title: Vertebral morphometry in children and adolescents with osteogenesis imperfecta: effect of intravenous pamidronate treatment.
abstract: Results in small patient series suggest that cyclical intravenous treatment with pamidronate can lead to reshaping of compressed vertebral bodies in children and adolescents with osteogenesis imperfecta (OI), but more detailed analyses are lacking. In this study of patients with moderate to severe OI (age range 0.1 to 16.7 years), we used vertebral morphometry to longitudinally assess changes in lumbar vertebral shape before (n = 17 patients) and during 2 to 4 years of pamidronate treatment (n = 72 patients). Anterior, posterior and midpoint vertebral heights of lumbar vertebrae L1 to L4 were determined on lateral lumbar spine X-rays and were related to vertebral body length in the antero-posterior direction. Before pamidronate treatment, vertebral body height ratios did not change significantly, but the mean concavity index (defined as the ratio between midpoint and posterior vertebral body heights) decreased by 22% (P = 0.002). Pamidronate treatment was associated with an increase in vertebral height ratio at each of the 12 sites that were analyzed. Consequently, patients who had received pamidronate for an average of 3 years had less compressed vertebrae than a historical control group of patients who had the same OI type, age and sex but who had not received pamidronate. Multiple regression analysis revealed that age was negatively and lumbar spine areal bone mineral density z score was positively associated with vertebral shape at baseline. The main determinant of treatment response was the severity of vertebral deformities at baseline. These results suggest that vertebral deformations worsen in patients with moderate to severe OI who do not receive medical treatment and that pamidronate helps to reverse this trend. In moderate to severe forms of OI, pamidronate should be started as early as possible to treat or to prevent vertebral deformations.
authors.affiliation: Genetics Unit, Shriners Hospital for Children, 1529 Cedar Avenue, Montréal, Québec, Canada H3G 1A6.
authors.forename: Christof
authors.initials: C
authors.lastname: Land
authors.forename: Frank
authors.initials: F
authors.lastname: Rauch
authors.forename: Craig F
authors.initials: CF
authors.lastname: Munns
authors.forename: Solmaz
authors.initials: S
authors.lastname: Sahebjam
authors.forename: Francis H
authors.initials: FH
authors.lastname: Glorieux
mesh_terms: D015502:Absorptiometry, Photon / Q000379:methods; D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000494:pharmacology / Q000627:therapeutic use; D015519:Bone Density / Q000187:drug effects; D002648:Child; D002675:Child, Preschool; D004164:Diphosphonates / Q000494:pharmacology / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008137:Longitudinal Studies; D008159:Lumbar Vertebrae / Q000000981:diagnostic imaging / Q000187:drug effects / Q000473:pathology; D008297:Male; D010013:Osteogenesis Imperfecta / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D000077268:Pamidronate; D012044:Regression Analysis; D013131:Spine / Q000000981:diagnostic imaging / Q000187:drug effects* / Q000473:pathology; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D004164:Diphosphonates; D000077268:Pamidronate