ICCBH2015 Poster Presentations (1) (201 abstracts)
MRI features as surrogate markers of X-linked hypophosphatemic rickets activity
Marta Lempicki 1 , Anya Rothenbuhler 2 , Valérie Merzoug 1 , Stéphanie Franchi-Abella 1 , Catherine Chaussain 3 , Agnès Linglart 2 & Catherine Adamsbaum 1
1APHP, Department of Pediatric Radiology, Hôpital de Bicêtre and Paris Sud University, Le Kremlin-Bicêtre, France; 2APHP, Department of Pediatric Endocrinology, Reference Center for Rare Disorders of Calcium and Phosphorus Metabolism, Bicêtre Hospital and Paris Sud University, Le Kremlin-Bicêtre, France; 3APHP, Department of Odontology, Bretonneau Hospital and 6EA2496 University Paris Descartes, Paris, France.
Background and objectives: X-linked hypophosphatemic rickets (XLH) is the most common form of inheritable rickets. The treatment of rickets is monitored via laboratory tests such as alkaline phosphatase (ALP), clinical features, and plain X-rays. The objectives of this study were to describe the MRI features in XLH and to look for correlations between those features and XLH activity.
Study design: Twenty-seven patients (younger than 18 years with XLH due to a PHEX mutation) were included in this prospective single-center observational study. All patients consented to an MRI of the distal femur (frontal T1-weighted and 3D SPAIR sequences). XLH activity was assessed using height, leg bowing, dental abscess history and ALP. We looked for correlations between the MRI features and serum ALP and clinical features.
Results: The median maximum width of the physis was 5.6 mm (4.8–7.8), and 82% of the patients had a width >3 mm. The median transverse extent of the widening was 55% (42.9–66.2). Zone of provisional calcification appearance was abnormal on 21 MRIs (78%). Harris lines were present on 24 MRIs (89%). There were bone marrow signal abnormalities on 16 MRIs (59%), predominantly in the epiphysis (14 MRIs). Two patients (7%) presented with an osteochondritis. Serum ALP was correlated with the maximum physeal widening and with the transverse extent of the widening. There were no correlations between the clinical parameters and the MRI features.
Conclusion: MRI of the knee provides precise rickets patterns that are correlated with the biochemical marker of the disease.
Disclosure: The authors declared no competing interests.
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