Abstract：
:Hypophosphatemic rickets (HR) is a group of rare disorders caused by excessive renal phosphate wasting. The purpose of this cross-sectional study of 38 HR patients was to characterize the phenotype of adult HR patients. Moreover, skeletal and endodontic severity scores were defined to assess possible gender differences in disease severity in patients with genetically verified X-linked HR. Compared to normal reference data, i.e., z = 0, HR patients had significantly lower final height, with a mean difference in z-score of -1.9 (95% CI -2.4 to -1.4, P < 0.001). Compared to paired z-scores of final height, z-scores of leg length were significantly lower and those of sitting height were significantly higher (P < 0.001), resulting in disproportion as indicated by the significantly elevated sitting height ratio, mean difference in z-score of 2.6 (95% CI 2.1-3.1, P < 0.001). Z-scores of head circumference (median 1.4, range -0.4 to 5.5, P < 0.001) and z-scores of bone mineral density (BMD) of the lumbar spine (median 1.9, range -1.5 to 8.6, P < 0.001) were significantly elevated compared to normal reference data. The relative risk (RR) of fracture was reduced (RR = 0.34, 95% CI 0.20-0.57, P < 0.001). The skeletal severity score tended to be higher in males compared to females (P = 0.07), and no gender difference in endodontic severity was found. In conclusion, adult HR patients were characterized by short stature and were disproportioned. They had elevated BMD of the lumbar spine and a reduced risk of fractures. We found a tendency for males to be more severely affected than females.
journal_name
Calcif Tissue Intjournal_title
Calcified tissue internationalauthors
Beck-Nielsen SS,Brusgaard K,Rasmussen LM,Brixen K,Brock-Jacobsen B,Poulsen MR,Vestergaard P,Ralston SH,Albagha OM,Poulsen S,Haubek D,Gjørup H,Hintze H,Andersen MG,Heickendorff L,Hjelmborg J,Gram Jdoi
10.1007/s00223-010-9373-0subject
Has Abstractpub_date
2010-08-01 00:00:00pages
108-19issue
2eissn
0171-967Xissn
1432-0827journal_volume
87pub_type
杂志文章abstract：:The type IA osteogenesis imperfecta (OI) phenotype is characterized by multiple fractures, blue sclerae, and minimal skeletal deformity without dentinogenesis imperfecta. The object of this study was to determine the effect of treatment with intravenous pamidronate (30 mg) every 3 months on bone density and bone histo...
journal_title：Calcified tissue international
pub_type： 临床试验,杂志文章
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journal_title：Calcified tissue international
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journal_title：Calcified tissue international
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journal_title：Calcified tissue international
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journal_title：Calcified tissue international
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journal_title：Calcified tissue international
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journal_title：Calcified tissue international
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journal_title：Calcified tissue international
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journal_title：Calcified tissue international
pub_type： 杂志文章
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journal_title：Calcified tissue international
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journal_title：Calcified tissue international
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更新日期：1996-01-01 00:00:00
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journal_title：Calcified tissue international
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journal_title：Calcified tissue international
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journal_title：Calcified tissue international
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