title: Inspiratory muscle training in a child with nemaline myopathy and organ transplantation.
abstract: OBJECTIVE
  To report the use of inspiratory muscle strength training to treat repeated ventilatory insufficiency in a child with nemaline myopathy who underwent cardiac and renal transplantation.
  
  
  METHODS
  Case report.
  
  
  METHODS
  Pediatric intensive care unit of a tertiary care university teaching hospital.
  
  
  METHODS
  A 16-yr-old female with nemaline myopathy affecting cardiorespiratory function, recent organ transplantation, and delayed postoperative ventilatory recovery.
  
  
  METHODS
  Inspiratory muscle strength training was provided 5 days weekly for 2 wks, accompanied by progressive weaning from noninvasive ventilation.
  
  
  RESULTS
  Maximal inspiratory pressure increased from -36.7 cm H2O to -77.8 cm H2O, accompanied by improved inspiratory flow, volume, pressure activation and power. During the training period, the patient was weaned from continuous noninvasive ventilatory assist to her preoperative level of ventilatory function.
  
  
  CONCLUSIONS
  Inspiratory muscle training may be a beneficial component of care for children with nemaline myopathy who experience acute ventilatory insufficiency.
authors.affiliation: Department of Physical Therapy University of Florida, Gainesville, FL, USA. bksmithphhp.ufl.edu
authors.forename: Barbara K
authors.initials: BK
authors.lastname: Smith
authors.forename: Mark S
authors.initials: MS
authors.lastname: Bleiweis
authors.forename: Joni
authors.initials: J
authors.lastname: Zauhar
authors.forename: A Daniel
authors.initials: AD
authors.lastname: Martin
mesh_terms: D000293:Adolescent; D001945:Breathing Exercises; D005260:Female; D016027:Heart Transplantation; D006784:Hospitals, Teaching; D006801:Humans; D015278:Intensive Care Units, Pediatric; D016030:Kidney Transplantation; D017696:Myopathies, Nemaline / Q000503:physiopathology*; D017063:Outcome Assessment, Health Care; D012129:Respiratory Function Tests / Q000379:methods; D012131:Respiratory Insufficiency / Q000628:therapy; D012138:Respiratory Therapy; D015300:Ventilator Weaning
references.citation: Chest. 2001 Apr;119(4):1108-13
references.pmid: 11296177
references.citation: Neuromuscul Disord. 2000 Jun;10(4-5):299-306
references.pmid: 10838258
references.citation: Muscle Nerve. 2000 Aug;23(8):1257-60
references.pmid: 10918264
references.citation: Curr Opin Neurol. 2007 Oct;20(5):583-9
references.pmid: 17885449
references.citation: Med Sci Sports Exerc. 1982;14(5):377-81
references.pmid: 7154893
references.citation: Respir Care. 2008 Nov;53(11):1490-4
references.pmid: 18957152
references.citation: Intensive Care Med. 2004 Jun;30(6):1117-21
references.pmid: 14767593
references.citation: Neurology. 2003 Feb 25;60(4):665-73
references.pmid: 12601110
references.citation: Respiration. 1988;54(3):153-61
references.pmid: 3247515
references.citation: Am J Respir Crit Care Med. 2002 Aug 15;166(4):518-624
references.pmid: 12186831
references.citation: Chest. 1994 Feb;105(2):475-82
references.pmid: 8306750
references.citation: Pediatr Neurol. 1997 May;16(4):344-6
references.pmid: 9258972
references.citation: Hum Mol Genet. 2004 Nov 1;13(21):2633-45
references.pmid: 15367485
references.citation: J Mol Med (Berl). 2008 Nov;86(11):1197-204
references.pmid: 18574571
references.citation: Muscle Nerve. 2009 Mar;39(3):406-8
references.pmid: 19208402
references.citation: Muscle Nerve. 2003 Jul;28(1):93-100
references.pmid: 12811779
references.citation: Muscle Nerve. 1991 Nov;14(11):1103-9
references.pmid: 1745285
references.citation: Chest. 2001 Sep;120(3):765-9
references.pmid: 11555507
references.citation: Paediatr Respir Rev. 2003 Sep;4(3):243-9
references.pmid: 12880760
references.citation: Crit Care Med. 2007 Sep;35(9 Suppl):S602-8
references.pmid: 17713416
references.citation: Curr Heart Fail Rep. 2009 Jun;6(2):95-101
references.pmid: 19486593
references.citation: Thorax. 1996 Jun;51(6):601-5
references.pmid: 8693441
references.citation: Muscle Nerve. 2004 Oct;30(4):470-80
references.pmid: 15372535