Bone densitometry measurements in children with neurofibromatosis Type 1 using quantitative computed tomography

Introduction: Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disease characterised by multisystemic involvement, including bone tissue. Deformities and reduced bone mass are the main bone manifestations in NF1. Quantitative computed tomography (QCT) provides true volumetric b...

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Bibliographic Details
Published in:Singapore medical journal 2022-09, Vol.63 (9), p.520-526
Main Authors: Tezol, Ozlem, Balcı, Yuksel, Alakaya, Mehmet, Gundogan, Begumhan, Cıtak, Elvan
Format: Article
Language:English
Subjects:
Bone densitometry
Bones
Children
Density
Measurement
Neurofibromatosis
Original
Pediatrics
Phosphatases
Phosphates
Physiological aspects
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Summary:Introduction: Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disease characterised by multisystemic involvement, including bone tissue. Deformities and reduced bone mass are the main bone manifestations in NF1. Quantitative computed tomography (QCT) provides true volumetric bone mineral density (BMD) measurement. This study aimed to evaluate bone metabolism parameters and BMD in children with NF1 using QCT. Methods: The data of 52 paediatric NF1 patients (23 female, 29 male) was evaluated retrospectively. We investigated anthropometric measurements, biochemical parameters like total calcium, phosphate, magnesium, alkaline phosphatase, 25-hydroxyvitamin D (25OHD), parathyroid hormone, calcitonin, urinary calcium/creatinine ratio, and QCT parameters like lumbar trabecular and cortical BMD, trabecular area and cortical thickness. Comparisons of gender and puberty status were performed. Results: 25% of patients had skeletal deformities and 42.3% had 25OHD inadequacy (
ISSN:0037-5675
2737-5935
DOI:10.11622/smedj.2021052