Cases reported "Sclerosis"

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1/19. Lipoprotein (a) immunapheresis in the treatment of familial lipoprotein (a) hyperlipoproteinemia in a patient with coronary heart disease.

    This paper reports 2 years' experience with lipoprotein (a) (Lp[a]) immunapheresis which was successfully handled on a now 40-year-old patient with familial Lp(a) hyperlipoproteinemia inducing severe coronary heart disease with 2 myocardial infarctions and diffuse coronary sclerosis. Continued treatment by Lp(a) immunabsorption with specific sheep antibodies reduced stenosis in coronary vessels more than 50% and stopped the progression of coronary heart disease. A special apheresis technique and the results of continued absorption effects are described.
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2/19. Histopathological findings of X-linked retinoschisis with neovascular glaucoma.

    BACKGROUND: X-linked retinoschisis (XLRS) is rarely complicated by neovascular glaucoma. Only a few reports of XLRS histopathological findings with neovascular glaucoma have been published. methods: A 41-year-old man with XLRS complicated by neovascular glaucoma in his left eye was examined with electroretinography, B-scan, ultrasound biomicroscopy and computed tomography. He was examined by ophthalmoscopy and fluorescein angiography in the other eye. An enucleation was performed in his left eye due to uncontrollable high intraocular pressure and persistent ocular pain. We examined the enucleated eye histopathologically. RESULTS: Examination of the enucleated eye showed nuclear sclerosis of the lens, pigmented retrolental membrane and retinoschisis which separated the inner layer of the retina and made a large space in the vitreous cavity without any apparent detachment of the outer layers of the retina. Sclerotic vessels were present histopathologically in both the inner and outer layers of the retina. There was a peripheral anterior synechia, ectropion uveae and a fibrovascular membrane, which contained many lumina of neovascularization, indicating marked rubeosis iridis. Small cystic spaces were observed in both the schitic retina in the peripheral region and the foveal schisis at the outer layer of the retina. The photoreceptor cells had become markedly atrophied and multiple regions of calcification were observed. The optic nerve showed severe atrophy with gliosis, but the central retinal artery and vein were still open within the nerve. CONCLUSIONS: These histopathological findings suggest that rubeosis iridis may have developed secondarily to retinal ischemia due to occlusion of the retinal blood vessels.
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ranking = 11.955168498893
keywords = blood vessel, vessel
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3/19. Sclerosing encapsulating peritonitis and non-occlusive mesenteric infarction found at autopsy in a man who had undergone continuous ambulatory peritoneal dialysis: a histochemical and immunohistochemical study.

    This is a report of a post-mortem histological, histochemical, and immunohistochemical examination of a rare case of sclerosing encapsulating peritonitis (SEP) and non-occlusive mesenteric infarction (NOMI), two serious complications of continuous ambulatory peritoneal dialysis (CAPD), with which a man suffering hepatitis c virus (HCV)-induced liver cirrhosis for 7 years and trauma-induced paraplegia for 50 years had been treated for 1 year. The direct cause of death was encephalopathy caused by extreme hyperammonemia (11 250 microg/dL in serum). The autopsy revealed that the SEP had drastically reduced the length of the small intestine to 210 cm, 180 cm of which presented acute ischemic enteritis with Gram-negative bacterial infection. Histological examination of the SEP revealed that the exterior was composed of normal serosal elastic lamina, but with a cocoon-like appearance remarkably thickened by fibrosis to 3-8 times that of the normal subserosal layer and consisting of spindle cells and blood vessels, with some infiltration of mast cells and lymphocytes. The immunohistochemical examination of the spindle cells revealed few AE1/AE3( ) cells, HHF35( ) cells, and CD34( ) cells, many CD117( ) cells with slight proliferative activity based on MIB-1 positivity (proliferation index <1%), but no CD44( ) cells. It was concluded that either the few CD34( ) and/or the many CD117( ) cells were mesenteric stem cells that had originated from the serosa, proliferated, then differentiated into myofibroblasts or fibroblasts, producing collagen and hyaluronic acid in the matrix, leading to the gradual formation of the SEP, which was induced by the continual irritation of CAPD.
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ranking = 10.955168498893
keywords = blood vessel, vessel
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4/19. Bilateral submandibular salivary gland swelling--a report of chronic sialodochitis with eosinophilia.

