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)

Intratracheal injection of rabbit whole leukocyte homogenate induced emphysema-like lesions in rabbits. The lesions were produced only by preparations having elastinolytic activity. The pathological aspects appeared similar to those induced by the administration of porcine pancreatic elastase. The pulmonary changes, resembling several of the anatomic appearances of panacinar human emphysema, may be a suitable experimental model for studying histogenesis of panacinar human emphysema. Numerous abnormal fenestrations were present in air spaces walls and were a constant feature in opposition to that has been reported in elastase-treated hamsters. For the presence of a prominent dilatation of the periarterial lymphatic network, this experimental model might be used also for studying the ultrastructural features of pulmonary lymphatic vessels.
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PMID:[Pulmonary damage in the rabbit caused by porcine pancreatic elastase and leukocyte lysate: unusual microscopic aspects]. 26 66

A premature male infant with hyaline membrane disease succumbed suddenly during continuous positive pressure respirator therapy. Autopsy revealed a very rare complication to be the cause of sudden death: air embolization to pulmonary veins, right ventricle and superior and inferior venae cavae as well as interstitial and subpleural emphysema, pneumothorax, pneumomediastinum and pneumopericardium. The important role of lymphatic vessels as a port of entry for air is stressed.
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PMID:[Sudden death caused by massive air embolism during respirator therapy in the newborn]. 37 5

Open lung biopsy in a 38-year-old female with Pringle-Bourneville syndrome and recurrent pneumothorax revealed a micronodular pneumocyte II hyperplasia, a new entity probably associated with the tuberous sclerosis syndrome. The lesion caused an obstruction of the alveolar lymphatic vessels and alveolar ducts, resulting in an emphysema-like picture. This cystic dilation of alveoli and draining lymphatics followed by rupture caused the recurrent pneumothorax. The epithelial pneumocytic nature of the lesion was confirmed by immunohistochemistry and electronmicroscopy.
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PMID:Micronodular hyperplasia of type II pneumocytes--a new lung lesion associated with tuberous sclerosis. 207 Oct 93

A left upper lobectomy was performed on a 27-day-old male infant with clinically suspected congenital lobar emphysema. At operation a cranial pericardial hiatus was also noted. The infant died ten days postoperatively of a septicaemia of extrapulmonary origin. An autopsy was not performed. Histologic examination of the left upper lobe revealed the changes to be pulmonary lymphangiectasis, which is only rarely unilateral or unilobar. In this infant alveolar rupture is thought to have provided air entry into the primarily ectatic lymphatic vessels with further spread of air. The clinical symptoms were due to this complication. This interpretation is supported by the finding of numerous foreign-body giant cells in the lymph vessels, an extraordinary finding in congenital pulmonary lymphangiectasis.
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PMID:[Congenital unilobar pulmonary lymphangiectasis]. 394 19

In a 50-year old patient with a long history of chronic obstructive airway disease and pulmonary emphysema, unusual solid spongious areas adjacent to bullous tissue were detected by bullectomia because of mediastinal displacement and dyspnoea on exertion. Pathological anatomical diagnosis showed villous framework in the marginal regions of bullous transformed parenchyma. According to pathognomonic histological finding the lesion is known as placentoid malformation or placentoid bullous transmogrification, respectively. This disease must be differentiated against rare cystic tumours such as alveolar adenoma or sclerosing haemangioma as well as congenital lesions e.g. adenomatoid cystic malformation. The lesion presented here includes hamartomatous features, such as the presence of leiomyomatoid proliferations of smooth muscle cells and fatty tissue embedded in the villous stroma. The clinically predominant emphysematous transformation of the adjacent lung tissue is pathogenetically the result of a valve formation in combination with unphysiological traction forces. The ectatic lymphatic vessels in peripheral tissue may perhaps be of etiological importance. According to former studies this may be an congenital malformation with progressive development. Resection of affected lung parenchyma seems to be curative: so far, no recurrences have been noticed.
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PMID:[Placentoid malformation of the lung as differential diagnosis of localized emphysema]. 933 86

We report the case of 2-week-old female infant with cystic lung disease who presented with mild tachypnea and had no history of mechanical ventilation. Chest CT demonstrated multiple air-filled cystic lesions in right upper lobe, and the patient subsequently underwent a right upper lobectomy. Histology revealed cystic lesions located in the pulmonary parenchyma and showed that the lesions were lined by lymphatic endothelium and were communicating with dilated lymphatic vessels in the interstitium. Additionally, multinucleated foreign body giant cells were attached to the lumen of the cyst. On the basis of these findings, we considered this a case of persistent interstitial pulmonary emphysema (PIPE) with massive pneumatic expansion of the lymphatic vessels, resulting in cystic lesions with lymphatic endothelium in the pulmonary parenchyma. While PIPE is extremely rare in term non-ventilated infants, our case demonstrated that this disease should be added to the differential diagnosis of cystic lung diseases with lymphatic endothelium even in infants without mechanical ventilation. When cystic lesions and symptoms persist despite conservative treatment, open or thoracoscopic resection is an appropriate option for diagnosis and treatment.
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PMID:Massive pneumatic expansion of lymphatic vessel resulting in cystic lesions in the pulmonary parenchyma: a rare case of persistent interstitial pulmonary emphysema in a non-ventilated infant. 2321 11

Congenital pulmonary lymphangiectasia (CPL) is a rare congenital disorder that typically presents with intractable respiratory failure in the first few days of life. There is an association non-immun hydrops and CPL. In this study we reviewed four CPL cases between January 2006 and January 2014 among 684 fetal-pediatric autopsies. All cases were in the second trimester. In light microscopy there were marked dilatated channels in the subpleural -peribronchial-subseptal region of the lungs. The channels were lined with flattened cells which were expressing CD 31 and D2-40, negative for CD34. Although pulmonary interstitial emphysema (PIE) was considered an important differential diagnosis, a giant cell reaction surrounding the interstitial cystic lesions, a histological hallmark of PIE. CPL is characterized by dilatation of the pulmonary lymphatic vessels and occurs as a congenital anomaly. Noonan classified it into three groups. Primary developmental defect of pulmonary lymphatics is group 3. Group 3 is called also as CPL; normal regression of the connective tissue elements fails to occur after the 16th week of fetal life, associated with an aggressive clinical course, poor prognosis. In fetal autopsy examination CPL should be recognized if there is a fetus with pleural effusion, non-immune hydrops. There is no clinical evidence for CPL.
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PMID:Rare congenital pulmonary malformation with diagnostic challenging: congenital pulmonary lymphangiectasia, report of four autopsy cases and review of literature. 2514 8