Erdheim-Chester disease: a rare syndrome with a characteristic bone scintigraphy pattern
Erdheim-Chester disease is a rare noninherited, non-Langerhans’ cell histiocytosis, with multiorgan involvement. The skeleton is frequently involved in as many as 70–80% of all cases. In nearly half of the cases, there is an involvement of other organs such as the cardiovascular system, lung, kidney...
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| Veröffentlicht in: | Annals of nuclear medicine 2008-05, Vol.22 (4), p.323-326 |
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| creator | Spyridonidis, Trifon J. Giannakenas, Costas Barla, Panagiota Apostolopoulos, Dimitrios J. |
| description | Erdheim-Chester disease is a rare noninherited, non-Langerhans’ cell histiocytosis, with multiorgan involvement. The skeleton is frequently involved in as many as 70–80% of all cases. In nearly half of the cases, there is an involvement of other organs such as the cardiovascular system, lung, kidneys, brain, and orbits. Extra-skeletal involvement is correlated with increased morbidity and mortality. In recent years, the disease is being described with increasing frequency although fewer than 200 cases have been identified worldwide. Besides its rarity, the disease has a characteristic almost pathognomonic bone scan appearance, which in some cases facilitates diagnosis of the syndrome. Bone scans also contribute to the qualitative assessment of skeletal involvement. |
| doi_str_mv | 10.1007/s12149-007-0110-3 |
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The skeleton is frequently involved in as many as 70–80% of all cases. In nearly half of the cases, there is an involvement of other organs such as the cardiovascular system, lung, kidneys, brain, and orbits. Extra-skeletal involvement is correlated with increased morbidity and mortality. In recent years, the disease is being described with increasing frequency although fewer than 200 cases have been identified worldwide. Besides its rarity, the disease has a characteristic almost pathognomonic bone scan appearance, which in some cases facilitates diagnosis of the syndrome. 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The skeleton is frequently involved in as many as 70–80% of all cases. In nearly half of the cases, there is an involvement of other organs such as the cardiovascular system, lung, kidneys, brain, and orbits. Extra-skeletal involvement is correlated with increased morbidity and mortality. In recent years, the disease is being described with increasing frequency although fewer than 200 cases have been identified worldwide. Besides its rarity, the disease has a characteristic almost pathognomonic bone scan appearance, which in some cases facilitates diagnosis of the syndrome. 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diagnostic imaging</topic><topic>Bone and Bones - pathology</topic><topic>Bone Diseases - diagnostic imaging</topic><topic>Bone Diseases - pathology</topic><topic>Case Report</topic><topic>Diaphyses - diagnostic imaging</topic><topic>Diaphyses - pathology</topic><topic>Epiphyses - diagnostic imaging</topic><topic>Epiphyses - pathology</topic><topic>Erdheim-Chester Disease - diagnostic imaging</topic><topic>Erdheim-Chester Disease - pathology</topic><topic>Humans</topic><topic>Imaging</topic><topic>Leg - diagnostic imaging</topic><topic>Leg - pathology</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Middle Aged</topic><topic>Nuclear Medicine</topic><topic>Radiology</topic><topic>Radionuclide Imaging</topic><topic>Upper Extremity - diagnostic imaging</topic><topic>Upper Extremity - pathology</topic><topic>Whole Body Imaging</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Spyridonidis, Trifon J.</creatorcontrib><creatorcontrib>Giannakenas, Costas</creatorcontrib><creatorcontrib>Barla, Panagiota</creatorcontrib><creatorcontrib>Apostolopoulos, Dimitrios J.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Nursing & Allied Health Database</collection><collection>Neurosciences Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Technology Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>Research Library (Alumni Edition)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>Advanced Technologies & Aerospace Collection</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Technology Collection</collection><collection>Natural Science Collection</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>Research Library Prep</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>ProQuest Biological Science Collection</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Research Library</collection><collection>Biological Science Database</collection><collection>Research Library (Corporate)</collection><collection>Nursing & Allied Health Premium</collection><collection>Advanced Technologies & Aerospace Database</collection><collection>ProQuest Advanced Technologies & Aerospace Collection</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>ProQuest Central Basic</collection><collection>Biotechnology Research Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>MEDLINE - 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The skeleton is frequently involved in as many as 70–80% of all cases. In nearly half of the cases, there is an involvement of other organs such as the cardiovascular system, lung, kidneys, brain, and orbits. Extra-skeletal involvement is correlated with increased morbidity and mortality. In recent years, the disease is being described with increasing frequency although fewer than 200 cases have been identified worldwide. Besides its rarity, the disease has a characteristic almost pathognomonic bone scan appearance, which in some cases facilitates diagnosis of the syndrome. Bone scans also contribute to the qualitative assessment of skeletal involvement.</abstract><cop>Japan</cop><pub>Springer Japan</pub><pmid>18535884</pmid><doi>10.1007/s12149-007-0110-3</doi><tpages>4</tpages></addata></record> |
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| subjects | Bone and Bones - diagnostic imaging Bone and Bones - pathology Bone Diseases - diagnostic imaging Bone Diseases - pathology Case Report Diaphyses - diagnostic imaging Diaphyses - pathology Epiphyses - diagnostic imaging Epiphyses - pathology Erdheim-Chester Disease - diagnostic imaging Erdheim-Chester Disease - pathology Humans Imaging Leg - diagnostic imaging Leg - pathology Male Medicine Medicine & Public Health Middle Aged Nuclear Medicine Radiology Radionuclide Imaging Upper Extremity - diagnostic imaging Upper Extremity - pathology Whole Body Imaging |
| title | Erdheim-Chester disease: a rare syndrome with a characteristic bone scintigraphy pattern |
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