{"Name":"Immunotactoid glomerulopathy","DiseaseID__c":"GARD:0012048","id":12048,"encodedName":"immunotactoid-glomerulopathy","IsDeleted":false,"Disease_Name_Full__c":"Immunotactoid glomerulopathy","Xref_IDs__c":"73305009; C158968; C5202806; C96182; MEDGEN:1684714; MONDO:0019991; ORPHA:97567","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":3,"Disease_Characteristics_Score__c":4,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0019991","Disease_Description__c":"Immunotactoid glomerulopathy (ITG) is a very rare condition characterized by glomerular accumulation of microtubules in the mesangium and the glomerular basement membrane, that mainly presents with proteinuria, micro-hematuria, nephrotic syndrome, renal insufficiency and hematologic malignancy. ITG and non-amyloid fibrillary glomerulopathy (non-amyloid FGP, see this term) are often grouped together as pathogenetically related diseases.","GARD_Name__c":"Immunotactoid glomerulopathy","GARD_Synonym__c":"fgn; fibrillary glomerulonephritis; immunotactoid glomerulonephritis","Curated_Disease_Description_Source__c":"GARD:0012048","Curated_Disease_Description__c":"Immunotactoid glomerulopathy, also known as glomerulonephritis with organized monoclonal microtubular immunoglobulin deposits (GOMMID), is a very uncommon cause of glomerular disease. It is related to a similar disease known as fibrillary glomerulopathy, which is more common. Both disorders probably result from deposits derived from immunoglobulins, but in most cases the cause is idiopathic (unknown). On electron microscopy, Immunotactoid glomerulopathy is characterized by the formation of microtubules which are much larger than the fibrils observed in fibrillary glomerulonephritis (30 to 50 versus 16 to 24 nm in diameter). The signs and symptoms include blood (hematuria) and protein (proteinuria) in the urine, kidney insufficiency and high blood pressure. Both fibrillary glomerulonephritis and Immunotactoid glomerulopathy have been associated with hepatitis C virus infection and with malignancy and autoimmune disease. Also, patients with Immunotactoid glomerulopathy have a greater risk to have chronic lymphocytic leukemia and B cell lymphomas and should be screened for all of these conditions.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":"as an Adult","SourceID__c":"ORPHA:97567","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0019991","ORPHANET_ID__c":"ORPHA:97567","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Glomerulopatía inmunotactoide","Spanish_Description_Source__c":"ORPHA:97567","Spanish_Description__c":"La glomerulopatía inmunotactoide (GIT) es una enfermedad muy poco frecuente caracterizada por una acumulación de microtúbulos en el mesangio y en la membrana basal glomerular, que se presenta principalmente con proteinuria, microhematuria, síndrome nefrótico, insuficiencia renal y neoplasia hematológica. La GIT y la glomerulopatía fibrilar no-amiloide (GPF no-amiloide) se agrupan normalmente como enfermedades patogenéticamente relacionadas.","Spanish_Disease_Name__c":"glomerulopatía inmunotactoide","Spanish_GARD_Synonym__c":null,"Category_Linearization__c":"ORPHA:93626","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":"Immunotactoid glomerulopathy, also known as glomerulonephritis with organized monoclonal microtubular immunoglobulin deposits (GOMMID), is a very uncommon cause of glomerular disease. It is related to a similar disease known as fibrillary glomerulopathy, which is more common. Both disorders probably result from deposits derived from immunoglobulins, but in most cases the cause is idiopathic (unknown). On electron microscopy, Immunotactoid glomerulopathy is characterized by the formation of microtubules which are much larger than the fibrils observed in fibrillary glomerulonephritis (30 to 50 versus 16 to 24 nm in diameter). The signs and symptoms include blood (hematuria) and protein (proteinuria) in the urine, kidney insufficiency and high blood pressure. Both fibrillary glomerulonephritis and Immunotactoid glomerulopathy have been associated with hepatitis C virus infection and with malignancy and autoimmune disease. Also, patients with Immunotactoid glomerulopathy have a greater risk to have chronic lymphocytic leukemia and B cell lymphomas and should be screened for all of these conditions.","Curated_Disease_Description_Source__c":"GARD:0012048","GARD_Synonym__c":"fgn; fibrillary glomerulonephritis; immunotactoid glomerulonephritis","Name":"Immunotactoid glomerulopathy","estimateUsa":""}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Nephrology","Tag_Category__c":"Account;Disease Category;Specialist","category_description":"Kidney diseases affect the kidneys' ability to remove waste and water from blood, create urine, or make certain hormones.","curated_tag_name":"Kidney diseases"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Adult","Provided_By__c":"ORPHA:97567"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=1684714","Source__c":"C5202806","Xref__c":"MEDGEN:1684714"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C158968","Source__c":"C5202806; MONDO:0019991","Xref__c":"C158968"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=73305009","Source__c":"MONDO:0019991","Xref__c":"73305009"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C5202806","Source__c":"C5202806","Xref__c":"C5202806"},{"URL__c":"https://www.orpha.net/en/disease/detail/97567","Source__c":"C5202806; MONDO:0019991","Xref__c":"ORPHA:97567"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C96182","Source__c":"MONDO:0019991","Xref__c":"C96182"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0019991","Source__c":"GARD:0012048","Xref__c":"MONDO:0019991"}],"tags":{"Account":["Nephrology"],"Disease Category":["Nephrology"],"Specialist":["Nephrology"]},"synonyms":["fgn"," fibrillary glomerulonephritis"," immunotactoid glomerulonephritis"]}