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1/217. Digital clubbing in a patient with progressive mantle cell lymphoma.

    Digital clubbing with or without hypertrophic osteoarthropathy may occur in a variety of neoplastic diseases. We present the first description of digital clubbing in a patient with progressive mantle cell lymphoma, in the absence of any other apparent etiology. We suggest that clubbing might be a paraneoplastic manifestation in mantle cell lymphoma. The literature on digital clubbing in hematological neoplasms is reviewed. ( info)

2/217. Epithelioid haemangioendothelioma.

    Epithelioid haemangioendothelioma is a rare pulmonary neoplasm with less than 40 cases described world wide. We describe the only case to have presented with hypertrophic pulmonary osteoarthropathy who has been treated with azathioprine and has remained alive and well with no deterioration in pulmonary function since being diagnosed 16 years ago. The progression of the chest radiograph and spiral CT appearances of this rare neoplasm are described, and current views regarding the cellular origin of the neoplasm, its cytological appearance, clinical presentation and prognosis are discussed. ( info)

3/217. Hypertrophic osteoarthropathy of one leg--a sign of aortic graft infection.

    We report a rare case of hypertrophic osteoarthropathy (HOA) confined to the right leg secondary to aortic graft infection. The development of HOA exclusively localized to areas distal to a vascular prosthesis may be the presenting manifestation of graft infection and a crucial diagnostic clue in the early detection of vascular graft infection. HOA is diagnosed by its characteristic radiographic and scintigraphic pattern. Most prosthetic, especially aortic, graft infections are uniformly fatal if not treated by aggressive surgical and antibiotic therapy. Recognition of this uncommon association may facilitate an early diagnosis, which usually requires immediate surgical therapy. ( info)

4/217. Lupoid hepatitis, Rendu-Osler-Weber syndrome, clubbing cyanosis and hypertrophic osteoarthropathy.

    Chronic liver disease typical of chronic active 'lupoid' hepatitis together with cyanosis, clubbing and hypertrophic osteoarthropathy in a 42-year-old female is described. In addition she had severe nose bleeds, gastro-intestinal haemorrhages, syncopal attacks with generalised convulsive seizures, pulmonary arterio-venous fistulae as manifestations of Rendu-Osler-Weber syndrome. A study of the literature revealed that similar associations are far more frequent than can be attribtued to chance. Possible mechanisms of the cyanosis, clubbing and osteoarthropathy and possible common pathogenesis for these seemingly unrelated disorders are discussed. ( info)

5/217. Respiratory bronchiolitis associated with severe dyspnea, exertional hypoxemia, and clubbing.

    Respiratory bronchiolitis-associated interstitial lung disease (RBILD) is a distinct clinicopathologic disease described almost exclusively in cigarette smokers.(1) (2) The disease usually presents with mild symptoms and is associated with a good prognosis. (2) Severe lung dysfunction has not been reported with RBILD, which is often confused clinically and radiographically with desquamative interstitial lung disease or idiopathic pulmonary fibrosis (IPF). Two patients with RBILD who developed severe dyspnea, hypoxemia, and clubbing are described. Initially, IPF was diagnosed in both patients. The severity of symptoms was such that the first patient's room air saturation was 85% and the second patient had severe impairment of lung function, with FEV(1) of 39% and FVC of 40%. Advanced lung disease required supplemental home oxygen therapy in the first patient and referral for lung transplant evaluation in the second patient. After a detailed review of histology revealed a diagnosis of RBILD, both patients were encouraged to stop smoking; smoking cessation led to considerable improvement in symptoms and lung function tests. We conclude that advanced lung dysfunction occurs in some patients with RBILD and should not dissuade that diagnosis. ( info)

6/217. exophthalmos, pretibial myxedema, osteoarthropathy syndrome associated with papillary fibroelastoma in the left ventricle.

    EMO syndrome, a rare complication of Graves' disease, exhibits exophthalmos, pretibial myxedema, and osteoarthropathy. The presence of functional thyrotropin receptors (TSHR) in adipocytes and osteoblasts, both of which we have recently observed, may be related to these extrathyroidal manifestations of Graves' disease. In addition, the expression of TSHR in the heart has recently been reported. We describe here a patient with Graves' disease exhibiting EMO syndrome with a papillary fibroelastoma in the left ventricle. Pathological examinations showed that the fibroelastoma contained alcian blue-stained mucinous materials that were also observed in the subcutaneous tissue of pretibial myxedema. ( info)

7/217. Hypertrophic pulmonary osteoarthropathy in acquired immunodeficiency syndrome. Case report and review.

    We describe a case of hypertrophic pulmonary osteoarthropathy (HPOA) in an adult patient with acquired immunodeficiency syndrome (AIDS). This is the ninth case of HPOA associated with AIDS in adults, reported in the literature. The presence of pulmonary tuberculosis was also suspected, based on clinical grounds. Cases of clubbing associated with AIDS infection are reviewed. ( info)

8/217. Secondary hypertrophic osteoarthropathy. An unusual cause of arthritis in childhood.

    Although an uncommon occurrence in childhood, hypertrophic osteoarthropathy secondary to tumors- most commonly to osteogenic sarcoma with pulmonary metastasis-may cause severe join pain and swelling. The syndrome should be considered in the differential diagnosis of acute arthritis in childhood ( info)

9/217. Hypertrophic osteoarthropathy in lung cancer: are the radiographic bone changes reversible after curative resection?

    Lack of relief of bone and joint pains and persistence of the radiographic abnormalities in patients with secondary hypertrophic osteoarthropathy, after lung tumour removal, are considered as signs of an unfavourable prognosis. Two patients are documented in whom the lack of reversion of periosteal changes after tumour removal was compatible with a favourable long-term prognosis, suggesting that radiographic exploration is an insensitive technique to appreciate involutive bone changes occurring in these patients. ( info)

10/217. Palmar fasciitis and polyarthritis associated with gastric carcinoma: complete resolution after total gastrectomy.

    Palmar fasciitis and polyarthritis (PFA) is a rare paraneoplastic rheumatic syndrome characterized by flexion contractures of both hands and thickening of palmar fascia. Several reports have suggested that this syndrome is a tumor-associated autoimmune disorder. We report a 44-year-old Japanese man who presented with flexion contractures of both hands associated with thickening of palmar fascia and polyarthritis. These clinical pictures were suggestive of PFA associated with occult neoplasm. Upper gastrointestinal endoscopic examination revealed advanced gastric cancer. Resection of the cancer resulted in a gradual resolution of palmar fasciitis and polyarthritis. This clinical course suggests an underlying tumor-related immunologic process in this syndrome. ( info)
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