1/8. Obstructive nephropathy as a result of retroperitoneal fibrosis: a review of its pathogenesis and associations.retroperitoneal fibrosis is a rare disease, typically with an insidious clinical course. It is thought that this disease process is perhaps an exaggerated reaction to an inciting inflammatory event. In this study, a case of retroperitoneal fibrosis is reported, in which the patient presented with typical symptoms of retroperitoneal fibrosis, along with some atypical vasculitic symptomatology. retroperitoneal fibrosis is a disease process with an unknown etiology, which has been observed to be associated with a number of different possible inciting factors. Two factors that have been documented in the literature as being associated with retroperitoneal fibrosis include the use of beta-blocking agents, and connective tissue disease processes such as systemic lupus erythematosus. The patient discussed was using beta-blocker medication and also had signs and symptoms suggestive of a lupus syndrome. There are no reported cases of the combined association of beta-blocker usage, lupus, and retroperitoneal fibrosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/8. Inflammatory vitiligo in Vogt-Koyanagi-Harada disease.Vogt-Koyanagi-Harada disease is a rare disease characterized by uveitis, meningitis, dysacusis, alopecia, poliosis, and vitiligo. We describe a 48-year-old patient with Vogt-Koyanagi-Harada disease associated with thin inflammatory raised erythema and plaque-type inflammatory erythema superimposed on vitiligo. Interestingly, inflammatory raised erythema was separated from the perfect vitiligo, and the incomplete vitiligo lay between them initially. Thereafter, incomplete vitiligo became completely depigmented with diminution of inflammatory raised erythema. This is the second case of vitiligo with inflammatory raised borders associated with Vogt-Koyanagi-Harada disease. Our results of immunohistochemical and electron microscopic studies suggested the involvement of T-cell-mediated cytotoxicity and apoptosis in the development of skin lesions.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/8. Inflammatory myofibroblastic tumour of the liver in a child: CT and MR findings.Inflammatory myofibroblastic tumour of the liver is a rare disease in children and is characterized by an inflammatory mass of uncertain aetiology which simulates a true neoplasm, often resulting in a diagnostic dilemma. We report a pathologically proven case of inflammatory myofibroblastic tumour of the liver in a 14-month-old boy with jaundice. CT and MRI showed an ill-defined, homogeneously enhancing mass located in the hilar portion of the liver with biliary obstruction. He underwent percutaneous transhepatic biliary drainage to relieve obstructive jaundice, followed by lobectomy and, finally, liver transplantation.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/8. Inflammatory myofibroblastic tumor of the stomach with peritoneal dissemination in a young adult: imaging findings.Inflammatory myofibroblastic tumors are lesions that most often affect young adults and children. These tumors have been found in numerous extrapulmonary sites but rarely in the stomach. It is unknown whether this process is reactive or neoplastic. They are infiltrative lesions and often extend through the gastric wall, sometimes reaching adjacent organs including the esophagus, duodenum, peritoneal cavity, spleen. pancreas, and liver. These features mimic malignancy on endoscopy and radiology. We report the ultrasound, color Doppler ultrasound, and helical computed tomographic findings of a gastric inflammatory myofibroblastic tumor with peritoneal dissemination in a young adult. To our knowledge, this is the first report of color Doppler ultrasound and helical computed tomographic findings of this rare disease entity.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/8. Granulomatous hypophysitis mimicking pituitary adenoma.Granulomatous inflammation of the pituitary gland is a rare disease that mimics non-functioning pituitary adenoma. Its incidence is high in middle aged and elderly women. Two such cases of histopathologically confirmed granulomatous hypophysitis are reported which presented with clinical and radiological impression of pituitary adenoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/8. Effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia.INTRODUCTION: Hyperzincaemia and hypercalprotectinaemia with systemic inflammation, recurrent infections, hepatosplenomegaly, arthritis, anemia, cutaneous inflammation, and failure to thrive is an extremely rare disease and no therapy is reported. AIM: To evaluated the effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia in terms of serum cytokine level changes before and after treatment. methods: A 10-year-old girl was admitted suffering from pyoderma gangrenosum, hepatosplenomegaly, anemia that was unresponsive to iron supplementation, persistent inflammation, arthritis, and increased serum zinc. The level of serum calprotectin was extremely high; therefore, we diagnosed hyperzincaemia and hypercalprotectinaemia and started cyclosporine A treatment. Twelve cytokines in serum were measured before and one year after treatment. RESULTS: cyclosporine A was very effective. Her skin lesion and joint pain were alleviated and quality of life was markedly improved. c-reactive protein had decreased and anemia had improved. While zinc levels had fallen, calprotectin remained at an extremely high level. Of the cytokines examined, interleukin -6 serum levels had fallen and interleukin -8 showed a marked reduction after treatment. CONCLUSION: cyclosporine A is effective for hyperzincaemia and hypercalprotectinaemia. serum interleukin -8 may be useful in assessing the therapeutic effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/8. Ureteritis cystica with 17-year followup.Ureteritis cystica is a rare disease that must be considered in the differential diagnosis of ureteral and renal pelvic filling defects. The characteristic radiographic findings often regress spontaneously when the process instigating the inflammation is eradicated. However, in our patient radiological findings remained unchanged during a 17-year period in the absence of malignancy or infection. Although it sometimes coexists with cancer, we believe that ureteritis cystica is usually benign and indolent.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/8. Ligneous (pseudomembranous) inflammation of the female genital tract. A report of two cases.Two women, aged 28 and 32, presented with primary infertility and severe dysmenorrhea. The younger woman developed recalcitrant and recurrent firm vaginal, cervical and possibly endometrial lesions over a period of six years; the older patient had similar material in the cervix, endometrium and a fallopian tube. The lesions were composed of amorphous eosinophilic hyaline or necrotic material, shown by histochemical and immunoperoxidase stains to be partly fibrin. Pathologic examination showed that the lesions were similar to those of ligneous (pseudomembranous) conjunctivitis, a rare disease that may occasionally affect the lower female genital tract. However, neither patient had eye disease, and ligneous conjunctivitis has not previously been reported to involve the upper genital tract. Endometrial and tubal involvement could readily explain primary infertility and dysmenorrhea in these patients.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |