{"Name":"Necrobacillosis","DiseaseID__c":"GARD:0006882","id":6882,"encodedName":"necrobacillosis","IsDeleted":false,"Disease_Name_Full__c":"Necrobacillosis","Xref_IDs__c":"52542005; C0027537; D057831; DOID:11337; MEDGEN:6541; MONDO:0015306; ORPHA:137839","USA_Estimate__c":"200,000","No_of_Specialist_Tagsa__c":2,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":"800,000 to 5,000,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":2,"Disease_Characteristics_Score__c":4,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0015306","Disease_Description__c":"Lemierre syndrome is a rare, potentially lethal, oropharyngeal infectious disease occurring in immunocompetent adolescents and young adults that is mainly due to <i>Fusobacterium necrophorum</i> and that is characterized by septic thrombophlebitis of the internal jugular vein that leads to septic, usually pulmonary, embolism, associated with ENT (ear, nose, and throat) infection that manifests with fever, neck pain, and tonsillopharyngitis.","GARD_Name__c":"Necrobacillosis","GARD_Synonym__c":"human necrobacillosis; lemierre postanginal sepsis; lemierre syndrome; postanginal sepsis; postanginal sepsis secondary to orophyngeal infection; septic phlebitis of the internal jugular vein","Curated_Disease_Description_Source__c":"GARD:0006882","Curated_Disease_Description__c":"Lemierre syndrome is a rare and potentially severe complication of bacterial infections that usually affects previously-healthy adolescents and young adults. It most commonly develops in association with a bacterial throat infection, but it may develop in association with an infection involving the ears, salivary glands (parotitis), sinuses, or teeth; or in association with an Epstein-Barr infection. The bacteria typically responsible for infection in Lemierre syndrome is Fusobacterium necrophorum, although a variety of bacteria can be responsible. In people with Lemierre syndrome, the initial infection spreads into tissues and deep spaces within the neck, leading to the formation of an infected blot clot (septic thrombophlebitis), sometimes made up of pus, in the internal jugular vein (the blood vessel that carries blood away from the brain, face, and neck). The infected clot then circulates in the blood (septicemia), resulting in the infection also spreading to the lungs (most commonly), skeletal system, and/or other parts of the body such as the spleen, liver, kidney, heart, or brain. This can lead to severe complications such as respiratory distress syndrome due to pulmonary emboli (blood clots in the lung), damage to other affected organs, and/or septic shock (in about 7% of cases). Lemierre syndrome may be diagnosed based on signs and symptoms, various blood tests, and imaging studies.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"200,000","Age_at_Onset_Snippet_Text__c":"as a Teenager","SourceID__c":"ORPHA:137839","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0015306","ORPHANET_ID__c":"ORPHA:137839","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Síndrome de lemierre","Spanish_Description_Source__c":"ORPHA:137839","Spanish_Description__c":"Es una enfermedad infecciosa orofaríngea poco frecuente y potencialmente letal que se presenta en adolescentes y adultos jóvenes inmunocompetentes y que se debe principalmente a&#160;<i>Fusobacterium necrophorum</i>. Está caracterizada por una tromboflebitis séptica de la vena yugular interna que conduce a una embolia séptica, generalmente pulmonar, asociada a infección ORL (oído, nariz y garganta) que se manifiesta con fiebre, dolor de cuello y faringoamigdalitis.","Spanish_Disease_Name__c":"síndrome de lemierre","Spanish_GARD_Synonym__c":"flebitis séptica de la vena yugular interna; sepsis postangina secundaria a infección orofaríngea; sepsis postanginal de lemierre; sepsis postanginal secundaria a una infección orofaríngea","Category_Linearization__c":"ORPHA:68416","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":"Lemierre syndrome is a rare and potentially severe complication of bacterial infections that usually affects previously-healthy adolescents and young adults. It most commonly develops in association with a bacterial throat infection, but it may develop in association with an infection involving the ears, salivary glands (parotitis), sinuses, or teeth; or in association with an Epstein-Barr infection. The bacteria typically responsible for infection in Lemierre syndrome is Fusobacterium necrophorum, although a variety of bacteria can be responsible. In people with Lemierre syndrome, the initial infection spreads into tissues and deep spaces within the neck, leading to the formation of an infected blot clot (septic thrombophlebitis), sometimes made up of pus, in the internal jugular vein (the blood vessel that carries blood away from the brain, face, and neck). The infected clot then circulates in the blood (septicemia), resulting in the infection also spreading to the lungs (most commonly), skeletal system, and/or other parts of the body such as the spleen, liver, kidney, heart, or brain. This can lead to severe complications such as respiratory distress syndrome due to pulmonary emboli (blood clots in the lung), damage to other affected organs, and/or septic shock (in about 7% of cases). Lemierre syndrome may be diagnosed based on signs and symptoms, various blood tests, and imaging studies.","Curated_Disease_Description_Source__c":"GARD:0006882","GARD_Synonym__c":"human necrobacillosis; lemierre postanginal sepsis; lemierre syndrome; postanginal sepsis; postanginal sepsis secondary to orophyngeal infection; septic phlebitis of the internal jugular vein","Name":"Necrobacillosis","Curated_USA_Estimate__c":"200,000","estimateUsa":"200,000"}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Infectious Disease","Tag_Category__c":"Disease Category;Specialist","category_description":"Infectious diseases are caused by bacteria, viruses, fungi, or parasites entering the body, multiplying, and spreading illness.","curated_tag_name":"Infectious diseases"},{"Tag_Name__c":"Rare Bacterial Infectious Disease","Tag_Category__c":"Cause","curated_tag_name":"Bacterial Infectious Disease"},{"Tag_Name__c":"Pediatrics","Tag_Category__c":"Specialist"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Adolescent","Provided_By__c":"ORPHA:137839"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.orpha.net/en/disease/detail/137839","Source__c":"C0027537; MONDO:0015306","Xref__c":"ORPHA:137839"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=6541","Source__c":"C0027537","Xref__c":"MEDGEN:6541"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=52542005","Source__c":"C0027537; MONDO:0015306","Xref__c":"52542005"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0027537","Source__c":"C0027537","Xref__c":"C0027537"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C057831","Source__c":"MONDO:0015306","Xref__c":"D057831"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A11337","Source__c":"MONDO:0015306","Xref__c":"DOID:11337"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0015306","Source__c":"GARD:0006882","Xref__c":"MONDO:0015306"}],"tags":{"Disease Category":["Infectious Disease"],"Specialist":["Infectious Disease","Pediatrics"],"Cause":["Rare Bacterial Infectious Disease"]},"synonyms":["human necrobacillosis"," lemierre postanginal sepsis"," lemierre syndrome"," postanginal sepsis"," postanginal sepsis secondary to orophyngeal infection"," septic phlebitis of the internal jugular vein"]}