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It is not a pooled estimate for the umbrella condition."}],"comparison":{"collapseColumnsLabel":"Show initial comparisons","defaultColumnKeys":["MONDO:0013128/OMIM:613092"],"expandColumnsLabel":"Compare all source entries","intro":"Compare the original source entries. Different entries may describe different patient groups.","primaryFeatureIds":[],"scopeNoteHtml":"<p>Each column keeps its native condition scope. Initial features are displayed alphabetically, not ranked by clinical importance. Counts remain separate, and an empty cell does not mean a feature cannot occur.</p>"},"coverage":{"countRecords":0,"emptyColumnKeys":[],"hpoRelease":"2026-06-23","narrativeSourceChanges":[],"records":9,"sourceGroups":{"omim":1,"orphanet":0,"published":8}},"features":[{"hpoId":"HP:0001903","label":"Anemia","primary":false},{"hpoId":"HP:0012622","label":"Chronic kidney disease","primary":false},{"hpoId":"HP:0000097","label":"Focal segmental glomerulosclerosis","primary":false},{"hpoId":"HP:0004719","label":"Hyperechogenic kidneys","primary":false},{"hpoId":"HP:0002149","label":"Hyperuricemia","primary":false},{"hpoId":"HP:0000093","label":"Proteinuria","primary":false},{"hpoId":"HP:0000089","label":"Renal hypoplasia","primary":false},{"hpoId":"HP:0000092","label":"Renal tubular atrophy","primary":false},{"hpoId":"HP:0005576","label":"Tubulointerstitial fibrosis","primary":false}],"ledeHtml":"REN-related kidney disease is an inherited condition that affects kidney function. This condition causes slowly progressive kidney disease that usually becomes apparent during childhood. As this condition progresses, the kidneys become less able to filter fluids and waste products from the body, resulting in kidney failure. Individuals with REN-related kidney disease typically require dialysis (to remove wastes from the blood) or a kidney transplant between ages 40 and 70.","mondoId":"MONDO:0013128","records":[{"frequencyKind":"reported","key":"039b0dd0f3b47124248e5f024f8d265ecc50105976cc29f96fd0bc8452f11e97","raw":{"aspect":"P","body_system":"genitourinary","disease_id":"OMIM:613092","disease_name":"Tubulointerstitial kidney disease, autosomal dominant, 4","evidence":"PCS","frequency_raw":null,"hpo_id":"HP:0000089","is_excluded":false,"label":"Renal hypoplasia","onset_id":null,"reference":"PMID:19664745","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"reported","key":"0f25a6919f2f5a6779a46568ab37e7465cc4bc0aa27b3769c73e2be01de777be","raw":{"aspect":"P","body_system":"genitourinary","disease_id":"OMIM:613092","disease_name":"Tubulointerstitial kidney disease, autosomal dominant, 4","evidence":"PCS","frequency_raw":null,"hpo_id":"HP:0000097","is_excluded":false,"label":"Focal segmental glomerulosclerosis","onset_id":"HP:0003621","reference":"PMID:19664745","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"reported","key":"122ec26d964b045e60524da3ab4343a761663cb627a590e52f00f3925016b3b2","raw":{"aspect":"P","body_system":"genitourinary","disease_id":"OMIM:613092","disease_name":"Tubulointerstitial kidney disease, autosomal dominant, 4","evidence":"PCS","frequency_raw":null,"hpo_id":"HP:0012622","is_excluded":false,"label":"Chronic kidney disease","onset_id":null,"reference":"PMID:19664745","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"reported","key":"20ff238aa5cbc22dfab230f990a41b9c744a5ad2bcd598187756f8fa20ffa234","raw":{"aspect":"P","body_system":"genitourinary","disease_id":"OMIM:613092","disease_name":"Tubulointerstitial kidney disease, autosomal dominant, 4","evidence":"PCS","frequency_raw":null,"hpo_id":"HP:0005576","is_excluded":false,"label":"Tubulointerstitial fibrosis","onset_id":"HP:0003621","reference":"PMID:19664745","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"reported","key":"42788041d857133521c5897a903b825d96e1959cca8d101d5e0f281986162293","raw":{"aspect":"P","body_system":"genitourinary","disease_id":"OMIM:613092","disease_name":"Tubulointerstitial kidney disease, autosomal dominant, 4","evidence":"PCS","frequency_raw":null,"hpo_id":"HP:0000092","is_excluded":false,"label":"Renal tubular atrophy","onset_id":"HP:0003621","reference":"PMID:19664745","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"reported","key":"51755adf50b5f24b0b5da3457e6f6f354353105e15962644e71d604b2753d10d","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613092","disease_name":"Tubulointerstitial kidney disease, autosomal dominant, 