ICD-11 编码查询_国际疾病分类第 11 版中文版_在线疾病编码检索工具
4A01.10

严重联合免疫缺陷

Severe combined immunodeficiencies

类目第 04 章 免疫系统疾病
上级条目
4A01.1 联合免疫缺陷
层级
5 级

定义

严重联合免疫缺陷（SCID）是一组罕见的单基因原发性免疫缺陷疾病，其特征是缺乏功能性外周血T淋巴细胞，导致早期严重呼吸道感染和发育不良。

Severe combined immunodeficiency (SCID) comprises a group of rare monogenic primary immunodeficiency disorders characterised by a lack of functional peripheral T lymphocytes resulting in early-onset severe respiratory infections and failure to thrive.

索引词 可用于检索该条目的同义词与别名

  • 严重联合免疫缺陷
  • SCID[严重联合免疫缺陷]
  • 重症联合免疫缺陷
  • 严重联合免疫缺陷病 [possible translation]
  • 严重联合免疫缺陷病
  • 严重联合免疫缺陷伴网状组织发育不全
  • SCID[严重联合免疫缺陷]伴网状组织发育不全
  • 广泛性造血发育不全
  • 严重联合免疫缺陷伴白细胞减少
  • 先天性白细胞减少症
  • 网状组织发育不全
  • De Vaal病
  • 严重联合免疫缺陷伴T细胞和B细胞数量降低
  • SCID[严重免疫缺陷]伴T细胞和B细胞数量降低
  • T-B-严重联合免疫缺陷
  • RAG1缺陷引起的严重联合免疫缺陷T-B-
  • RAG1[重组激活基因1]缺陷引起的SCID[严重联合免疫缺陷]T-B-
  • 重组激活基因1缺陷引起的严重联合免疫缺陷T-B-
  • 重组活化基因1缺陷引起的严重联合免疫缺陷T-B-
  • RAG2缺陷引起的严重联合免疫缺陷T-B-
  • RAG2[重组激活基因2]缺陷引起的SCID[严重联合免疫缺陷]T-B-
  • 重组激活基因2缺陷引起的严重联合免疫缺陷T-B-
  • 重组活化基因2缺陷引起的严重联合免疫缺陷T-B-
  • 严重联合免疫缺陷伴嗜酸性粒细胞增多症
  • SCID[严重联合免疫缺陷]伴嗜酸性粒细胞增多症
  • Omenn综合征
  • DCLRE1C[DNA交联修复1C]缺乏引起的SCID[严重联合免疫缺陷]
  • SCID[严重联合免疫缺陷]，Athabaskan型
  • DNA交联修复蛋白C1缺乏引起的SCID[严重联合免疫缺陷]
  • Artemis蛋白缺乏引起的SCID[严重联合免疫缺陷]
  • 严重联合免疫缺陷[SCID]，淋巴细胞缺乏型
  • 无丙种球蛋白血症，淋巴细胞缺乏型
  • LCK缺乏引起的严重联合免疫缺陷
  • LCK[淋巴细胞特异性酪氨酸蛋白激酶]缺乏引起的严重联合免疫缺陷[SCID]
  • DNA-PKcs缺乏引起的SCID[严重联合免疫缺陷]
  • DNA-PKcs[DNA依赖性蛋白激酶催化亚基]缺乏引起的SCID[严重联合免疫缺陷]
  • 腺苷脱氨酶缺乏引起的严重联合免疫缺陷T-B-
  • ADA[腺苷脱氨酶]缺乏
  • ADA[腺苷氨基水解酶]缺乏
  • 腺苷脱氨酶缺乏引起的SCID[严重联合免疫缺陷]T-B-
  • 腺苷脱氨酶缺乏
  • 短肢型骨发育不全伴严重联合免疫缺陷
  • 淋巴细胞减少性无丙种球蛋白血症-短肢侏儒综合征
  • SCID[严重联合免疫缺陷]伴短肢侏儒
  • 短肢免疫缺陷
  • 免疫缺陷伴短肢身材
  • 严重联合免疫缺陷伴短肢侏儒症
  • 软骨发育不全或瑞士型无丙种球蛋白血症
  • 软骨发育不全-严重联合免疫缺陷
  • 免疫缺陷伴短肢侏儒症
  • 严重联合免疫缺陷[SCID]伴B细胞数量减少或正常
  • SCID[严重联合免疫缺陷]伴B细胞数量减少或正常
  • T-B+严重联合免疫缺陷
  • γ链缺陷引起的严重联合免疫缺陷T-B+
  • γ链缺陷引起的SCID[严重联合免疫缺陷]T-B+
  • 严重联合免疫缺陷T-B+，X连锁
  • JAK3缺陷引起的T-B+严重联合免疫缺陷病
  • JAK3[Janus激酶3]缺陷引起的SCID[严重联合免疫缺陷]T-B+
  • IL-7Rα缺陷引起的严重联合免疫缺陷T-B+
  • IL-7Rα[白细胞介素7受体α]缺陷引起的SCID[严重联合免疫缺陷]T-B+
  • CD45缺陷引起的严重联合免疫缺陷T-B+
  • CD45缺陷引起的SCID[严重联合免疫缺陷]T-B+
  • CD3δ、CD3ε或者CD3ζ引起的严重联合免疫缺陷T-B+
  • CD3δ/CD3ε/CD3ζ/引起的SCID[严重联合免疫缺陷]T-B+
  • CORO1A缺陷引起的严重联合免疫缺陷
  • Coronin-1A缺陷引起的SCID[严重联合免疫缺陷]
英文词条（62）
  • Severe combined immunodeficiencies
  • SCID - [Severe combined immunodeficiencies]
  • severe combined immunodeficiency disease
  • Severe combined immunodeficiency with reticular dysgenesis
  • Congenital aleukocytosis
  • De Vaal disease
  • Reticular dysgenesis
  • SCID - [Severe combined immunodeficiencies] with reticular dysgenesis
  • Severe combined immunodeficiency with leukopenia
  • generalised haematopoietic hypoplasia
  • Severe combined immunodeficiency with low T- and B-cell numbers
  • SCID - [Severe combined immunodeficiencies] with low t- and b-cell numbers
  • T- B- severe combined immunodeficiency
  • Severe combined immunodeficiency T- B- due to RAG1 deficiency
  • SCID - [severe combined immunodeficiency] T- B- due to RAG1 - [recombination-activating gene 1] deficiency
  • Severe combined immunodeficiency T- B- due to recombination activating gene 1deficiency
  • Severe combined immunodeficiency T- B- due to RAG2 deficiency
