chains ineffective erythropoiesis haemolysis and increased red blood cell turnover Cooley etal 1927 described the first patient with anemia splenomegaly cranial facial bone enlargement Pathophysiology of bone denisity changes in beta thalassemia major Several studies had been previously evaluated shown that multiple factors may act in concert to produce bone disease in beta thalassemia major TM including bone marrow expansion Shamshirsaz etal 2003 hypogonadism AnapliotouSakaJensen1998 defective growth hormone-insulin-like growth factor-1 GH-IGF-1 axis Solimanetal1998 altered pattern of cytokines Morabitoetal2007 iron deposit in bone Bordatetal1993deferoxamine bone toxicity Chan etal 2002and vitamin D deficiency Dandona etal 1987 Some of these pathogenic factors directly andor indirectly affect osteoblastic population leading to depressed bone formation while others often increase osteoclastic bone resorption
Complications of transfusion dependent poorly controlled beta thalassemia major are1-Osteoprosis Iron overload impairs osteoid maturation and inhibits local mineralization to form focal osteomalacia In addition integration of iron in calcium hydroxyapatite affects the growth of crystals which causes mineralization failure Chan etal 2002 defective growth hormone-insulin-like growth factor-1 GH-IGF-1 axis Soliman etal 1998altered pattern of cytokines Morabitoetal 2007 iron deposit in bone Bordat etal 1993 deferoxamine bone toxicity Chan etal2002 and vitamin D deficiency Dandona etal 1987 2-Fractures The introduction of red blood cell transfusion and concomitant iron chelation therapy has led to improved bone health through various mechanisms It leads to a reduction in medullary expansion and cortical bone thinning the reduced incidence of hypogonadism and a reduction in other endocrine complications such as hypoparathyroidism and metabolic disorders that predispose to low bone density and fractures Multicentre study italian working group 1995 Z-score of bone density will be calculated Z score is the preferred parameter in children which is calculated as the number of standard deviations above or below the mean for the patients age sex
Complications of transfusion dependent poorly controlled beta thalassemia major are1-Osteoprosis Iron overload impairs osteoid maturation and inhibits local mineralization to form focal osteomalacia In addition integration of iron in calcium hydroxyapatite affects the growth of crystals which causes mineralization failure Chan etal 2002 defective growth hormone-insulin-like growth factor-1 GH-IGF-1 axis Soliman etal 1998altered pattern of cytokines Morabitoetal 2007 iron deposit in bone Bordat etal 1993 deferoxamine bone toxicity Chan etal2002 and vitamin D deficiency Dandona etal 1987 2-Fractures The introduction of red blood cell transfusion and concomitant iron chelation therapy has led to improved bone health through various mechanisms It leads to a reduction in medullary expansion and cortical bone thinning the reduced incidence of hypogonadism and a reduction in other endocrine complications such as hypoparathyroidism and metabolic disorders that predispose to low bone density and fractures Multicentre study italian working group 1995 Z-score of bone density will be calculated Z score is the preferred parameter in children which is calculated as the number of standard deviations above or below the mean for the patients age sex
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None