Cor pulmonale and acute liver necrosis, due to upper airway obstruction as part of pycnodysostosis


A male patient with pycnodysostosis suffered from chronic respiratory insufficiency and pulmonary hypertension. This was caused by concomitant upper airway obstruction, resulting from a low implanted uvula and a long soft palate, in combination with glossoptosis and retrognathia due to the flattened mandibular angles. An inter-current respiratory infection… (More)
DOI: 10.1007/BF00572773

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