title: Tartrate-Resistant Acid Phosphatase 5b in Young Patients With Sickle Cell Disease and Trait Siblings: Relation to Vasculopathy and Bone Mineral Density.
abstract: Bone involvement is a frequent cause of acute morbidity in sickle cell disease (SCD). Tartrate-resistant acid phosphatase 5b (TRACP 5b), a bone resorption marker, is produced specifically by activated osteoclasts. We assessed bone mineral density (BMD) in 30 young patients with SCD and 17 asymptomatic patients with sickle cell trait (SCT) compared with 32 healthy controls and determined TRACP 5b levels in relation to vascular complications. Serum ferritin, alkaline phosphatase (ALP), and TRACP 5b were measured. Echocardiography was performed with assessment of BMD using dual energy X-ray absorptiometry (DXA). The BMD was decreased in patients with SCD compared with SCT and controls (P = .005), with no significant difference between the latter 2 groups. Patients with SCD had higher incidence of bone complications than SCT group and controls (P = .03). The SCD group with abnormal DXA scan had higher ferritin and ALP than normal BMD. Serum TRACP 5b was significantly higher in patients with SCD than SCT and controls (P = .003). The TRACP 5b levels were associated with severe vaso-occlusive crisis (P = .022). Patients treated with hydroxyurea and those on chelation therapy had lower TRACP 5b levels than untreated patients. The TRACP 5b level was positively correlated with lactate dehydrogenase, while there was no relation with ferritin, ALP, or BMD. We suggest that bone complications frequently occur in SCD as reflected by low BMD and high ALP and TRACP 5b. Hemolysis and iron overload may be involved in the occurrence of these complications. The lack of correlation between abnormal DXA scan and high TRACP 5b suggests that bone disease in SCD is multifactorial.
authors.affiliation: Pediatrics Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Galila Mohamed
authors.initials: GM
authors.lastname: Mokhtar
authors.affiliation: Pediatrics Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt azatantawy@hotmail.com.
authors.forename: Azza Abdel Gawad
authors.initials: AA
authors.lastname: Tantawy
authors.affiliation: Pediatrics Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Ahmed Al-Saeed
authors.initials: AA
authors.lastname: Hamed
authors.affiliation: Pediatrics Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Amira Abdel Moneam
authors.initials: AA
authors.lastname: Adly
authors.affiliation: Clinical Pathology Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Eman Abdel Rahman
authors.initials: EA
authors.lastname: Ismail
authors.affiliation: Pediatrics Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Sara Mostafa
authors.initials: SM
authors.lastname: Makkeyah
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000401:mortality; D015519:Bone Density; D001862:Bone Resorption; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D035781:Siblings; D000071681:Tartrate-Resistant Acid Phosphatase / Q000378:metabolism*
chemical_list: D000071681:Tartrate-Resistant Acid Phosphatase
keywords: ALP; TRACP 5b; bone mineral density; sickle cell disease; vasculopathy