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Bronchomalacia can best be described as a birth defect of the bronchus in the respiratory tract. Congenital malacia of the large airways is one of the few causes of irreversible airways obstruction in children, with symptoms varying from recurrent wheeze and recurrent lower airways infections to severe dyspnea and respiratory insufficiency.
Differential diagnosis includes non-specific interstitial pneumonia, pulmonary Langerhans cell histiocytosis, respiratory bronchiolitis-associated interstitial lung disease, and hypersensitivity pneumonia. DIP is primarily treated by quitting smoking, but it may not be enough in all cases. In moderate to severe cases, corticosteroids are used.
In most cases the outcome of a fetus with CPAM is very good. In rare cases, the cystic mass grows so large as to limit the growth of the surrounding lung and cause pressure against the heart. In these situations, the CPAM can be life-threatening for the fetus. CPAM can be separated into five types, based on clinical and pathologic features. [2]
The lung tissue lies within the same visceral pleura as the lobe in which it occurs. [1] Males and females are equally affected. [1] The arterial supply is usually derived from the lower thoracic or upper abdominal aorta. Venous drainage is usually to the left atrium via pulmonary veins establishing a left to left shunt.
The treatment is dependent on the severity of respiratory impairment and the underlying etiology of the disorder. [13] In most cases, surgical resection is performed to remove the nonfunctioning lobe or the entire defected lung. [13] Removing the malformed part of the lung helps reduce symptoms and chances of lung infection. [18]
Imaging shows upper-lobe emphysema, and lower-lobe interstitial fibrosis. [4] CFPE is often complicated by pulmonary hypertension , acute lung injury , lung cancer , and coronary artery disease . [ 4 ]
Necrotizing pneumonia (NP), also known as cavitary pneumonia or cavitatory necrosis, is a rare but severe complication of lung parenchymal infection. [1] [2] [3] In necrotizing pneumonia, there is a substantial liquefaction following death of the lung tissue, which may lead to gangrene formation in the lung.
Around 80% of people with primary ciliary dyskinesia experience respiratory problems beginning within a day of birth. Many have a collapsed lobe of the lung and blood oxygen low enough to require treatment with supplemental oxygen. [1] Within the first few months of life, most develop a chronic mucus-producing cough and runny nose. [1]