  • SCID - [severe combined immunodeficiency] T- B- due to RAG2 - [recombination-activating gene 2] deficiency
  • Severe combined immunodeficiency T- B- due to recombination activating gene 2 deficiency
  • Severe combined immunodeficiency with hypereosinophilia
  • Omenn syndrome
  • SCID - [severe combined immunodeficiency] with hypereosinophilia
  • SCID - [Severe combined immunodeficiency] due to DCLRE1C- [DNA cross-link repair 1C] deficiency
  • Agammaglobulinaemia, alymphocytotic type
  • SCID - [Severe combined immunodeficiency] due to Artemis protein deficiency
  • SCID - [Severe combined immunodeficiency] due to DNA cross-link repair protein 1C deficiency
  • SCID - [Severe combined immunodeficiency], Athabaskan type
  • Severe combined immunodeficiency [SCID], alymphocytotic type
  • Severe combined immunodeficiency due to LCK deficiency
  • SCID - [severe combined immunodeficiency due to LCK - [lymphocyte-specific protein tyrosine kinase] deficiency
  • SCID - [Severe combined immunodeficiency] due to DNA-PKcs deficiency
  • SCID - [Severe combined immunodeficiency] due to DNA-PKcs - [DNA-dependent protein kinase, catalytic subunit] deficiency
  • Severe combined immunodeficiency T- B- due to adenosine deaminase deficiency
  • ADA - [adenosine aminohydrolase] deficiency
  • ADA - [adenosine deaminase] deficiency
  • Adenosine deaminase deficiency
  • SCID - [severe combined immunodeficiency] T- B- due to adenosine deaminase deficiency
  • Short-limb skeletal dysplasia with severe combined immunodeficiency
  • Achondroplasia - severe combined immunodeficiency
  • Achondroplasia or Swiss-type agammaglobulinaemia
  • Immunodeficiency with short limb dwarfism
  • SCID - [severe combined immunodeficiency] with short-limbed dwarfism
  • immunodeficiency with short-limbed stature
  • lymphopenic agammaglobulinaemia - short-limbed dwarfism syndrome
  • severe combined immunodeficiency with short-limbed dwarfism
  • short-limbed immunodeficiency
  • Severe combined immunodeficiency with low or normal B-cell numbers
  • SCID - [severe combined immunodeficiency] with low or normal b-cell numbers
  • T- B+ severe combined immunodeficiency
  • Severe combined immunodeficiency T- B+ due to gamma chain deficiency
  • SCID - [severe combined immunodeficiency] T- B+ due to gamma chain deficiency
  • Severe combined immunodeficiency T- B+, X-linked
  • Severe combined immunodeficiency T- B+ due to JAK3 deficiency
  • SCID - [severe combined immunodeficiency] T- B+ due to JAK3- [Janus kinase 3] deficiency
  • Severe combined immunodeficiency T- B+ due to IL-7Ralpha deficiency
  • SCID - [severe combined immunodeficiency] T- B+ due to IL-7Ralpha - [Interleukin 7 receptor alpha] deficiency
  • Severe combined immunodeficiency T-B+ due to CD45 deficiency
  • SCID - [severe combined immunodeficiency] T- B+ due to CD45 deficiency
  • Severe combined immunodeficiency T- B+ due to CD3delta, CD3epsilon or CD3zeta
  • SCID - [severe combined immunodeficiency] T- B+ due to CD3delta/CD3epsilon/CD3zeta
  • Severe combined immunodeficiency due to CORO1A deficiency
  • SCID - [severe combined immunodeficiency] due to coronin-1A deficiency

后组配轴 Postcoordination

该条目未定义后组配轴，直接使用上面的编码即可。后组配是在主干编码之后追加侧别、具体解剖部位、严重程度、病因等细节的可选组合方式，不是必填项。