1/30. Ultrasound guided percutaneous fine-needle biopsy in a case of eosinophilic gastroenteritis.Eosinophilic gastroenteritis is a rare disease; clinical features depend on which intestinal layer is involved. In our report a 70-year-old woman presented with intestinal subocclusion and ascites. Endoscopic biopsies of gastric mucosa were negative. Ultrasound guided percutaneous fine-needle biopsy showed muscle infiltration by eosinophils of muscle layer of the stomach and jejunum. Muscular and serosal disease are usually diagnosed only by laparotomy or laparoscopy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/30. Primary sclerosing cholangitis in a child.Primary sclerosing cholangitis (PSC) is a rare disease in taiwan and has not been described in Taiwanese children previously. We report a 4-year-old girl who presented with prolonged fever, eosinophilia (11%), hepatomegaly, and markedly elevated serum levels of alkaline phosphatase (3,318 IU/L) and gamma-glutamyl transpeptidase (475 IU/L). Subsequent investigations including endoscopic retrograde cholangiopancreatography and liver histology confirmed the diagnosis fo PSC. Treatment with a low dose of prednisolone for 2 months and ursodeoxycholic acid during 32 months of follow-up resulted in clinical remission and halted disease progression. A high index of suspicion is necessary for physicians to diagnose this disorder in children with chronic liver disease. Our experience in this case indicates that therapy with prednisolone and ursodeoxycholic acid may be helpful for the treatment of PSC in children, and suggests the need for more trials of combined therapy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/30. Mast cell sarcoma with tissue eosinophilia arising in the ascending colon.Mast cell sarcoma is a rare disease. We report an unusual case of this neoplasm arising in the ascending colon of a 32-year-old Japanese woman who presented with abdominal pain. An ulcerating mass in the colon was resected, along with enlarged mesenteric lymph nodes. Two years after surgery, the neoplasm recurred as left cervical lymphadenopathy and an intra-abdominal mass. Despite predonine and radiation therapy, the disease progressed, and the patient died. The tumor cells had abundant fine granular or clear cytoplasm, and oval, lobulated, or indented nuclei. Numerous mature eosinophils were intermingled with the tumor cells. Immunohistologic studies on paraffin sections demonstrated that the majority of the tumor cells were strongly positive for CD45RB, CD68, and mast cell tryptase. They were unreactive, however, with a broad spectrum of antibodies against myelomonocytic and lymphocytic antigens. The mast cell nature of this rare type of tumor can be best identifiable by immunostains for mast cell tryptase.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/30. Eosinophilic cystitis--diagnosis and treatment in denmark.OBJECTIVE: The purpose of this study was to evaluate the incidence, diagnostic criteria and treatment of eosinophilic cystitis (EC) in denmark during the period 1989-94. MATERIAL AND methods: A questionnaire was sent to all Danish departments of surgery, urology and pathology. RESULTS: Fifty-six cases of EC were diagnosed in the departments of surgery and urology, and 135 patients were diagnosed in the departments of pathology. The therapy was most often steroid instillation in the bladder or peroral antihistamines. Success rates of treatment were reported to be acceptable. CONCLUSION: The questionnaire confirmed that EC is a rare disease, which is often confused with other bladder diseases. We recommend strict histological criteria combined with clinical information when making the diagnosis of EC. Therapy is generally symptomatic. A diagnostic code number from the Danish health Board is needed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/30. Eosinophilic fasciitis--progression to linear scleroderma: a case report.Eosinophilic fasciitis is a rare disease in children. Although changes similar to linear scleroderma have been reported, the outcome is usually good. In this report, a 10-year-old boy who developed eosinophilic fasciitis without a good response to steroids is presented. He progressed to linear scleroderma within months. Our case reinforces the hypothesis that eosinophilic fasciitis may be an early manifestation or a variant of localized scleroderma similar to the other cases in the literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/30. Synchronous first manifestation of an idiopathic eosinophilic gastroenteritis and bronchial asthma.Eosinophilic gastroenteritis is a rare disease of the gastrointestinal tract in which the eosinophils seem to play an important role in the inflammation of the gut wall. We report on a case with a synchronous first manifestation of eosinophilic gastroenteritis and bronchial asthma, which also occurred synchronously in all further episodes. The diagnosis was first made at the end of the second episode during which the patient lost more than 13 kg in weight. Under steroid therapy, symptoms of both diseases disappeared quickly in the third episode. We assume that participation of the gastrointestinal tract in patients with bronchial asthma occurs more frequently than expected. In asthma patients with abdominal symptomatology, eosinophilic gastroenteritis should also be considered.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/30. Successful medical treatment of two patients with eosinophilic oesophagitis.BACKGROUND/PURPOSE: Significant oesophageal eosinophilia is associated with oesophagitis and gastroesophageal reflux (GER). Eosinophilic oesophagitis is a rare disease that causes thickening of the oesophageal wall, narrowing of the oesophageal lumen, and severe motor disturbance. methods AND RESULTS: Two 12 yr-old patients with eosinophilic oesophagitis were studied prospectively. Clinical and investigation details are presented. Elemental formula was administered until complete remission of disease. Final outcome was assessed after 3 months on regular diet with exclusion of specific allergic components. Both patients responded to the dietary manipulation. CONCLUSIONS: Eosinophilic oesophagitis must be included in the differential diagnosis when dealing with oesophageal strictures of unknown or unclear aetiology. Elimination diet therapy plays a crucial role in ameliorating the course of the illness. blood eosinophilia correlates with therapeutic response and with improvement of the disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/30. Clinical, haemodynamic, and angiographic findings in Loffler's eosinophilic endocarditis.Detailed haemodynamic and angiographic findings in Loffler's endocarditis are presented for the first time in a report of 3 cases of this rare disease. In 2 of the cases the right ventricular cavity was obliterated; in one of them this was shown by biopsy to be caused by organized thrombus. In the third case, there was progressive mitral regurgitation.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/30. Focal eosinophilic infiltration of the liver mimicking hepatocellular carcinoma: case reports.Focal eosinophilic infiltration is a rare disease entity that in patients with malignancies may mimic malignant hepatic nodules. We describe two cases in which focal eosinophilic infiltration of the liver, as well as primary liver cancer, occurred. In these patients, imaging findings similar to those observed in hepatocellular carcinoma (HCC) were noted: Enhancement at dynamic magnetic resonance (MR) imaging substantially increased perfusion at computed tomography during hepatic arteriography (CTHA) and a perfusion defect at computed tomography during arterial portography (CTAP).- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/30. Eosinophilic gastroenteritis: presentation of two patients with unusual affect of terminal ileum and caecum with manifestations of acute abdomen and literature review.Eosinophilic gastroenteritis is a rare disease; the long-term personal history with digestive symptoms and the course of the disease with relapses and remissions is the key for the disease to be suspected. endoscopy, CT scan and sonographic studies may provide important indirect signs of the disease and in combination with histological examination the diagnosis can be achieved. The administration of corticosteroids is an important factor for the treatment or the remission of the disease. In this study two cases with unusual location of the disease, on the terminal ileum and caecum, are presented and a literature review is attempted. The disease process, clinical and laboratory findings as well as the surgical approach used are described. Eosinophilic gastroenteritis is a very rare disease with its surgical complications. The disease is a non-surgical disease, thus presurgical diagnosis is important because the entity discussed can be under control by conservative treatment. A high disease suspicious index must be kept in the physicians' mind.- - - - - - - - - - ranking = 2keywords = rare disease (Clic here for more details about this article) |
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