ORPHA:314701
Primary systemic amyloidosis
Synonyms: Systemic AL amyloidosis
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Status
- active
This disease is described under AL amyloidosis
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary systemic amyloidosis | Systemic AL amyloidosis |
| fr | Amylose primitive systémique | Amylose AL systémique |
| de | Amyloidose, primäre systemische | AL-Amyloidose, systemische |
| es | Amiloidosis sistémica primaria | Amiloidosis AL sistémica |
| it | Amiloidosi sistemica primitiva | Amiloidosi AL sistemica |
| nl | Primaire systemische amyloïdose | Systemische AL-amyloïdose |
| pt | Amiloidose sistémica primária | Amiloidose AL sistémica · Amiloidose imunoglobulinica sistémica |
| pl | Pierwotna amyloidoza układowa | Układowa amyloidoza AL · Układowa amyloidoza immunoglobulinowa |
| cs | Primární systémová amyloidóza | Systémová AL amyloidóza |
| uk | Первинний системний амілоїдоз | Системний AL-амілоїдоз |
| zh | 原发性系统性淀粉样变 | 系统性AL淀粉样变 |
| tr | Primer sistemik amiloidoz | Sistemik AL amiloidoz |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 254500 | NTBT | validated | |
| ICD-10 | E85.0 | BTNT | specific | validated |
| ICD-10 | E85.1 | BTNT | specific | validated |
| ICD-10 | E85.2 | BTNT | specific | validated |
| ICD-10 | E85.3 | BTNT | specific | validated |
| MONDO | 0017816 | E | validated | |
| UMLS | C0281479 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:85443AL amyloidosis
rare cardiac diseases; rare neurological diseases; rare renal diseases; rare hematological diseases; rare systemic and rheumatological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
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Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 30 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2003[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
6
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/314701
- This record as JSON: /data/d/314701.json