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  • Title: Respiratory failure and pulmonary hypertension associated with Klippel-Feil syndrome.
    Author: Suga K, Motoyama K, Hara A, Kume N, Matsunaga N, Kametani R, Matsuzaki M.
    Journal: Ann Nucl Med; 1999 Dec; 13(6):441-6. PubMed ID: 10656282.
    Abstract:
    A 28-year-old woman with a deformed thorax and kyphoscoliosis associated with Klippel-Feil syndrome developed respiratory failure with pulmonary hypertension. Pulmonary 133Xe ventilation and 99mTc-MAA perfusion scintigraphies showed maldistributions of lung ventilation and perfusion, and noticeably delayed 133Xe washout from the lungs. Dynamic breathing MR imaging showed poor and/or asynchronous respiratory movements of the chest wall and diaphragm. These findings indicate that the perfusion-ventilation imbalance, the decreased ventilatory turnover, and expiratory flow from the alveolar space partly derived from the impaired respiratory mechanics may be responsible for the respiratory complications in this patient.
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