Acid maltase deficiency: treatment of respiratory insufficiency with cuirass respirator.
Three patients with the late-onset form of acid maltase deficiency showed a gradual weakening of proximal limb and trunk muscles leading to severe respiratory insufficiency. Considerable deterioration of pulmonary function occurred owing to a vicious cycle of hypoventilation and exhaustion. Treatment by nocturnal cuirass ventilation with tailor-made shells was successful. The muscle weakness still progressed very slowly but there were no more respiratory problems.
D000328:Adult; D006008:Glycogen Storage Disease / Q000150:complications*; D006009:Glycogen Storage Disease Type II / Q000150:complications*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012131:Respiratory Insufficiency / Q000209:etiology / Q000628:therapy*; D012122:Ventilators, Mechanical