Polycystic kidney disease (PKD): Nursing

With polycystic kidney disease, poly- means multiple and cystic refers to fluid-filled sacs. So, polycystic kidney disease describes a condition in which fluid-filled sacs form in the kidney. There are three types of polycystic kidney disease: infantile polycystic disease, which appears in infancy or childhood; adult polycystic disease, which appears in adulthood; these first two conditions are inherited. The third type is not inherited, and is therefore called acquired polycystic disease.

First, let’s quickly review the anatomy and physiology of the kidneys. These organs are made up of an outer cortex and an inner medulla. The cortical tissue extends towards the medulla, forming renal columns that divide the medulla into pyramidal-shaped structures called the renal pyramids. Now, the cortex and the medulla house the functional units of the kidney, called the nephrons, which filter the blood and create urine.

This urine drains from the tips of renal pyramids to the minor calyces, which then drain into the major calyces. The major calyces then merge to form the renal pelvis, which drains urine into the ureters. The two ureters carry urine to the urinary bladder, which is a pelvic organ that stores urine.

During urination, urine passes from the bladder to the urethra and to the outside of the body. The kidneys can also produce hormones, such as renin and erythropoietin. Renin raises blood pressure when it falls below normal, while erythropoietin stimulates red blood cell production from the bone marrow.

Now, the causes of polycystic kidney disease depends on the type of disease. Infantile polycystic disease is caused by a mutation of the polycystic kidney and hepatic disease gene, or PKHD1 gene for short. This type is inherited in an autosomal recessive pattern, meaning that the individual needs to receive one gene mutation from each parent to have the disease, so it’s also known as autosomal recessive polycystic kidney disease, or ARPKD.