Avascular Bone Necrosis in Sickle Cell Disease: a Pediatric Study. (OsteoSCD)

San Luigi Gonzaga Hospital

Status: Completed

Conditions: Sickle Cell Disease

Study type: Observational

Funder types: Other

Identifiers

NCT05203991

Osteonecrosis SCD

Details and patient eligibility

About

Avascular necrosis (AVN) is a serious complication of sickle cell disease, especially in pediatric patients where the prevalence is between 3% and 8% and are more frequent in patients with multiple vaso-occlusive crisis (VOC). The prevalence of AVN is usually made by a study of the hip through radiography, whereas other possible sites of ischemic infarcts are evaluated only in case of specific symptoms. In addition, bone infarcts may be the trigger for additional VOC.

In this study, we want to investigate the presence of possible bone lesions even in asymptomatic or paucisymptomatic children.

This is a prospective interventional and monocentric study whose objective is to describe the prevalence of osteonecrosis in children with sickle cell disease in Italy

Enrollment: 32 patients

Sex: All

Ages: 7 to 17 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Female or male patients diagnosed with sickle cell anemia (HbSS, HbS/β0, HbS/β+, HbSC) with or without known avascular lesions
• Age ≥ 7 and < 18 years at the moment of signed consent
• Written informed consent/assent, according to local guidelines, signed by patient and/or guidelines, signed by the patient and/or at least one parent or legal guardian

Exclusion criteria

• • Any contraindication to perform Nuclear Magnetic Resonance Magnetic Resonance Imaging (MRI)
• Inability to obtain informed consent/assent