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ACD is a rare disease. As of 2011, about 100 cases had been reported. It is likely an under-recognized cause of death shortly after birth because diagnosis requires microscopic examination of lung tissue or specialized genetic testing, or death can be attributed to severe heart or intestinal congenital abnormalities which frequently occur in ACD.
Rare [2] Swyer–James syndrome ( SJS ), also called Swyer–James–MacLeod syndrome , is a rare disease of the lungs , characterized by a small lung or part of lung. [ 1 ] Typical symptoms are of recurrent respiratory tract infections , but some have no symptoms.
Computed Tomography (CT): CT scans provide a more detailed view, revealing a clear demarcation between the calcified lung parenchyma and the spared subpleural area. The black pleura sign is evident as a peripheral lucent rim beneath the pleura, highlighting the contrast between the dense, calcified lung tissue and the less affected subpleural ...
Childhood interstitial lung disease, sometimes abbreviated as ChILD, is a family of rare chronic and complex disorders that affect the lungs of children. [1] In the lungs, these disorders affect the interstitium, which is the space around the alveoli. The alveoli are the air sacs of the lungs. [2]
Pulmonary Alveolar Microlithiasis patients, families, and caregivers are encouraged to join the NIH Rare Lung Diseases Consortium Contact Registry. This is a privacy protected site that provides up-to-date information for individuals interested in the latest scientific news, trials, and treatments related to rare lung diseases. [citation needed]
Primary ciliary dyskinesia (PCD) is a rare, autosomal recessive genetic ciliopathy, that causes defects in the action of cilia lining the upper and lower respiratory tract, sinuses, Eustachian tube, middle ear, fallopian tube, and flagella of sperm cells.
Birt-Hogg-Dubé Syndrome patients, families, and caregivers are encouraged to join the NIH Rare Lung Diseases Consortium Contact Registry. This is a privacy-protected site that provides up-to-date information for individuals interested in the latest scientific news, trials, and treatments related to rare lung diseases.
The morbidity associated with DIPNECH is due to the associated obstructive lung disease. The lung disease tends to be slowly progressive, but given enough time can lead to significant disability and require supplemental oxygen therapy. [8] There have been reports of lung transplantation in the setting of end-stage DIPNECH. [11]