Non-amyloid monoclonal immunoglobulin deposition disease
Synonyms: Non-amyloid MIDD · Randall disease
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A rare, secondary glomerular disease characterized by proteinuria, dysproteinemias, nephrotic syndrome, and nodular glomerulopathy leading to renal failure, with or without extra-renal manifestations. The renal biopsy shows typical deposits of monoclonal immunoglobulins that do not show a fibrillar organization and are negative for Congo red staining. Associated signs and symptoms depend on the involvement of other organs, liver, heart, nerve fibers, gastrointestinal tract, or skin.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Non-amyloid monoclonal immunoglobulin deposition disease | Non-amyloid MIDD · Randall disease |
| fr | Maladie des dépôts d'immunoglobuline monoclonale non-amyloïde | MDIM non-amyloïde · MIDD non-amyloïde · Maladie de Randall |
| de | Nicht-amyloide monoklonale Immunglobulin-Ablagerungskrankheit | Immunoglobulin-Speicherkrankheit, monoklonale nichtamyloide · MIDD, nichtamyloide · Randall-Krankheit |
| es | Enfermedad por depósito no amiloides de inmunoglobulinas monoclonales | |
| it | Malattia da deposito di immunoglobuline monoclonali non amiloidi | MIDD non amiloide · Malattia da depositi d'immunoglobulina monoclonale non amiloide · Malattia di Randall |
| nl | Ziekte van niet-amyloïde depositie van monoklonaal immunoglobuline | Niet-amyloïde MIDD · Ziekte van Randall |
| pt | Doença de deposição de imunoglobulinas monoclonais | |
| pl | Nieamyloidowa choroba monoklonalnych depozytów immunoglobulinowych | Choroba Randalla · Nieamyloidowa MIDD |
| cs | Non-amyloidní onemocnění z depozice monoklonálních imunoglobulinů | Non-amyloidní MIDD · Randallova nemoc |
| uk | Неамілоїдна хвороба відкладення моноклональних імуноглобулінів | Неамілоїдна МІДД · Хвороба Рендела |
| zh | 非淀粉样单克隆免疫球蛋白沉积病 | 非淀粉样MIDD |
| tr | Amiloid olmayan monoklonal immünoglobulin birikimi hastalığı | Randall hastalığı · Amiloid olmayan MIDD |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D89.8 | NTBT | attributed | validated |
| MONDO | 0019463 | E | validated | |
| UMLS | C5394673 | 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:567558Non-genetic systemic disease with glomerulopathy as a major feature
rare renal diseases; rare transplant-related disorders - ORPHA:98282Plasma cell tumor
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/86861
- This record as JSON: /data/d/86861.json