Abstract
Respiratory and others physiological functions were followed in four infants presenting clinical and radiological findings of Jeune's syndrome. Three infants died. The principal respiratory disturbances were tachypnoea, hypoxemia /paO2=40–60 mmHg/ compensed respiratory acidosis /paCO2=42–60mmHg/ compensed respiratory acidosis /paCO2=42–60mmHg/. It was present also a decrease of VT and an increase of physiological dead speace /VD phisiol./ and of venous asmixture. The signs of hypoxia were evident on the EEG. Electromyographical examinations revealed in several sites including intercostal muscles widespread signs of neurogenic muscolar sufference. The surviving infant shows at the age of 11 months: respiratory rate=66/min, VT=0,81 ml/cm, VE=599 ml/kg, VD phisiol.=35,2%, QVA/QT=25,4%. It is suggested that should be taken into account, these findings when considering surgical therapy.
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Bevilacqua, G., Grzincich, G. ASPHYXIATING THORACIC DYSTROPHY. Pediatr Res 10, 556 (1976). https://doi.org/10.1203/00006450-197605000-00011
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DOI: https://doi.org/10.1203/00006450-197605000-00011
