FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W1996724286> ?p ?o ?g. }

• W1996724286 endingPage "828" @default.
• W1996724286 startingPage "828" @default.
• W1996724286 abstract "Skeletal muscle NMR imaging has two distinct roles: 1/ to diagnose disorders and 2/ to monitor disease progression. Both have been played to the benefit of type II glycogenosis patients (GSDII). In this study, we quantified muscle water T2, a marker of disease activity, in thigh and leg muscles of GSDII. We also investigated the relationship between T2 and the extension and progression of fatty degenerative changes in skeletal muscle. The degree of fatty infiltration in 11 thigh and 8 leg muscle heads was calculated for both lower limbs from a 3-point 3D Dixon acquisition. Since quantitative imaging has become standard in our department for the routine follow-up of GSDII in 2009, 36 patients have been examined, 17 were rescanned after 1 yr and 6 rescanned after 2 yrs. Twenty-one patients were treated by enzyme substitution, 2 stopped treatment during the observation. Quite a large number of muscles had abnormally elevated muscle water T2 (>39 ms): 84/296 in the thighs and 62/253 in the legs. Abnormal muscle water T2s were associated with higher percentages of fatty infiltration at 1 yr follow up: in the thighs: 11.6% vs 5.8% in normal T2 muscles, p < 0.0001; in the legs: 6.6% vs 4.9% in normal T2 muscles, p = 0.001. Treated patients had higher muscle T2s and fat fractions, which likely reflects the decision to treat the more severe cases in this open-label cohort, as indicated by the higher Mercuri score in the treated group. This study demonstrated the ability of quantitative NMR imaging to identify muscles with active disease in GSDII, and identified a link between disease activity and muscle fatty degenerative change severity. Skeletal muscle NMR imaging has two distinct roles: 1/ to diagnose disorders and 2/ to monitor disease progression. Both have been played to the benefit of type II glycogenosis patients (GSDII). In this study, we quantified muscle water T2, a marker of disease activity, in thigh and leg muscles of GSDII. We also investigated the relationship between T2 and the extension and progression of fatty degenerative changes in skeletal muscle. The degree of fatty infiltration in 11 thigh and 8 leg muscle heads was calculated for both lower limbs from a 3-point 3D Dixon acquisition. Since quantitative imaging has become standard in our department for the routine follow-up of GSDII in 2009, 36 patients have been examined, 17 were rescanned after 1 yr and 6 rescanned after 2 yrs. Twenty-one patients were treated by enzyme substitution, 2 stopped treatment during the observation. Quite a large number of muscles had abnormally elevated muscle water T2 (>39 ms): 84/296 in the thighs and 62/253 in the legs. Abnormal muscle water T2s were associated with higher percentages of fatty infiltration at 1 yr follow up: in the thighs: 11.6% vs 5.8% in normal T2 muscles, p < 0.0001; in the legs: 6.6% vs 4.9% in normal T2 muscles, p = 0.001. Treated patients had higher muscle T2s and fat fractions, which likely reflects the decision to treat the more severe cases in this open-label cohort, as indicated by the higher Mercuri score in the treated group. This study demonstrated the ability of quantitative NMR imaging to identify muscles with active disease in GSDII, and identified a link between disease activity and muscle fatty degenerative change severity." @default.
• W1996724286 created "2016-06-24" @default.
• W1996724286 creator A5005884967 @default.
• W1996724286 creator A5009848505 @default.
• W1996724286 creator A5014378465 @default.
• W1996724286 creator A5031903426 @default.
• W1996724286 creator A5045631879 @default.
• W1996724286 creator A5055570301 @default.
• W1996724286 creator A5073654406 @default.
• W1996724286 creator A5075931186 @default.
• W1996724286 date "2013-10-01" @default.
• W1996724286 modified "2023-10-16" @default.
• W1996724286 title "P.17.7 Quantitative NMR imaging of lower limb musculature in type II glycogenosis patients: Preliminary analysis of a 4-year follow-up" @default.
• W1996724286 doi "https://doi.org/10.1016/j.nmd.2013.06.657" @default.
• W1996724286 hasPublicationYear "2013" @default.
• W1996724286 type Work @default.
• W1996724286 sameAs 1996724286 @default.
• W1996724286 citedByCount "0" @default.
• W1996724286 crossrefType "journal-article" @default.
• W1996724286 hasAuthorship W1996724286A5005884967 @default.
• W1996724286 hasAuthorship W1996724286A5009848505 @default.
• W1996724286 hasAuthorship W1996724286A5014378465 @default.
• W1996724286 hasAuthorship W1996724286A5031903426 @default.
• W1996724286 hasAuthorship W1996724286A5045631879 @default.
• W1996724286 hasAuthorship W1996724286A5055570301 @default.
• W1996724286 hasAuthorship W1996724286A5073654406 @default.
• W1996724286 hasAuthorship W1996724286A5075931186 @default.
• W1996724286 hasConcept C105702510 @default.
• W1996724286 hasConcept C126322002 @default.
• W1996724286 hasConcept C141071460 @default.
• W1996724286 hasConcept C2779018429 @default.
• W1996724286 hasConcept C2779959927 @default.
• W1996724286 hasConcept C2989316616 @default.
• W1996724286 hasConcept C3019295976 @default.
• W1996724286 hasConcept C71924100 @default.
• W1996724286 hasConcept C72563966 @default.
• W1996724286 hasConcept C99508421 @default.
• W1996724286 hasConceptScore W1996724286C105702510 @default.
• W1996724286 hasConceptScore W1996724286C126322002 @default.
• W1996724286 hasConceptScore W1996724286C141071460 @default.
• W1996724286 hasConceptScore W1996724286C2779018429 @default.
• W1996724286 hasConceptScore W1996724286C2779959927 @default.
• W1996724286 hasConceptScore W1996724286C2989316616 @default.
• W1996724286 hasConceptScore W1996724286C3019295976 @default.
• W1996724286 hasConceptScore W1996724286C71924100 @default.
• W1996724286 hasConceptScore W1996724286C72563966 @default.
• W1996724286 hasConceptScore W1996724286C99508421 @default.
• W1996724286 hasIssue "9-10" @default.
• W1996724286 hasLocation W19967242861 @default.
• W1996724286 hasOpenAccess W1996724286 @default.
• W1996724286 hasPrimaryLocation W19967242861 @default.
• W1996724286 hasRelatedWork W2085796019 @default.
• W1996724286 hasRelatedWork W2410799512 @default.
• W1996724286 hasRelatedWork W2485950312 @default.
• W1996724286 hasRelatedWork W4306176347 @default.
• W1996724286 hasRelatedWork W1987836531 @default.
• W1996724286 hasRelatedWork W2761752626 @default.
• W1996724286 hasRelatedWork W2762557234 @default.
• W1996724286 hasRelatedWork W2764262185 @default.
• W1996724286 hasRelatedWork W2777935312 @default.
• W1996724286 hasRelatedWork W2982976481 @default.
• W1996724286 hasVolume "23" @default.
• W1996724286 isParatext "false" @default.
• W1996724286 isRetracted "false" @default.
• W1996724286 magId "1996724286" @default.
• W1996724286 workType "article" @default.