Application Version: 4.0.6.9
Content Version: 2024.01.20
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Code System Concept
Code System Concept Code | 241955009 |
Code System Concept Name | Acquired C1 esterase inhibitor deficiency (disorder) |
Code System Preferred Concept Name | Acquired C1 esterase inhibitor deficiency (disorder) |
Concept Status | Published |
Concept Status Date | 03/01/2024 |
Code System Name | SNOMED-CT |
Concept Details
Code System Code | PH_SNOMED-CT |
Code System OID | 2.16.840.1.113883.6.96 |
HL7 Table 0396 Code | SCT |
Superseded By Concept | |
SDO Concept Status | Active |
SDO Concept Status Date | 01/31/2002 |
SDO Date Created | 01/31/2002 |
SDO Date Revised | 01/31/2002 |
Description | A rare non-histaminic angioedema characterized by potentially life-threatening episodes of edema of subcutaneous and/or mucosal tissues without urticaria, caused by excessive consumption of C1 esterase inhibitor (C1-INH) in the context of lymphoproliferative or autoimmune diseases. Patients typically present in the fourth decade of life or later and without a family history of angioedema. Clinical manifestation includes nonpitting edema of the skin predominantly involving the face, but also the limbs or genitals, as well as abdominal pain due to involvement of the gastrointestinal mucosa and severe edema of the upper airway and oral mucosa. Laboratory examination shows low C1-INH activity and low C3, C4, and C1q levels. Autoantibodies to C1-INH are frequently detectable. |
Sequence |
Extended Properties
No extended properties.
Concept Alternate Designations
SDO Designation ID | Concept Designation Description | Status | Designation Type | PHIN Preferred Term Flag | Code Flag |
---|---|---|---|---|---|
362175013 | Acquired C1 esterase inhibitor deficiency | Active | Synonym | false | false |
3915188019 | Acquired angioedema | Active | Synonym | false | false |
3915189010 | Acquired angio-oedema | Active | Synonym | false | false |
5034960018 | Acquired angioedema with C1Inh (C1 esterase inhibitor) deficiency | Active | Synonym | false | false |
5034961019 | Acquired angioneurotic edema with C1Inh (C1 esterase inhibitor) deficiency | Active | Synonym | false | false |
5034963016 | Acquired angioneurotic oedema with C1Inh (C1 esterase inhibitor) deficiency | Active | Synonym | false | false |
5034964010 | Acquired angioneurotic edema with C1 inhibitor deficiency | Active | Synonym | false | false |
5034967015 | Acquired angioneurotic oedema with C1 inhibitor deficiency | Active | Synonym | false | false |
Parent Concepts
No parent concepts present.
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Other Relationships
No other relationships present.
|
Child Concepts
No child concepts present.
|
Application Version: 4.0.6.9
Content Version: 2024.01.20
Release Notes [PDF285KB]
User Guide [PDF6.08MB]
Code System Update Calendar [PDF161KB]
Contact Us
Subscribe
Related Links
- PHIN Vocabulary Services
- Quick Links / Mapping Tools
- External Standards Resources
- Message Guides
- Developer's Guide
Downloads
- Page last reviewed:December 5, 2018
- Page last updated:December 5, 2018
- Content source: