Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034067 (emphysema)
 11,506 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One-year-old girl was admitted because of recurrent pneumonia and pectus excavatum. Chest X-ray showed hyperlucency at the upper lung field and infiltrated shadow at the lower field of the left lung. Bronchoscopy and bronchograms revealed marked collapse in a long segment of the left main bronchus during expiration. CT scan showed an emphysematous change and a giant bulla of left lung. Angiogram showed right aortic arch. Sternoturnover was performed at 5 years of age for pectus excavatum. After 10 months, left pneumonectomy was performed for bronchomalacia and lobar emphysema. Pathologic findings of the bronchus revealed that the rings were flattened, while the cartilage was microscopically normal.
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PMID:[Congenital bronchomalacia of left main bronchus combined with lobar emphysema, pectus excavatum and right aortic arch--a case report]. 189 73

Tracheal bronchus is an aberrant bronchus that arises most often from the right tracheal wall above the carina and is the result of an additional tracheal outgrowth early in embryonic life. Its incidence ranges between 0.1 and 5%. This anomaly usually is diagnosed incidentally during bronchoscopy or bronchography performed for various respiratory problems. Occasionally, it represents the underlying etiology for chronic pulmonary disease such as emphysema, atelectasis, and persistent or recurrent pneumonia, especially if it involves the right upper lobe and reflects an abnormal pulmonary clearing mechanism. Tracheal bronchus may be associated with other bronchopulmonary anomalies, tracheal stenosis, or Down's syndrome. In the absence of clinical symptoms, a diagnosis of tracheal bronchus does not require any treatment. In patients with recurrent right upper lobe disease and a tracheal bronchus, therapy should include resection of the aberrant bronchus as well as the lobe it supplies.
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PMID:Tracheal bronchus. 359 78

Laryngeal papilloma in children is a frequent disease caused by human papilloma virus (HPV) type 6 or type 11. This disease has a tendency to recur and the changes are histologically benign. In some cases papilloma may affect the lower levels of the respiratory tract. In this study, among 90 patients treated for laryngeal papillomatosis, in four children papilloma of trachea, bronchi and lung tissue were detected in endoscopic and radiological examination. This constitutes 4.4% of all patients. Compact nodules and acquired cysts between 5 and 50 mm long were found in chest X-rays and in computerised tomography. These cysts appeared from 4 to 8 years after establishing a diagnosis of laryngeal papilloma, and 1 year after recognising papilloma in the trachea. In all four children the presence of nodules and cysts in the lungs was preceded by recurrent pneumonia, emphysema or atelectasis of the lungs. All children with laryngeal papillomatosis should have a chest X-ray. Detection of acquired cyst-like changes in lung tissue in children with laryngeal papillomatosis is a warning of future papilloma in the trachea and bronchi, with involvement of lung tissue. In differential diagnosis of these changes in the lungs we should take into consideration the presence of papilloma in the bronchi. A prognosis of papillomatosis in the lower airways in children is always serious.
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PMID:Lower airway papillomatosis in children. 1562 62

This patient was a 28-year-old man who had been treated with steroids for recurrent asthmatic attacks since around the age of 20. At one time the steroid therapy was discontinued and other treatments, including bronchodilator therapy and desensitization therapy, were substituted. At age 28, he first consulted our hospital due to the recurrence of attacks. We found peripheral eosinophlia and a cytologic examination revealed Curschmann spirals in his sputum. Migratory infiltrate recurred, and steroids and antibiotics were prescribed each time. A confirmed diagnosis of allergic bronchopulmonary aspergillosis was made at age 35. The patient was treated with continuous oral steroids for recurrent infiltrates. After the start of inhale steroid therapy at age 44, the frequency of attacks decreased and tapering of the oral steroids became possible. It is notable that lung tissue destruction progressed by recurrent pneumonia and emphysema extended into all portions of the lung. Furthermore, this case history is of value since it yields a series of images taken over a period of 27 years.
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PMID:[A case of allergic bronchopulmonary aspergillosis followed by diagnostic imaging for 27 years]. 1599 90

Mutations in the gene encoding surfactant protein C (SP-C) SFTPC have been found to be associated with chronic interstitial lung disease. A 5-year-old girl oxygen dependent from birth and affected by interstitial lung disease (ILD) is heterozygous for a T to C change in exon 3 resulting in the substitution of threonine for isoleucine at codon 73 (173T), already described in association with ILD. We studied 25 members of her family where the 173T mutation in the SP-C gene is associated to chronic pulmonary diseases. Five members in the mother's family showed respiratory diseases with great diversity in clinical features: her mother was affected by restrictive pneumopathy and emphysema, her grand-mother by asthma and recurrent pneumonia, 2 uncles underwent lung transplantation in the adult age, an aunt was clinically diagnosed having pulmonary fibrosis. All the family members affected by pulmonary diseases and one with no clinical symptoms showed the presence of the mutation 173T. Among the other family members the mutation was found in six subjects for whom no clinical data were available, yet. Our results confirm that heterozygosity for the mutation 173T may cause chronic inflammation of the lung or progressive pulmonary fibrosis. In addition, the possibility to study a large pedigree allowed us to perform a genotype-phenotype correlation indicating a marked phenotypic variability. The diversity in symptoms, age at onset, clinical course, duration of lung disease in the relatives sharing this mutation indicates an incomplete penetrance of the mutation. This might be due to the influence of other genetic factors thus indicating that the phenotype may be complicated by additional components.
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PMID:[Genetic basis in chronic interstitial familial pneumopathy. Familial study of SFTPC]. 1691 Apr 60

Chronic Obstructive Pulmonary Disease (COPD) is a progressive disease. Frequent pneumonias and exacerbations are known to accelerate its progression. We present a case of severe emphysema whose lung function paradoxically improved following recurrent pneumonia, without lung volume reduction surgery (LVRS). A 54-year-old female with severe COPD presented for LVRS evaluation. She was not a candidate for the surgery because of the unsuitable anatomic distribution of her emphysema. The patient experienced recurrent pneumonia over the years but her lung function and oxygen requirement showed marked improvement. Follow-up imaging studies showed decreased lung volumes and focal fibrotic changes. We believe that the improvement in her lung function overtime is the reflection of lung volume reduction as a result of parenchymal remodeling due to repeated lung infection. These findings seen in our patient contribute important information for the continued effort in developing nonsurgical lung volume reduction techniques.
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PMID:Lung Volume Reduction following Recurrent Pneumonia: An Unusual Finding in a COPD Patient. 2837 84

A 50-year-old former smoker presented with recurrent pneumonia, fever and atypical chest pain on and off for a year and was treated with antibiotics several times. Meanwhile, her lung function deteriorated with an obstructive pattern and therefore she was treated with inhalation medication without effect. Tests of the heart, bronchoscopy, gastroscopy and a CT-scan of the lungs did not reveal the cause. Finally, an FDG-PET scan showed inflammation of the tissue on the backside of a 21-year-old silicone chest implant, which was intact but encapsulated. The chest implants were removed after which the patient has remained free of infections. However, the lung function impairment did not improve, and a new HRCT-scan demonstrated widespread emphysema.
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PMID:21-year-old silicone breast implants causing recurrent pneumonia, chest pain and coughing. 3209 86