{"orpha":263665,"name":"NK-cell enteropathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Natural killer (NK)-cell enteropathy is a benign NK-cell lymphoproliferative disease characterized by minor abdominal symptoms (abdominal pain, diverticulosis, constipation and reflux) due to NK cell-derived lesions in the mucosal layer of the gastrointestinal tract and often mistaken for NK or T-cell lymphoma.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-263665/","orphanet_url":"https://www.orpha.net/en/disease/detail/263665","names":{"fr":{"name":"Entéropathie à cellules NK","definition":"Maladie lymphoproliférative bénigne à cellules NK caractérisée par des symptômes abdominaux mineurs (douleurs abdominales, diverticulose, constipation et reflux) dus à des lésions de la muqueuse gastro-intestinale dérivées des cellules NK et souvent confondue avec un lymphome NK ou T."},"de":{"name":"NK-Zellen-Enteropathie","definition":"Die <I>Natural killer</I> (NK)-Zellen-Enteropathie ist eine benigne NK-Zellen-lymphoproliferative Krankheit und gekennzeichnet durch leichtere abdominelle Symptome (Leibschmerzen, Divertikulose, Obstipation, Reflux). Ursache sind Läsionen in der Mukosaschicht des Magendarmtraktes, die sich von NK-Zellen herleiten und oft als NK- oder T-Zell-Lymphom (s. dort) fehlgedeutet werden."},"es":{"name":"Enteropatía de células NK","definition":"La enteropatía de células natural killer (NK) es una enfermedad linfoproliferativa de células NK que se caracteriza por síntomas abdominales menores (dolor abdominal, diverticulosis, estreñimiento y reflujo), debidos a lesiones derivadas de las células NK en la capa mucosa del tracto gastrointestinal y a menudo confundidas con un linfoma de células NK o T (consulte estos términos)."},"it":{"name":"Enteropatia a cellule NK","definition":"L'enteropatia a cellule natural killer (NK) è una malattia linfoproliferativa benigna a cellule NK caratterizzata da lievi sintomi addominali (dolore addominale, diverticolosi, costipazione e reflusso) da lesioni derivate dalle cellule NK nello strato mucoso del tratto intestinale ed è spesso confusa con il linfoma a cellule NK o T (si vedano questi termini)."},"nl":{"name":"NK-cel-enteropathie","definition":"Een goedaardige lymfoproliferatieve aandoening van natuurlijke killer (NK)-cellen, gekenmerkt door minieme abdominale symptomen (abdominale pijn, diverticulose, constipatie en reflux) door van NK-cellen afgeleide laesies in slijmvlies van maag-darmstelsel, en vaak verkeerdelijk aanzien voor NK- of T-cellymfoom."},"pt":{"name":"Enteropatia de células NK"},"pl":{"name":"Enteropatia komórek NK"},"cs":{"name":"Enteropatie NK-buněk"},"uk":{"name":"NK-клітинна ентеропатія","definition":"Natural killer (NK)-cell enteropathy is a benign NK-cell lymphoproliferative disease characterized by minor abdominal symptoms (abdominal pain, diverticulosis, constipation and reflux) due to NK cell-derived lesions in the mucosal layer of the gastrointestinal tract and often mistaken for NK or T-cell lymphoma (see these terms)."},"zh":{"name":"NK细胞肠病","definition":"自然杀伤(NK)细胞肠病是一种良性的NK细胞淋巴增生性疾病，其特征是胃肠道粘膜层的NK细胞源性病变引起的腹部轻微症状(腹痛、憩室、便秘和反流)，常被误诊为NK或T细胞淋巴瘤(见该类词条)。"},"tr":{"name":"NK-hücresi enteropatisi"}},"xrefs":[{"source":"ICD-10","code":"K52.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DA94.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0016996","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4509932","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97935,"name":"Rare gastroenterologic disease"},"parents":[{"orpha":117569,"name":"Rare intestinal disease","classifications":[152]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":8.0,"area":"Worldwide","status":"Validated","source":"20966166[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-06-14","source":"20966166[PMID]","terms":[{"hpo":"HP:0000969","label":"Edema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100828","label":"Increased T cell count","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0005523","label":"Lymphoproliferative disorder","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004295","label":"Abnormality of the gastric mucosa","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002253","label":"Colonic diverticula","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002019","label":"Constipation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002588","label":"Duodenal ulcer","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002592","label":"Gastric ulcer","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002020","label":"Gastroesophageal reflux","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002573","label":"Hematochezia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005266","label":"Intestinal polyp","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012425","label":"Stercoral ulcer","frequency":"Occasional (29-5%)","criteria":null}]}}