INT53184
From wiki-pain
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Sentences Mentioned In
Key: | Protein | Mutation | Event | Anatomy | Negation | Speculation | Pain term | Disease term |
Five inborn errors with consequences for bile acid biosynthesis have been described: 7-dehydrocholesterol 7-reductase deficiency, 3 beta-hydroxysteroid delta 5-oxidoreductase/isomerase deficiency, 3-oxo-delta 4-steroid 5 beta-reductase deficiency, sterol 27-hydroxylase deficiency (cerebrotendinous xanthomatosis), and peroxisomal disease(s) with absence of peroxisomes. | |||||||||||||||
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These results are highly suggestive, but not conclusive, that the newly identified transversion in the CYP27 gene accounts for the sterol 27-hydroxylase (EC 1.14.13.15) deficiency in these patients. | |||||||||||||||
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Additionally, in contrast to placebo, adalimumab has been shown to decrease uCTX-II levels in patients with RA (17.3% decrease, p < 0.01) (Garnero P, 2004). | |||||||||||||||
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Finally, Landewe and colleagues demonstrated a decrease in uCTX-II levels in RA patients after 3 months of treatment with disease modifying anti-rheumatic drug (DMARD) therapy. | |||||||||||||||
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In the case of sterol 27-hydroxylase deficiency, early treatment with chenodeoxycholic acid may prevent the development of progressive neurological dysfunction, dementia, and ataxia. | |||||||||||||||
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This decline in uCTX-II at 3 months predicted long term (5 year) improvement in radiographic outcome (Landewe et al. 2004).
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Cerebrotendinous xanthomatosis (CTX) or sterol 27-hydroxylase deficiency is an autosomal recessive disorder characterized by defect in bile acid biosynthesis and storage of sterols, and early childhood onset. | |||||||||||||||
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Decreased CTX at various time points during bisphosphonate therapy is strongly correlated with BMD increases at 6 months and 1 year (Leeming et al 2006). | |||||||||||||||
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In the present study, an elevated urinary CTX-I level, a marker for bone resorption, was found to reflects increased disease activity and decreased BMD in men with AS. | |||||||||||||||
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Levels of both CTX-II and CTX-I, as markers of cartilage and bone degradation respectively, decreased by approximately 50% in the treatment group compared with baseline, and CTX-II levels were restored to premenopausal levels. | |||||||||||||||
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Levels of both CTX-II and CTX-I, as markers of cartilage and bone degradation respectively, decreased by approximately 50% in the treatment group compared with baseline, and CTX-II levels were restored to premenopausal levels. | |||||||||||||||
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In the combined sCT treatment group analysis, robust reductions in both CTX-I and CTX-II were observed shortly after the morning dose, albeit with a more rapid reduction in CTX-I than CTX-II (Figure 5B). | |||||||||||||||
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In the combined sCT treatment group analysis, robust reductions in both CTX-I and CTX-II were observed shortly after the morning dose, albeit with a more rapid reduction in CTX-I than CTX-II (Figure 5B). | |||||||||||||||
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In the combined sCT treatment group analysis, robust reductions in both CTX-I and CTX-II were observed shortly after the morning dose, albeit with a more rapid reduction in CTX-I than CTX-II (Figure 5B). | |||||||||||||||
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The third infusion of zoledronic acid led to a 60% reduction of CTx levels within 911 days; this was followed by a gradual increase, indicating the persistence of bone resorption in patients receiving zoledronic acid. | |||||||||||||||
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CTX-I in the HRT group was reduced by 62 ± 5% (mean ± SEM) after 12 months and 53 ± 6% after 24 months. | |||||||||||||||
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A small reduction of CTX-I was also noticed in the control group at the end of first year, which possibly could be due to the treatment with calcium and vitamin D3. | |||||||||||||||
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HRT caused a pronounced decrease in the collagen type I degradation marker, CTX-I, both when the HRT and control groups were compared (P < 0.001) and within the HRT group (P < 0.001) (Fig. 1a). | |||||||||||||||
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A reduction in ICTP was correlated with improved BMD in the lumbar spine (P = 0.002) and total hip (P = 0.027) and with a decrease in ESR (P = 0.006), CTX-II (P = 0.001), and COMP (P = 0.005), besides the correlation with CTX-I. | |||||||||||||||
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A decrease in CTX-I was correlated with increased bone mass in the total hip (P < 0.001) and lumbar spine (P < 0.001) and with a reduction in ICTP (P < 0.001), PICP (P = 0.005) and ESR (P = 0.019). | |||||||||||||||
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