A longitudinal study on BIO14.6 hamsters with dilated cardiomyopathy: micro-echocardiographic evaluation

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• 作者：Maria Paola Belfiore (1)
  Daniela Berritto (1)
  Francesca Iacobellis (1)
  Claudia Rossi (1)
  Gerardo Nigro (2)
  Ida Luisa Rotundo (2)
  Santolo Cozzolino (3)
  Salvatore Cappabianca (1)
  Antonio Rotondo (1)
  Roberto Grassi (1)
  
• 关键词：μ ; US ; muscular dystrophy ; gene therapy ; animal model
• 刊名：Cardiovascular Ultrasound
• 出版年：2011
• 出版时间：December 2011
• 年：2011
• 卷：9
• 期：1
• 全文大小：567KB
• 参考文献：1. Grassi R, Cavaliere C, Cozzolino S, Mansi L, Cirillo S, Tedeschi G, Franchi R, Russo P, Cornacchia S, Rotondo A: Small imaging facility: new perspectives for the radiologist. / Radiol Med 2009, 114:152-7. CrossRef
  2. Losi MA, Nistri S, Galderisi M, Betocchi S, Cecchi F, Olivotto I, Agricola E, Ballo P, Buralli S, D'Andrea A, D'Errico A, Mele D, Sciomer S, Mondillo S: Echocardiography in patients with hypertrophic cardiomyopathy: usefulness of old and new techniques in the diagnosis and pathophysiological assessment. / Cardiovasc Ultrasound 2010, 8:7.
  3. Thomas DE, Wheeler R, Yousef ZR, Masani ND: The role of echocardiography in guiding management in dilated cardiomyopathy. / Eur J Echocardiogr 2009, 10:iii15-1. CrossRef
  4. Turk R, Sterrenburg E, van der Wees CG, de Meijer EJ, de Menezes RX, Groh S, Campbell KP, Noguchi S, van Ommen GJ, den Dunnen JT, 't Hoen PA: Common pathological mechanisms in mouse models for muscular dystrophies. / FASEB J 2006, 20:127-.
  5. Godfrey C, Foley AR, Clement E, Muntoni F: Dystroglycanopathies: coming into focus. / Curr Opin Genetic Dev 2011, 21:278-5. CrossRef
  6. Vitiello C, Faraso S, Sorrentino NC, Di Salvo G, Nusco E, Nigro G, Cutillo L, Calabrò R, Auricchio A, Nigro V: Disease rescue and increased lifespan in a model of cardiomyopathy and muscolar dystrophy by combined AAV treatments. / PLoS One 2009, 4:e5051. CrossRef
  7. Aurino S, Piluso G, Saccone V, Cacciottolo M, D'amico F, Dionisi M, Totaro A, Belsito A, Di Vicino U, Nigro V: Candidate-gene testing for orphan limb-girdle muscular dystrophies. / Acta Myol 2008, 27:90-.
  8. Fanim M, Nascimbeni AC, Aurino S, Tasca E, Pegoraro E, Nigro V, Angelini C: Frequency of LGMD gene mutations in Italian patients with distinct clinical phenotypes. / Neurology 2009, 72:1432-. CrossRef
  9. Gregorevic P, Blankiship MJ, Allen JM, Crawford RW, Meuse L, Miller DG, Russell DW, Chamberlain JS: Systemic delivery of genes to striated muscles using adeno-associated viral vectors. / Nat Med 2004, 10:828-4. CrossRef
  10. Wang Z, Zhu T, Qiao C, Zhou L, Wang B, Zhang J, Chen C, Li J, Xiao X: Adeno-associated virus serotype 8 efficiently delivers gene to muscle and heart. / Nat Biotechnol 2005, 23:321-. CrossRef
  11. Russell WM, Burch RL: The progress of humane technique. In / The Principles of Humane Experimental Technique. Edited by: Russell WM, Burch RL. Springfield IL: Charles C. Thomas; 1959.
  12. Flecknell P: Replacement, reduction and refinement. / ALTEX 2002, 19:73-.
  13. Gao XM, Dart AM, Dewar E, Jennings G, Du XJ: Serial echocardiographic assessment of left ventricular dimensions and function after myocardial infarction in mice. / Cardiovasc Res 2000, 45:330-. CrossRef
  14. Collins KA, Korcarz CE, Lang RM: Use of echocardiography for the phenotypic assessment of genetically altered mice. / Physiol Genomics 2003, 13:227-9.
  15. Ahmad N, Bygrave M, De Zordo T, Fenster A, Lee TY: Detecting degenerative changes in myotonic murine models of Duchenne muscular dystrophy using high-frequency. / J Ultrasound Med 2010, 29:367-5.
  
• 作者单位：Maria Paola Belfiore (1)
  Daniela Berritto (1)
  Francesca Iacobellis (1)
  Claudia Rossi (1)
  Gerardo Nigro (2)
  Ida Luisa Rotundo (2)
  Santolo Cozzolino (3)
  Salvatore Cappabianca (1)
  Antonio Rotondo (1)
  Roberto Grassi (1)
  
  1. Institute of Radiology, Second University of Naples (SUN), P.zza Miraglia 2, 80138, Napoli, Italy
  2. Telethon Institute of Genetics and Medicine (TIGEM), Via Pietro Castellino 111, 80131, Napoli, Italy
  3. Biotechnology Center, “A. Cardarelli-Hospital, Via Cardarelli 9, 80131, Napoli, Italy
  

文摘

Background In recent years, several new technologies for small-animal imaging have been developed. In particular, the use of ultrasound in animal imaging has focused on the investigation of accessible biological structures such as the heart, of which it provides a morphological and functional assessment. The purpose of this study was to investigate the role of micro-ultrasonography (μ-US) in a longitudinal study on BIO14.6 cardiomyopathic hamsters treated with gene therapy. Methods Thirty hamsters were divided into three groups (n = 10): Group I, untreated BIO 14.6 hamsters; Group II, BIO 14.6 hamsters treated with gene therapy; Group III, untreated wild type (WT) hamsters. All hamsters underwent serial μ-US sessions and were sacrificed at predetermined time points. Results μ-US revealed: in Group I, progressive dilation of the left ventricle with a change in heart morphology from an elliptical to a more spherical shape, altered configuration of the mitral valve and subvalvular apparatus, and severe reduction in ejection fraction; in Group II, mild decrease in contractile function and ejection fraction; in Group III, normal cardiac chamber morphology and function. There was a negative correlation between the percentage of fibrosis observed at histology and the ejection fraction obtained on μ-echocardiography (Spearman r: -0.839; p < 0.001). Conclusions Although histological examination remains indispensable for a conclusive diagnosis, high-frequency μ-echocardiography, thanks to the high spatial and contrast resolution, can be considered sufficient for monitoring therapeutic efficacy and/or the progression of dilated cardiomyopathy, providing an alternative tool for repeatable and noninvasive evaluation.