A Short Note on Polycystic Kidney Disease

Girma Mulugeta Emeru^*
*Correspondence: Girma Mulugeta Emeru, Department of Renal Diseases and Hypertension, University of Colorado at Denver, Aurora, United States, Email:

Description

For individuals with polycystic kidney disease (PKD), life can be a constant cycle of symptoms, such as aches and pains, abdominal swelling, kidney stones, and hypertension. The infection adequately leads to major issues such as kidney failure, cysts in the liver and vascular issues, including strokes. As per the specialists, PKD is a common genetic disorder, influencing around 600,000 individuals in the United States, with the more common autosomal dominant (AD) structure influencing approximately one out of 500 to 1,000 individuals [1].

Most patients will ultimately have these cystic kidneys, and they require dialysis or a kidney transplantation, the two of which are not great choices. For the instance, treatment of different symptoms and complications set a heavy economic burden on the medical care framework and drastically bring down patients' quality of life.

In a step toward disrupting the cycle that leads to cyst formation in the kidneys, the Weimbs Lab has now revealed a formerly unrecognized component that accelerates cystogenesis. The reaction is intended to safeguard the kidneys, the rapid dilation of the tubules that eliminates wastes from the kidneys as urine has been found to be a third-hit trigger that results in rapid development of cyst.

The kidneys are the filtration frameworks for our blood. Blood enters the nephrons (the kidneys' basic functional unit) where waste and fluids pass through the renal tubules, while cells and proteins stay in the blood. A few fluid and nutrient supplements get reabsorbed into circulation while excess of fluids and waste gets converted to urine that flows to the bladder. There are around millions of such tubules in every human kidney [2,3].

During this filtration process, waste products like calcium oxalate, calcium phosphate and uric acid tend to concentrate and precipitate into crystals in the renal tubules. In the healthy individuals, these formed microscopic crystals however are flushed away with the urine, while other factors prevent the runaway growth and retention of these crystals in the tubules. The formation and accumulation of these crystals, when left unchecked, could eventually lead to kidney stones. To eliminate out these crystals from the renal tubules has been found rapidly to dilate and then return to normal after the crystals have cleared. This dilation is a mechanism that had not been previously recognized.

It was not understood how the majority of these crystals are flushed out. Up to this point, these crystals were thought to cross through into the kidneys' interstitial tissue to be reabsorbed, yet research shows that isn't true for most crystals.

In normal-functioning kidneys, as per the study, the tubule dilation is viewed as a defensive mechanism. The deposition of oxalate crystals specifically sets off the rapid activation of protein signalling pathways (mTOR and Src/STAT3) that regulate cell growth and proliferation, accompanied by the rapid dilation of the whole tubule system to dislodge the microcrystals [4].

Kidneys are genetically preconditioned to form these cysts and it was observed that these crystals can trigger the same dilation, yet rather than returning to normal, those tubules eliminate and form cysts.

In people with Autosomal dominant polycystic kidney disease (ADPKD), the rapid and constant tubule dilation is viewed as a third-hit physical injury that results in cyst formation. As per the third-hit model of cystogenesis, three events should happen to form individual cysts: the initial two are genetic mutations, while the third is a physiological damage resulting in overcompensation by the renal tubule that leads to the formation of the fluid-filled sacs. Trauma and other different strikes to the kidneys are fairly rare, yet the microcrystals could introduce a persistent and relevant type of injury in ADPKD patients that could trigger the damage/repair response [5].

Conclusion

The researcher’s results suggest that contrary to conventional assumptions that abnormalities in tissue design or metabolic abnormalities during ADPKD progression leads to increased kidney stones, the inverse might be the situation; more crystals may lead to the progression of ADPKD. Also, as per the study, it is possible that ADPKD progression and kidney stone formation reinforce each other.

This opens up the possibility that similar well-established practices for keeping kidney stones under control may likewise prove effective for slowing the progression of ADPKD. Research suggests that the rate of progression could be at least in part determined by something like diet. Kidney stones can be prevented by avoiding intake of specific foods, increasing water intake and remedy citrate treatment, could likewise demonstrate advantageous for those with polycystic kidney illness.

References

1. Torres, Jacob A., Mina Rezaei, Caroline Broderick and Louis Lin, et al. "Crystal deposition triggers tubule dilation that accelerates cystogenesis in polycystic kidney disease." J Clin Investig 10 (2019): 4506-4522.
2. Dimitrakov, D., and S. Simeonov. "Studies on nephrolithiasis in patients with autosomal dominant polycystic kidney disease." Folia medica . “World Energy Outlook.” Paris: International Energy Agency, France (2010).
3. Torres, Vicente E., David M. Wilson, Robert R. Hattery, and Joseph W. Segura. "Renal stone disease in autosomal dominant polycystic kidney disease." Am J Kidney Dis 4 (1993):513-519.
4. Mejías, Edwin, Jorge Navas, Rosa Lluberes, and Manuel Martínez-Maldonado. "Hyperuricemia, gout, and autosomal dominant polycystic kidney disease." Am J Med Sci 3 (1989): 145-148.
5. Schrier, Robert W., Godela Brosnahan, Melissa A. Cadnapaphornchai and Michel Chonchol, et al. "Predictors of autosomal dominant polycystic kidney disease progression." J Am Soc Nephrol 11 (2014): 2399-2418.