{"Name":"Non-amyloid fibrillary glomerulopathy","DiseaseID__c":"GARD:0012740","id":12740,"encodedName":"non-amyloid-fibrillary-glomerulopathy","IsDeleted":false,"Disease_Name_Full__c":"Non-amyloid fibrillary glomerulopathy","Xref_IDs__c":"718192000; C4273674; MEDGEN:907201; MONDO:0019990; ORPHA:97566","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:0019990","Disease_Description__c":"Non-amyloid fibrillary glomerulopathy (non-amyloid FGP) is a rare cause of glomerulonephritis (GN) characterized by glomerular accumulation of non-amyloid fibrils in the mesangium and the glomerular (and rarely tubular) basement membrane, that mainly presents with renal insufficiency, micro-hematuria and nephrotic range proteinuria. Non-amyloid FGP and immunotactoid glomerulopathy (ITG, see this term) are often grouped together as pathogenetically related diseases.","GARD_Name__c":"Non-amyloid fibrillary glomerulopathy","GARD_Synonym__c":"congo red negative amyloidosis like glomerulopathy; congo red-negative amyloidosis-like glomerulopathy; fibrillary glomerulonephritis ; non-amyloid fibrillary glomerulonephritis","Curated_Disease_Description_Source__c":"GARD:0012740","Curated_Disease_Description__c":"Non-amyloid fibrillary glomerulopathy is an uncommon cause of glomerular disease. A more rare disorder known as immunotactoid glomerulpathy is a very similar condition. Both disorders probably result from deposits derived from immunoglobulins but in most cases the cause is idiopathic (unknown). The diagnosis is made with a kidney biopsy and by electron microscopy. Non-amyloid fibrillary glomerulopathy and immunotactoid glomerulopathy can be differentiated from each other by electron microscopy; the fibrils in non-amyloid fibrillary glomerulopathy are smaller and randomly oriented as opposed to the larger and often organized fibrils of immunotactoid glomerulopathy. The signs and symptoms include blood (hematuria) and protein (proteinuria) in the urine, kidney insufficiency and high blood pressure. Both non-amyloid fibrillary glomerulopathy and immunotactoid glomerulopathy have been associated with hepatitis C virus infection and with malignancy and autoimmune disease. Because of this, patients should be screened for 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:97566","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0019990","ORPHANET_ID__c":"ORPHA:97566","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Glomerulopatía fibrilar no amiloide","Spanish_Description_Source__c":"ORPHA:97566","Spanish_Description__c":"La glomerulopatía fibrilar no-amiloide (GPF no-amiloide) es un caso poco frecuente de glomerulonefritis (GN) caracterizado por una acumulación de fibrillas no amiloides en el mesangio y en la membrana basal glomerular (y raramente tubular), que principalmente se presenta con insuficiencia renal, micro-hematuria y proteinuria en rango nefrótico. La GPF no-amiloide y la glomerulopatía inmunotactoide (GIT) están frecuentemente agrupadas como enfermedades patogenéticamente relacionadas.","Spanish_Disease_Name__c":"glomerulopatía fibrilar no amiloide","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":"Non-amyloid fibrillary glomerulopathy is an uncommon cause of glomerular disease. A more rare disorder known as immunotactoid glomerulpathy is a very similar condition. Both disorders probably result from deposits derived from immunoglobulins but in most cases the cause is idiopathic (unknown). The diagnosis is made with a kidney biopsy and by electron microscopy. Non-amyloid fibrillary glomerulopathy and immunotactoid glomerulopathy can be differentiated from each other by electron microscopy; the fibrils in non-amyloid fibrillary glomerulopathy are smaller and randomly oriented as opposed to the larger and often organized fibrils of immunotactoid glomerulopathy. The signs and symptoms include blood (hematuria) and protein (proteinuria) in the urine, kidney insufficiency and high blood pressure. Both non-amyloid fibrillary glomerulopathy and immunotactoid glomerulopathy have been associated with hepatitis C virus infection and with malignancy and autoimmune disease. Because of this, patients should be screened for these conditions.","Curated_Disease_Description_Source__c":"GARD:0012740","GARD_Synonym__c":"congo red negative amyloidosis like glomerulopathy; congo red-negative amyloidosis-like glomerulopathy; fibrillary glomerulonephritis ; non-amyloid fibrillary glomerulonephritis","Name":"Non-amyloid fibrillary 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:97566"}],"External_Identifier_Disease__c":[{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=718192000","Source__c":"C4273674; MONDO:0019990","Xref__c":"718192000"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=907201","Source__c":"C4273674","Xref__c":"MEDGEN:907201"},{"URL__c":"https://www.orpha.net/en/disease/detail/97566","Source__c":"C4273674; MONDO:0019990; ORPHA:97566","Xref__c":"ORPHA:97566"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C4273674","Source__c":"C4273674","Xref__c":"C4273674"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0019990","Source__c":"GARD:0012740","Xref__c":"MONDO:0019990"}],"tags":{"Account":["Nephrology"],"Disease Category":["Nephrology"],"Specialist":["Nephrology"]},"synonyms":["congo red negative amyloidosis like glomerulopathy"," congo red-negative amyloidosis-like glomerulopathy"," fibrillary glomerulonephritis "," non-amyloid fibrillary glomerulonephritis"]}