{"Name":"IgA glomerulonephritis","DiseaseID__c":"GARD:0000863","id":863,"encodedName":"iga-glomerulonephritis","IsDeleted":false,"Disease_Name_Full__c":"IgA glomerulonephritis","Xref_IDs__c":"68779003; C0017661; C34643; D005922; DOID:2986; MEDGEN:9032; MONDO:0005342; ORPHA:34145","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":1,"World_Estimate__c":null,"No_of_HRSA_records__c":0,"Evidence_Based_Score__c":1,"No_of_Disease_Descriptions__c":4,"Disease_Characteristics_Score__c":5,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0005342","Disease_Description__c":"Inflammation of a specific segment of glomeruli within the kidney.","GARD_Name__c":"IgA glomerulonephritis","GARD_Synonym__c":"berger's disease; berger's iga or igg nephropathy; focal glomerulonephritis; iga nephropathy; igan - iga nephropathy; immunoglobulin a nephropathy; nephritis, iga type; non rare in europe: berger disease; primary iga nephropathy; segmental glomerulonephritis","Curated_Disease_Description_Source__c":"GARD:0000863","Curated_Disease_Description__c":"IgA nephropathy is a kidney disorder that occurs when IgA (immunoglobulin A), a protein that helps the body fight infections, settles in the kidneys. In the early stages, IgA nephropathy has no symptoms. The first sign of this condition may be blood in the urine. End-stage kidney disease may develop. In most instances, the cause of this condition is unknown; however, certain disorders have been linked with IgA nephropathy, such as cirrhosis of the liver, celiac disease, and HIV infection. Although IgA nephropathy usually occurs in a family with no other affected members, several cases of familial IgA nephropathy have been reported. Familial IgA nephropathy is suspected to run through families in an autosomal dominant manner and is linked to genetic material on the long arm of chromosome 6 (6q22-23).","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":null,"SourceID__c":null,"Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0005342","ORPHANET_ID__c":"ORPHA:34145","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Nefropatía por inmunoglobulina a","Spanish_Description_Source__c":null,"Spanish_Description__c":null,"Spanish_Disease_Name__c":"nefropatía por inmunoglobulina a","Spanish_GARD_Synonym__c":"enfermedad de berger; nefropatía por iga","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":"IgA nephropathy is a kidney disorder that occurs when IgA (immunoglobulin A), a protein that helps the body fight infections, settles in the kidneys. In the early stages, IgA nephropathy has no symptoms. The first sign of this condition may be blood in the urine. End-stage kidney disease may develop. In most instances, the cause of this condition is unknown; however, certain disorders have been linked with IgA nephropathy, such as cirrhosis of the liver, celiac disease, and HIV infection. Although IgA nephropathy usually occurs in a family with no other affected members, several cases of familial IgA nephropathy have been reported. Familial IgA nephropathy is suspected to run through families in an autosomal dominant manner and is linked to genetic material on the long arm of chromosome 6 (6q22-23).","Curated_Disease_Description_Source__c":"GARD:0000863","GARD_Synonym__c":"berger's disease; berger's iga or igg nephropathy; focal glomerulonephritis; iga nephropathy; igan - iga nephropathy; immunoglobulin a nephropathy; nephritis, iga type; non rare in europe: berger disease; primary iga nephropathy; segmental glomerulonephritis","Name":"IgA glomerulonephritis","estimateUsa":""}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"HealthTree Foundation","Website__c":"https://healthtree.org/"},{"Account_Name__c":"IgA Nephropathy Foundation","Website__c":"https://igan.org/"},{"Account_Name__c":"NephCure Kidney International","Website__c":"https://nephcure.org/"},{"Account_Name__c":"Global Autoimmune Institute","Website__c":"https://www.autoimmuneinstitute.org/"},{"Account_Name__c":"National Kidney Foundation","Website__c":"https://www.kidney.org/"},{"Account_Name__c":"The Kidney Foundation of Canada","Website__c":"https://kidney.ca/"},{"Account_Name__c":"Autoimmune Association","Website__c":"https://autoimmune.org/"}],"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"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=9032","Source__c":"C0017661","Xref__c":"MEDGEN:9032"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C34643","Source__c":"C0017661; MONDO:0005342","Xref__c":"C34643"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C005922","Source__c":"C0017661; MONDO:0005342","Xref__c":"D005922"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A2986","Source__c":"MONDO:0005342","Xref__c":"DOID:2986"},{"URL__c":"https://www.orpha.net/en/disease/detail/34145","Source__c":"C0017661; MONDO:0005342; ORPHA:34145","Xref__c":"ORPHA:34145"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0017661","Source__c":"C0017661","Xref__c":"C0017661"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=68779003","Source__c":"MONDO:0005342","Xref__c":"68779003"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0005342","Source__c":"GARD:0000863","Xref__c":"MONDO:0005342"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=236407003","Source__c":"C0017661","Xref__c":"236407003"},{"URL__c":"https://www.niddk.nih.gov/health-information/kidney-disease/iga-nephropathy"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C35280","Source__c":"C0017661","Xref__c":"C35280"}],"GARD_Disease_Feature__c":[{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Occasional (5-29%)","Feature__r":{"HPO_Description__c":"A reduction in the level of performance of the kidneys in areas of function comprising the concentration of urine, removal of wastes, the maintenance of electrolyte balance, homeostasis of blood pressure, and calcium metabolism.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0000083","HPO_Synonym__c":"Renal failure; Renal failure in adulthood","HPO_Name__c":"Renal insufficiency","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Occasional (5-29%)","Feature__r":{"HPO_Description__c":"A chronic disorder of the liver in which liver tissue becomes scarred and is partially replaced by regenerative nodules and fibrotic tissue resulting in loss of liver function.