834721
Description
Cystic kidney diseases
- APKD
- Polycystin 1 (85%),
Polycystin 2 (15%)
- Baloons that affect tubular part, not
glomerular -> no proteinuria
- They can compress abdominal organs
- They can compress abdominal organs
- Recessive form is more dramatic
- Extrarenal maifestations
- Therapy
- Transplant (done in later stages)
- Tolvaptan
- Transplant (done in later stages)
- Symptoms
- Polycystin 1 (85%),
Polycystin 2 (15%)
- Uromodulin
- Mechanisms against infection
- It inhibits calcium oxalate and calcium
phosphate crystals formation
- linked to nnate immunity
- Danger signal
- When mutated, it is not deleted
- The natural effect of mutation is misfolding and ER retention
- Novel target for lowering BP
- Lower uromodulin levels are associated
with better kidney aging
- It can trigger red blood cell alteration
- Mechanisms against infection
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