Abstrakt: |
Dentin mineralization defects were found across all regions of dentin in affected teeth: outer mantle dentin, circumpulpal dentin, and the most recently formed proximal pulpal dentin. Three-dimensional quantification of interglobular dentin volume confirmed an average 585-fold mean increase with mild (no different from controls; patients 1, 3, 4, and 6) or severe effects (patients 2 and 5, and one tooth from patient 3) noted (Fig. Dentin was the most severely affected hard tissue, consistent with numerous prior reports. SP ( sp 6,8-10,12,13,22,24-31 SP ) sp Several patients exhibited reduced dentin density and thickness, and hypomineralization was detected across mantle, circumpulpal, and proximal pulpal dentin regions. Keywords: CEMENTUM; DENTIN; PERIODONTAL TISSUES/PERIODONTIUM; MINERALIZED TISSUE/DEVELOPMENT; HYPOPHOSPHATEMIA; RICKETS EN CEMENTUM DENTIN PERIODONTAL TISSUES/PERIODONTIUM MINERALIZED TISSUE/DEVELOPMENT HYPOPHOSPHATEMIA RICKETS 1 11 11 04/16/21 20210401 NES 210401 ABSTRACT X-linked hypophosphatemia rickets (XLH) is caused by inactivating mutations in the phosphate-regulating endopeptidase homolog, X-linked ( I PHEX i ) gene, leading to renal phosphate wasting and hypophosphatemia. [Extracted from the article] |