In the previous article, we briefly learned what PKD (As we mentioned before, since ARPKD is a rare disease, all PKD discussed below refer to ADPKD) is, and also know that BHB has a positive effect on PKD. In the following articles, we will get more detail about PKD and how BHB act on PKD.

Polycystic kidney disease (PKD) is a common hereditary nephropathy. The cause of polycystic kidney disease is mainly genetic factors. About half of the patients have a family history, dominant inheritance in adults, and infants are recessive. Pathologically, the kidney is often swollen, 2-3 times larger than normal.

The abnormal gene of 90% polycystic kidney disease is located on the short arm of chromosome 16, called ADPKD1 gene, and the gene product is still unclear. Many of the coding genes in this region have been elucidated and cloned, and ADPKD1 is expected to be confirmed in the near future. Another 10% of patients with abnormal genes are located on the short arm of chromosome 4, called ADPKD2 gene, and the encoded product is not clear. The ages of the two groups differed between onset, hypertension, and renal failure.

Although symptoms usually appear after adulthood, they begin to form during the fetal period. Cysts originate in the renal tubules and their fluid properties vary depending on the site of origin. If originated from the proximal tubule, the components in the cyst fluid such as Na+, K+, CI-, H+, creatinine, urea, etc. are similar to those in plasma. If it originates from the distal end, the concentration of Na+ and K+ in the cyst fluid is lower, and the concentrations of CI-, H+, creatinine and urea are higher.

In the next article, we will continue this subject. Please keep follow our next article.

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