emphysema

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Related to lobar emphysema: congenital lobar emphysema

emphysema

(ĕmfĭsē`mə), pathological or physiological enlargement or overdistention of the air sacs of the lungs. A major cause of pulmonary insufficiency in chronic cigarette smokers, emphysema is a progressive disease that commonly occurs in conjunction with chronic bronchitis. It is found predominantly in people over age 45, but a genetically based early-onset form also exists. Symptoms are difficulty in breathing, cough with thick sticky sputum, and a bluish tinge of the skin. Progressive disease can result in disability, and in severe cases heart or respiratory failure and death.

Causes

Cigarette smoking is the cause of most cases of emphysema. Tobacco smoke damages the lungs' alveoli, the tiny air sacs through which inhaled oxygen is transferred to the bloodstream and carbon dioxide is passed back to the lungs to be exhaled. The lungs become less elastic and breathing becomes increasingly difficult. The genetic form of emphysema occurs earlier in life (worsened by, but not dependent upon cigarette smoking). It is caused by a rare genetic deficiency of the protein alpha1-antitrypsin. In the absence of antitrypsin, which normally functions to protect the lungs from damage, the walls of the alveoli are attacked by chemicals released in alveoli in response to tobacco smoke and air pollutants.

Treatment

Emphysematous lung damage is irreversible. Its progression can be slowed by giving up smoking. Treatment is aimed at increasing the functional capacity of the lungs and may include bronchodilators, administration of supplemental oxygen, or lung transplantation. Surgical removal of affected lung tissue (lung volume reduction surgery), aimed at allowing healthy areas of the lung room to function, is being studied for its effectiveness and safety. The genetic form is treated with supplemental antitrypsin administered by infusion or by a gene therapy technique that uses T cells (special immune cells that identify diseased or deformed cells) to deliver it to the desired cell sites.

emphysema

[‚em·fə′sē·mə]
(medicine)
A pulmonary disorder characterized by overdistention and destruction of the air spaces in the lungs.

emphysema

Pathol
1. a condition in which the air sacs of the lungs are grossly enlarged, causing breathlessness and wheezing
2. the abnormal presence of air in a tissue or part
References in periodicals archive ?
Congenital lobar emphysema is a rare cause of severe respiratory distress in neonates, which is characterized by overdistension of a lobe or segment of lobe by a "check valve" mechanism leading to mediastinal shift and progressive respiratory distress.
Fiorino, "A novel case report of congenital lobar emphysema in a patient with williams-beuren syndrome," Chest, vol.
Yalpin, "Congenital lobar emphysema: evaluation and long-term follow-up of thirty cases at a single center," Pediatric Pulmonology, vol.
Ismeal, "Bilateral congenital lobar emphysema: a rare cause for respiratory distress in infancy," Annals of Thoracic Medicine, vol.
1)CT thorax showed congenital lobar emphysema left upper lobe and partially obliterated left upper lobe bronchus.
Congenital lobar emphysema is characterized by over inflation of the pulmonary lobe (gas trapping) and is caused by localized bronchial obstruction.9,10The causative factor can be found only in half of the cases which include either partial bronchial obstruction or intrinsic alveolar disease.
The reason for male predominance is unknown.4,5 It usually presents in the neonatal period but diagnosis may be delayed up to 5-6 months in 5% patients.3 It can also be diagnosed on antenatal ultrasound.6 The signs of presentation range from mild tachypnoea, wheeze to severe dyspnoea with cyanosis.4 Congenital heart disease has an association with congenital lobar emphysema.7 In literature 12-20% concomitant CHD have been repor ted.9 The treatment of congenital lobar emphysema is surgical excision of the affected lobe.10 We share our experience of successfully managing a case of congenital lobar emphysema.
Findings were due to congenital lobar emphysema involving left upper lobe.
"Congenital Lobar Emphysema Mimicking as Persistent Pneumonia in a Newborn." Clinics and Practice 1.4 (2011): e101.PMC.
Congenital lobar emphysema usually presents at birth (33%) or within the first month of life (50%) with moderate respiratory distress.
Congenital lobar emphysema: Differential diagnosis and therapeutic approach.
The most prominent late complication of these malformations include repeated infections, haemorrhage, erosion into adjacent structures or spontaneous pneumothorax.3 Surgical removal of the symptomatic cyst is the rule; however, observation is being carried out by pulmonologists in asymptomatic individuals or those with mild symptoms in Congenital Lobar Emphysema on the premise that spontaneous resolution has been documented.