juvenile Paget disease

An extremely rare (40 cases in the world literature) autosomal recessive osteopathy (OMIM:239000) that presents in infancy or early childhood and is characterised by rapid remodelling of woven bone, osteopenia, debilitating fractures and deformities, due to a marked acceleration of bone remodelling throughout the skeleton.
Management
Agents to block osteoclastic bone resorption, which can be fatal.
Molecular pathology
Caused by defects in TNFRSF11B, which encodes an osteoblast-secreted decoy receptor of the TNF receptor superfamily that functions as a negative regulator of bone resorption.

单词	juvenile paget disease
释义	DictionarySeedisease juvenile Paget disease juvenile Paget disease An extremely rare (40 cases in the world literature) autosomal recessive osteopathy (OMIM:239000) that presents in infancy or early childhood and is characterised by rapid remodelling of woven bone, osteopenia, debilitating fractures and deformities, due to a marked acceleration of bone remodelling throughout the skeleton. Management Agents to block osteoclastic bone resorption, which can be fatal. Molecular pathology Caused by defects in TNFRSF11B, which encodes an osteoblast-secreted decoy receptor of the TNF receptor superfamily that functions as a negative regulator of bone resorption.
随便看	nzoma nzop nzoq nzord nzosm nzoss nzot nzoug nzow nzp nzpa nzpaf nzpb nzpbb nzpbc nzpbf nzpbra nzpc nzpca nzpcc nzpcg nzpd nzpe nzpec nzpersonals