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Biomedical subjects

A A Liebow

Publications and source records attributed to A A Liebow.

At least 19 recordsLinked to original sources

Corrosion cast studies of the bronchial arteries.

The bronchial a. are the nutrient vessels of the lung. Despite being high pressure (systemic) vessels they are of very small size compared to the pulmonary a. and their side branches are normally so minute that they are extremely difficult to visualize during in vivo studies--yet these branches also supply many important mediastinal structures including the esophagus, trachea, lymph nodes, pericardium, aorta, and mediastinal parietal pleura. The bronchial a. can react vigorously to pathological changes in the lung by hypertrophy and increased flow and their precise anatomy has become of increasing importance with the development of interventional techniques involving the systemic circulation of the lungs. In order to demonstrate the distribution and extent of these vessels we have adopted a casting technique using injections of various colors of latex to distinguish between pulmonary a. and veins, bronchial a. and veins and their connections with the pulmonary vascular bed in both normal and diseased lungs.

Adult

Intravascular, bronchiolar, and alveolar tumor of the lung (IVBAT). An analysis of twenty cases of a peculiar sclerosing endothelial tumor.

Twenty cases of an unusual tumor of the lung are described. This tumor usually presents with multiple small, slowly growing pulmonary nodules. Many cases are detected incidentally. Eighty percent are women, and 50% are less than 40 years of age. Survival with tumor can be quite long. However, one half the patients have died, usually of progressive pulmonary insufficiency. This is a peculiar sclerosing tumor of endothelial cell origin.

Adenocarcinoma, Bronchiolo-Alveolar

Eosinophilic granuloma of lung. Clinical aspects of primary histiocytosis in the adult.

One hundred cases of eosinophilic granuloma diagnosed by open lung biopsy were reviewed. There were 60 women and 40 men, ages 18 to over 60 years. Radiographs and clinical histories were contributed by physicians referring the cases for pathological consultation. Clinical follow-up information was obtained in 60 cases. The outcome was generally benign; the 16 asymptomatic patients remained well; 17 others had complete remission of symptoms, 22 had persistent symptoms, though half had partial improvement; 4 patients had progressive disease despite treatment, but only 1 patient died (of bilateral pneumothoraces complicating severe fibrosis). The more severe manifestations were found in young men, who had a higher incidence of pneumothorax, fibrosis and honeycombing, and diabetes insipidus. Many previously reported cases have had a less favorable outcome; milder cases of primary pulmonary histiocytosis have probably been overlooked in the past. In this series the prognostic value of histologic, clinical, or radiologic findings was limited. The diagnosis can often be strongly suggested radiologically, though infectious granulomata must be excluded; it is arguable whether exclusion of sarcoidosis is clinically important. The effectiveness of treatment with adrenal corticosteroids could not be assessed because of lack of controls; some individuals appeared to benefit, but relapse was very unusual in any case. The etiology of EG remains obscure, but in view of the paucity of patients with a history of allergy or asthma, and the lack of eosinophilia in any case, hypersensitivity seems unlikely. Smoking was far more common among these patients (97% altogether; 80% current) than in the general population (about 35%), an unexplained finding.

Adolescent

Lymphomatoid granulomatosis: a clinicopathologic study of 152 cases.

A retrospective study of 152 cases of lymphomatoid granulomatosis (LYG) was undertaken to expand current knowledge about clinical manifestations and therapy, and to identify prognostic indicators in this disease. The major clinical and radiographic features and extra-pulmonary sites of involvement noted in previous studies were essentially confirmed in our study. Adverse prognostic factors included neurologic manifestations, and large numbers of atypical lymphoreticular cells within the pulmonary infiltrate. Unilateral chest lesions and large numbers of small lymphocytes and histiocytes within the infiltrate were associated with a better prognosis. Malignant lymphoma involving lymph nodes developed in 12% of patients. Almost two-thirds of patients have died and the median survival was only 14 months. No particular mode of therapy was found to be satisfactory for LYG, although there was some suggestion that corticosteroids may be useful. Randomized prospective studies are necessary for definitive recommendations regarding therapy.

Adolescent

Pulmonary hyalinizing granuloma.

Twenty patients with pulmonary nodules consisting of concentric hyaline lamellae, usually accompanied by perivascular collections of plasma cells and lymphocytes, were studied. In most instances, the lesions were multiple, bilateral, and mildly symptomatic. Many of these nodules showed all of the staining characteristics of amyloid, but others had an atypical birefringence pattern. No infectious agents were identified, and no consistent pattern of dysproteinemia was observed. Two of the patients had prior histories of tuberculosis. In other cases, the nodules were of unknown origin and pathogenesis. Four cases were complicated by sclerosing mediastinitis, and one, by retroperitoneal fibrosis and amyloidosis. Our current working hypothesis is that these lesions represent an exaggerated and, possibly, continuing immune response, perhaps to one of a number of agents.

Adult

Pulmonary lymphangiomyomatosis. A review.

Anatomic and clinical observations of 28 cases, including 23 previously unpublished, of pulmonary lymphangiomyomatosis are recorded and discussed. This brings the total reported to 57. All patients were women in the reproductive age group with the major complaint of breathlessness. This was usually progressive, and death from pulmonary insufficiency resulted within 10 years. Functional changes were obstructive or restrictive, or both. Pneumothorax, chylous effusions and hemoptysis were frequent complications. Radiographically the lesions initially appear as fine, linear and nodular, predominantly basal densities, and progress to a pattern of bullous change, or honeycombing, involving all portions of the lungs not sparing the region of the costophrenic sinuses as is typical of eosinophilic granuloma. There may be associated pleural effusions. A progressively increasing lung volume is characteristic. The lesions consist of an irregular, nodular or laminar "irrational" proliferation of smooth muscle within all portions of the lung, with loss of parenchyma leading to honeycombing. Proliferated muscle can obstruct bronchioles (with air trapping and formation of bullae often complicated by pneumothorax), venules (with pulmonary hemorrhage and hemosiderosis accompanied clinically by hemoptysis) and lymphatics (with chylothorax or chyloperitoneum). Both thoracic and abdominal lymph nodes and the thoracic duct can also be involved in the myoproliferative process with formation of subsidiary minute channels and obstruction. Renal or perirenal angiomyolipomas can also occur, as exemplified by 2 patients in the present series. Identical pulmonary lesions occasionally occur in tuberous sclerosis. Especially since these patients usually have no neurologic disturbances and are almost women, the possibility of a relationship between tuberous sclerosis and lymphangiomyomatosis must be considered. One feature of note in pulmonary lesions of tuberous sclerosis is the presence of adenomatoid proliferations of epithelium. Such changes were also observed in 2 patients of the present series, and it is remarkable that both of these women had "retarded"children. At present the question of whether by lymphangiomyomatosis is a forme fruste of tuberous sclerosis must be considered as unresolved. It may yield to further investigation, possibility including chromosomal studies.

Adolescent