1/6. Reversible hyperinflation in emphysema.pulmonary emphysema results in hyperinflation of the lungs and concomitant changes in the configuration of the thoracic cavity. We describe a patient who underwent bilateral lung transplantation for emphysema due to alpha 1 antitrypsin deficiency. Dramatic changes in chest dimensions and configuration occurred following transplantation, demonstrating the dynamic and reversible nature of the thoracic cavity abnormalities of emphysema.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/6. Berry aneurysms, cirrhosis, pulmonary emphysema, and bilateral symmetrical cerebral calcifications: a new syndrome.Familial idiopathic nonarteriosclerotic cerebral calcification (FINCC) constitutes a rare but pathologically well defined disorder. Thus far, central nervous system symptoms and signs have been the only recorded expression of this disease. Autosomal dominant and autosomal recessive inheritance have both been postulated as cause. We describe three sibs who had symmetrical cerebral calcifications, but three also had cirrhosis and pulmonary emphysema; two had congenital cerebral aneurysms. All were male and of short stature; they also had delayed development and seizures, and two had other neurologic deficits. One sib died at age 3 years of hepatic failure and portal hypertension. Ruptured cerebral aneurysms led to the death of the other two boys at ages 8 and 13 years. The cerebral calcifications symmetrically involved the basal ganglia and thalami, the dentate nucleus, and the cortical and subcortical areas of the cerebrum. The liver was studied by sequential biopsies in two of the children and in all three by autopsy. Fatty degeneration and portal fibrosis preceded a periportal and micronodular cirrhosis. Severe bilateral pulmonary emphysema was present in one sib at age 12 years, whereas all three had bullae and cysts at autopsy. Ruptured left middle cerebral artery aneurysms were demonstrated in two sibs, and one also had aneurysms of the anterior and posterior communicating arteries. We conclude that in this family FINCC is a complex pleiotropic mendelian mutation, either of autosomal or X-linked recessive nature, whose basic pathogenesis remains unknown but may involve a metabolic defect. This form of FINCC may be a previously undescribed syndrome or a form of FINCC in which extraneural manifestations were previously overlooked.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/6. The vanishing lung syndrome associated with pulmonary sarcoidosis.Two patients are reported with "vanishing lungs' associated with sarcoidosis or necrotizing sarcoid granulomatosis. This syndrome is a rare outcome of sarcoidosis, but sarcoidosis may be an underlying cause of giant bullous emphysema. If bullectomy is undertaken, tissue should be removed from relatively normal lungs and mediastinal nodes to demonstrate the presence and nature of any granulomas.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/6. proteus syndrome: emphasis on the pulmonary manifestations.Published articles on the radiologic aspects of proteus syndrome are sparse. This report highlights the features of this disease with specific attention to the serious pulmonary manifestations that may occur at an early age. Two cases of proteus syndrome and severe lung disease are presented, with complete autopsy in one case and correlative surgical pathologic data in the other. Multiple superficial and visceral vascular abnormalities were present in both cases. Both patients developed rapidly progressive diffuse cystic emphysematous pulmonary disease leading to the death of one patient at age 18 years and a heart-lung transplant in the other at age 8 1/2 years. Extensive gross pulmonary cysts were evident pathologically with diffuse panlobar emphysema microscopically. Studies of collagen and cultured fibroblasts in one patient revealed no abnormality. The early presentation, rapid progression and potentially lethal nature of lung involvement has not previously been emphasized.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/6. Postpneumonectomy pulmonary edema: can it be predicted preoperatively?Postpneumonectomy pulmonary edema (PPE) is a rarely reported form of acute lung injury which occurs in up to 4% of all pneumonectomies. The details of two well-documented cases of PPE are described with special emphasis paid to the preoperative lung functions. Both cases illustrated a striking disparity between preserved spirometric lung function and advanced emphysema as detected by quantitative CT emphysema scores and single-breath diffusion of carbon monoxide measurements. Though retrospective in nature, these results suggest a restricted capillary volume plays a critical role in the development of PPE.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/6. Combined lung volume reduction and mitral valve reconstruction.Combined lung volume reduction and mitral valve reconstruction was performed in a 66-year-old man with end-stage emphysema and severe mitral regurgitation. quality of life, pulmonary function, 6-minute walk, echocardiographic degree of mitral regurgitation, and new york Heart association heart failure classification all improved substantially. A lung volume reduction operation can safely be combined with complex cardiac operations for patients with disabling dyspnea of a multifactorial nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |