Literature DB >> 35904681

Sickle cell bone disease and response to intravenous bisphosphonates in children.

C Grimbly1,2, P Diaz Escagedo3, J L Jaremko4, A Bruce5,6, N Alos3, M E Robinson7,8, V N Konji8, M Page7,8, M Scharke8, E Simpson7, Y D Pastore3, R Girgis5,6, R T Alexander5,6, L M Ward7,8.   

Abstract

Children with sickle cell disease (SCD) have the potential for extensive and early-onset bone morbidity. This study reports on the diversity of bone morbidity seen in children with SCD followed at three tertiary centers. IV bisphosphonates were effective for bone pain analgesia and did not trigger sickle cell complications.
INTRODUCTION: To evaluate bone morbidity and the response to intravenous (IV) bisphosphonate therapy in children with SCD.
METHODS: We conducted a retrospective review of patient records from 2003 to 2019 at three Canadian pediatric tertiary care centers. Radiographs, magnetic resonance images, and computed tomography scans were reviewed for the presence of avascular necrosis (AVN), bone infarcts, and myositis. IV bisphosphonates were offered for bone pain management. Bone mineral density was assessed by dual-energy X-ray absorptiometry (DXA).
RESULTS: Forty-six children (20 girls, 43%) had bone morbidity at a mean age of 11.8 years (SD 3.9) including AVN of the femoral (17/46, 37%) and humeral (8/46, 17%) heads, H-shaped vertebral body deformities due to endplate infarcts (35/46, 76%), and non-vertebral body skeletal infarcts (15/46, 32%). Five children (5/26, 19%) had myositis overlying areas of AVN or bone infarcts visualized on magnetic resonance imaging. Twenty-three children (8/23 girls) received IV bisphosphonate therapy. They all reported significant or complete resolution of bone pain. There were no reports of sickle cell hemolytic crises, pain crises, or stroke attributed to IV bisphosphonate therapy.
CONCLUSION: Children with SCD have the potential for extensive and early-onset bone morbidity. In this series, IV bisphosphonates were effective for bone pain analgesia and did not trigger sickle cell complications.
© 2022. The Author(s).

Entities:  

Keywords:  (4–6): children; Avascular necrosis; Bisphosphonate; Bone infarcts; Myositis; Sickle cell disease.

Mesh:

Substances:

Year:  2022        PMID: 35904681      PMCID: PMC9568449          DOI: 10.1007/s00198-022-06455-2

Source DB:  PubMed          Journal:  Osteoporos Int        ISSN: 0937-941X            Impact factor:   5.071


  48 in total

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Review 9.  Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members.

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10.  Height adjustment in assessing dual energy x-ray absorptiometry measurements of bone mass and density in children.

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Journal:  J Clin Endocrinol Metab       Date:  2010-01-26       Impact factor: 5.958

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