    A case of bilateral swelling of the submandibular salivary glands is presented. The histopathological features were diffuse periductal sclerosis with a lymphoplasmacytic infiltrate which was rich in eosinophils. Some acinar atrophy was seen. salivary ducts showed mucous and squamous prosoplasia, with focal inspissated mucin. Numerous dilated and congested blood vessels were prominent throughout the stroma. These features overlapped with those of Kimura's disease and angiolymphoid hyperplasia with eosinophils. The features of these conditions and a differential diagnosis are discussed.
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ranking = 10.955168498893
keywords = blood vessel, vessel
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5/19. Asymptomatic chronic intestinal ischemia caused by idiopathic phlebosclerosis of mesenteric vein.

    Phlebosclerosis of the mesenteric vein is a rare condition causing chronic intestinal ischemia, it has only been reported in japan. A 56-year-old man with liver cirrhosis and hepatic tumor presented with phlebosclerosis of mesenteric vein without any abdominal symptoms. He was admitted for examination of suspected hepatic tumor. Abdominal plain x-ray films and computed tomography revealed calcification of the mesenteric vein. barium enema revealed narrowing and thumbprinting from the cecum to transverse colon. On colonoscopic examination, blue-black vessels were visible in the terminal ileum, and hyperemic nodular mucosa with small irregular ulcers surrounded by dark purple mucosa was found from the cecum to transverse colon. The etiology of mesenteric vein phlebosclerosis is unknown, although a physical mechanism rather than inflammatory changes appear to be involved in this rare and usually progressive condition of chronic intestinal ischemia.
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6/19. Mesenteric phlebosclerosis: a new disease entity causing ischemic colitis.

    PURPOSE: Nonthrombotic stenosis or occlusion of the mesenteric veins is a rare cause of intestinal ischemia. The aim of this study was to describe a new disease entity causing chronic ischemic colitis. methods: Seven patients were diagnosed as having mesenteric phlebosclerosis. All seven patients had calcifications in the small mesenteric veins and their intramural branches. No evidence of vasculitis or portal hypertension was recognized. None of the patients had a history of gastrointestinal disease or of prolonged drug use. We report clinical, laboratory, radiographic, endoscopic, and histopathologic findings. RESULTS: Clinical findings included abdominal pain and diarrhea of a gradual onset and chronic course. A positive fecal occult blood test and mild anemia were often found. The patients had linear calcifications and stenosis in the right colon, which were discovered by plain abdominal radiography and barium enema, respectively. Endoscopic findings included edematous, dark colored mucosa and ulcerations. Four patients underwent a subtotal colectomy because of persistent abdominal pain or ileus. The histopathologic findings were macroscopically characterized by a dark purple or dark brown colored colonic surface, the swelling and disappearance of plicae semilunares coli, and marked thickening of the colonic wall, while they were microscopically characterized by marked fibrous thickening of the venous walls with calcifications, marked submucosal fibrosis, deposition of collagen in the mucosa, and foamy macrophages within the vessel walls. CONCLUSIONS: These peculiar lesions have not previously been fully described. The cause and pathogenesis still remain unknown. We conclude that such lesions represent a new clinicopathologic disease entity and propose the term "idiopathic mesenteric phlebosclerosis."
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7/19. nephrogenic fibrosing dermopathy with systemic involvement.

