{"orpha":71273,"name":"Renal nutcracker syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Left renal vein entrapment syndrome","RNS"],"definition":"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.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-71273/","orphanet_url":"https://www.orpha.net/en/disease/detail/71273","names":{"fr":{"name":"Syndrome de la pince mésentérique","synonyms":["Pince aorto-mésentérique"],"definition":"Syndrome rénal rare en rapport avec une compression de la veine rénale gauche (VRG) entre l'artère mésentérique supérieure (AMS) et l'aorte abdominale. Cela conduit à une augmentation de la pression dans la lumière veineuse, à l'apparition de varices au niveau du hile rénal et d'hématuries et, au niveau tissulaire, à une déchirure des veines à paroi fine au niveau des fornix."},"de":{"name":"Renales Nussknackersyndrom","synonyms":["Kompressionssyndrom der linken Nierenvene","RNS"],"definition":"Eine seltene, syndromale Nierenerkrankung, die durch die Einklemmung der linken Nierenvene (LRV) zwischen der A. mesenterica superior (SMA) und der Bauchaorta gekennzeichnet ist und zu erhöhtem Luminaldruck, Nierenhilusvarizen, Hämaturie und, auf mikroskopischer Ebene, zur Ruptur dünnwandiger Venen in das Sammelsystem des Nierengewölbes führt."},"es":{"name":"Síndrome renal del cascanueces","definition":"Es una enfermedad renal sindrómica y poco frecuente caracterizada por el atrapamiento de la vena renal izquierda (VRI) entre la arteria mesentérica superior (AMS) y la aorta abdominal, resultando en un incremento de la presión luminal, varices a nivel del hilio renal, hematuria y, a nivel microscópico, ruptura de la delgada pared venosa al sistema colector en el fórnix renal."},"it":{"name":"Sindrome renale dello schiaccianoci","definition":"La sindrome renale dello schiaccianoci (NCS) è una malattia renale sindromica rara, caratterizzata dall'intrappolamento della vena renale sinistra (VRS) tra l'arteria mesenterica superiore (AMS) e l'aorta addominale, che causa un aumento della pressione nel lume, varici dell'ilo renale, ematuria e, a livello microscopico, rottura delle vene con pareti sottili nel sistema collettore nei fornici renali."},"nl":{"name":"Renaal notenkrakersyndroom","synonyms":["Ingeklemde linkernieradersyndroom","RNS"],"definition":"Een zeldzame, syndromale nierziekte gekarakteriseerd door beknelling van de vena renalis sinistra (linker nierader; LRV) tussen de arteria mesenterica superior (AMS) en de aorta abdominalis (buikaorta), wat leidt tot verhoogde luminale druk, spataders ter hoogte van de nierhilus, hematurie en, op microscopisch niveau, ruptuur van dunwandige aders in het verzamelsysteem in de renale fornices."},"pt":{"name":"Síndrome quebra-nozes renal","synonyms":["Encarceramento da veia renal esquerda, síndrome","RNS","Síndrome encarceramento da veia renal esquerda"]},"pl":{"name":"Zespół dziadka do orzechów","synonyms":["RNS","Zespół uwięźnięcia lewej żyły nerkowej"]},"cs":{"name":"Renální louskáčkový syndrom","synonyms":["Syndrom útlaku levé renální žíly","RNS"]},"uk":{"name":"Нирковий синдром Лускунчика","synonyms":["Синдром защемлення лівої ниркової вени","RNS"],"definition":"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."},"zh":{"name":"肾胡桃夹综合征","synonyms":["左肾静脉受压综合征"]},"tr":{"name":"Böbrek fındıkkıran sendromu","synonyms":["RNS","Sol renal entrapman sendromu"]}},"xrefs":[{"source":"ICD-10","code":"Q27.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA90.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0019105","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D059228","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3178770","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"11971","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93626,"name":"Rare renal disease"},"parents":[{"orpha":165711,"name":"Rare abdominal surgical disease","classifications":[182]},{"orpha":93547,"name":"Syndromic renal or urinary tract malformation","classifications":[147,188,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-06-12","source":"34815965[PMID]_38038139[PMID]_28356209[PMID]_32644615[PMID]","terms":[{"hpo":"HP:0000716","label":"Depression","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011934","label":"Dilatation of mesenteric artery","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030157","label":"Flank pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000790","label":"Hematuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003419","label":"Low back pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000093","label":"Proteinuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001920","label":"Renal artery stenosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012871","label":"Varicocele","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002619","label":"Varicose veins","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001903","label":"Anemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100518","label":"Dysuria","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002907","label":"Microscopic hematuria","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0034267","label":"Pelvic pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100677","label":"Vulval varicose vein","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012332","label":"Abnormal autonomic nervous system physiology","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100607","label":"Dysmenorrhea","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0030016","label":"Dyspareunia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000789","label":"Infertility","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001278","label":"Orthostatic hypotension","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001279","label":"Syncope","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001649","label":"Tachycardia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Very rare (<4-1%)","criteria":null}]}}