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Early bisphosphonate treatment in infants with severe osteogenesis imperfecta
Franco Antoniazzi1, Giorgio Zamboni, Silvana Lauriola
1Pediatric Clinic and Rheumatological Rehabilitation, University of Verona, Verona, Italy. franco.antoniazzi@univr.it
Insights
Early bisphosphonate treatment for osteogenesis imperfecta (OI) in infants improves growth and reduces fractures. Starting neridronate therapy at birth, rather than after six months, yields superior outcomes in severe OI cases.
Area of Science:
- Pediatric Endocrinology
- Orthopedics
- Pharmacology
Background:
- Osteogenesis imperfecta (OI) is a genetic disorder characterized by fragile bones and frequent fractures.
- Severe forms of OI, such as type III, present significant challenges in growth and skeletal development.
- Bisphosphonates are a class of drugs known to inhibit bone resorption and are used in OI treatment.
Purpose of the Study:
- To prospectively assess the efficacy of bisphosphonate (neridronate) treatment in infants with severe osteogenesis imperfecta (OI).
- To compare the outcomes of early-initiated (at birth) versus delayed-initiated (at 6 months) neridronate therapy.
- To evaluate the impact of neridronate on growth, fracture incidence, and biochemical markers in infants with OI.
Main Methods:
- A prospective study involving 10 infants with OI type III, divided into two groups: Group A (treatment at birth) and Group B (treatment at 6 months).
- A historical control group (Group C) of untreated children with OI was used for comparison.
- Measurements included weight, length, fracture count, and various serum and urinary biochemical markers, along with vertebral radiography.
Main Results:
- Group A demonstrated superior growth and a lower fracture incidence compared to Groups B and C within the first six months.
- Both treated groups (A and B) showed reduced fracture rates compared to the control group (C) after 12 months.
- Neridronate treatment led to increased osteocalcin and IGF-I levels, decreased urinary Ca/Cr and NTx/Cr ratios, and improved vertebral bone structure, particularly in Group A.
Conclusions:
- Cyclical neridronate treatment initiated at birth positively impacts growth and fracture rates in infants with severe OI.
- Early intervention with bisphosphonates is crucial for optimizing therapeutic benefits in pediatric osteogenesis imperfecta.
- Neridronate therapy shows promise in improving skeletal health and reducing fracture burden in infants with severe OI.
Objective:
To evaluate prospectively the efficacy of bisphosphonate treatment in infants with severe forms of osteogenesis imperfecta (OI).
Study Design:
Of 10 children (6 females) with OI type III, 5 (group A) started treatment (2 mg/kg neridronate administered intravenously for 2 consecutive days, every 3 months) just after diagnosis at birth and 5 (group B) after 6 months. Ten untreated children, matched for sex, age, and clinical severity of OI, constituted a historical control group (group C). We measured weight, length, and number of fractures every 3 months and serum and urinary levels of calcium, phosphorus, creatinine, serum alkaline phosphatase, 25-hydroxyvitamin D, insulin-like growth factor I, parathyroid hormone, and osteocalcin, urinary type I collagen N-terminal telopeptide, and lateral radiography of vertebral column every 6 months.
Results:
Group A had better growth and a lower incidence of fractures than groups B and C in the first 6 months of treatment. In the second 6 months, both groups A and B had lower fracture rates than group C. After 12 months of therapy, osteocalcin and insulin-like growth factor I levels significantly increased only in group A. The urinary Ca/Cr ratio and N-terminal telopeptide/Cr ratio significantly declined only in treated patients. Vertebral body area and the structure of vertebral bodies improved in all treated patients, but especially in group A.
Conclusions:
Cyclical neridronate treatment, started just after diagnosis at birth, had positive effects on growth and fracture rate.
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