Inspiratory muscle training in Morquio's syndrome: a case study.
We reported a case of MPS IV A presented with dyspnea on exertion and respiratory muscle weakness. The patient underwent inspiratory muscle training (IMT) using threshold loading for 18 weeks. After 6 weeks of initial IMT, aerobic exercise training consisting of walking was added to the treatment program. Inspiratory muscle strength increased 70%, and 6-minute walk test (6MWT) distance increased to 47 m. With the inclusion of aerobic exercise training, additional increases in inspiratory muscle strength (7%) and 6MWT distance (26.5 m) were obtained. Exertional dyspnea improved from severe to slight after 6 weeks of IMT, and to very slight after additional 12 weeks of combined aerobic training and IMT. Health-related quality of life improved especially in social function, emotional function, vitality, and physical role. In conclusion, inspiratory muscles can be trained with the improvement of muscle strength in a patient with Morquio's syndrome.
Savci
Sema
S
School of Physical Therapy and Rehabilitation, Hacettepe University, Ankara, Turkey. semasavci@yahoo.com
Ozturk
Melda
M
Inal-Ince
Deniz
D
Gultekin
Zuhal
Z
Arikan
Hulya
H
Sivri
H Serap Kalkanoglu
HS
D000293:Adolescent; D004417:Dyspnea / Q000209:etiology / Q000503:physiopathology / Q000534:rehabilitation*; D005081:Exercise Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D009085:Mucopolysaccharidosis IV / Q000150:complications / Q000503:physiopathology / Q000534:rehabilitation*; D011788:Quality of Life; D012132:Respiratory Muscles / Q000503:physiopathology*; D012138:Respiratory Therapy / Q000379:methods*