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Chest, Vol 68, 41-45, Copyright © 1975 by American College of Chest Physicians

ARTICLES

Pulmonary function in Morquio's disease: A study of two siblings

WJ Buhain, G Rammohan and HW Berger

Two siblings with characteristic clinical and radiologic features of Morquio's disease are presented. Detailed pulmonary function tests, including vital capacity, flow rates, lung volumes, airway resistance, and lung compliance, were performed. The ventilatory studies suggested a restricted pattern due to chest cage dysfunction. Hypoxemia was noted in one patient and was shown to be due to right-to-left shunting, probably related to the microatelectasis as a result of the restricted chest cage.


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 ThoraxHome page
P P Walker, E Rose, and J G Williams
Upper airways abnormalities and tracheal problems in Morquio's disease
Thorax, May 1, 2003; 58(5): 458 - 459.
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Copyright © 1975 by the American College of Chest Physicians.