    BACKGROUND: There is a growing literature regarding sclerotic and panniculitic cutaneous conditions seen in patients with end-stage renal disease (eg, calciphylaxis and soft tissue calcification). nephrogenic fibrosing dermopathy (NFD) is a recent designation to describe cutaneous findings in patients with end-stage renal disease who developed sclerotic plaques with scleromyxedema-like histologic features. Soft tissue calcification is rare in patients with NFD and systemic involvement has not been reported. OBSERVATIONS: We describe a patient with end-stage renal disease who developed diffuse indurated woody plaques consistent with NFD in association with soft tissue calcification with catastrophic sequelae. A deep excisional biopsy specimen from the patient revealed thickened collagen bundles in the reticular dermis, plump bipolar spindle cells, and increased mucin. Focally, there were zones of calcium deposition in dermal collagen without vessel calcification. autopsy of the patient revealed extensive fibrosis and calcification of the diaphragm, psoas muscle, renal tubules, and rete testes. The patient died 11 months after developing NFD. CONCLUSION: A subset of patients with NFD may have significant systemic involvement.
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8/19. Hyalinized hemangioma of the liver.

    We report the case of a 65-yr-old woman with hyalinized hemangioma of the liver which, on radiological examination, resembled primary or metastatic carcinoma of the liver. She had undergone a partial colectomy for a sigmoid adenocarcinoma, followed by the diagnosis of a hepatic tumor with ultrasonic echogram 5 months later. The tumor was depicted as a low-density mass on plain computed tomography (CT), and an enhancement at the peripheral portion was noted by contrast CT. Hepatic angiography disclosed a faint pooling of contrast medium in segment 8. A subsementectomy of the liver was performed under the diagnosis of metastatic adenocarcinoma or hepatocellular carcinoma. Histologically, the tumor was composed of dense collagenous tissues with marked hyalinization and scattered sclerotic vessels. Elastic fibers were distributed concentrically around the vessels. Totally hyalinized sclerosis of hemangioma is uncommon, and can be erroneously diagnosed as carcinoma by radiologic examination. This unusual hemangioma is reported, with pertinent literature.
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9/19. Phlebosclerotic colitis coincident with carcinoma in adenoma.

    Phlebosclerosis of the colon is a rare disease characterized by a thickening of the wall of the colon with fibrosis, hyalinization and calcification to the affected veins. These symptoms result in a type of ischemic colitis known as phlebosclerotic colitis. A case of phlebosclerotic colitis coincident with carcinoma in adenoma is reported. A 74-year-old Japanese woman was admitted to hospital because of a mass in her right lower abdomen. Abdominal computed tomography examination revealed linear calcifications in the wall of the cecum and the ascending colon. colonoscopy revealed dark purple mucosa with multiple ulcers in the cecum and the ascending colon. biopsy specimens showed a marked hyalinous thickening of the wall of small blood vessels in the mucosa. Phlebosclerotic colitis was suspected because of negative results with amyloid stain. Alternative ileocolic angiography showed the serpentine of the peripheral nature blood vessels and pooling at the late venous phase. Microscopic examination of the surgically resected colon revealed mucosal and submucosal fibrosis, and a thickening of the venous wall with fibrosis, hyalinization and calcification from the mucosa to the serosa, which caused a marked luminal narrowing. A small polypoid lesion was also found in the affected region and was diagnosed histologically as carcinoma in adenoma. To our knowledge, this is the first reported case of phlebosclerotic colitis complicated by carcinoma.
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ranking = 21.910336997786
keywords = blood vessel, vessel
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10/19. nephrogenic fibrosing dermopathy in pediatric patients.

    nephrogenic fibrosing dermopathy (NFD) is a rare and recently recognized sclerosing skin disorder of unknown etiology. Reported cases have occurred in patients with chronic renal failure, with or without renal replacement therapy. All previous cases have been reported in older adult patients. We describe two pediatric patients who recently developed this condition and review the existing literature for NFD. Our patients included an 8-year-old boy on peritoneal dialysis with no prior renal transplant and a 19-year-old boy on hemodialysis with a history of previous failed renal transplants. We speculate that the recent emergence of this condition and occurrence in patients with chronic renal failure suggest an association with some newer pharmacological agent that has recently come into wide use. Since both our patients also had previously experienced large vessel thrombosis, hypercoagulable states may also be implicated.
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