Chronic idiopathic hyperphosphatasia with unusual dental findings: a case report
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Chronic idiopathic hyperphosphatasia with unusual dental findings: a case report

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Chronic idiopathic hyperphosphatasia with unusual dental findings: a case report

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dc.contributor.author Sreejan, Cheriya K. es
dc.contributor.author Gopakumar, Nair es
dc.contributor.author Subhas Babu, Gogineni es
dc.date.accessioned 2014-05-21T12:45:32Z
dc.date.available 2014-05-21T12:45:32Z
dc.date.issued 2012 es
dc.identifier.uri http://hdl.handle.net/10550/35292
dc.source Sreejan, Cheriya K. ; Gopakumar, Nair ; Subhas Babu, Gogineni. Chronic idiopathic hyperphosphatasia with unusual dental findings: a case report. En: Journal of Clinical and Experimental Dentistry, 2012, Vol. 4, No. 5: 313-316 es
dc.subject Odontología es
dc.subject Ciencias de la salud es
dc.title Chronic idiopathic hyperphosphatasia with unusual dental findings: a case report es
dc.type info:eu-repo/semantics/article en
dc.type info:eu-repo/semantics/publishedVersion en
dc.subject.unesco UNESCO::CIENCIAS MÉDICAS es
dc.description.abstractenglish Chronic idiopathic hyperphosphatasia(CIH) or juvenile Paget disease is believed to be a distinct disease characterized by an increase in the serum alkaline phosphatase, cortical thickening and bowing of the long bones, especially the femora. It is a rare autosomal recessive bone disorder, with excessive bone resorption and bone formation. Skeletal malformations in the legs may cause problems in walking and may eventually result in short stature. The radiographic appearances include widening of the diaphyses, vertebral osteoporosis, acetabular protrusion, and thickening of the skull vault. Intensive bisphosphonate treatment prevented the development of deformity and disability but there is no published data on long-term efficacy. Bisphosphonate therapy showed suppression of bone turnover, doubling of trabecular thickness with no mineralization defect, and no osteopetrosis. We report a female of 21 years, a case of chronic idiopathic hyperphosphatasia congenital form, with a history of fracture, short stature and malformed teeth. She had a waddling gait, bone deformities, kyphoscoliosis and curvature of her limbs. es

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