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0001394","HPO_Synonym__c":"Hepatic cirrhosis; Scar tissue replaces healthy tissue in the liver","HPO_Name__c":"Cirrhosis","Feature_System__c":"Digestive System","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Occasional (5-29%)","Feature__r":{"HPO_Description__c":"The presence of chronic increased pressure in the systemic arterial system.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0000822","HPO_Synonym__c":"Arterial hypertension; Systemic hypertension","HPO_Name__c":"Hypertension","Feature_System__c":"Cardiovascular System","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Occasional (5-29%)","Feature__r":{"HPO_Description__c":"Inflammation of the renal glomeruli.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0000099","HPO_Synonym__c":"Glomerular nephritis","HPO_Name__c":"Glomerulonephritis","Feature_System__c":"Immune System","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Occasional (5-29%)","Feature__r":{"HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0000282","HPO_Synonym__c":"Facial puffiness; Facial swelling; Swelling of the face","HPO_Name__c":"Facial edema","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Occasional (5-29%)","Feature__r":{"HPO_Description__c":"Accumulation of fluid in the peritoneal cavity (between the layers of the peritoneum that lines the abdomen).","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0001541","HPO_Synonym__c":"Accumulation of fluid in the abdomen; Peritoneal effusion","HPO_Name__c":"Ascites","Feature_System__c":"Digestive System","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Occasional (5-29%)","Feature__r":{"HPO_Description__c":"Celiac disease (CD) is an autoimmune condition affecting the small intestine, triggered by the ingestion of gluten, the protein fraction of wheat, barley, and rye. Clinical manifestations of CD are highly variable and include both gastrointestinal and non-gastrointestinal features. The hallmark of CD is an immune-mediated enteropathy. This term is included because the occurrence of CD is seen as a feature of a number of other diseases.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0002608","HPO_Synonym__c":"Celiac disease; Celiac sprue","HPO_Name__c":"Celiac disease","Feature_System__c":"Immune System; Digestive System","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"Hematuria that is visible upon inspection of the urine.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0012587","HPO_Synonym__c":"Bloody urine; Gross hematuria","HPO_Name__c":"Macroscopic hematuria","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"The presence of immunoglobulin A deposits in the glomerulus.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0000794","HPO_Name__c":"IgA deposition in the glomerulus","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Occasional (5-29%)","Feature__r":{"HPO_Description__c":"Glomerular crescent refers hyperplastic lesions involving 10% or more of the circumference of Bowman's capsule. Crescents can be composed of a variable mixture of epithelial/leukocyte hypercellularity, fibrous matrix, and fibrin.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0033316","HPO_Name__c":"Glomerular crescent formation","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"Microscopic hematuria detected by dipstick or microscopic examination of the urine.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0002907","HPO_Synonym__c":"Microhematuria; Occult hematuria; Small amount of blood in urine","HPO_Name__c":"Microscopic hematuria","HPO_Feature_Type__c":"Lab"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"Mildly increased levels of protein in the urine (150-500 mg per day in adults).","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0012595","HPO_Name__c":"Mild proteinuria","HPO_Feature_Type__c":"Lab"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Occasional (5-29%)","Feature__r":{"HPO_Description__c":"Crackles are discontinuous, explosive, and nonmusical adventitious lung sounds normally heard in inspiration and sometimes during expiration. Crackles are usually classified as fine and coarse crackles based on their duration, loudness, pitch, timing in the respiratory cycle, and relationship to coughing and changing body position.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0030830","HPO_Synonym__c":"Crepitations; Rales","HPO_Name__c":"Crackles","Feature_System__c":"Respiratory system","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Uncommon (<1-4%)","Feature__r":{"HPO_Description__c":"Severely increased amount of excretion of protein in the urine, defined as 3.5 grams per day or more in adults and 40 mg per meter-squared body surface area per hour in children.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0012593","HPO_Name__c":"Nephrotic range proteinuria","HPO_Feature_Type__c":"Lab"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Occasional (5-29%)","Feature__r":{"HPO_Description__c":"An abnormally increased level of immunoglobulin A in blood.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0003261","HPO_Synonym__c":"Elevated IgA; Elevated serum IgA; IgA hypergammaglobulinemia; Increased circulating IgA level; Increased levels of IgA; Increased serum IgA","HPO_Name__c":"Increased circulating IgA concentration","Feature_System__c":"Immune System; Blood and Blood-Forming Tissue","HPO_Feature_Type__c":"Lab"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Occasional (5-29%)","Feature__r":{"HPO_Description__c":"Urine has an increased amount of frothy fine bubbles.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0031504","HPO_Synonym__c":"Frothy urine","HPO_Name__c":"Foamy urine","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:34145","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"Sudden loss of renal function, as manifested by decreased urine production, and a rise in serum creatinine or blood urea nitrogen concentration (azotemia).","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0001919","HPO_Synonym__c":"Acute kidney failure; Acute renal failure; AKI","HPO_Name__c":"Acute kidney injury","HPO_Feature_Type__c":"Symptom"}}],"tags":{"Account":["Nephrology"],"Disease Category":["Nephrology"],"Specialist":["Nephrology"]},"synonyms":["berger's disease"," berger's iga or igg nephropathy"," focal glomerulonephritis"," iga nephropathy"," igan - iga nephropathy"," immunoglobulin a nephropathy"," nephritis, iga type"," non rare in europe: berger disease"," primary iga nephropathy"," segmental glomerulonephritis"]}