ESPE Abstracts

Introduction: Primary osteoporosis (POP) is a rare and important problem in childhood that can cause serious skeletal deformities and morbidity. In this study, we aimed to reveal the spectrum of POP in childhood, and also to assess the effectiveness and safety of bisphosphonates in increasing BMD, reducing fractures, and improving quality of life.

Method: Patients with POP and who received at least one course of pamidronate (PA) or zolendronic acid (ZA) between 2000 and 2020 were included in the study. Patients were divided into two groups as OI and other POP patients. We retrospectively enrolled demographic, anthropometric, clinical, laboratory, and radiological findings. Bone densitometer parameters, activation scores, pain status, deformity status, number of fractures per year were evaluated in all patients at admission, and after the treatment.

Results: Of 31; 21 OI, and 3 spondyloocular syndromes, 2 Bruck Syndrome, 5 idiopathic juvenile osteoporosis were included. The mean age at diagnosis in our patients (F/M = 17/14) was 6.95 ± 4.53 [5.29 ± 3.97 for OI] years. Patients with OI were diagnosed at a younger age. They had similar height and BMD with the other POP group. Thirteen of these had a family history of OI. According to the expanded Sillence classification, 10 were type I, 5 were type III, 5 were was type IV, and 1 was type VI OI. All patients with POP had received bisphosphonate therapy. Of 21 had received PA treatment, while only 4 received ZA and 6 of them switched from PA to ZA. At the end of the treatment, the total BMD (g /cm²) increased from 0.31 ± 0.11 to 0.53 ± 0.13. Mean BMD Z score increased from -4.04 ± 2.08 to -0.95 ± 1.34, and height adjusted Z score increased from -3.39 ± 1.30 to -0.95 ± 1.34. There was no difference between the two groups in terms of improvement in BMD increase. The number of fractures per year decreased from 2.28 ± 2.67 to 0.29 ± 0.69. The activation score increased from 2.81 ± 1.47 to 3.16 ± 1.48. The pain decreased significantly. There was no difference in BMD increase treated with PA or ZA.

Conclusion: POP is a heterogeneous problem that most often arises as a result of OI. Those with OI were diagnosed at an earlier age with severe deformity and fractures. Bisphosphonate treatment (PA or ZA) increased both BMD and improved quality of life in all with POP.