1/6. Pulmonary hypertension associated with pulmonary occlusive vasculopathy after allogeneic bone marrow transplantation.BACKGROUND: Pulmonary vasculature abnormalities, including pulmonary veno-occlusive disease, have been demonstrated in marrow allograft recipients. However, it is often difficult to make a correct diagnosis of pulmonary lesions. methods: An open lung biopsy was performed on a patient who developed severe pulmonary hypertension after bone marrow transplantation for T-cell lymphoma. RESULTS: An open lung biopsy specimen demonstrated pulmonary arterial occlusion due to intimal fibrosis and veno-occlusion. The most striking alteration was partial to complete occlusion of the small arteries by fibrous proliferation of the intima. CONCLUSION: High-dose preparative chemotherapy and radiation before transplantation are thought to have contributed to the development of vasculopathy in this patient, because arterial occlusion by intimal fibrosis and atypical veno-occlusion are often associated with lung injury due to chemoradiation. An open lung biopsy is essential for diagnosing pulmonary vascular disease presenting signs compatible with posttransplantation pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/6. breast conservation therapy. Severe breast fibrosis after radiation therapy in patients with collagen vascular disease.Two patients with collagen vascular disease (rheumatoid arthritis and scleroderma) had extremely poor cosmetic results after breast radiation therapy (RT). The patient with rheumatoid arthritis received 5251 cGy at 210 cGy per day, followed by a 1600 cGy iridium-192 implant boost. Between 8 and 11 months post-RT she had severe breast fibrosis, retraction, and pain that required a mastectomy for relief. The patient with scleroderma received 5040 cGy at 180 cGy per day without a boost. Between 1 and 4 months post-RT the systemic symptoms of scleroderma progressed and the breast became hard and retracted. Both rheumatoid arthritis and scleroderma are chronic systemic diseases characterized by severe inflammation and an autoimmune component. The presence of scleroderma at or before treatment should be considered a contraindication to breast RT, whereas the presence of active rheumatoid arthritis should be considered a relative contraindication. An autoimmune mechanism will be presented to explain both the fibrosis and the systemic progression of collagen vascular disease that was observed.- - - - - - - - - - ranking = 6keywords = vascular disease (Clic here for more details about this article) |
3/6. Fibrocalculous pancreatic diabetes in the elderly.Fibrocalculous pancreatic diabetes (FCPD) is a form of diabetes seen in tropical countries. It is secondary to chronic, calcific, non-alcoholic pancreatitis. FCPD is usually a disease of youth. This paper reports on two elderly onset cases of FCPD. Macrovascular complications are usually rare in FCPD patients. These two patients had evidence of macrovascular diseases probably due to the older age group of the patients.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
4/6. Extensive corporeal fibrosis after penile irradiation.A potent man with early signs and symptoms of Peyronie's disease 3 months in duration received 1,200 rad of external beam radiation to the penis and presented 5 months later with impotence. physical examination revealed diffusely woody indurated corporeal tissue. Nocturnal penile tumescence testing was abnormal and pharmaco-cavernosometry demonstrated diffuse corporeal veno-occlusive dysfunction. Treatment by penile injections was unsuccessful. During penile prosthesis implantation bilateral rubbery erectile tissue was encountered, requiring extensive bilateral corporotomy and sharp corporeal tissue excision for prosthesis insertion. Histological analysis of excised corporeal tissue demonstrated extensive corporeal fibrosis and arterial vasculopathy. Computer assisted color histomorphometry revealed that the mean percentage of trabecular smooth muscle area to total erectile tissue area was 26.5 /- 15.8 (normal 40 to 52%). Immunohistochemical staining with desmin confirmed extensive fibrosis. The most likely explanation for severe corporeal fibrosis is penile irradiation. The hypothesized mechanism of radiation associated fibrosis is ionizing injury to the endothelial cells of the lacunar spaces and cavernous/helicine arteries, which induced irreversible corporeal extracellular matrix structural changes. Penile irradiation, like vascular disease and priapism, is a potential cause of diffuse corporeal fibrosis.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/6. mixed connective tissue disease and radiation toxicity. A case report.BACKGROUND: Several cases of long term radiation sequelae have been reported in patients with lupus erythematosus and systemic sclerosis after breast or chest wall irradiation. To the authors' knowledge, no experience with such complications in patients with mixed connective tissue disease (MCTD) has been reported previously. methods: A case of a woman with occult breast carcinoma metastatic to the axilla and preexisting MCTD is presented. To the authors' knowledge, this is the first case report of the adverse effects of breast irradiation in a patient with MCTD. The pathophysiology of such radiation injury to specific anatomic structures and technical dosimetric considerations of the radiation therapy and radiation dose are analyzed. The relevant literature on other collagen vascular diseases with features related to MCTD is reviewed. RESULTS: A moderate dose of radiation to the breast and regional lymphatics resulted in marked early and late toxicity to skin and subcutaneous tissues. The tissue injury was similar to that observed in patients with lupus erythematosus and systemic sclerosis. The early skin reaction (moist desquamation) was related to the daily radiation dose delivered at the depth of the epidermis, and the late reaction (subcutaneous fibrosis) was related to the dose at the depth of the dermal capillaries and dermal connective tissue. CONCLUSIONS: patients with MCTD may develop exaggerated radiation reactions similar to those in patients with lupus erythematosus and systemic sclerosis. Although the incidence of such radiation reactions in patients with MCTD is difficult to assess, the risks and benefits of radiation therapy should be carefully weighed in these patients, particularly if an alternative therapy is available. If there is no alternative, judicious attention to radiotherapy technique may reduce or prevent skin toxicity.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
6/6. Surgical treatment for Scheie's syndrome (mucopolysaccharidosis type I-S): report of two cases.Scheie's syndrome (mucopolysaccharidosis type I-S) is a rare genetic lysosomal storage disease affecting mucopolysaccharide metabolism, and is known to include cardiovascular disease. Surgical treatment was carried out in 2 patients with Scheie's syndrome. Patient 1 was a 56-year-old man with triple-vessel coronary artery disease, who successfully underwent coronary artery bypass grafting. Patient 2 was a 52-year-old man with aortic and mitral valve stenosis, who successfully underwent combined aortic and mitral valve replacement. The literature on Scheie's syndrome associated with valvular and coronary artery disease is also reviewed.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |