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Newborn infants present with grunting, chest retractions and cyanosis followed by rapidly progressive respiratory failure refractory to ventilation and ECMO,
and have also presented as persistent pulmonary hypertension of the newborn .

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Pulmonary opacification, reticular¨Cgranular
infiltrates and air bronchograms are seen on chest radiographs.
ABCA3 (_/_) mice have grossly reduced surfactant phosphatidyl
glycerol levels and die of respiratory failure soon after birth .
There are no known treatments for lung disease resulting from this
mutation and the infants die within the first month of life despite
maximal medical therapy.
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