Literature DB >> 21289561

The natural history of cardiac and pulmonary function decline in patients with duchenne muscular dystrophy.

Rolando Roberto1, Anto Fritz, Yolanda Hagar, Braden Boice, Andrew Skalsky, Hosun Hwang, Laurel Beckett, Craig McDonald, Munish Gupta.   

Abstract

STUDY
DESIGN: Retrospective review of scoliosis progression, pulmonary and cardiac function in a series of patients with Duchenne Muscular Dystrophy (DMD).
OBJECTIVE: To determine whether operative treatment of scoliosis decreases the rate of pulmonary function loss in patients with DMD. SUMMARY OF BACKGROUND DATA: It is generally accepted that surgical intervention should be undertaken in DMD scoliosis once curve sizes reach 35° to allow intervention before critical respiratory decline has occurred. There are conflicting reports, however, regarding the effect of scoliosis stabilization on the rate of pulmonary function decline when compared to nonoperative cohorts.
METHODS: We reviewed spinal radiographs, echocardiograms, and spirometry, hospital, and operative records of all patients seen at our tertiary referral center from July 1, 1992 to June 1, 2007. Data were recorded to Microsoft Excel (Microsoft, Redmond, WA) and analyzed with SAS (SAS Institute, Cary, NC) and R statistical processing software (www.r-project.org).
RESULTS: The percent predicted forced vital capacity (PPFVC) decreased 5% per year before operation. The mean PPFVC was 54% (SD = 21%) before operation with a mean postoperative PPFVC of 43% (SD = 14%). Surgical treatment was associated with a 12% decline in PPFVC independent of other treatment variables. PPFVC after operation declined at a rate of 1% per year and while this rate was lower, it was not significantly different than the rate of decline present before operation (P = 0.18). Cardiac function as measured by left ventricular fractional shortening declined at a rate of 1% per year with most individuals exhibiting a left ventricular fractional shortening rate of more than 30 before operation.
CONCLUSION: Operative treatment of scoliosis in DMD using the Luque Galveston method was associated with a reduction of forced vital capacity related to operation. The rate of pulmonary function decline after operation was not significantly reduced when compared with the rate of preoperative forced vital capacity decline.

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Mesh:

Year:  2011        PMID: 21289561      PMCID: PMC3120906          DOI: 10.1097/BRS.0b013e3181fea1ed

Source DB:  PubMed          Journal:  Spine (Phila Pa 1976)        ISSN: 0362-2436            Impact factor:   3.468


  27 in total

1.  Comparison of pelvic fixation techniques in neuromuscular spinal deformity correction: Galveston rod versus iliac and lumbosacral screws.

Authors:  Michael W Peelle; Lawrence G Lenke; Keith H Bridwell; Brenda Sides
Journal:  Spine (Phila Pa 1976)       Date:  2006-09-15       Impact factor: 3.468

2.  The effect of scoliosis surgery on lung function in the immediate postoperative period.

Authors:  Nanci Yuan; Javier A Fraire; Monique M Margetis; David L Skaggs; Vernon T Tolo; Thomas G Keens
Journal:  Spine (Phila Pa 1976)       Date:  2005-10-01       Impact factor: 3.468

3.  Pulmonary function in Duchenne muscular dystrophy related to stage of disease.

Authors:  S R Inkley; F C Oldenburg; P J Vignos
Journal:  Am J Med       Date:  1974-03       Impact factor: 4.965

4.  Prolongation of life in Duchenne's muscular dystrophy.

Authors:  Y Rideau; G Gatin; J Bach; G Gines
Journal:  Acta Neurol (Napoli)       Date:  1983-04

5.  Deflazacort treatment of Duchenne muscular dystrophy.

Authors:  W D Biggar; M Gingras; D L Fehlings; V A Harris; C A Steele
Journal:  J Pediatr       Date:  2001-01       Impact factor: 4.406

Review 6.  Duchenne muscular dystrophy.

Authors:  Michael Sussman
Journal:  J Am Acad Orthop Surg       Date:  2002 Mar-Apr       Impact factor: 3.020

7.  Posterior spinal fusion for scoliosis in duchenne muscular dystrophy diminishes the rate of respiratory decline.

Authors:  Maria V Velasco; Andrew A Colin; David Zurakowski; Basil T Darras; Frederic Shapiro
Journal:  Spine (Phila Pa 1976)       Date:  2007-02-15       Impact factor: 3.468

8.  Correlation of scoliosis and pulmonary function in Duchenne muscular dystrophy.

Authors:  L T Kurz; S J Mubarak; P Schultz; S M Park; J Leach
Journal:  J Pediatr Orthop       Date:  1983-07       Impact factor: 2.324

9.  Evaluation of pulmonary function in muscular dystrophy patients requiring spinal surgery.

Authors:  J G Jenkins; D Bohn; J F Edmonds; H Levison; G A Barker
Journal:  Crit Care Med       Date:  1982-10       Impact factor: 7.598

10.  Respiratory function in the muscular dystrophies.

Authors:  Y Rideau; L W Jankowski; J Grellet
Journal:  Muscle Nerve       Date:  1981 Mar-Apr       Impact factor: 3.217

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  17 in total

1.  A population-based study of scoliosis among males diagnosed with a dystrophinopathy identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet).

