1/4. Renal failure resulting from infiltration by inflammatory myofibroblastic tumor responsive to corticosteroid therapy.Inflammatory myofibroblastic tumor (IMT) is a rare disease of inflammatory and spindle cell proliferative lesions in multiple sites. Most frequently reported in the lungs, a variety of extrapulmonary sites have been described. We report the first case of IMT causing renal failure by massive bilateral renal infiltration. Renal function continued to deteriorate (peak serum creatinine level, 8.4 mg/dL) despite placement of a ureteral stent. The kidneys were diffusely enlarged on computed tomographic (CT) scan. Renal biopsy showed myofibroblastic proliferation and inflammatory infiltration. Renal failure responded favorably over weeks to corticosteroid therapy (serum creatinine level, 2.8 mg/dL) with a marked reduction in bilateral renal enlargement by CT scan.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/4. Pancreatic involvement in primary amyloidosis: radiologic findings.amyloidosis is a rare disease that can involve a great variety of organs. As a consequence, clinical and radiological manifestations are very different as well. We report on a patient with primary amyloidosis who presented with diffuse pancreatic involvement detected by ultrasound, helical CT, and MRI. There are very few imaging descriptions about this glandular involvement in amyloidosis in the literature. Despite the apparent uncommon radiological appearance, we believe that this entity should be taken into account in the differential diagnosis of diffuse infiltrative pancreatic lesions in the appropriate clinical and analytical setting.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/4. Malabsorption of flucytosine in a pediatric patient with Shwachman syndrome.The malabsorption of drugs from the gastrointestinal tract in patients with pancreatic insufficiency is not well documented in the literature. We describe a case of flucytosine malabsorption in a pediatric patient with Shwachman syndrome, a rare disease in the pediatric age group characterized by pancreatic insufficiency. A significant increase in serum concentrations of flucytosine was noted when the drug was administered in a lipophilic vehicle, possibly due to enhanced absorption.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/4. Pancreatic tuberculosis in non-immunocompromised patients: reports of two cases, and a literature review.We present two cases of biopsy proven tuberculosis of the pancreas in non-immunocompromised patients diagnosed and treated in our unit within the last 14 years. The first case presented with abdominal pain and fever, and the second with iron deficiency anaemia and severe weight loss. In both cases abdominal ultrasound and computed tomography suggested a pancreatic carcinoma. There was no pulmonary or intestinal tuberculosis. The tuberculin skin test was positive. Upon exploratory laparotomy the macroscopic appearance of the pancreas was that of an inoperable pancreatic carcinoma. Following the histological diagnosis of pancreatic tuberculosis, both patients were successfully treated with triple antituberculous therapy for 6 months. Isolated pancreatic tuberculosis is an extremely rare disease with only 41 cases in non-immunocompromised patients reported worldwide (1966-1997). It is a curable disease and should be considered in the differential diagnosis of a pancreatic mass or abscess shown on ultrasound or computed tomography, especially in developing countries, where tuberculosis is common.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |