FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2951376987> ?p ?o ?g. }

• W2951376987 abstract "Mucopolysaccharidosis type III (MPS III), or Sanfilippo syndrome, is caused by a deficiency in one of the four enzymes involved in the lysosomal degradation of heparan sulfate. Cardiac abnormalities have been observed in patients with all types of MPS except MPS IX, however few studies have focused on cardiac alterations in patients with MPS III. We reviewed medical records, echocardiograms, and electrocardiograms of 26 Taiwanese patients with MPS III (five with IIIA, 20 with IIIB, and one with IIIC; 14 males and 12 females; median age, 7.4 years; age range, 1.8–26.5 years). The relationships between age and each echocardiographic parameter were analyzed. Echocardiographic examinations (n = 26) revealed that 10 patients (38%) had valvular heart disease. Four (15%) and eight (31%) patients had valvular stenosis or regurgitation, respectively. The most prevalent cardiac valve abnormality was mitral regurgitation (31%), followed by aortic regurgitation (19%). However, most of the cases of valvular heart disease were mild. Three (12%), five (19%) and five (19%) patients had mitral valve prolapse, a thickened interventricular septum, and asymmetric septal hypertrophy, respectively. The severity of aortic regurgitation and the existence of valvular heart disease, aortic valve abnormalities and valvular stenosis were all positively correlated with increasing age (p < 0.05). Z scores > 2 were identified in 0, 38, 8, and 27% of left ventricular mass index, interventricular septal end-diastolic dimension, left ventricular posterior wall end-diastolic dimension, and aortic diameter, respectively. Electrocardiograms in 11 patients revealed the presence of sinus arrhythmia (n = 3), sinus bradycardia (n = 2), and sinus tachycardia (n = 1). Six patients with MPS IIIB had follow-up echocardiographic data at 1.9–18.1 years to compare with the baseline data, which showed some patients had increased thickness of the interventricular septum, as well as more patients had valvular abnormalities at follow-up. Cardiac involvement in MPS III is less common and milder compared with other types of MPS. The existence of valvular heart disease, aortic valve abnormalities and valvular stenosis in the patients worsened with increasing age, reinforcing the concept of the progressive nature of this disease." @default.
• W2951376987 created "2019-06-27" @default.
• W2951376987 creator A5004782890 @default.
• W2951376987 creator A5029271104 @default.
• W2951376987 creator A5031629187 @default.
• W2951376987 creator A5033926474 @default.
• W2951376987 creator A5064448888 @default.
• W2951376987 creator A5068739516 @default.
• W2951376987 creator A5086777464 @default.
• W2951376987 date "2019-06-13" @default.
• W2951376987 modified "2023-10-18" @default.
• W2951376987 title "Cardiac characteristics and natural progression in Taiwanese patients with mucopolysaccharidosis III" @default.
• W2951376987 cites W1522987887 @default.
• W2951376987 cites W1603932632 @default.
• W2951376987 cites W1953621380 @default.
• W2951376987 cites W1990717001 @default.
• W2951376987 cites W1997859168 @default.
• W2951376987 cites W2002083318 @default.
• W2951376987 cites W2008316788 @default.
• W2951376987 cites W2025180168 @default.
• W2951376987 cites W2030045021 @default.
• W2951376987 cites W2044861416 @default.
• W2951376987 cites W2048367113 @default.
• W2951376987 cites W2050557934 @default.
• W2951376987 cites W2063222638 @default.
• W2951376987 cites W2073620213 @default.
• W2951376987 cites W2084103627 @default.
• W2951376987 cites W2085237653 @default.
• W2951376987 cites W2092327321 @default.
• W2951376987 cites W2096776225 @default.
• W2951376987 cites W2101099004 @default.
• W2951376987 cites W2106865163 @default.
• W2951376987 cites W2111183899 @default.
• W2951376987 cites W2124364276 @default.
• W2951376987 cites W2124624517 @default.
• W2951376987 cites W2132481477 @default.
• W2951376987 cites W2134923697 @default.
• W2951376987 cites W2137541148 @default.
• W2951376987 cites W2141084326 @default.
• W2951376987 cites W2144310582 @default.
• W2951376987 cites W2163220775 @default.
• W2951376987 cites W2179366637 @default.
• W2951376987 cites W2181968881 @default.
• W2951376987 cites W2288110872 @default.
• W2951376987 cites W2338559137 @default.
• W2951376987 cites W2473662354 @default.
• W2951376987 cites W2558542370 @default.
• W2951376987 cites W2568253215 @default.
• W2951376987 cites W2574983263 @default.
• W2951376987 cites W2800635415 @default.
• W2951376987 cites W2805116397 @default.
• W2951376987 cites W2885251753 @default.
• W2951376987 cites W2889113749 @default.
• W2951376987 cites W2890928166 @default.
• W2951376987 cites W2899889744 @default.
• W2951376987 cites W2911511001 @default.
• W2951376987 cites W2913338401 @default.
• W2951376987 cites W4244020249 @default.
• W2951376987 cites W4249896096 @default.
• W2951376987 doi "https://doi.org/10.1186/s13023-019-1112-7" @default.
• W2951376987 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6567572" @default.
• W2951376987 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/31196149" @default.
• W2951376987 hasPublicationYear "2019" @default.
• W2951376987 type Work @default.
• W2951376987 sameAs 2951376987 @default.
• W2951376987 citedByCount "12" @default.
• W2951376987 countsByYear W29513769872020 @default.
• W2951376987 countsByYear W29513769872021 @default.
• W2951376987 countsByYear W29513769872022 @default.
• W2951376987 countsByYear W29513769872023 @default.
• W2951376987 crossrefType "journal-article" @default.
• W2951376987 hasAuthorship W2951376987A5004782890 @default.
• W2951376987 hasAuthorship W2951376987A5029271104 @default.
• W2951376987 hasAuthorship W2951376987A5031629187 @default.
• W2951376987 hasAuthorship W2951376987A5033926474 @default.
• W2951376987 hasAuthorship W2951376987A5064448888 @default.
• W2951376987 hasAuthorship W2951376987A5068739516 @default.
• W2951376987 hasAuthorship W2951376987A5086777464 @default.
• W2951376987 hasBestOaLocation W29513769871 @default.
• W2951376987 hasConcept C126322002 @default.
• W2951376987 hasConcept C164705383 @default.
• W2951376987 hasConcept C2776002628 @default.
• W2951376987 hasConcept C2777495988 @default.
• W2951376987 hasConcept C2777543888 @default.
• W2951376987 hasConcept C2777953023 @default.
• W2951376987 hasConcept C2778921608 @default.
• W2951376987 hasConcept C2779416428 @default.
• W2951376987 hasConcept C2779736815 @default.
• W2951376987 hasConcept C2779740085 @default.
• W2951376987 hasConcept C2780007028 @default.
• W2951376987 hasConcept C2780074459 @default.
• W2951376987 hasConcept C2780168065 @default.
• W2951376987 hasConcept C2781235965 @default.
• W2951376987 hasConcept C2993373945 @default.
• W2951376987 hasConcept C71924100 @default.
• W2951376987 hasConcept C84393581 @default.
• W2951376987 hasConceptScore W2951376987C126322002 @default.
• W2951376987 hasConceptScore W2951376987C164705383 @default.
• W2951376987 hasConceptScore W2951376987C2776002628 @default.
• W2951376987 hasConceptScore W2951376987C2777495988 @default.

• next