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| has gloss | (noun) a congenital disease affecting T cells that can result from a mutation in any one of several different genes; children with it are susceptible to infectious disease; if untreated it is lethal within the first year or two of life severe combined immunodeficiency, SCID, severe combined immunodeficiency disease |
| has gloss | eng: Severe combined immunodeficiency (SCID), or Boy in the Bubble Syndrome, (also known as "Alymphocytosis," "Glanzmann–Riniker syndrome," "Severe mixed immunodeficiency syndrome," and "Thymic alymphoplasia" ) is a genetic disorder in which both "arms" (B cells and T cells) of the adaptive immune system are crippled, due to a defect in one of several possible genes. SCID is a severe form of heritable immunodeficiency. It is also known as the "bubble boy" disease because its victims are extremely vulnerable to infectious diseases and some of them, such as David Vetter, became famous of living in sterile environment. |
| lexicalization | eng: SCID |
| lexicalization | eng: Severe combined immuno deficiency |
| lexicalization | eng: severe combined immunodeficiency disease |
| lexicalization | eng: Severe Combined Immunodeficiency |
| subclass of | (noun) immunological disorder in which some part of the body's immune system is inadequate and resistance to infectious diseases is reduced immunodeficiency |
| subclass of | (noun) an inherited disease controlled by a single pair of genes monogenic disease, monogenic disorder |
| has subclass | (noun) SCID resulting from mutation of a gene that codes for adenosine deaminase ADA-SCID |
| has subclass | (noun) SCID in male children resulting from mutation of a gene that codes for a protein on the surface of T cells that allows them to develop a growth factor receptor X-linked SCID, X-SCID |
| Meaning | |
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| Catalan | |
| lexicalization | cat: immunodeficiència combinada greu |
| German | |
| has gloss | deu: SCID (engl. severe combined immunodeficiency; schwerer kombinierter Immundefekt) ist eine Sammelbezeichnung für Krankheiten oder ein Syndrom, die als Gemeinsamkeit eine angeborene schwere Störung des Immunsystems aufweisen. Typisch ist für alle SCID-Formen eine Fehlfunktion oder ein Mangel an T-Lymphozyten. Damit ist eine Störung der zellulären Immunantwort bei allen SCID-Formen bedingt. Je nach SCID-Form ist auch die Funktionen oder Anzahl von B-Lymphozyten und NK-Zellen fehlerhaft. Dementsprechend kann auch die humorale Immunität (Antikörper-gestützte Abwehr) beeinträchtigt sein. Ursache für die einzelnen SCID-Formen sind angeborene Defekte oder Störungen von Genen. Die Erkrankungen manifestieren sich durch wiederholte schwere Infektionen mit "ungewöhnlichen" Erregern und Entwicklungsverzögerung bereits im Säuglings- und Kleinkindesalter. |
| lexicalization | deu: Severe Combined Immunodeficiency |
| Galician | |
| lexicalization | glg: SCID |
| Indonesian | |
| has gloss | ind: Severe combined immunodeficiency atau defisiensi imunitas kombinasi adalah kelainan genetik yang menyebabkan bagian utama dari sistem kekebalan tiruan (Sel B dan Sel T) lumpuh, akibat berbagai kemungkinan yang disebabkan gen. Pasien dengan penyakit ini mengalami kekurangan kekebalan. Penyakit ini disebut juga dengan "bocah gelembung" karena pasien akan sangat rentan terhadap penyakit infeksi. |
| lexicalization | ind: Severe combined immunodeficiency |
| Korean | |
| has gloss | kor: 중증합병면역결핍증 또는 SCID(Severe combined immunodeficiency)는 유전자 이상으로 면역 체계의 B 세포와 T 세포가 기형이 되는 증후군으로, 심한 유전성 면역결핍증이다. 이 증후군이 있는 환자는 감염성 질환에 취약하기 때문에 치료되지 않는다면 완벽하게 소독된 환경에서 살아야 한다. 중증합병면역결핍증은 80000 분의 1 확률로 일어나며, 그렇게 태어난 아이는 반복되는 감염에 의해 1년 이내에 사망한다. |
| lexicalization | kor: 중증합병면역결핍증 |
| Dutch | |
| has gloss | nld: SCID (Severe Combined Immune Deficiency syndrome), ook wel Boy in the Bubble Syndroom genoemd, is de verzamelnaam voor een heterogene groep erfelijke, aangeboren afwijkingen van het immuunsysteem. Kinderen geboren met SCID zijn ernstig immuungecompromitteerd, doordat zowel de humorale (B-cel gemedieerde) als cellulaire (T-cel gemedieerde) immuniteit ernstig gestoord is. Het wordt ook wel Boy in the Bubble Syndroom genoemd omdat de patiëntjes extreem vatbaar zijn voor infectie ziekten, waardoor ze in een steriele luchtbel moeten leven. Het meest bekende patiëntje met SCID is het jongetje David Vetter. |
| lexicalization | nld: SCID |
| Polish | |
| has gloss | pol: Zespół SCID (ang. Severe Combined Immuno Deficiency) - ciężki złożony niedobór immunologiczny - to choroba dziedziczna, o sposobie dziedziczenia autosomalnym recesywnym spowodowany mutacją w obrębie genów RAG-1 lub RAG-2 (tzw. agammoglobulinemia typu szwajcarskiego) bądź sprzężonym z chromosomem X, w którym dochodzi do nieprawidłowego różnicowania tymocytów i braku limfocytów T i NK. |
| lexicalization | pol: Zespół SCID |
| Swedish | |
| has gloss | swe: Svår kombinerad immunbrist, på engelska Severe Combined Immunodeficiency (SCID) är en mycket allvarlig immunbristsjukdom där individen har få eller helt saknar lymfocyter (B-celler och T-celler). Brist på dessa lymfocyter gör att individen i fråga helt saknar ett adaptivt immunförsvar, och därmed blir ytterst känslig för alla typer av infektioner. |
| lexicalization | swe: Svår kombinerad immunbrist |
| Links | |
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| similar | e/Severe combined immunodeficiency (non-human) |
| similar | e/Severe combined immunodeficiency |
| Media | |
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| media:img | Lymphocyte GL.jpg |
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