1/369. Idiopathic chronic intestinal pseudo-obstruction. Use of central venous nutrition.patients with idiopathic chronic intestinal pseudo-obstruction suffer from malnutrition because of inability to maintain adequate oral intake without the development of obstructive symptoms. We have successfully used central venous nutrition in two patients with this syndrome, both on a short-term and long-term home-maintenance basis. Hyperalimentation can provide adequate nutrition in patients with intestinal pseudo-obstruction until normal bowel function returns or until definitive therapy for this chronic disease is found.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
2/369. Chronic urticaria as a presenting sign of hairy cell leukemia.Chronic urticaria is a common clinical disorder that is idiopathic in over 75% of cases. Less commonly, urticaria may be the presenting manifestation of an allergic or infectious disease, endocrinopathy, inherited syndrome, or autoimmune disorder. Rarely, urticaria may be a sign of underlying malignancy, including leukemia. C.C. is a 48-year-old white female who was referred for evaluation of recurrent urticaria for 3 years. The pruritic, erythematous wheals were pinpoint, and appeared to be precipitated by heat, stress, and effort. Prick tests were negative except to D. pteronyssinus. CBCs over the past 5 years revealed WBCs of 2,300-5,000 cells/mm3. skin biopsy revealed interstitial edema with infiltration of eosinophils and mast cells consistent with urticaria. The impression was probable cholinergic urticaria, for which hydroxyzine was prescribed with fair symptomatic control. One year later, she presented with bright red blood per rectum. Repeat physical examination revealed lymphadenopathy and splenomegaly. Subsequent laboratory studies showed pancytopenia. endoscopy was normal except for small, nonbleeding hemorrhoids. bone marrow biopsy revealed histologic evidence of hair, cell leukemia that was treated with 2-chlorodeoxyadenosine. Upon initiation of chemotherapy her pruritus and urticaria subsided. Recent CBC revealed Hgb 9.2 g/dL, platelets 290,000 cells/mm3, and WBC 4,100 cells/mm3. Peripheral blood smear showed no hairy cells.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
3/369. myasthenia gravis and polymyositis as manifestations of chronic graft-versus-host-disease.myasthenia gravis and polymyositis are each a rare manifestation of immune dysregulation in chronic graft-versus-host disease (cGVHD). We report a 4-year-old boy with idiopathic acquired aplastic anemia who developed myasthenia gravis 22 months and polymyositis 69 months after an allogeneic BMT (5/6 matched, MLC-nonreactive). The occurrence of both syndromes in one patient is unique. Autoimmune dysfunction may be associated with the development of cGVHD as demonstrated by the high incidence of prior aplastic anemia in BMT patients presenting with myasthenia gravis and polymyositis. Recognition of these neurologic manifestations is important in the diagnosis and treatment of cGVHD.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
4/369. Misdiagnosis of specific cytomegalovirus infection of the ileoanal pouch as refractory idiopathic chronic pouchitis: report of two cases.PURPOSE: Chronic nonspecific reservoir ileitis (pouchitis) occurs in 5 to 10 percent of patients who undergo ileal pouch-anal anastomosis for ulcerative colitis. Specific infection of the ileal pouch-anal anastomosis with cytomegalovirus has not been reported. AIM: We report two patients with specific cytomegalovirus infection of the ileal pouch-anal anastomosis, initially misdiagnosed as idiopathic chronic pouchitis. CASE SERIES: Patient 1 had ileal pouch-anal anastomosis for ulcerative colitis. Three years later she had diarrhea, fever, pelvic pain, and pouch inflammation at endoscopy consistent with pouchitis. She had no response to medical therapy. Repeat endoscopy showed persistent inflammation and biopsies showed cytomegalovirus. She had symptomatic improvement after treatment with intravenous ganciclovir, 10 mg/kg/day for ten days (stopped for rash). Repeat pouch biopsies were negative for cytomegalovirus. Patient 2 had ileal pouch-anal anastomosis for ulcerative colitis. Nine years later she had resection of obstructing stricture at previous loop ileostomy site. She underwent reoperation with ileostomy and pouch defunctionalization for peritonitis. Four weeks later she had fever and bloody discharge from the diverted pouch. Pouch endoscopy with biopsy showed inflammation consistent with pouchitis. She had no response to medical therapy. Re-examination of pouch biopsies with a specific monoclonal immunofluorescent stain showed cytomegalovirus. She had symptomatic improvement after treatment with intravenous ganciclovir, 10 mg/kg/day for 21 days. Repeat pouch biopsies were negative for cytomegalovirus. CONCLUSIONS: Specific cytomegalovirus infection of the ileal pouch-anal anastomosis may be misdiagnosed as idiopathic refractory chronic pouchitis. cytomegalovirus must be excluded before immune modifier therapy or pouch excision in these patients.- - - - - - - - - - ranking = 6keywords = idiopathic (Clic here for more details about this article) |
5/369. hydroa vacciniforme persistent in a 60-year-old man.hydroa vacciniforme (HV) is an idiopathic photodermatosis with onset in childhood, a chronic-recurrent course, and spontaneous resolution in adolescence or early adulthood. We present a patient with typical HV in whom lesions began in childhood and continued until 60 years of age.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
6/369. Eosinophilic enterocolitis and visceral neuropathy with chronic intestinal pseudo-obstruction.A patient is described who was found to have both eosinophilic enterocolitis and visceral neuropathy with chronic idiopathic intestinal pseudo-obstruction. The etiology and pathogenesis of this rare combined disorder of the gastrointestinal tract are discussed. Although eosinophilic enterocolitis is amenable to conservative treatment, surgery may be necessary for palliation in selected patients with pseudo-obstruction due to visceral neuropathy.- - - - - - - - - - ranking = 2320.8507270481keywords = chronic idiopathic, idiopathic (Clic here for more details about this article) |
