Swyer–James Syndrome
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Swyer–James syndrome (SJS), also called Swyer–James–MacLeod syndrome, is a disease of the lungs, characterized by a small lung or part of lung. Typical symptoms are of recurrent respiratory tract infections, but some have no symptoms. As a result of childhood
bronchiolitis obliterans Bronchiolitis obliterans (BO), also known as obliterative bronchiolitis, constrictive bronchiolitis and popcorn lung, is a disease that results in obstruction of the smallest airways of the lungs (bronchioles) due to inflammation. Symptoms include ...
, typically following adenovirus infection, there is a reduction in blood vessels and overextended
alveoli Alveolus (; pl. alveoli, adj. alveolar) is a general anatomical term for a concave cavity or pit. Uses in anatomy and zoology * Pulmonary alveolus, an air sac in the lungs ** Alveolar cell or pneumocyte ** Alveolar duct ** Alveolar macrophage * ...
, resulting in air trapping in one or part of one lung. It is rare.


Signs and symptoms

Often, there are no symptoms and the disease is detected when investigating another problem. Sometimes there is shortness of breath on exertion, wheeze, reduced ability to exercise, cough and chest pain.


Cause and mechanism

Swyer–James syndrome is a manifestation of postinfectious obliterative bronchiolitis. In SJS, the involved
lung The lungs are the primary organs of the respiratory system in humans and most other animals, including some snails and a small number of fish. In mammals and most other vertebrates, two lungs are located near the backbone on either side of t ...
or portion of the lung does not grow normally and is slightly smaller than the opposite lung. The characteristic radiographic appearance is that of pulmonary hyperlucency, caused by overdistention of the
alveoli Alveolus (; pl. alveoli, adj. alveolar) is a general anatomical term for a concave cavity or pit. Uses in anatomy and zoology * Pulmonary alveolus, an air sac in the lungs ** Alveolar cell or pneumocyte ** Alveolar duct ** Alveolar macrophage * ...
in conjunction with diminished
arterial flow Hemodynamics or haemodynamics are the dynamics of blood flow. The circulatory system is controlled by homeostatic mechanisms of autoregulation, just as hydraulic circuits are controlled by control systems. The hemodynamic response continuously m ...
. and has been linked to
adenovirus Adenoviruses (members of the family ''Adenoviridae'') are medium-sized (90–100 nm), nonenveloped (without an outer lipid bilayer) viruses with an icosahedral nucleocapsid containing a double-stranded DNA genome. Their name derives from the ...
type 21, a type of adenovirus infection.


Diagnosis

In appearance Swyer–James normally leaves shadowing in a
CT scan A computed tomography scan (CT scan; formerly called computed axial tomography scan or CAT scan) is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers ...
in the upper lobar regions of one or (rarely) both lungs. People with the illness operate in much the same way as patients with mild
bronchiectasis Bronchiectasis is a disease in which there is permanent enlargement of parts of the bronchi, airways of the lung. Symptoms typically include a chronic cough with sputum, mucus production. Other symptoms include shortness of breath, hemoptysis, co ...
. As a result, the illness can go undiagnosed for some time. With current pharmaceutical developments, the prognosis is good for sufferers of the illness to lead normal and healthy lives.


History

It was found by English chest physician William Mathiseon MacLeod, and (simultaneously) by physician Paul Robert Swyer and radiologist George James in the 1950s in Canada.


References


External links

{{DEFAULTSORT:Swyer-James syndrome Syndromes affecting the lung Lung disorders