AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE: CURRENT TREATMENT AND FUTURE THERAPEUTIC OPTIONS
Formerly Published in Vol. 7 No. 2 Jul. - Dec., 2022
DOI:
https://doi.org/10.83120/pmj.vol3no2.24Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease. Cyst initiation and expansion arise from a combination of abnormality in cell proliferation, secretion of fluid and extracellular matrix defects. The consequence is kidney enlargement and interstitial fibrosis. The disease has extra-renal manifestations including polycystic liver disease, intracranial aneurysms, cardiac valve defect and development of cyst in many other organs. This clinical review focused on existing treatment and future therapies in ADPKD using a clinical scenario in a developing world. We report a 49 year old woman who presented to the nephrology clinic with abdominal pains, recurrent painless haematuria and generalized body weakness. She was found to have ballotable kidneys, hypertension and uraemia. There was a radiologic confirmation of polycystic kidneys with extra-renal cysts in the liver. This clinical review draws attention to this cause of chronic kidney disease, current treatments and promising therapies.
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