Learning objectives
To illustrate the imaging of musculoskeletal manifestations of sickle cell disease (SCD) in 97 children followed in our center.
To demonstrate usefulness of imaging in the diagnosis of the various musculoskeletal manifestations in SCD.
Background
Sickle cell disease disease is one of a group of genetic disorders known as hemoglobinopathies,
and is characterized by the inheritance of sickle haemoglobin (HbS).
A mutation in the beta-globin gene on 11th chromosome,
resulting in replacement of glutamic acid in position 6 of the beta-globin chain by valine,
resulting in an abnormal haemoglobin HbS molecule.
The term sickle cell disease applies to those patients who have at least one abnormal HbS chain and another abnormal beta chain.
If the second abnormal beta chain is...
Imaging findings OR Procedure Details
This retrospective study reports the osteo-articular manifestations associated with sickle-cell disease encountered in our institution,
in children with at least one hematologic appointment since 2013 till the end of 2014.
Osteo-articular manifestations with imaging findings were reported in 28 patients out of 97.
There were 14 females and 14 males. There were 23 homozygous (SS) patients and 5 composite heterozygous (SC,
Sb) out of 28 patients with osteo-articular manifestations.
These cases were retrospectively studied and we depicted 11 major manifestations like infections and avascular necrosis....
Conclusion
Familiarity with the imaging features of SCD is important for the diagnosis and management of musculoskeletal complications.
Acute osteomyelitis may be particularly difficult to distinguish from bone infarction,
and MRI could be an important tool in those cases.
References
1-Wang WC,
Sickle cell anemia and other sickling syndromes.
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Foerster J,
Rodgers GM,
Paraskevas F,
Glader B,
Arber DA,
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Wintrobe’s Clinical Hematology.
12th ed .
Philadelphia: Lippincott Williams and Wilkins; 2009.
p1038-82.
2-Schnong JB,
Duits AJ,
Muskiet FAJ et al.
Sickle cell disease; a general overview.
Neth J Med 2004; 62 : 364-374.
3-Junior G,
Daher E,
Rocha F.
Osteoarticular involvement in sickle cell disease.
Rev Bras Hematol Hemoter 2012; 34 (2):156-64.
4-Almeida A,
Roberts I.
Bone involvement...
Personal Information
Pedro Luís Figueiredo Pegado
MD,
Radiology Lecturer at New University of Lisbon,
Faculty of Medicine.
Department of Radiology,
Centro Hospitalar Lisboa Central – Hospital de São José,
Rua Antonio Serrano 1000-199,
Lisboa,
Portugal
[email protected]
Sara Serpa
MD
Department of Radiology,
Hospital do Divino Espírito Santo -Av.
D.
Manuel I,
9500-370 Ponta Delgada,
Portugal
Rosário Perry
MD
Department of Pediatrics,
Centro Hospitalar Lisboa Central - Hospital Dona Estefânia,R.
Jacinta Marto,
1169-045 Lisboa
Carina Ruano
MD,
Radiology Lecturer at New University of Lisbon,
Faculty of Medicine.
Department...