1/8. otitis media as a sign of Wegener's granulomatosis in childhood.Wegener's granulomatosis (WG) is a rare disease among paediatric patients. Chronic otitis media with or without facial nerve dysfunction is a known manifestation of the disease among adults. A case of a 15-year-old boy with WG, whose initial symptoms were acute otitis media and facial nerve paralysis, is presented. The otorhinolaryngological manifestations, as well as diagnostic and current treatment modalities in paediatric patients with WG, are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/8. Limited Wegener's disease presenting as pharyngolaryngeal tumor.Wegener's disease (WD) which is mostly a systemic illness rarely presents as isolated, monoorganic, limited disease. Limited pharyngolaryngeal WD is thus a very rare occurrence. We report the case of a 29 years old man who developed a pharyngolaryngeal tumor with clinically benign evolution, histologically showing granulomatous inflammation and small vessel vasculitis, with no signs of: tuberculosis, sarcoidosis, fungal disease, Hodgkin's disease or foreign body aspiration. p-ANCA's were positive. He was considered a limited form of WD and treated with moderate doses of corticoids and cotrimoxazole. One month later, the lesion diminished significantly. The finding of a pharyngolaryngeal tumor with granulomatous inflammation and vasculitis, in the context of p-ANCA positivity and without any evidence for another systemic granulomatous disease, suggested the diagnosis of limited WD. The response to treatment favoured this presumption. Limited pharyngolaryngeal WD is a rare disease, with a potential for life-threatening (even fatal) complications. It should be recognized early and treated promptly. Remissions can be achieved (even without the use of cyclophosphamide).- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/8. Wegener's granulomatosis of the breast.Wegener's granulomatosis is a multisystem disorder characterized by necrotizing granulomatous inflammation and vasculitis of small vessels and can affect any organ system. The most common sites of involvement are upper and lower respiratory tracts, and kidneys. breast involvement is unusual and very rare. We report a case of breast Wegener's granulomatosis in a 32-year-old woman who presented with pulmonary lesions and palpable masses in the left breast. mammography showed multiple, sharply delineated nodules without microcalcifications. ultrasonography revealed multiple hypoechoic solid lesions, some of them with anechoic areas of necrosis. Computed tomography showed multiple nodules. Histopathology of excision biopsy specimens of breast lesions revealed necrotizing granulomatous material consistent with Wegener's granulomatosis. Twenty reports of breast involvement in this rare disease were found in the literature; however, the respective ultrasonographic and computed tomography findings have not hitherto been described.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/8. Wegener's granulomatosis.Wegener's granulomatosis is a rare disease of unknown etiology. Until recently it was considered uniformly fatal. family physicians should be aware of the variable presentations of this disease and keep the diagnosis in mind when faced with a puzzling, chronic, progressive multisystem process. New laboratory markers can lead to earlier diagnosis, and aggressive treatment can improve the prognosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/8. Paediatric Wegener's granulomatosis: two case histories and a review of the literature.Wegener's granulomatosis is a rare disease in children, with only 37 previous cases described in the literature. We report two new cases, one in an 11-year-old child who presented with cavitating nodules in the right upper lung field as well as a maxillary sinusitis and nasal perforation, and the other in a 16-year-old female who initially presented with a purulent maxillary sinusitis and only later developed an orbital pseudotumour.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/8. wegener granulomatosis of the breast.Two cases of wegener granulomatosis of the breast are reported. In both cases, this rare disease was detected on mammographic examination and was diagnosed for the first time by a fine-needle aspiration biopsy of the breast. The mammographic findings can lead to misinterpretations and can be confused with advanced mammary carcinoma or the alterations caused by lymphoblastoma, leukemia, or hodgkin disease. The total regression of the tumorlike lesions in the breast, evidence of involvement of the lung with only slight clinical symptoms, and a very protracted course of the disease were remarkable in both cases.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/8. Wegener's granulomatosis diagnosed by open lung biopsy.A 21 year old man was diagnosed as having Wegener's granulomatosis at thoracotomy. Treatment with prednisolone and cyclophosphamide was initiated but the patient died 4 months later. Wegener's granulomatosis is a rare disease which should be considered in the differential diagnosis of pulmonary malignancies, tuberculosis and lung abscess. early diagnosis is mandatory for a favourable outcome. Open lung biopsy is recommended, if other examinations have failed, as large representative biopsies can be obtained.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/8. Wegener's granulomatosis in childhood. review of the literature and case report.Wegener's granulomatosis (WG) is a rare disease in pediatric patients. The case of a 14-year-old girl with generalized WG is described and the pediatric literature reviewed. Thirty additional cases of WG in childhood have been traced in previous studies. Diagnostic problems are discussed in the context of clinical, radiological and biopsy findings.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |