ORPHA:91500
Tubulointerstitial nephritis and uveitis syndrome
Synonyms: Dobrin syndrome · TINU syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare renal tubular disease characterized by early-onset tubulointerstitial nephritis associated with anterior uveitis.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Tubulointerstitial nephritis and uveitis syndrome | Dobrin syndrome · TINU syndrome |
| fr | Néphrite tubulo-interstitielle et uvéite | Syndrome TINU · Syndrome de Dobrin |
| de | Tubulointerstitielle Nephritis und Uveitis-Syndrom | Dobrin-Syndrom · TINU-Syndrom |
| es | Síndrome de nefritis tubulointersticial y uveitis | Síndrome TINU · Síndrome de Dobrin |
| it | Sindrome da nefrite tubulointerstiziale e uveite | Sindrome TINU · Sindrome di Dobrin |
| nl | Tubulo-interstitiële nefritis en uveïtis-syndroom | Syndroom van Dobrin · TINU-syndroom |
| pt | Síndrome de nefrite túbulointersticial e uveíte | Síndrome Dobrin · Síndrome TINU |
| pl | Zespół kanalikowo-śródmiąższowego zapalenia nerek i zapalenia błony naczyniowej | Ostre zapalenie kanalikowo-śródmiąższowe nerek i zapalenie błony naczyniowej · Zespół TINU |
| cs | Syndrom tubulointersticiální nefritidy a uveitidy | Akutní tubulointersticiální nefritida s uveitidou · TINU syndrom · Tubulointersticiální nefritida s uveitidou |
| uk | Тубулоінтерстиціальний нефрит та синдром увеїту | Синдром Добріна · Синдром TINU |
| zh | 肾小管间质性肾炎和葡萄膜炎综合征 | 急性肾小管间质性肾炎和葡萄膜炎综合征 |
| tr | Tubulointerstisyel nefrit ve üveit sendromu | TINU sendromu · Dobrin sendromu · Akut tubulointerstisyel nefrit ve üveit sendromu |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 607665 | E | validated | |
| ICD-10 | N10 | NTBT | attributed | validated |
| ICD-11 | 9A96.1 | NTBT | index | validated |
| MONDO | 0011885 | E | validated | |
| MeSH | C536922 | E | validated | |
| UMLS | C1843273 | E | validated | |
| GARD | 9252 | E | validated | |
| MedDRA | 10069034 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:93603Rare renal tubular disease
rare renal diseases - ORPHA:280926Systemic diseases with anterior uveitis
rare ophthalmic disorders
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET_28709457[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
53 annotations · validated 2021-02-12| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0025466 | Beta 2-microglobulinuria | |
| HP:0007813 | Nongranulomatous uveitis | |
| HP:0032637 | Renal interstitial edema | |
| HP:0032589 | Renal lymphocytic tubulitis | |
| HP:0032590 | Renal neutrophilic tubulitis | |
| HP:0001970 | Tubulointerstitial nephritis | |
| Frequent (79-30%) | ||
| HP:0012122 | Anterior uveitis | |
| HP:0012213 | Decreased glomerular filtration rate | |
| HP:0003259 | Elevated circulating creatinine concentration | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | |
| HP:0002907 | Microscopic hematuria | |
| HP:0012595 | Mild proteinuria | |
| HP:0200026 | Ocular pain | |
| HP:0001085 | Papilledema | |
| HP:0012123 | Posterior uveitis | |
| HP:0025337 | Red eye | |
| HP:0100586 | Sterile pyuria | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0003355 | Aminoaciduria | |
| HP:0002039 | Anorexia | |
| HP:0031616 | Anterior chamber flare | |
| HP:0002829 | Arthralgia | |
| HP:0000518 | Cataract | |
| HP:0011506 | Choroidal neovascularization | |
| HP:0011505 | Cystoid macular edema | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0030157 | Flank pain | |
| HP:0004918 | Hyperchloremic metabolic acidosis | |
| HP:0003237 | Increased circulating IgG level | |
| HP:0010702 | Increased circulating immunoglobulin concentration | |
| HP:0012124 | Intermediate uveitis | |
| HP:0040049 | Macular edema | |
| HP:0003326 | Myalgia | |
| HP:0001895 | Normochromic anemia | |
| HP:0001897 | Normocytic anemia | |
| HP:0007906 | Ocular hypertension | |
| HP:0012121 | Panuveitis | |
| HP:0000613 | Photophobia | |
| HP:0011484 | Posterior synechiae of the anterior chamber | |
| HP:0031851 | Reduced hematocrit | |
| HP:0001994 | Renal Fanconi syndrome | |
| HP:0008682 | Renal tubular epithelial necrosis | |
| HP:0025188 | Retinal vasculitis | |
| HP:0100532 | Scleritis | |
| HP:0000988 | Skin rash | |
| HP:0000505 | Visual impairment | |
| HP:0007902 | Vitreous hemorrhage | |
| Very rare (<4-1%) | ||
| HP:0007703 | Abnormality of retinal pigmentation | |
| HP:0007777 | Chorioretinal scar | |
| HP:0011525 | Iris nevus | |
Run the differential on the 6 obligate / very frequent terms
Annotation source: 28806188[PMID]_31719109[PMID]_32463299[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/91500
- This record as JSON: /data/d/91500.json