Literature DB >> 23616069

A study of glycogen storage disease with 99Tcm-MIBI gated myocardial perfusion imaging.

L G Wei1, J Q Gao, X M Liu, J M Huang, X Z Li.   

Abstract

OBJECTIVE: Gated myocardial perfusion imaging (G-MPI) is regularly performed using single-photon emission computed tomography. The objective of this study was to evaluate the clinical value of 99Tcm-methoxyisobutylisonitrile (MIBI) myocardial imaging in glycogen storage disease (GSD).
METHODS: 99Tcm-MIBI G-MPI was performed in nine patients with clinically proven GSD. QGS quantitative software was used for processing and interpretation. Left ventricular ejection fraction (LVEF), end-diastolic volume (EDV) and end-systolic volume (ESV) were automatically generated. The myocardium was divided into seven segments, 20 sub-segments and a five-point scoring system was used.
RESULTS: Seven out of nine cases were abnormal and the positive rate of G-MPI was 77.8 %. Sixty-two sub-segments of injured myocardium were detected in 140 sub-segments of seven abnormal patients. One injured segment was observed in one patient (14.3 %), two segments were detected in two patients (28.6 %) and three or more abnormal segments were observed in four patients (57.1 %).
CONCLUSION: 99Tcm-MIBI G-MPI can detect myocardial damage in GSD as a non-invasive method. It plays an important role in the clinic.

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Year:  2013        PMID: 23616069     DOI: 10.1007/s11845-013-0939-5

Source DB:  PubMed          Journal:  Ir J Med Sci        ISSN: 0021-1265            Impact factor:   1.568


  23 in total

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Authors:  Reinout P Hesselink; Gert Schaart; Anton J M Wagenmakers; Maarten R Drost; Ger J van der Vusse
Journal:  Muscle Nerve       Date:  2006-04       Impact factor: 3.217

2.  Pathology of skeletal muscle cells in adult-onset glycogenosis type II (Pompe disease): ultrastructural study.

Authors:  Eliza Lewandowska; Teresa Wierzba-Bobrowicz; Rafał Rola; Joanna Modzelewska; Tomasz Stepień; Agnieszka Lugowska; Elzbieta Pasennik; Danuta Ryglewicz
Journal:  Folia Neuropathol       Date:  2008       Impact factor: 2.038

3.  Molecular analysis and protein processing in late-onset Pompe disease patients with low levels of acid α-glucosidase activity.

Authors:  Deeksha S Bali; Adviye A Tolun; Jennifer L Goldstein; Jian Dai; Priya S Kishnani
Journal:  Muscle Nerve       Date:  2011-05       Impact factor: 3.217

4.  Myocardial late gadolinium enhancement in specific cardiomyopathies by cardiovascular magnetic resonance: a preliminary experience.

Authors:  Caterina Silva; James C Moon; Andrew G Elkington; Anna S John; Raad H Mohiaddin; Dudley J Pennell
Journal:  J Cardiovasc Med (Hagerstown)       Date:  2007-12       Impact factor: 2.160

5.  A new mutation in PRKAG2 gene causing hypertrophic cardiomyopathy with conduction system disease and muscular glycogenosis.

Authors:  Pascal Laforêt; Pascale Richard; Mina Ait Said; Norma Beatriz Romero; Emmanuelle Lacene; Jean-Paul Leroy; Christiane Baussan; Jean-Yves Hogrel; Thomas Lavergne; Karim Wahbi; Bernard Hainque; Denis Duboc
Journal:  Neuromuscul Disord       Date:  2006-02-17       Impact factor: 4.296

6.  Assessment of cardiac abnormalities in Duchenne's muscular dystrophy by (99m)Tc-MIBI gated myocardial perfusion imaging.

Authors:  Peng Fu; Lingge Hu; Jing Sinzinger; Jianmin Huang; Xiaomei Liu
Journal:  Hell J Nucl Med       Date:  2012 May-Aug       Impact factor: 1.102

Review 7.  Lysosomal dysfunction in muscle with special reference to glycogen storage disease type II.

Authors:  Reinout P Hesselink; Anton J M Wagenmakers; Maarten R Drost; Ger J Van der Vusse
Journal:  Biochim Biophys Acta       Date:  2003-03-20

8.  Glycogen storage disease type III diagnosis and management guidelines.

Authors:  Priya S Kishnani; Stephanie L Austin; Pamela Arn; Deeksha S Bali; Anne Boney; Laura E Case; Wendy K Chung; Dev M Desai; Areeg El-Gharbawy; Ronald Haller; G Peter A Smit; Alastair D Smith; Lisa D Hobson-Webb; Stephanie Burns Wechsler; David A Weinstein; Michael S Watson
Journal:  Genet Med       Date:  2010-07       Impact factor: 8.822

9.  Pompe disease diagnosis and management guideline.

Authors:  Priya S Kishnani; Robert D Steiner; Deeksha Bali; Kenneth Berger; Barry J Byrne; Laura E Case; Laura Case; John F Crowley; Steven Downs; R Rodney Howell; Richard M Kravitz; Joanne Mackey; Deborah Marsden; Anna Maria Martins; David S Millington; Marc Nicolino; Gwen O'Grady; Marc C Patterson; David M Rapoport; Alfred Slonim; Carolyn T Spencer; Cynthia J Tifft; Michael S Watson
Journal:  Genet Med       Date:  2006-05       Impact factor: 8.822

10.  The electrodiagnostic characteristics of Glycogen Storage Disease Type III.

Authors:  Lisa D Hobson-Webb; Stephanie L Austin; Deeksha S Bali; Priya S Kishnani
Journal:  Genet Med       Date:  2010-07       Impact factor: 8.822

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