7/369. Complete agenesis of the dorsal pancreas.Pancreatic anomalies are occasionally reported, but complete agenesis of the dorsal pancreas is extremely rare. We report a 47-year-old woman with complete agenesis of the dorsal pancreas. This patient initially presented with jaundice. Computed tomography did not reveal the pancreatic corpus or tail. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography did not visualize the dorsal pancreatic duct. Choledochojejunostomy was performed because she had obstructive jaundice. At laparotomy, there was an enlarged pancreatic head, but no distal pancreas was seen. Histological examination of the pancreatic biopsy specimen showed scattered islets of langerhans in diffuse fibrosis, with destruction of the glandular parenchyma. This case was diagnosed as complete agenesis of the dorsal pancreas.- - - - - - - - - - ranking = 0.62161475840778keywords = jaundice (Clic here for more details about this article) |
8/369. Fatal strongyloidosis following corticosteroid therapy in a patient with chronic idiopathic thrombocytopenia.A patient with chronic idiopathic thrombocytopenia and fatal strongyloides hyperinfection syndrome following prolonged corticosteroid therapy is briefly described. Diagnosis was difficult to perform due to absence of eosinophilia and diarrhea at presentation, as well as to the negativity of multiple stool specimens examined by direct microscopy of saline smear, formol-ether concentration techniques, and Baermann's test. The striking hypoalbuminemia in the setting of the normal results of liver function tests and prothrombin time was assumed to be due to enteropathy. Therefore, an upper endoscopy was undertaken, revealing strongyloides stercoralis (SS) larvae in the biopsy specimens of the gastric and duodenal mucosa. The SS larvae were also demonstrated in the multiple specimens of the concentrated sputum. Despite thiabendazol treatment, death ensued. On autopsy, SS larvae were recovered in the gastrointestinal tract and lungs. The importance of early diagnosis and of ruling out strongyloidosis prior to administration of corticosteroids are discussed, as well as the pathogenetic aspects of strongyloidosis in the patient under corticosteroids.- - - - - - - - - - ranking = 2324.8507270481keywords = chronic idiopathic, idiopathic (Clic here for more details about this article) |
9/369. Chronic pancreatitis and inflammatory bowel disease: true or coincidental association?OBJECTIVE: Several cases of pancreatitis have been described during the course of Crohn's disease (CD) or ulcerative colitis (UC), but many of them were related to either biliary lithiasis or drug intake. We tried to evaluate the clinical and morphological features of so-called idiopathic pancreatitis associated with inflammatory bowel disease and to define their pathological characteristics. methods: Chronic idiopathic pancreatitis was diagnosed on the basis of abnormal pancreatograms suggestive of chronic pancreatitis associated with or without impaired exocrine pancreatic function, or pathological examination in patients undergoing pancreatic resection. We found 6 patients presenting with features of chronic idiopathic pancreatitis and UC and 2 patients with CD seen between 1981 and 1996 in three hospital centers of the south of france. A review of the literature has identified 6 cases of pancreatitis associated with UC and 14 cases of pancreatitis associated with CD based on the above criteria. RESULTS: hyperamylasemia was not a sensitive test since it was present in 44% and 64% of patients with UC or CD. In UC, pancreatitis was a prior manifestation in 58% of patients. In contrast, the pancreatitis appeared after the onset of CD in 56% of the cases. In patients with UC, pancreatitis were associated with severe disease revealed by pancolitis (42%) and subsequent surgery. Bile duct involvement was more frequent in patients with UC than with CD (58% vs 12%) mostly in the absence of sclerosing cholangitis (16% vs 6%). weight loss and pancreatic duct stenosis were also more frequent in UC than in CD (41% vs 12% and 50% vs 23%, respectively). Pathological specimens were analyzed in 5 patients and demonstrated the presence of inter- and intralobular fibrosis with marked acinar regression in 3 and the presence of granulomas in 2 patients, both with CD. CONCLUSIONS: pancreatitis is a rare extraintestinal manifestation of inflammatory bowel disease. Chronic pancreatitis associated with UC differs from that observed in CD by the presence of more frequent bile duct involvement, weight loss, and pancreatic duct stenosis, possibly giving a pseudotumor pattern.- - - - - - - - - - ranking = 2322.8507270481keywords = chronic idiopathic, idiopathic (Clic here for more details about this article) |
10/369. Chronic subdural haematomas and Parkinsonian syndromes.We describe three men with parkinsonian syndromes caused or aggravated by chronic subdural haematomas. A 63-year-old man developed tremor at rest, rigidity and bradykinesia one week after he fell and hit his head. A 70-year-old patient suffering from tardive dyskinesia and drug-induced parkinsonism experienced deterioration of his bradykinetic symptoms over two weeks. There was no history of trauma. The third patient, a 82-year-old man with idiopathic Parkinson's disease had a marked increase of his left-sided parkinsonian symptoms. Again, there was no history of trauma. In all three patients chronic subdural haematomas were demonstrated by computed tomography. Evacuation of the chronic subdural haematoma resulted in disappearance respectively improvement of the movement disorder. Diagnostic evaluations appear to be delayed and initial misinterpretations are frequent. The findings of our report and review of the literature point out that a favourable outcome after appropriate surgical treatment is achieved in most instances.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
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