Cases reported "Chronic Disease"

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1/602. Chronic eosinophilic leukemia: successful treatment with an unrelated bone marrow transplantation.

    The optimal treatment of eosinophilic leukemia is still uncertain. We report the successful treatment of a 21-year-old patient with eosinophilic leukemia, without cytogenetic abnormalities, by bone marrow transplantation from an unrelated donor. The conditioning regimen for the transplantation consisted of fractionated total body irradiation and cyclophosphamide. Acute GVHD, grade I, post-transplantation was successfully treated. No other severe complications occured. The patient is alive in complete remission 21 months after unrelated bone marrow transplantation.
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keywords = bone
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2/602. Focal malakoplakia in chronic periapical periodontitis.

    AIMS: Three cases of chronic periapical periodontitis including focal areas with malakoplakia changes are reported. methods AND RESULTS: These areas included both von Hansemann-type macrophages and periodic acid-Schiff-positive, iron- and calcium-containing concretions. Some concretions corresponded to spherules with a targetoid configuration, thus fitting the morphological criteria for classical Michaelis-Gutmann bodies. CONCLUSION: The vast majority of the cases of malakoplakia that have been reported in the literature corresponded to a characteristic, fairly homogeneous lesion, but a few instances of focal malakoplakia have been described in various chronic conditions. These considerations support the opinion that the local conditions for the production of Michaelis-Gutmann bodies may occur focally in diseases characterized by macrophage accumulation.
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keywords = macrophage
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3/602. The fourth-compartment syndrome: its anatomical basis and clinical cases.

    We propose a new term, the "fourth-compartment syndrome" to describe chronic dorsal wrist pain of the fourth compartment. Five main causes responsible for this syndrome are thought to be as follows: 1. Ganglion involvement, including an occult ganglion; 2. Extensor digitorum brevis manus muscle; 3. Abnormal extensor indicis muscle; 4. Tenosynovialitis; 5. Anomaly or deformity of carpal bones. Should the above mentioned conditions occur in the fourth compartment, pressure within the fourth compartment increases, ultimately compressing the posterior interosseous nerve directly or indirectly. Anatomical studies of the fourth compartment of the wrist and the posterior interosseous nerve are presented and the fourth-compartment syndrome is summarized with twelve clinical cases (six cases of occult ganglions, two cases of extensor digitorum brevis manus, two cases of tenosynovialitis, one case of abnormal extensor indicis muscle, and one case of carpal bossing).
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keywords = bone
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4/602. Correction of bone marrow failure in dyskeratosis congenita by bone marrow transplantation.

    dyskeratosis congenita is recognized by its dermal lesions and constitutional aplastic anemia in some cases. We report successful allogeneic bone marrow transplantation in two siblings with this disease from their sister, and their long term follow-up. We used reduced doses of cyclophosphamide and busulfan for conditioning instead of total body irradiation. Also, we report late adverse effects of transplantation which are not distinguishable from the natural course of disease.
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5/602. Disseminated nocardiosis in a bone marrow transplant recipient with chronic GVHD.

    We describe a case of disseminated nocardiosis in a 53-year-old male allogeneic marrow recipient with chronic GVHD, 15 years post BMT. Six months prior to admission he was treated for recurrent chronic GVHD with corticosteroids with a good response. He deteriorated subsequently while still on steroids requiring admission for fever, anorexia, weight loss, productive cough and progressive dyspnoea. On admission he had multiple nodular lesions on chest roentgenogram and subsequently grew nocardia farcinica in blood culture. N. farcinica is rare post BMT, has a high mortality, is resistant to various antibiotics and needs prolonged antimicrobial therapy. We report the successful management of our patient with single agent trimethoprim-sulphamethoxazole.
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keywords = bone
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6/602. Destruction of the glenoid joint fossa by a tenosynovial giant-cell tumour of the skull base: a case report.

