Enamel and dentin mineralization in familial hypophosphatemic rickets: a micro-CT study
نویسندگان
چکیده
منابع مشابه
Dentin noncollagenous matrix proteins in familial hypophosphatemic rickets.
Familial hypophosphatemic rickets is transmitted in most cases as an X-linked dominant trait and results from the mutation of the PHEX gene predominantly expressed in osteoblast and odontoblast. Patients with rickets have been reported to display important dentin defects. Our purpose was to explore the structure, composition and distribution of noncollagenous proteins (NCPs) of hypophosphatemic...
متن کاملFamilial Hypophosphatemic Rickets: Pathophysiology and Medical Management
Chronic hypophosphatemia is one major cause of rickets and osteomalacia in growing children (see Preface of this volume). There are acquired and congenital forms. In most instances, the acquired forms can be controlled by acting on the underlying cause (insufficient phosphate intake, increased renal loss secondary to a mesenchymal tumor, or an altered renal tubular function). The inherited synd...
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Introduction Familial hypophosphatemic or X-linked hypophosphatemic (XLH) rickets is the most common form of non-nutritional rickets1. The prevalence of XLH rickets yet remain unknown in Bangladesh. It is an Xlinked dominant disorder characterized by renal phosphate wasting with consequent defect of bone mineralization1. Some form of the disease are observed to be transmitted which followed an ...
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In a case of familial hypophosphatemic rickets, marked bone thickening caused narrowing of the optic canals, resulting in bilateral optic atrophy. The case also showed metastatic calcification in the walls of both globes.
متن کاملTertiary hyperparathyroidism during high phosphate therapy of familial hypophosphatemic rickets.
We report the development of severe tertiary hyperparathyroidism in three girls treated for familial hypophosphatemic rickets and characterize parathyroid function in vivo and in vitro. All patients had been previously treated with relatively large doses of inorganic phosphorus (125 mm/day) and ergocalciferol or calcitriol for several years and had radiographic evidence of long-standing hyperpa...
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ژورنال
عنوان ژورنال: Dentomaxillofacial Radiology
سال: 2015
ISSN: 0250-832X,1476-542X
DOI: 10.1259/dmfr.20140347