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Imaging of malignant infantile osteopetrosis before and after bone marrow transplantation

Malignant infantile osteopetrosis (MIOP) is a sclerosing bone disease caused by absence or defective function of osteoclasts. Since these are of haemopoietic origin, the disease can be cured by allogeneic stem-cell transplantation, but there are no detailed studies of radiological follow-up of these...

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Published in:Pediatric radiology 2001-12, Vol.31 (12), p.869-875
Main Authors: CHEOW, Heok K, STEWARD, Colin G, GRIER, David J
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STEWARD, Colin G
GRIER, David J
description Malignant infantile osteopetrosis (MIOP) is a sclerosing bone disease caused by absence or defective function of osteoclasts. Since these are of haemopoietic origin, the disease can be cured by allogeneic stem-cell transplantation, but there are no detailed studies of radiological follow-up of these procedures. To investigate the radiological findings at presentation and follow-up in children undergoing bone marrow transplantation (BMT) for MIOP. Examination of the records and imaging studies of nine paediatric patients undergoing BMT for MIOP during 1988-2000. Presentation findings included characteristic features such as fractures, subperiosteal new bone formation and rachitic appearances. Five children engrafted successfully, allowing assessment of the nature and speed of resolution of radiological features after transplantation. Radiological improvement was apparent within 2 months of successful engraftment with almost complete resolution of abnormalities after 1 year. Studies in two children who are, respectively, 58 and 83 months post-transplant show complete resolution of all bone changes.
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source Springer Nature
subjects Biological and medical sciences
Bone Marrow Transplantation
Diseases of the osteoarticular system
Extremities - diagnostic imaging
Female
Follow-Up Studies
Humans
Infant
Infant, Newborn
Male
Malformations and congenital and or hereditary diseases involving bones. Joint deformations
Medical sciences
Osteopetrosis - diagnostic imaging
Osteopetrosis - therapy
Pelvis - diagnostic imaging
Radiography
Ribs - diagnostic imaging
Time Factors
title Imaging of malignant infantile osteopetrosis before and after bone marrow transplantation
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