{"orpha":293208,"name":"Celiac artery compression syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Dunbar syndrome","MALS","Median arcuate ligament syndrome"],"definition":"A rare disease caused by compression of the celiac axis by an abnormally shaped arcuate ligament (the part of the diaphragm in which both pillars join in the midline around the aorta). Patients have recurrent abdominal pain, anorexia and weight loss. The pain is epigastric, and diarrhea or constipation may be present as well. Onset of pain will usually, although not always, be after food intake, and may be associated with nausea and emesis. Other symptoms may include lassitude, exercise intolerance and vomiting. Occasionally, a patient may show an abdominal murmur upon auscultation.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-293208/","orphanet_url":"https://www.orpha.net/en/disease/detail/293208","names":{"fr":{"name":"Syndrome de compression du tronc coeliaque","synonyms":["Syndrome de Dunbar","Syndrome du ligament arqué médian"],"definition":"Maladie rare causée par la compression du tronc coeliaque par un ligament arqué de forme anormale (la partie du diaphragme dans laquelle les deux piliers se rejoignent sur la ligne médiane autour de l'aorte). Les patients manifestent des douleurs abdominales récurrentes, une anorexie et une perte de poids. La douleur est épigastrique, la diarrhée ou la constipation peuvent également être présentes. La douleur se présente généralement, mais pas toujours, après l'ingestion d'aliments et peut être associée à des nausées et à des vomissements. Les autres signes cliniques peuvent être la lassitude, l'intolérance à l'effort et les vomissements. Parfois, un patient peut manifester un souffle abdominal à l'examen médical."},"de":{"name":"Arteria-coeliaca-Kompressions-Syndrom","synonyms":["Dunbar-Syndrom","Ligamentum arcuatum medianum-Syndrom","MALS"],"definition":"Eine seltene Störung, die durch eine Kompression der Zöliakie-Achse durch ein abnorm geformtes Ligamentum arcuatum (der Teil des Zwerchfells, in dem die beiden Säulen in der Mittellinie um die Aorta zusammenlaufen) verursacht wird. Die Patienten leiden unter wiederkehrenden Bauchschmerzen, Anorexie und Gewichtsverlust. Die Schmerzen sind epigastrisch, und es kann auch zu Durchfall oder Verstopfung kommen. Die Schmerzen treten in der Regel, wenn auch nicht immer, nach der Nahrungsaufnahme auf und können mit Übelkeit und Erbrechen einhergehen. Weitere Symptome können Abgeschlagenheit, Bewegungsunverträglichkeit und Erbrechen sein. Gelegentlich kann der Patient bei der Auskultation ein abdominales Herzgeräusch wahrnehmen."},"es":{"name":"Síndrome de compresión de la arteria celíaca","synonyms":["MALS","Síndrome de Dunbar","Síndrome del ligamento arcuato medio"],"definition":"Es una enfermedad poco frecuente causada por la compresión del eje celíaco por el ligamento arcuato de forma anómala (parte del diafragma en la que ambos pilares se unen en la línea media alrededor de la aorta). Los pacientes presentan dolor abdominal recurrente, anorexia y pérdida de peso. El dolor es epigástrico y también puede haber diarrea o estreñimiento. El inicio del dolor suele ser, aunque no siempre, posterior a la ingestión del alimento y puede estar asociado a náusea y emesis. Otros síntomas pueden incluir cansancio, intolerancia al ejercicio y vómitos. En ocasiones, los pacientes pueden mostrar un soplo abdominal en la auscultación."},"it":{"name":"Sindrome da compressione del tronco celiaco","definition":"Si tratta di una malattia rara causata dalla compressione del tronco celiaco da parte di un ligamento arcuato anomalo (la parte del diaframma nella quale entrambi i pilastri si congiungono lungo la linea mediana attorno all'aorta). I pazienti presentano dolore addominale ricorrente, anoressia e perdita di peso. Si tratta di un dolore epigastrico, che può associarsi a diarrea o costipazione. In genere, ma non sempre, il dolore si manifesta dopo l'assunzione di cibo e può associarsi a nausea e vomito. Altri sintomi comprendono la debolezza e l'intolleranza all'esercizio fisico. Occasionalmente i pazienti presentano all'auscultazione un soffio nell'addome."},"nl":{"name":"Compressie van truncus coeliacus-syndroom","synonyms":["Ligamentum arcuatum medianum-syndroom","MALS","Mediaan gebogen ligament-syndroom","Syndroom van Dunbar"],"definition":"Een zeldzame ziekte, veroorzaakt door compressie van truncus coeliacus (ingewandsslagader) door een abnormaal gevormd ligamentum arcuatum (deel van diafragma waar beide pijlers samenkomen op de middellijn rond de aorta). Patiënten lijden aan terugkerende abdominale pijn, anorexie en gewichtsverlies. Pijn is epigastrisch, en diarree of constipatie kan ook aanwezig zijn. Pijn start meestal, maar niet altijd, na inname van voedsel, en kan gepaard gaan met misselijkheid en braken. Andere symptomen die kunnen voorkomen, zijn onder meer asthenie, inspanningsintolerantie en braken. Occasioneel kan een patiënt bij auscultatie buikgeruis vertonen."},"pt":{"name":"Síndrome de compressão do tronco celíaco"},"pl":{"name":"Zespół ucisku pnia trzewnego","synonyms":["Dunbar syndrome","MALS","Median arcuate ligament syndrome"]},"cs":{"name":"Syndrom komprese truncus coeliacus","synonyms":["Syndrom komprese celiakální arterie","Syndrom ligamentum arcuatum medianum","Dunbarův syndrom"]},"uk":{"name":"Синдром здавлення чревної артерії","synonyms":["Синдром Данбара","MALS","Синдром серединної дугоподібної звязки"],"definition":"A rare disease caused by compression of the celiac axis by an abnormally shaped arcuate ligament (the part of the diaphragm in which both pillars join in the midline around the aorta). Patients have recurrent abdominal pain, anorexia and weight loss. The pain is epigastric, and diarrhea or constipation may be present as well. Onset of pain will usually, although not always, be after food intake, and may be associated with nausea and emesis. Other symptoms may include lassitude, exercise intolerance and vomiting. Occasionally, a patient may show an abdominal murmur upon auscultation."},"zh":{"name":"腹腔动脉压迫综合征","synonyms":["Dunbar综合征"],"definition":"一种罕见的疾病，由畸形的弓形韧带（横膈膜的一部分，两根横膈膜柱在主动脉周围中线连接）压迫腹腔干引起。患者常出现腹痛、厌食和体重减轻。腹痛为上腹痛，也可出现腹泻或便秘。腹痛常在餐后发作，但并不总是如此，并可伴恶心和呕吐。其他症状可能包括疲乏、运动不耐受和呕吐。听诊偶可闻及腹部杂音。"},"tr":{"name":"Çölyak arter kompresyon sendromu","synonyms":["MALS","Dunbar sendromu","Median arkuat ligament sendromu"]}},"xrefs":[{"source":"ICD-10","code":"I77.4","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"BD52.5","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1666443751"},{"source":"MONDO","code":"0017388","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"17388","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1861783","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12308","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10009838","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":165711,"name":"Rare abdominal surgical disease"},"parents":[{"orpha":165711,"name":"Rare abdominal surgical disease","classifications":[182]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}