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Pulmonary agenesis | |
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Chest radiograph of a woman with unilateral pulmonary agenesis | |
Specialty | Pulmonology |
Pulmonary agenesis is an inborn lung underdevelopment that is rare and potentially lethal.[1] The disorder is caused by a complete developmental arrest of the primitive lung during embryonic life, and it is often associated with other developmental defects.[2] Bilateral and unilateral pulmonary agenesis are classified, depending on whether one side of the lung or both sides are affected. Bilateral pulmonary agenesis is lethal, while the mortality rate of unilateral pulmonary agenesis is higher than 50%.[3] Depending on the severity, the symptom ranges from none to various respiratory complaints.[4] It is detectable prenatally, however, its nonspecific clinical features act as the obstacle for diagnosing.[2] The exact cause of pulmonary agenesis is still obscure. However, theories have been raised regarding the vascular, iatrogenic, viral and genetic causes of pulmonary agenesis in an attempt to explain the pathogenesis of the disorder.[5] In most cases of pulmonary agenesis, surgical resection is performed to remove the malformed lobe or the entire defected lung of the patient depending on the severity of the respiratory impairment.[6]
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