1/368. 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 = edema (Clic here for more details about this article) |
2/368. retinitis pigmentosa, nanophthalmos, and optic disc drusen: a case report.OBJECTIVE: Although the associations of retinitis pigmentosa (RP) with nanophthalmos/microphthalmos and RP with optic disc drusen have previously been recognized, the concurrence of all three features, as far as the authors are aware, has not previously been reported. DESIGN: Case report. RESULTS: The authors report a sporadic case of nanophthalmos, RP, and optic nerve drusen with the additional complication of chronic angle closure glaucoma. CONCLUSIONS: Visual loss may be secondary to the complications of nanophthalmos, RP, or optic nerve drusen. Chronic angle closure may be caused by choroidal effusion with serous retinal detachment, which may, in turn, cause a pseudo-RP picture. It is therefore important to recognize the possible association of true RP with nanophthalmos as a cause for visual deterioration.- - - - - - - - - - ranking = 15.372795148881keywords = optic (Clic here for more details about this article) |
3/368. Systemic lupus erythematosus with optical neuromyelitis (Devic's syndrome). A case with a 35-year follow-up.Optical neuromyelitis or Devic's syndrome is a very uncommon neurological manifestation of systemic lupus erythematosus. It is also associated with antiphospholipid antibodies, limited responsiveness to glucocorticoid treatment and a poor prognosis. We report the case of a female systemic lupus erythematosus patient who developed recurrent flares of optical neuritis and transverse myelitis. These flares consistently responded to glucocorticoid therapy. Despite the absence of overt anticardiolipin antibodies in the course of the disease, long-term anticoagulant therapy has been introduced with positive results. Treatments are usually of limited efficacy in Devic's syndrome. In our patient, however, aggressive glucocorticoid treatment resulted in prolonged survival.- - - - - - - - - - ranking = 10.980567963486keywords = optic (Clic here for more details about this article) |
4/368. Papillary endothelial hyperplasia presenting as a chest wall neoplasm.Soft tissue hematomas generally resolve but may persist and develop into slow-growing, organized masses. These chronic expanding hematomas are characterized by a pseudocapsule and a predominantly necrotic central cavity, with foci of newly formed capillaries. These have been called chronic expanding hematomas or Masson's papillary endothelial hyperplasia. These lesions can mimic vascular neoplasms and must be considered in the evaluation of expanding soft tissue vascular malformations.- - - - - - - - - - ranking = 1.136642615546keywords = papilla (Clic here for more details about this article) |
5/368. optic atrophy and chronic acquired polyneuropathy.Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic, multifocal disorder usually defined as limited to the peripheral nervous system. Multifocal motor neuropathy, an acquired demyelinating neuropathy with conduction block affecting motor neurons only, may be a pathogenically distinct syndrome or a predominantly motor variant of chronic inflammatory demyelinating polyneuropathy. central nervous system demyelination including optic neuropathy has been reported uncommonly previously in these entities. We report two cases and review the literature on the possible association of optic neuropathy and chronic acquired polyneuropathy.- - - - - - - - - - ranking = 4.3922271853945keywords = optic (Clic here for more details about this article) |
6/368. lymphangiosarcoma in chronic lymphoedema. Stewart-Treves syndrome.A case of post-mastectomy lymphangiosarcoma is reported. lymphangiosarcoma is an extremely rare but highly lethal complication of chronic lymphoedema. Our patient was treated by amputation and died 6 months later. Treatment of post-mastectomy lymphangiosarcoma is still unsatisfactory. Early recognition and radical ablative surgery seem to provide best chance for survival.- - - - - - - - - - ranking = 5keywords = edema (Clic here for more details about this article) |
7/368. Autoimmune atrophic gastritis with hypergastrinemia.Elevation in fasting serum gastrin levels was found in three patients being evaluated for persistent upper abdominal pain without radiographic evidence of peptic ulcer disease. Fiberoptic endoscopy of the upper gastrointestinal tract in each patient revealed characteristic changes of chronic atrophic gastritis. Gastric biopsies showed diffuse chronic inflammation in the lamina propria, a decrease in the number of parietal cells, and "intestinalization" of gastric mucosa. Total achlorhydria was demonstrated after a maximal histalog stimulus; however, serum levels of vitamin B12 and schilling test values were normal in all three patients. Parietal cell antibodies were found in the serum in all patients in a dilution of 1:20 to 1:80. These cases represent autoimmune (type A) chronic atrophic gastritis and should be distinguished from chronic simple (type B) gastritis, in which serum gastrin levels are normal and no parietal cell antibodies are found in the serum. patients with autoimmune gastritis should be observed at frequent intervals for the occurrence of pernicious anemia or gastric carcinoma.- - - - - - - - - - ranking = 2.1961135926973keywords = optic (Clic here for more details about this article) |
8/368. Extraction of a rubber bullet from a bronchus after 1 year: complete resolution of chronic pulmonary damage.inhalation of a foreign body (FB) into the bronchial tree rarely occurs asymptomatically and, if leading to recurrent pneumonia, can be very difficult to diagnose. The present report deals with the case of a 10-year-old boy who had three episodes of pneumonia in the left lower lobe caused by the asymptomatic inhalation of a FB 12 months before. Standard thoracic CT, done during the third episode, revealed a slight reduction in the volume of the left lung with air bronchograms, multiple areas of bronchiectasis, and parenchymal consolidation of a segment of the lower lobe. Flexible fiberoptic bronchoscopy revealed a FB at the distal end of the left lower lobar bronchus, surrounded by granulation tissue and fully obstructing the anterior basal segmental bronchus. High-resolution CT (HRCT) images showed an inverted C-shaped image obstructing a bronchus. Removal of the FB was successful only with rigid bronchoscopy under total anesthesia. The FB was an air-pistol rubber bullet that the boy remembered playing with 12 months before. Two months after removal of the FB (ie, 14 months from its asymptomatic inhalation) and treatment with oral steroids, antibiotics, and respiratory physiotherapy, the patient recovered completely, and HRCT showed complete normalization of the lung. We conclude that, when the radiographic density of the FB is greater than the surrounding pulmonary parenchyma, HRCT can reveal the FB, and diagnostic flexible fiberoptic bronchoscopy can be avoided.- - - - - - - - - - ranking = 4.3922271853945keywords = optic (Clic here for more details about this article) |
9/368. A GLC1A gene Gln368Stop mutation in a patient with normal-tension open-angle glaucoma.PURPOSE: To present a case involving a patient with normal-tension glaucoma with a Gln368Stop mutation of the myocilin/trabecular meshwork inducible glucocorticoid response protein (MYOC/TIGR) gene. methods: Slit-lamp biomicroscopic and gonioscopic examination, morphometry of the optic disc, 24-hour intraocular pressure (IOP) profile, and perimetry were performed to determine the phenotype of the patient. neurologic examination and a computed tomographic (CT) scan of the brain were performed to rule out a neurologic disorder. Single-strand confirmation polymorphism (SSCP) analysis and subsequent sequence analysis of blood was performed for genotyping of the GLC1A gene. RESULTS: A nonsense codon, namely a Gln368Stop mutation in the third exon of the GLC1A gene, was found in this patient with normal-tension glaucoma. CONCLUSION: In contrast to previous reports, a Gln368Stop mutation of the GLC1A gene need not be confined to patients with glaucomatous optic atrophy due to high IOP. The pathogenesis of glaucoma associated with GLC1A gene mutations might be more complex than expected, and (unknown) suppressor mechanisms have to be considered.- - - - - - - - - - ranking = 4.3922271853945keywords = optic (Clic here for more details about this article) |
10/368. A successful case of pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension with a thrombus in the right ventricle.Chronic thromboembolism is a frequent cause of progressive hypertension and carries a poor prognosis. Medical treatment is not effective and surgery provides the only potential for a cure at present. We herein report a successful case of thromboendarterectomy treated via a median sternotomy with intermittent circulatory arrest. A 43-year-old man was admitted to our hospital complaining of progressive dyspnea, edema of the lower extremities, and a fever with an unknown origin. A subsequent definitive evaluation showed him to be suffering from surgically accessible chronic thromboembolic pulmonary hypertension with a thrombus in the right ventricle. He underwent a pulmonary thromboendarterectomy and thrombectomy via a median sternotomy with intermittent circulatory arrest on November 24, 1994. Postoperatively he showed a marked improvement in his hemodynamic status and blood gas analysis. He has also returned to work with no trouble. Deep vein thrombosis appeared to be the pathogenesis of this case, but we could not find the origin of his unknown fever. He is currently being controlled by treatment with methylprednisolone as before.- - - - - - - - - - ranking = 1keywords = edema (Clic here for more details about this article) |
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