    A 63-year-old man is presented in whom a tenosynovial giant-cell tumour had destroyed the temporomandibular joint fossa and expanded intracranially. The lesion was not diagnosed for a period of at least two years. Treatment included wide resection including the surrounding bone, dura and condyle.
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keywords = bone
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7/602. thalidomide in children undergoing bone marrow transplantation: series at a single institution and review of the literature.

    thalidomide has one of the most notorious drug histories because of its teratogenicity. Its widespread use in the 1960s led to a worldwide epidemic of phocomelia in inborns; this in turn led to its complete ban in most of the world. However, it has now been licensed for selected indications including graft-versus-host-disease (GVHD) after bone marrow transplantation, wasting associated with tuberculosis and human immunodeficiency virus infection, and leprosy. Little is known, however, about its use in children in these settings. Therefore, we report our experience and review the literature on thalidomide in children for GVHD after bone marrow transplantation. We studied 6 patients, 2 with chronic GVHD, 2 with acute GVHD, and 2 with acute GVHD progressing into chronic disease. One patient with chronic GVHD had a complete response, whereas the other had a partial response. Side effects consisted primarily of sedation and constipation, which are reported previously and well known side effects. None had neuropathy. One patient had rash, eosinophilia, and early pancreatitis that began shortly after initiation of thalidomide, persisted, and resolved only after discontinuation of thalidomide. eosinophilia and pancreatitis are both previously unreported side effects or associated findings of thalidomide treatment. review of the literature reveals three major studies of thalidomide in GVHD; of these two included children and adults together, and one in which age range of patients was not mentioned. In addition, four series of children receiving only thalidomide are reported. These series contained 1 to 14 patients each. Results show efficacy in at least 50% of children with chronic GVHD and little or no efficacy in children with exclusively acute GVHD. Side effects are similar to those reported in adults and consisted mostly of sedation and constipation, both of which subsided over time and resolved after discontinuing the drug. We speculate on the reasons for which thalidomide is more effective in chronic, compared with acute, GVHD in children, and make recommendations for future study.
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keywords = bone
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8/602. Incomplete nondisplaced tibial osteotomy for treatment of osteoarthritic knee pain.

    Intraosseous venous hypertension is considered a significant factor in the production of knee pain secondary to degenerative arthrosis, thus vascular decompression by means of tibial osteotomy is a rational option for treatment of selected patients. A preliminary report is presented of six patients with symptoms of primary or secondary degenerative arthrosis who were treated by incomplete nondisplaced proximal tibial osteotomy with good or excellent results. These patients were refractory to nonoperative treatment and were not deemed suitable candidates for angulated osteotomy, arthroscopic surgery alone, or total knee arthroplasty. patients ranged in age from 36 to 61 years (mean age: 47 years). Follow-up ranged from .8 to 6.7 years (mean: 3.1 years). The subjects were studied postoperatively by interview, physical examination, radiographs, and bone scans. Results were assessed using the knee rating system of The Hospital for Special Surgery.
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keywords = bone
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9/602. Gustatory otalgia and wet ear syndrome: a possible cross-innervation after ear surgery.

    HYPOTHESIS: The chorda tympani and Arnold's nerves have close approximation to each other and their cross-innervation is possible after ear surgery. STUDY DESIGN: A retrospective study was performed with a temporal bone pathology case and two clinical cases as representatives of such a possibility. patients had severe otalgia and wet ear during gustatory stimulation. methods: A temporal bone pathology case was studied under a light microscope. earache and/or wet ear were provoked during gustatory stimulation. Wet ear was tested with iodine-starch reaction after the subject tasted lemon juice. RESULTS: The temporal bone specimen has clusters of regenerated fibers in the tympanic cavity in the area of the chorda tympani and Arnold's nerves, suggesting a possibility of mixing. There are regenerated fibers in the iter chordae anterius, showing successful bridging of the chorda tympani nerves across a long gap. Detachment of the skin over the operated mastoid bowl obscured signs in one clinical case. Another clinical case of gustatory wet ear showed objective evidence of cross-innervation with iodine-starch reaction. CONCLUSION: The detachment procedure and iodine-starch reaction were the proofs that the signs were related to regenerated fibers. This is the first report of gustatory otalgia and wet ear after ear surgery.
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keywords = bone
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10/602. Angiocentric lymphoma involving the temporal bone in a child.

    Involvement of the temporal bone in patients with malignant lymphomas is very rare. Most of the reported cases have been clinically asymptomatic and were diagnosed only by post-mortem examinations. We present a nasal, paranasal, nasopharyngeal lymphoma that occurred in a 12-year-old child and also involved the temporal bone. Clinical presentation began with bilateral chronic otitis media. Histopathologically, tumor was found to be an angiocentric lymphoma of B-cell origin. association with Epstein-Barr virus could not be demonstrated. Despite combination chemotherapy (with cyclophosphamide, vincristine, doksorubicine, prednisolone, L-asparaginase, cytosine arabinoside, metotraxate) and radiotherapy (to 40 Gy), disease progressed locally as well as to cervical lymph nodes and the lungs.
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keywords = bone
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