Searchable abstracts of presentations at key conferences on calcified tissues
Bone Abstracts (2013) 2 OP10 | DOI: 10.1530/boneabs.2.OP10

ICCBH2013 Oral Posters (1) (15 abstracts)

Metacarpal width, length, medullary diameter and cortical thickness in hypophosphatemic rickets

Signe Sparre Beck-Nielsen 1, , Kim Brixen 1, , Jeppe Gram 4 & Mette Ramsdal Poulsen 1,

1Institute of Clinical Research, University of Southern Denmark, Odense, Denmark; 2Department of Pediatrics, Hospital of Southwest Denmark, Esbjerg, Denmark; 3Department of Endocrinology, Odense University Hospital, Odense, Denmark; 4Department of Endocrinology, Hospital of Southwest Denmark, Esbjerg, Denmark; 5Department of Clinical Radiology, Odense University Hospital, Odense, Denmark.

Hand X-rays from patients with hypophosphatemic rickets (HR) were assessed to evaluate if HR influences the dimensions of the metacarpal bones and the distribution between cortical and cancellous bone. In addition, we aimed to test the hypothesis that HR caused by mutation in DMP1 has a greater impact on bone dimensions than HR caused by mutations in PHEX.

Hand X-rays from 17 children with HR were evaluated. Three children had HR caused by a DMP1 mutation, and the remaining 14 had a verified PHEX mutation. BoneXpert® (Visiana) was used to calculate the dimensions of the second, third and fourth metacarpal bones. HR patients were compared with age- and gender matched healthy children from Switzerland. For the whole HR group, data are reported as mean Z-scores (95% CI) and p value, followed by the mean Z-score in subgroups of patients with a PHEX mutation followed by patients with a DMP1 mutation.

Overall, patients with HR had significantly broader metacarpal bones, +2.5 (95% CI 1.7–3.2) S.D., P<0.001, (2.0; 4.6) S.D., with a wider medullary diameter of +2.4 (95% CI 1.8–2.1) S.D., P<0.001, (2.1; 4.2) S.D. and a reduced cortical thickness of -0.6 (95% CI –1.3 to –0.3) S.D., P<0.02, (−0.8; 0.3) SD. The metacarpal length of +0.7 (0.2–1.2) S.D., P=0.3, (0.4; 1.9) S.D., was not different compared with controls (Fig. 1).

Figure 1 Distribution of metacarpal dimensions in children with PHEX and DMP1 mutations respectively.

Children with HR have significantly broader metacarpal bones, a wider medullary diameter, and decreased cortical thickness. The metacarpal bones in children with a DMP1 mutation appear longer and broader with a wider medullary diameter compared to children with a PHEX mutation. Our data suggest that DMP1 mutations affect bone size more severely than PHEX mutations.


Volume 2

6th International Conference on Children's Bone Health

Rotterdam, The Netherlands
22 Jun 2013 - 25 Jun 2013


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