{"Name":"Sclerosing mesenteritis","DiseaseID__c":"GARD:0008169","id":8169,"encodedName":"sclerosing-mesenteritis","IsDeleted":false,"Disease_Name_Full__c":"Sclerosing mesenteritis","Xref_IDs__c":"1092381000119100; C0267770; MEDGEN:75639; MONDO:0016544; ORPHA:238593","USA_Estimate__c":null,"No_of_Specialist_Tagsa__c":1,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":null,"No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":2,"Disease_Characteristics_Score__c":3,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0016544","Disease_Description__c":"Sclerosing mesenteritis (SM) is a rare pathological disease causing inflammation of the adipose tissue of the small bowel mesentery and is commonly associated with abdominal pain, diarrhea, nausea, weight loss, bloating and loss of appetite. The two subforms include mesenteric panniculitis (where inflammation and fatty necrosis are dominant features) and retractile mesenteritis (where fibrosis and retraction dominate).","GARD_Name__c":"Sclerosing mesenteritis","GARD_Synonym__c":"idiopathic sclerosing mesenteritis; igg4-related mesenteritis; isolated mesenteric lipodystrophy; lipomatous mesenteritis; liposclerotic mesenteritis; mesenteric fibromatosis; mesenteric lipodystrophy; mesenteric lipogranuloma; mesenteric panniculitis; peritoneal retractile mesenteritis; retractile mesenteritis","Curated_Disease_Description_Source__c":"GARD:0008169","Curated_Disease_Description__c":"IgG4-related mesenteritis is one of many terms that describes a spectrum of inflammatory disorders that affect the mesentery. The mesentery is the membrane that anchors the small intestine to the back of the abdominal wall. The condition mostly affects men between their 40s and 60s, but women and children can also be affected. It may result in a variety of symptoms including abdominal pain, nausea and vomiting, constipation or diarrhea, weight loss, and fever. Some people have an abdominal mass. The cause of the condition is poorly understood. Suggested possible causes have included previous abdominal surgery or trauma, autoimmunity, paraneoplastic syndromes, ischemic injury, and infection.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":"as an Adult","SourceID__c":"ORPHA:238593","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0016544","ORPHANET_ID__c":"ORPHA:238593","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Mesenteritis asociada a igg4","Spanish_Description_Source__c":"ORPHA:238593","Spanish_Description__c":"La mesenteritis esclerosante (SM) es una enfermedad patológica poco frecuente que causa inflamación del tejido adiposo del mesenterio del intestino delgado y está asociada normalmente con dolor abdominal, diarrea, náuseas, pérdida de peso, hinchazón y pérdida de apetito. Las dos subformas son la paniculitis mesentérica (en la que la inflamación y la necrosis grasa son las características dominantes) y la mesenteritis retráctil (en la que dominan la fibrosis y la retracción).","Spanish_Disease_Name__c":"mesenteritis asociada a igg4","Spanish_GARD_Synonym__c":"lipodistrofia mesentérica aislada; lipogranuloma mesentérico; mesenteritis esclerosante; mesenteritis lipoesclerótica; mesenteritis lipomatosa; paniculitis mesentérica","Category_Linearization__c":"ORPHA:165711","icd10_id__c":null,"mesh_id__c":null,"omim_id__c":null,"snomed_id__c":null,"umls_id__c":null,"GARD_Disease__c":[{"Curated_Disease_Description__c":"IgG4-related mesenteritis is one of many terms that describes a spectrum of inflammatory disorders that affect the mesentery. The mesentery is the membrane that anchors the small intestine to the back of the abdominal wall. The condition mostly affects men between their 40s and 60s, but women and children can also be affected. It may result in a variety of symptoms including abdominal pain, nausea and vomiting, constipation or diarrhea, weight loss, and fever. Some people have an abdominal mass. The cause of the condition is poorly understood. Suggested possible causes have included previous abdominal surgery or trauma, autoimmunity, paraneoplastic syndromes, ischemic injury, and infection.","Curated_Disease_Description_Source__c":"GARD:0008169","GARD_Synonym__c":"idiopathic sclerosing mesenteritis; igg4-related mesenteritis; isolated mesenteric lipodystrophy; lipomatous mesenteritis; liposclerotic mesenteritis; mesenteric fibromatosis; mesenteric lipodystrophy; mesenteric lipogranuloma; mesenteric panniculitis; peritoneal retractile mesenteritis; retractile mesenteritis","Name":"Sclerosing mesenteritis","estimateUsa":""}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"Alianza Iberoamericana de Enfermedades Raras o Poco Frecuentes","Website__c":"https://aliber.org/web/"},{"Account_Name__c":"Federación Mexicana de Enfermedades Raras (FEMEXER)","Website__c":"http://www.femexer.org/"},{"Account_Name__c":"Federación Española de Enfermedades Raras","Website__c":"https://enfermedades-raras.org/"},{"Account_Name__c":"Federación Colombiana de Enfermedades Raras","Website__c":"http://www.fecoer.org"},{"Account_Name__c":"Federación Argentina de Enfermedades Poco Frecuentes","Website__c":"https://fadepof.org.ar/"},{"Account_Name__c":"Asociación Todos Unidos Enfermedades Raras Uruguay","Website__c":"https://atueru.org.uy/"},{"Account_Name__c":"The Digestive Disease National Coalition","Website__c":"https://ddnc.org/"}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Rheumatology","Tag_Category__c":"Specialist"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Adult","Provided_By__c":"ORPHA:238593"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.orpha.net/en/disease/detail/238593","Source__c":"C0267770; MONDO:0016544; ORPHA:238593","Xref__c":"ORPHA:238593"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0267770","Source__c":"C0267770","Xref__c":"C0267770"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=75639","Source__c":"C0267770","Xref__c":"MEDGEN:75639"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=1092381000119100","Source__c":"C0267770; MONDO:0016544","Xref__c":"1092381000119100"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0016544","Source__c":"GARD:0008169","Xref__c":"MONDO:0016544"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=86422009","Source__c":"C0267770","Xref__c":"86422009"}],"tags":{"Specialist":["Rheumatology"]},"synonyms":["idiopathic sclerosing mesenteritis"," igg4-related mesenteritis"," isolated mesenteric lipodystrophy"," lipomatous mesenteritis"," liposclerotic mesenteritis"," mesenteric fibromatosis"," mesenteric lipodystrophy"," mesenteric lipogranuloma"," mesenteric panniculitis"," peritoneal retractile mesenteritis"," retractile mesenteritis"],"spanishId":13353,"spanishName":"mesenteritis-esclerosante"}