Abstract

Korapati Ramarao, Abdul Mushtaq Mohammed, Safi Ur-Rahman Mohammed, C. Sai Jayanth Guptha and Dr. S. P. Srinivas Nayak*

ABSTRACT

Autosomal dominant polycystic kidney disease is the most common inherited kidney disease, results in progressive loss of renal function due to the development and growth of cysts. Advances in understanding the nature of the disease have led to increased awareness of ADPKD, improvements in imaging modalities for diagnosis and assessment and the availability of effective therapies because patients with ADPKD often experience a range of renal and extrarenal complications. Approximately 78% of cases of ADPKD arise from PKD1 mutations. PKD2 mutations account for another 15%. These mutation-driven changes produce the hallmark disease process of ADPKD: development of large, fluid-filled cysts in the kidney, which over time increase kidney size and volume and compromise kidney function, leading to decreased life expectancy, need for dialysis and/or transplantation, cardiovascular/cerebro-vascular disease, and intracranial aneurysms. The involvement of the vasopressin system makes it a target for therapy designed to slow progression of ADPKD. Steps are to taken to slow down the progression of disease by early diagnosis and symptomatic treatment approaches are needed to be followed to prevent the complications. As RAAS mechanisms are prime factors for progression and worsening of the condition, steps should be taken to prevent the over activity of RAAS by using many of the newer therapeutic agents show promise in preventing or stabilizing cyst growth, providing much needed hope in this currently relentless condition. Hypertension should be kept in check to prevent any chances of strokes. Making lifestyle changes such as dash diet and maintaining adequate hydration to maintain the normal renal sufficiency are some of the key approaches to control or to prevent the progression of this condition and help the patient to lead normal life and life expectancy.