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Pulmonary Alveolar Proteinosis and Pulmonary Hypertension
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Pulmonary alveolar proteinosis and pulmonary hypertension occurs in approximately one-third of GATA2 deficiency patients. These pulmonary changes seen in this disease likely reflect pulmonary alveolar macrophage dysfunction and defective transcriptional regulation of endothelial nitric oxide synthetase.
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