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Bone Abstracts (2019) 7 P19 | DOI: 10.1530/boneabs.7.P19

Department of Pediatrics, Neonatal Pathology and Bone Metabolic Diseases, Łódź, Poland.

Fractures of long bone and ribs in the neonatal period may be expression of genetic disturbances of collagen type I production. The aim of the study was to present clinical symptoms, laboratory, radiological and densitometric data in 27 newborns with osteogenesis imperfecta type III.

Methods: Medical history, clinical examination and radiographs of 27 children hospitalized in the Department of Paediatrics, Neonatal Pathology and Bone Metabolism Diseases were evaluated. Serum levels of 25-hydroxyvitamin D (25OHD) and osteocalcin (bone formation marker) of the patients were measured. Assessment of urine excretion of bone resorption marker D-Pyrylinks was made. All of the newborns were qualified to pamidronate therapy and the treatment was commenced. After the first cycle of pamidronate, densitometric examination in Infant programme by dual-X-Ray absorptiometry (DXA method) was performed.

Results: In every family of evaluated patients osteogenesis imperfecta occurred by the first time. In clinical examination the disease was manifested by deformations in body proportions: shortened extremities, sabre shanks, flabby skull bones and reduction in activity. 23 of 27 children had blue sclera. In all X-ray baby-graphs bone fractures, developed in utero as well as after birth, were found. From biochemical tests only a few abnormalities were observed, such as lowered concentration of serum 25OHD in 9 of 27 patients. Due to orthopedic care of the multiple fractures DXA method is hard to obtain during first hospitalization, so that it is performed before second cycle of pamidronate.

Conclusion: 1. Osteogenesis imperfecta is one of the reasons of bone fractures in neonates and its diagnosis is based on family history, clinical manifestation and X-ray examination.

2. Based on our experience Dual X-ray absorptiometry is useful for the pamidronate therapy monitoring, not for making the diagnosis of osteogenesis imperfecta.

Disclosure: The authors declared no competing interests.

Volume 7

9th International Conference on Children's Bone Health


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