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retroperitoneal fibrosis

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retroperitoneal fibrosis,
a chronic inflammatory process, usually of unknown cause, in which fibrous tissue surrounds the large blood vessels in the lower lumbar area. It frequently causes constriction of the midportion of the ureters, which may lead to hydronephrosis and azotemia. Occasionally the fibrosis spreads upward to involve the duodenum, bile ducts, and superior vena cava. Symptoms include low-back and abdominal pain; weakness; weight loss; fever; and, with urinary tract involvement, frequency of urination, hematuria, polyuria, or anuria. Methysergide, taken to prevent migraine headaches, is one known cause of this condition. Treatment includes stopping methysergide and instituting surgical release of the ureters from the fibrosis with transplantation laterally or intraperitoneally.

fibrosis [fi-bro´sis]
formation of fibrous tissue; see also fibroid degeneration. adj., adj fibrot´ic.
congenital hepatic fibrosis a developmental disorder of the liver, marked by formation of irregular broad bands of fibrous tissue containing multiple cysts formed by disordered terminal bile ducts, resulting in vascular constriction and portal hypertension.
cystic fibrosis (cystic fibrosis of pancreas) see cystic fibrosis.
diffuse idiopathic interstitial fibrosis (diffuse interstitial pulmonary fibrosis) idiopathic pulmonary fibrosis.
endomyocardial fibrosis an idiopathic type of myocardiopathy that is endemic in various parts of Africa and rarely in other areas, characterized by cardiomegaly, marked thickening of the endocardium with dense white fibrous tissue that may extend to involve the inner myocardium, and by congestive heart failure.
idiopathic pulmonary fibrosis chronic inflammatory progressive fibrosis of the pulmonary alveolar walls, with steadily progressive dyspnea, resulting in death from oxygen lack or right heart failure. Most cases are of unknown origin, although some are thought to result from pneumoconiosis, hypersensitivity pneumonitis, scleroderma, and other diseases.
mediastinal fibrosis development of hard white fibrous tissue in the upper portion of the mediastinum, sometimes obstructing the air passages and large blood vessels; called also fibrosing or fibrous mediastinitis.
periureteral fibrosis retroperitoneal fibrosis.
pleural fibrosis fibrosis of the visceral pleura so that part or all of a lung becomes covered with a plaque or a thick layer of nonexpansible fibrous tissue. The more extensive form is called fibrothorax.
postfibrinous fibrosis that occurring in tissues in which fibrin has been deposited.
proliferative fibrosis that in which the fibrous elements continue to proliferate after the original causative factor has ceased to operate.
pulmonary fibrosis idiopathic pulmonary fibrosis.
retroperitoneal fibrosis deposition of fibrous tissue in the retroperitoneal space, producing vague abdominal discomfort, and often causing blockage of the ureters, with resultant hydronephrosis and impaired renal function, which may result in renal failure. Called also Ormond disease.
fibrosis u´teri a morbid condition characterized by overgrowth of the smooth muscle and increase in the collagenous fibrous tissue of the uterus, producing a thickened, coarse, tough myometrium.

retroperitoneal fibrosis
Ormond's disease Urology An idiopathic affliction of young adults–ages 30-45, ♂:♀ ratio, 2:1, characterized by retroperitoneal proliferation of fibrous tissue that encases the ureters, causing obstructive uropathy and possibly renal failure, which may evoke fibrosis elsewhere–eg, sclerosing cholangitis and mediastinitis, Riedel's thyroiditis, pseudotumor of orbit and generalized vasculitis Clinical Malaise, vomiting, backache, constipation, HTN Radiology Compression of intraabdominal structures Lab Oliguria, azotemia, proteinuria, ↑ ESR, anemia


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This condition has also been associated with such disorders as Riedel thyroiditis, orbital pseudotumor, sclerosing cholangitis, and retroperitoneal fibrosis.
Pathology results from the biopsy specimens revealed chronic inflammatory cell infiltrates admixed with plasma cells, histiocytes and eosinophils, confirming the diagnosis of retroperitoneal fibrosis (RPF) (Fig.
In patients with Cowden's syndrome, retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Riedel's thyroiditis, and inflammatory pseudotumor of the orbit can all manifest, either alone or in combination.
 
 
 
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