HISTIOCITOSIS DE LANGERHANS PDF

Archivos de Bronconeumologia http: www. Other types of articles such as reviews, editorials, special articles, clinical reports, and letters to the Editor are also published in the Journal. It is a monthly Journal that publishes a total of 12 issues, which contain these types of articles to different extents. All manuscripts are sent to peer-review and handled by the Editor or an Associate Editor from the team. The Journal is published both in Spanish and English. Therefore, the submission of manuscripts written in either Spanish or English is welcome.

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The Spanish Association of Pediatrics has as one of its main objectives the dissemination of rigorous and updated scientific information on the different areas of pediatrics. Annals of Pediatrics is the Body of Scientific Expression of the Association and is the vehicle through which members communicate. The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two receding years. CiteScore measures average citations received per document published.

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Langerhans' cell histiocytosis LCH , previously known as histiocytosis X, is a rare disease. It is characterized by the accumulation and proliferation of histiocytes, eosinophils and Langerhans' cells with Birbeck granules detected by electron microscopy. It involves single organs or systems or can present as a multisystem disease. The clinical presentation may vary widely, ranging from benign self-limiting types with spontaneous regression to slowlyprogressive malignant disease.

We report five cases of LCH with the same histopathologic basis but different outcome.. ISSN: Previous article Next article. Issue 5. Pages 01 November Langerhans' cell histiocytosis: various manifestations with the same histopathologic base. Download PDF. Corresponding author. Hospital de la Santa Creu i Sant Pau. Sant Antoni M. This item has received.

Article information. Palabras clave:. We report five cases of LCH with the same histopathologic basis but different outcome. Key words:. Langerhans' cell histiocytosis. Full text is only aviable in PDF. Lichtenstein, X. Arch Pathol Lab Med, 56 , pp.

Favara, A. Contemporany classification of histiocytic disorders. Med Pediatr Oncol, 29 , pp. Howarth, G. Gilchrist, B. Mullan, G. Wiseman, J. Edmonson, P. Langerhans cell histiocytosis. Diagnosis, natural history, management and outcome. Cancer, 85 , pp. Bhatia, M. Nesbit, R. Egeler, J. Buckley, A. Mertens, L. Epidemiologic study of Langerhans cell histiocytosis in children. J Pediatr, , pp. Hematol Oncol Clin North Am, 1 , pp. McClain, H. Jin, V.

Gresik, B. Langerhans cell histiocytosis: Lack of a viral etiology. Am J Hematol, 47 , pp. Kilpatrick, D. Wenger, G. Gilchrist, T. Shives, P. Wollan, K. Langerhans' cell histiocytosis histiocytosis X of bone. A clinicopathological analysis of pediatric and adult cases. Cancer, 76 , pp. Dunger, V. Broadbent, E. Yeoman, J. Seckl, S. Lightman, D. Grant, et al. The frequency and natural history of diabetes insipidus in chindren with Langerhans-cell histiocytosis.

N Engl J Med, , pp. Arico, R. Clinical aspects of Langerhans cell histiocytosis. Hematol Oncol Clin North Am, 12 , pp. Egeler, R. Thompson, P. Voute, M. Intralesional infiltration of costicosteroids in localized Langerhans cell histiocytosis.

J Pediratr Orthop, 12 , pp. Egeler, G. Broadbent, H. Current therapy for Langerhans cell histiocytosis. Ringden, B. Lancet, , pp. Braier, M. Ciocca, A. Cholestasis, sclerosing cholangitis, and liver transplantation in langerhans cell histiocytosis. Med Pediatr Oncol, 38 , pp. The Frech Langerhans' cell histiocytosis study group. A multicentre retrospective survey of Langerhans' cell histiocytosis: cases observed beetween and Arch Dis Child, 75 , pp.

Willis, A. Ablin, V. Weinberg, S. Zager, W. Wara, K. Disease course and late sequelae of Langerhans' cell histiocytosis: year experience at the University of California, San Francisco.

J Clin Oncol, 14 , pp. Actualizado el 23 de Marzo.

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Langerhans cell histiocytosis

Langerhans cell histiocytosis is a disorder in which excess immune system cells called Langerhans cells build up in the body. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin , lymph nodes, spleen , lungs, liver , and bone marrow. In Langerhans cell histiocytosis , excess immature Langerhans cells usually form tumors called granulomas. Many researchers now consider Langerhans cell histiocytosis to be a form of cancer, but this classification remains controversial. In approximately 80 percent of affected individuals, one or more granulomas develop in the bones, causing pain and swelling. The granulomas, which usually occur in the skull or the long bones of the arms or legs, may cause the bone to fracture. Granulomas also frequently occur in the skin, appearing as blisters, reddish bumps, or rashes which can be mild to severe.

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Langerhans cell histiocytosis LCH is a rare cancer involving clonal proliferation of Langerhans cells , abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Symptoms range from isolated bone lesions to multisystem disease. LCH is part of a group of syndromes called histiocytoses , which are characterized by an abnormal proliferation of histiocytes an archaic term for activated dendritic cells and macrophages. These diseases are related to other forms of abnormal proliferation of white blood cells , such as leukemias and lymphomas.

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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. Langerhans cell histiocytosis LCH is a systemic disease associated with the proliferation and accumulation usually in granulomas of Langerhans cells in various tissues. In the majority of cases, onset occurs during childhood. However, involvement of these organs does not affect the vital prognosis.

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Langerhans cell histiocytosis: a rare cause of cholestasis in adult patients. Case report. Histiocitosis de Langerhans: causa infrecuente de colestasis en el paciente adulto. Palabras clave: Histiocitosis. We present a 42 year old man, with a history of central diabetes insipidus since childhood, primary hyperthyroidism with negative antibodies and seizures since early adulthood. He had undergone a cholecistectomy and received treatment with levothyroxin, desmopressin and lamotrigine. One year before coming to our hospital for the first time, he had presented an episode of acute pancreatitis with pseudocyst formation.

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