Erdheim-Chester hastalığı

Hastalık uzun kemiklerde, akciğerde, plevrada, perikardiyumda, karaciğerde, dalakta, ince bağırsakta, retroperitoneal bölgede, nadiren merkezi sinir sisteminde görülür. Genelde intrakranial kitleler ekstra-aksiyel yerleşim gösterirler.

 İntra-aksiyel yerleşimli lezyonlar en sık hipotalamus ve serebellar bölgeleri tutar. Buna bağlı olarak psikonöroendokrin bozukluklar (diabetus insipitus) ve dizartri, dizmetri, nistagmus, ataksi gibi serebellar bulgular vardır. İntrakranial intraaksiyel yerleşimli ECH literatürde nadirdir. Yüksek dereceli glial tümörü taklit edebilen klinik ve radyolojik bulgularla ortaya çıkabilir. İntrakranial lezyonlarlada kesin tedavisi bilinmemekle beraber; literatürde kortikostreoid, kemoterapi, radyoterapi, interferon ve çıkarılabilecek kitleler için cerrahi önerilen tedavi seçenekleridir

Erdheim Chester

Erdheim Chester Disease
Synonyms of Erdheim Chester Disease
ECD
Lipoid Granulomatosis
Disorder Subdivisions


General Discussion
Erdheim-Chester disease (ECD) is a rare multisystem disorder of adulthood. It is characterized by excessive production and accumulation of histiocytes within multiple tissues and organs. Histiocytes are large phagocytic cells (macrophages) that normally play a role in responding to infection and injury. (A phagocytic cell is any scavenger cell that engulfs and destroys invading microorganisms or cellular debris.) In those with ECD, sites of involvement may include the long bones, skin, tissues behind the eyeballs, lungs, brain, pituitary gland, and/or additional tissues and organs. Associated symptoms and findings and disease course depend on the specific location and extent of such involvement. The specific underlying cause of ECD is unknown.
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Organizations related to Erdheim Chester Disease
ECD Global Alliance
 13065 Hwy 1146
DeRidder LA 70634
Phone #: 337-515-6987
800 #: N/A
e-mail: N/A
Home page: http://www.erdheim-chester.org
Histiocytosis Association of America
 332 North Broadway
Pitman NJ 08071
Phone #: 856-589-6606
800 #: 800-548-2758
e-mail: association@histio.org
Home page: http://www.histio.org
MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
 150 Custer Court
Green Bay WI 54301-1243
Phone #: 920-336-5333
800 #: 877-336-5333
e-mail: mums@netnet.net
Home page: http://www.netnet.net/mums/
NIH/National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse
 1 AMS Circle
Bethesda MD 20892-3675
Phone #: 301-495-4484
800 #: 877-226-4267
e-mail: NIAMSinfo@mail.nih.gov
Home page: http://www.niams.nih.gov/Health_Info
NIH/National Heart, Lung and Blood Institute Information Center
 P.O. Box 30105
Bethesda MD 20824-0105
Phone #: 301-592-8573
800 #: --
e-mail: nhlbiinfo@rover.nhlbi.nih.gov
Home page: N/A
National Institute of Neurological Disorders and Stroke (NINDS)
 31 Center Drive
Bethesda MD 20892-2540
Phone #: 301-496-5751
800 #: 800-352-9424
e-mail: braininfo@ninds.nih.gov
Home page: http://www.ninds.nih.gov/
http://www.hekimce.com/disease/erdheim-chester-disease

Erdheim –Chester Hastalığı

Erdheim –Chester Hastalığı, iskelet sistemi ve göz akciğer böbrekler hipofiz glandı gibi çeşitli organların ksantomatöz non-Langerhans hücreli histiyosit infiltrasyonu ile karakterize nadir bir hastalıktır. En sık kemik iliği tutulumu ile görülmektedir

Kaynak : http://www.elektronikposter.com/2013turkrad-eposter/index.html