4","evidence":"PCS","frequency_raw":null,"hpo_id":"HP:0001903","is_excluded":false,"label":"Anemia","onset_id":"HP:0011463","reference":"PMID:19664745","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"reported","key":"748139e22b080d5c5f81039808ac5105ffe531bed451f98a605b593f1c32110f","raw":{"aspect":"P","body_system":"metabolic","disease_id":"OMIM:613092","disease_name":"Tubulointerstitial kidney disease, autosomal dominant, 4","evidence":"PCS","frequency_raw":null,"hpo_id":"HP:0002149","is_excluded":false,"label":"Hyperuricemia","onset_id":"HP:0011463","reference":"PMID:19664745","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"reported","key":"b77a0b47b324b6e8d4e96acf997235a6b87b7ebf092e7d6845a6dfd55c886540","raw":{"aspect":"P","body_system":"genitourinary","disease_id":"OMIM:613092","disease_name":"Tubulointerstitial kidney disease, autosomal dominant, 4","evidence":"TAS","frequency_raw":null,"hpo_id":"HP:0004719","is_excluded":false,"label":"Hyperechogenic kidneys","onset_id":null,"reference":"OMIM:613092","source_release":"2026-06-23"},"sourceGroups":["omim"]},{"frequencyKind":"reported","key":"ef1334c37664d745aa04220c733394b92960e1ab7e32dee7f87ed5af0a596be0","raw":{"aspect":"P","body_system":"metabolic","disease_id":"OMIM:613092","disease_name":"Tubulointerstitial kidney disease, autosomal dominant, 4","evidence":"PCS","frequency_raw":null,"hpo_id":"HP:0000093","is_excluded":false,"label":"Proteinuria","onset_id":null,"reference":"PMID:19664745","source_release":"2026-06-23"},"sourceGroups":["published"]}],"referenceLabels":{},"references":{"causeHtml":"<ul><li><a href=\"https://medlineplus.gov/genetics/condition/ren-related-kidney-disease\">REN-related kidney disease</a></li></ul>","identityNote":"MONDO cross-references identify native condition entries. Ancestor membership does not transfer phenotype values between groups.","importantHtml":""},"sections":[{"bodyHtml":"<p>Mutations in the REN gene cause REN-related kidney disease. This gene provides instructions for making a protein called renin that is produced in the kidneys. Renin plays an important role in regulating blood pressure and water levels in the body.</p><p>Mutations in the REN gene that cause REN-related kidney disease result in the production of an abnormal protein that is toxic to the cells that normally produce renin. These kidney cells gradually die off, which causes progressive kidney disease.</p><p class=\"section-source\"><a href=\"https://medlineplus.gov/genetics/condition/ren-related-kidney-disease/#causes\">MedlinePlus Genetics: Causes</a></p>","key":"forms","title":"What causes REN-related kidney disease?"},{"bodyHtml":"<p>People with REN-related kidney disease sometimes have low blood pressure. They may also have mildly increased levels of potassium in their blood (hyperkalemia). In childhood, people with REN-related kidney disease develop a shortage of red blood cells (anemia), which can cause pale skin, weakness, and fatigue. In this disorder, anemia is usually mild and begins to improve during adolescence.</p><p>Many individuals with this condition develop high blood levels of a waste product called uric acid. Normally, the kidneys remove uric acid from the blood and transfer it to urine so it can be excreted from the body. In REN-related kidney disease, the kidneys are unable to remove uric acid from the blood effectively. A buildup of uric acid can cause gout, which is a form of arthritis resulting from uric acid crystals in the joints. Individuals with REN-related kidney disease may begin to experience the signs and symptoms of gout during their twenties.</p><p class=\"section-source\">Source: <a href=\"https://medlineplus.gov/genetics/condition/ren-related-kidney-disease\">MedlinePlus Genetics</a></p>","key":"overview","title":"Condition overview"}],"sharedSources":[],"slug":"ren-related-kidney-disease","sourceDocuments":[{"name":"REN-related kidney disease","reviewed":"2010-01","sections":{"causes":{"fetchedAt":"2026-10-20T20:24:40.521297+00:00","label":"Causes","pageSha256":"f2ff5141bb50edf6f80d48640399361902810c35509a5a4e2e5d470f346cd093","sourceUrl":"https://medlineplus.gov/genetics/condition/ren-related-kidney-disease/#causes","status":"available"}},"source_release":"2026-10-01","url":"https://medlineplus.gov/genetics/condition/ren-related-kidney-disease"}],"sourceNote":"MedlinePlus descriptions and Causes; original HPO annotations.","takeawayHtml":"Cause explanations are from MedlinePlus Genetics. Feature values below retain their original sources and patient groups.","title":"REN-related kidney disease"},"schemaVersion":1}