Authors:  Kristin M Conway; Amber Gedlinske; Katherine D Mathews; Seth Perlman; Nicholas Johnson; Russell Butterfield; Man Hung; Jerry Bounsanga; Dennis Matthews; Joyce Oleszek; Paul A Romitti
Journal:  Muscle Nerve       Date:  2021-12-01       Impact factor: 3.852

Review 2.  Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management.

Authors:  David J Birnkrant; Katharine Bushby; Carla M Bann; Benjamin A Alman; Susan D Apkon; Angela Blackwell; Laura E Case; Linda Cripe; Stasia Hadjiyannakis; Aaron K Olson; Daniel W Sheehan; Julie Bolen; David R Weber; Leanne M Ward
Journal:  Lancet Neurol       Date:  2018-02-03       Impact factor: 44.182

3.  Left ventricular dysfunction in Duchenne muscular dystrophy.

Authors:  Katherine A James; Jane Gralla; Leslie A Ridall; ThuyQuynh N Do; Angela S Czaja; Peter M Mourani; Emma Ciafaloni; Christopher Cunniff; Jennifer Donnelly; Joyce Oleszek; Shree Pandya; Elinora Price; Michele L Yang; Scott R Auerbach
Journal:  Cardiol Young       Date:  2020-01-22       Impact factor: 1.093

4.  Characterization of pulmonary function in Duchenne Muscular Dystrophy.

Authors:  O H Mayer; R S Finkel; C Rummey; M J Benton; A M Glanzman; J Flickinger; B-M Lindström; T Meier
Journal:  Pediatr Pulmonol       Date:  2015-03-09

5.  The lifetime risk of pneumonia in patients with neuromuscular scoliosis at a mean age of 21 years: the role of spinal deformity surgery.

Authors:  Heli Keskinen; Heikki Lukkarinen; Katariina Korhonen; Tuomas Jalanko; Antti Koivusalo; Ilkka Helenius
Journal:  J Child Orthop       Date:  2015-09-08       Impact factor: 1.548

6.  Comparison of pulmonary function and back muscle strength according to the degree of spinal curvature of healthy adults.

Authors:  Jae Eung You; Hye Young Lee; Kyoung Kim
Journal:  J Phys Ther Sci       Date:  2015-06-30

7.  Long-Term Efficacy, Safety, and Pharmacokinetics of Drisapersen in Duchenne Muscular Dystrophy: Results from an Open-Label Extension Study.

Authors:  Nathalie M Goemans; Már Tulinius; Marleen van den Hauwe; Anna-Karin Kroksmark; Gunnar Buyse; Rosamund J Wilson; Judith C van Deutekom; Sjef J de Kimpe; Afrodite Lourbakos; Giles Campion
Journal:  PLoS One       Date:  2016-09-02       Impact factor: 3.240

Review 8.  Assessment and management of respiratory function in patients with Duchenne muscular dystrophy: current and emerging options.

Authors:  Antonella LoMauro; Maria Grazia D'Angelo; Andrea Aliverti
Journal:  Ther Clin Risk Manag       Date:  2015-09-28       Impact factor: 2.423

9.  Upper limb strength and function changes during a one-year follow-up in non-ambulant patients with Duchenne Muscular Dystrophy: an observational multicenter trial.

Authors:  Andreea Mihaela Seferian; Amélie Moraux; Mélanie Annoussamy; Aurélie Canal; Valérie Decostre; Oumar Diebate; Anne-Gaëlle Le Moing; Teresa Gidaro; Nicolas Deconinck; Frauke Van Parys; Wendy Vereecke; Sylvia Wittevrongel; Michèle Mayer; Kim Maincent; Isabelle Desguerre; Christine Thémar-Noël; Jean-Marie Cuisset; Vincent Tiffreau; Severine Denis; Virginie Jousten; Susana Quijano-Roy; Thomas Voit; Jean-Yves Hogrel; Laurent Servais
Journal:  PLoS One       Date:  2015-02-02       Impact factor: 3.240

10.  Cardiomyocyte-produced miR-339-5p mediates pathology in Duchenne muscular dystrophy cardiomyopathy.

Authors:  Melanie Gartz; Margaret Beatka; Mariah J Prom; Jennifer L Strande; Michael W Lawlor
Journal:  Hum Mol Genet       Date:  2021-11-16       Impact factor: 5.121

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