ESPE Abstracts (2022) 95 P1-26

1Division of Pediatric Endocrinology, Dokuz Eylül University, Faculty of Medicine, İzmir, Turkey; 2Division of Pediatric Radiology, Dokuz Eylül University, Faculty of Medicine, İzmir, Turkey; 3Division of Pediatric Hematology, Dokuz Eylül University, Faculty of Medicine, İzmir, Turkey; 4Division of Pediatric Rheumatology, Dokuz Eylül University, Faculty of Medicine, İzmir, Turkey; 5Division of Pediatric Nephrology, Dokuz Eylül University, Faculty of Medicine, İzmir, Turkey

Background: Osteonecrosis (ON) is bone death caused by inadequate blood supply resulting in demineralization and trabecular thinning and, subsequently, mechanical failure. Although the pathophysiology of ON is not fully understood, the use of high-dose glucocorticoid (GC) is one of the triggers. Furthermore, its optimal management remains uncertain. The use of bisphosphonates (BP) for the treatment of ON has been reported, however, data on outcomes are limited.

Method: The patients who were evaluated in our unit regarding ON during the last two years were included. Data regarding clinical, laboratory, and imaging studies MRI, and bone mineral density measurements (BMD) were recorded before and one year after treatment (reevaluation). The severity of the clinical picture was assessed using Common Terminology Criteria for Adverse Events (CTCAE) criteria (between 1 (asymptomatic) and 5(death)). Patients were treated with pamidronate (1mg/kg/day, with a maximum dose of 60 mg/day, for two days, quarterly) and, when needed, calcium, vitamin D, and physiotherapy.

Results: There were four female patients (Patient 1, acute lymphoblastic leukemia treated with bone marrow transplantation; Patient 2, immune thrombocytopenic purpura, Patients 3 and 4, systemic lupus erythematosus) aged between 10 and 17 years. Only Patient 1 was prepubertal. According to CTCAE, ON was consistent with Grade 3 (Severe symptoms; limiting self-care activities of daily living; elective operative intervention indicated) and multifocal in lower limbs in all of them. Surgical core decompression in Patient 4, which had been performed before pamidronate, did result in clinical improvement. All of them had been treated with high-dose GC. None of them had any fractures. Calcium, phosphorus, alkaline phosphatase (ALP), vitamin D, and parathyroid hormone levels were normal in all patients before, during, and one year after pamidronate administration. The BMD SD scores were all normal for P1, P2, and P4. Patient 3 had a normal BMD SD score (1.2 SDS) before treatment, but it increased to 2.9 SD score at reevaluation. No other side effects related to the use of bisphosphonates were observed. At the time of reevaluation, the lesions were stable on MRI and their clinical condition was consistent with Grade 1 according to CTCAE (Asymptomatic; clinical or diagnostic observations only; intervention not indicated).

Conclusion: Pamidronate may be an effective treatment for the improvement of symptoms related to ON.

Volume 95

60th Annual ESPE (ESPE 2022)

Rome, Italy
15 Sep 2022 - 17 Sep 2022

European Society for Paediatric Endocrinology 

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