Renal nutcracker syndrome
Synonyms: Left renal vein entrapment syndrome · RNS
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Adult
- Status
- active
A rare, syndromic renal disease characterized by the entrapment of left renal vein (LRV) between the superior mesenteric artery (SMA) and the abdominal aorta, resulting in increased luminal pressure, renal hilar varices, hematuria and, at the microscopic level, rupture of thin-walled veins into the collecting system in renal fornices.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Renal nutcracker syndrome | Left renal vein entrapment syndrome · RNS |
| fr | Syndrome de la pince mésentérique | Pince aorto-mésentérique |
| de | Renales Nussknackersyndrom | Kompressionssyndrom der linken Nierenvene · RNS |
| es | Síndrome renal del cascanueces | |
| it | Sindrome renale dello schiaccianoci | |
| nl | Renaal notenkrakersyndroom | Ingeklemde linkernieradersyndroom · RNS |
| pt | Síndrome quebra-nozes renal | Encarceramento da veia renal esquerda, síndrome · RNS · Síndrome encarceramento da veia renal esquerda |
| pl | Zespół dziadka do orzechów | RNS · Zespół uwięźnięcia lewej żyły nerkowej |
| cs | Renální louskáčkový syndrom | Syndrom útlaku levé renální žíly · RNS |
| uk | Нирковий синдром Лускунчика | Синдром защемлення лівої ниркової вени · RNS |
| zh | 肾胡桃夹综合征 | 左肾静脉受压综合征 |
| tr | Böbrek fındıkkıran sendromu | RNS · Sol renal entrapman sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q27.8 | NTBT | attributed | validated |
| ICD-11 | LA90.Y | NTBT | attributed | validated |
| MONDO | 0019105 | E | validated | |
| MeSH | D059228 | E | validated | |
| UMLS | C3178770 | E | validated | |
| GARD | 11971 | 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:165711Rare abdominal surgical disease
rare abdominal surgical diseases - ORPHA:93547Syndromic renal or urinary tract malformation
rare developmental anomalies during embryogenesis; rare renal diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
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
25 annotations · validated 2024-06-12| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0000716 | Depression | |
| HP:0011934 | Dilatation of mesenteric artery | |
| HP:0030157 | Flank pain | |
| HP:0000790 | Hematuria | |
| HP:0003419 | Low back pain | |
| HP:0000093 | Proteinuria | |
| HP:0001920 | Renal artery stenosis | |
| HP:0012871 | Varicocele | |
| HP:0002619 | Varicose veins | |
| Occasional (29-5%) | ||
| HP:0001903 | Anemia | |
| HP:0100518 | Dysuria | |
| HP:0012378 | Fatigue | |
| HP:0002907 | Microscopic hematuria | |
| HP:0034267 | Pelvic pain | |
| HP:0100677 | Vulval varicose vein | |
| Very rare (<4-1%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0012332 | Abnormal autonomic nervous system physiology | |
| HP:0100607 | Dysmenorrhea | |
| HP:0030016 | Dyspareunia | |
| HP:0000789 | Infertility | |
| HP:0002018 | Nausea | |
| HP:0001278 | Orthostatic hypotension | |
| HP:0001279 | Syncope | |
| HP:0001649 | Tachycardia | |
| HP:0001824 | Weight loss | |
Annotation source: 34815965[PMID]_38038139[PMID]_28356209[PMID]_32644615[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/71273
- This record as JSON: /data/d/71273.json