Acquired monoclonal Ig light chain-associated Fanconi syndrome
Synonyms: Acquired Fanconi syndrome secondary to monoclonal gammopathy · Acquired monoclonal immunoglobulin light chain-associated Fanconi syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare monoclonal gammopathy characterized by renal proximal tubule dysfunction secondary to monoclonal kappa light chain deposits in proximal tubular cells. Clinical presentation is with variable chronic kidney disease, low molecular weight proteinuria, aminoaciduria, hyperphosphaturia, uricosuria, bicarbonaturia, and non-diabetic glycosuria. Renal phosphate and urate wasting may cause hypophosphatemia and hypouricaemia.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acquired monoclonal Ig light chain-associated Fanconi syndrome | Acquired Fanconi syndrome secondary to monoclonal gammopathy · Acquired monoclonal immunoglobulin light chain-associated Fanconi syndrome |
| fr | Syndrome de Fanconi acquis associé à la chaine légère des Ig monoclonales | Syndrome de Fanconi acquis associé à la chaine légère des immunoglobulines monoclonales · Syndrome de Fanconi acquis secondaire à une gammapathie monoclonale |
| de | Erworbenes monoklonales lg-Leichtketten-assoziiertes Fanconi-Syndrom | Sekundäres Fanconi-Syndrom als Folge monoklonaler Gammopathie |
| es | Síndrome de Fanconi secundario a gammapatía monoclonal de cadenas ligeras | Síndrome de Fanconi adquirido asociado a Ig monoclonal de cadenas ligeras · Síndrome de Fanconi adquirido asociado a inmunoglobulina monoclonal de cadena ligera · Síndrome de Fanconi adquirido secundario a una gammapatía monoclonal |
| it | Sindrome di Fanconi associata alla acquisizione di catene leggere Ig monoclonali | Sindrome di Fanconi associata al mieloma · Sindrome di Fanconi associata alle catene leggere kappa delle Ig monoclonali |
| nl | Verworven syndroom van Fanconi geassocieerd met monoklonale Ig lichte ketens | Verworven syndroom van Fanconi geassocieerd met monoklonale immunoglobuline lichte ketens · Verworven syndroom van Fanconi secundair aan monoklonale gammopathie |
| pt | Síndrome de Fanconi | |
| pl | Zespół Fanconiego związany z monoklonalnymi lekkimi łańcuchami Ig | Zespół Fanconiego ze szpiczakiem · Zespół Fanconiego związany z lekkimi łańcuchami kappa |
| cs | Získaný Fanconiho syndrom asociovaný s lehkými řetězci monoklonálních imunoglobulinů | Získaný Fanconiho syndrom způsobený monoklonální gamapatií |
| uk | Набутий синдром Фанконі, асоційований з легким ланцюгом моноклонального Ig | Набутий синдром Фанконі, вторинний до моноклональної гамопатії · Набутий моноклональний імуноглобулін легкого ланцюга, асоційований з синдромом Фанконі |
| zh | 获得性单克隆免疫球蛋白轻链相关性范科尼综合征 | 获得性单克隆免疫球蛋白轻链相关的Fanconi综合征 |
| tr | Edinsel monoklonal Ig hafif zincir-ilişkili Fanconi sendromu | Monoklonal gammopatiye sekonder edinsel Fanconi sendromu · Edinsel monoklonal immünoglobulin hafif zincir ile ilişkili Fanconi sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E72.0 | NTBT | attributed | validated |
| ICD-11 | GB90.42 | NTBT | attributed | validated |
| MONDO | 0019604 | E | validated | |
| UMLS | C4510369 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:506213Rare disorder potentially indicated for kidney transplant
rare transplant-related disorders - ORPHA:93603Rare renal tubular disease
rare renal diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 100 cases | Worldwide | Case(s) | Validated | [EXPERT] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_[EXPERT] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/91136
- This record as JSON: /data/d/91136.json