1/23. 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 = intima (Clic here for more details about this article) |
2/23. Uncommon arteriopathies: what the vascular surgeon needs to know.Uncommon arteriopathies encompass a diverse range of diseases, including inherited collagen vascular disorders such as marfan syndrome, Ehlers-Danlos Type IV, and pseudoxanthoma elasticum; vasculitides, including Takayasu's arteritis, extracranial giant cell arteritis, and Behcet's disease; neurofibromatosis type 1; intimal fibromuscular dysplasia; unusual bacterial and viral infections; and drug-induced arteriopathies. patients with uncommon arterial disorders may present to the vascular surgeon with common surgical problems, including intermittent claudication, renovascular hypertension, Raynaud's phenomenon, and aneurysmal disease. However, the disease manifestations, expected course, and outcomes may be much different than more common arterial disorders and this can have important surgical implications. This review centers around several interesting cases and the differential diagnoses that should be considered when encountering an unusual clinical presentation. Reference is made to the literature for diagnostic criteria, clinical pearls, and how to avoid pitfalls in the evaluation and management of patients with unusual arteriopathies.- - - - - - - - - - ranking = 0.33333333333333keywords = intima (Clic here for more details about this article) |
3/23. Generalized arterial calcification of infancy: different clinical courses in two affected siblings.Generalized arterial calcification of infancy (GACI) is a rare autosomal recessive disease caused by mutations in ENPP1. Due to extensive calcification of the arterial media associated with intimal proliferation leading to vascular occlusion, most affected children die within the first 6 months of life. We report on two Taiwanese siblings with an identical genotype, but different clinical course. The male sibling developed heart failure and severe hypertension, and died at the age of 6 weeks despite of treatment with bisphosphonates, ACE inhibitors, and hydralazine. The subsequent female, who was monitored closely pre- and post-natally, is having an uncomplicated clinical course up to the age of 1(1/2) year now. There were similar characteristic sonographic and roentgenographic findings in both siblings in early infancy. In both siblings, the same compound heterozygous mutations (c.1025G > T [p.Gly342Val] and c.1112A > T [Tyr371Phe]) in ENPP1 were identified. Despite the same genotype and similar sonographic and radiographic features in early infancy, the phenotype of GACI can vary to a great extent within one family.- - - - - - - - - - ranking = 0.33333333333333keywords = intima (Clic here for more details about this article) |
4/23. Development of vascular neoplasia in Castleman's disease. Report of seven cases.Seven cases of vascular neoplasia arising within lesions of hypervascular follicular hyperplasia (HFH) fulfilling the criteria of Castleman's disease are described. The patients did not have evidence of acquired immunodeficiency syndrome or other immunologic disorders. The masses were solitary and located in the retroperitoneum (five cases), mediastinum (one case), and axilla (one case). Grossly, they measured up to 20 cm and had a variegated appearance. In each case two morphologically distinct processes were present: a mesenchymal spindle-cell neoplasm with evidence of vascular differentiation and Castleman's disease of hyaline vascular type. The two processes blended with each other, with the neoplasm appearing to be continuous with the interfollicular proliferation of small vessels that is typical of Castleman's disease. The lesions behaved aggressively in two cases, both patients having died with metastatic disease. This remarkable association may be viewed as a pathologic manifestation of the intimate functional relationship that exists between the immune and the vascular systems. Other probable examples of this relationship are systemic Castleman's disease associated with Kaposi's sarcoma, localized Castleman's disease associated with vascular hamartoma, histiocytoid hemangioma/angiolymphoid hyperplasia with eosinophilia, and (possibly) angiomatoid malignant fibrous histiocytoma. Perhaps these associations are mediated by the production of angiogenic factors by the activated lymphoid cells.- - - - - - - - - - ranking = 0.33333333333333keywords = intima (Clic here for more details about this article) |
5/23. Pathological considerations of reoperative vascular patients.Since vascular disease is always progressive and a perfect vascular prosthesis has yet to be developed, postoperative complications are almost inevitable. In this paper, case histories of those who had to have a second operation or, in other words, re-operated patients, have been examined to ascertain the current problems in vascular surgery. Of 176 vascular reconstructive operations performed between January 1, 1980, and December 31, 1986, 29 re-operations were performed on 19 patients (mean age: 64 years; 15: male). The incidence of late graft failures was 8.4% and, of these, anastomotic aneurysms seemed to be the most serious complication (3.1% incidence rate). Late graft failures included intimal hyperplasia, occurring within two years in five cases, and four cases of progressing atherosclerosis, which appeared three years after the initial operation. In all cases of anastomotic aneurysm, arterial wall failure, possibly combined with the changing of implanted grafts, was considered to be related to the false aneurysmal formation. Knitted Dacron demonstrated susceptibility to atherosclerotic progression, whereas the major fault of polytetrafluoroethylene (PTFE) grafts was the insufficiency of the anastomotic diameter. In conclusion, it was revealed that many factors can provoke late graft failure. Improvement of long-term patency seems to be achieved by a more increased understanding of the pathological meaning of these factors, along with the proper application of medical techniques suited to the causes of vascular obstruction.- - - - - - - - - - ranking = 0.33333333333333keywords = intima (Clic here for more details about this article) |
6/23. Lupus glomerulonephritis with thrombotic microangiopathy.Well-documented cases of systemic lupus erythematosus (SLE) with thrombotic microangiopathy (TMA) are rare. Renal biopsy in a 25-year-old woman with SLE who was in renal failure demonstrated proliferative lupus glomerulonephritis with arteriolar thrombosis and the arterial intimal changes of TMA. No staining of vessels for immunoglobulins or complement was found by direct immunofluorescence. Fibrillar and flocculent deposits were seen in the widened and rarefied subendothelial space in a small artery and two glomeruli, one of which also contained electron-dense deposits. The vascular findings, which are those of TMA, are distinct from the immune complex vasculopathy of SLE.- - - - - - - - - - ranking = 0.33333333333333keywords = intima (Clic here for more details about this article) |
7/23. The pathology and pathogenesis of malignant atrophic papulosis (Degos' disease). A case study with reference to other vascular disorders.The morphology and immunohistology in a case of malignant atrophic papulosis (Degos' disease), a rare vascular disorder of unknown etiology, are described. The vascular lesions affected middle class and small arteries and veins throughout the body and were histologically characterized by intimal proliferation in the absence of any appreciable inflammation. The lesions were categorized as early, intermediate or late. Early lesions consisted of cellular proliferation and edema of the intima with signs of immune complex deposition (IgM, C3). thrombosis was occasionally present as a secondary phenomenon in the affected vessel segments. In intermediate lesions the edema decreased and smooth muscle proliferation became apparent. Late lesions consisted of acellular intimal sclerosis with hyalinization and narrowing or obliteration of the vascular lumen. The media of the vessels remained always intact. In comparing these features to the pathology and pathogenesis of other vascular disorders they resembled the vascular lesions in a murine model of lupus erythematodes in which also considerable intimal proliferation occurred with thrombotic occlusion, but without appreciable inflammation. The murine model is associated with sustained low levels of circulating immune complexes and it is tempting to assume the same for Degos' disease. The notion of an immune complex mediated non-inflammatory condition underlying this severe and often fatal vascular disorder of mainly young males may contribute to the eventual finding of a successful therapeutical regimen.- - - - - - - - - - ranking = 1.3333333333333keywords = intima (Clic here for more details about this article) |
8/23. Musculoelastosis: a change of small pulmonary arteries found in a case with atrial septal defect and pulmonary hypertension.Interesting findings were obtained in the lung biopsy of a 46 year old female patient with secundum atrial septal defect and pulmonary hypertension. In the intima of small pulmonary arteries, there were a marked increase in elastic fibers and marked proliferation of longitudinal smooth muscle cells. The vascular lumen was markedly stenotic, but reconstruction of small pulmonary arteries indicated that all vascular lumina were patent. The case was accordingly diagnosed as operable and closure of atrial septal defect was undertaken. Three years postoperatively, the patient is well with much improved condition and lowered pulmonary arterial pressure was demonstrated by cardiac catheterization. This case had apparently serious vascular lesions due to severe intimal thickening which we labeled "musculoelastosis", but it was found that such thickening alone did not cause peripheral pulmonary arterial occlusion. It is therefore thought to be a benign pulmonary vascular condition.- - - - - - - - - - ranking = 0.66666666666667keywords = intima (Clic here for more details about this article) |
9/23. Idiopathic arterial calcification of infancy.Idiopathic arterial calcification of infancy is an uncommon disease of unknown etiology, characterized by calcification along the internal elastic membrane and intimal proliferation of almost all the arteries of the body. When the diagnosis is made premortem, it is usually done by radiographic demonstration of arterial calcification. Because of its edge enhancement effect, xeroradiography should be useful in detecting the soft tissue calcifications of this disease.- - - - - - - - - - ranking = 0.33333333333333keywords = intima (Clic here for more details about this article) |
10/23. Percutaneous transluminal angioplasty (PTA) with the Gruntzig balloon catheter: technical problems encountered in the first forty patients.Forty patients have undergone percutaneous transluminal angioplasty (PTA) of the iliac or femoral-popliteal system with the Gruntzig balloon catheter. Technical problems ahve included experience with difficult antegrade puncture, production of intimal flaps, and loss of collateral vessels, as well as the need to develop an approach for recanalization of obstructions and for use of the balloon. Since problems in these areas may jeopardize the success of the procedure, they have been elucidated with some discussion of prevention. Preliminary results on the success of the procedure are included.- - - - - - - - - - ranking = 0.33333333333333keywords = intima (Clic here for more details about this article) |
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