Inspiratory muscle training in a child with nemaline myopathy and organ transplantation.
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.
Smith
Barbara K
BK
Department of Physical Therapy University of Florida, Gainesville, FL, USA. bksmithphhp.ufl.edu
Bleiweis
Mark S
MS
Zauhar
Joni
J
Martin
A Daniel
AD
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
Chest. 2001 Apr;119(4):1108-13
11296177
Neuromuscul Disord. 2000 Jun;10(4-5):299-306
10838258
Muscle Nerve. 2000 Aug;23(8):1257-60
10918264
Curr Opin Neurol. 2007 Oct;20(5):583-9
17885449
Med Sci Sports Exerc. 1982;14(5):377-81
7154893
Respir Care. 2008 Nov;53(11):1490-4
18957152
Intensive Care Med. 2004 Jun;30(6):1117-21
14767593
Neurology. 2003 Feb 25;60(4):665-73
12601110
Respiration. 1988;54(3):153-61
3247515
Am J Respir Crit Care Med. 2002 Aug 15;166(4):518-624
12186831
Chest. 1994 Feb;105(2):475-82
8306750
Pediatr Neurol. 1997 May;16(4):344-6
9258972
Hum Mol Genet. 2004 Nov 1;13(21):2633-45
15367485
J Mol Med (Berl). 2008 Nov;86(11):1197-204
18574571
Muscle Nerve. 2009 Mar;39(3):406-8
19208402
Muscle Nerve. 2003 Jul;28(1):93-100
12811779
Muscle Nerve. 1991 Nov;14(11):1103-9
1745285
Chest. 2001 Sep;120(3):765-9
11555507
Paediatr Respir Rev. 2003 Sep;4(3):243-9
12880760
Crit Care Med. 2007 Sep;35(9 Suppl):S602-8
17713416
Curr Heart Fail Rep. 2009 Jun;6(2):95-101
19486593
Thorax. 1996 Jun;51(6):601-5
8693441
Muscle Nerve. 2004 Oct;30(4):470-80
15372535