00:00 / 00:00
Renal tubular acidosis
Minimal change disease
Focal segmental glomerulosclerosis (NORD)
Rapidly progressive glomerulonephritis
IgA nephropathy (NORD)
Acute tubular necrosis
Renal papillary necrosis
Renal cortical necrosis
Chronic kidney disease
Polycystic kidney disease
Multicystic dysplastic kidney
Medullary cystic kidney disease
Medullary sponge kidney
Renal artery stenosis
Renal cell carcinoma
Nephroblastoma (Wilms tumor)
Posterior urethral valves
Hypospadias and epispadias
Lower urinary tract infection
Transitional cell carcinoma
Non-urothelial bladder cancers
Congenital renal disorders: Pathology review
Renal tubular defects: Pathology review
Renal tubular acidosis: Pathology review
Acid-base disturbances: Pathology review
Electrolyte disturbances: Pathology review
Renal failure: Pathology review
Nephrotic syndromes: Pathology review
Nephritic syndromes: Pathology review
Urinary incontinence: Pathology review
Urinary tract infections: Pathology review
Kidney stones: Pathology review
Renal and urinary tract masses: Pathology review
Polycystic kidney disease
0 / 14 complete
0 / 2 complete
polycystic kidney disease and p. 71
Tanner Marshall, MS
Polycystic kidney disease, or PKD, is a genetic disease in which the kidneys become filled with hundreds of cysts, or fluid-filled sacs, causing them to be larger than normal and to quit functioning over time.
These cysts develop in the outer layer—the cortex, as well as the inner layer—the medulla—of both kidneys.
These cysts, which are lined with renal tubular epithelium, fill up with fluid and get larger and larger over time, making the kidneys much larger than normal.
The blood vessels that feed neighboring healthy nephrons can get compressed by growing cysts, which literally starves them of oxygen.
Poorly perfused kidneys respond by activating the renin-angiotensin-aldosterone system, which facilitates fluid retention and leads to hypertension.
Also, expanding cysts can compress the collecting system, causing urinary stasis, and in some cases this can lead to kidney stones.
Additionally, destruction of the normal renal architecture can cause symptoms like flank pain and hematuria, or blood in the urine.
Over time, as enough nephrons are affected, it leads to renal insufficiency and eventually renal failure.
Now the first type of PKD is autosomal dominant PKD or ADPKD, which used to be called adult PKD, since symptoms usually manifest in adulthood.
The first gene responsible for ADPKD is PKD1, which when mutated causes the more severe and earlier onset variety, and PKD2, which when mutated causes less severe disease and is also later in onset. PKD1 and PKD2 code for the polycystin 1 and polycystin 2 proteins, respectively, which are components of the primary cilium.
Now, the primary cilium is an appendage that sticks out from most cells in the body and receives developmentally important signals.
More specifically, in the nephron, as the urinary filtrate flows by and cause it to bend, polycystin 1 and polycystin 2 respond by allowing calcium influx, which activates pathways in the cell that inhibit cell proliferation.
Polycystic kidney disease or PKD, is a genetic disorder in which the kidneys become filled with hundreds of cysts, causing them to be larger than normal and to fail over time. PKD presents with high blood pressure, headaches, abdominal pain, blood in the urine, and excessive urination. Other symptoms include pain in the back, and cyst formation (renal and other organs). PKD comes in two varieties: autosomal dominant, which presents in adulthood, and autosomal recessive, which presents in infancy or even before birth. Treatment of PKD typically involves medications to control symptoms, lifestyle changes, and in some cases, surgery.
Copyright © 2023 Elsevier, except certain content provided by third parties
Cookies are used by this site.
USMLE® is a joint program of the Federation of State Medical Boards (FSMB) and the National Board of Medical Examiners (NBME). COMLEX-USA® is a registered trademark of The National Board of Osteopathic Medical Examiners, Inc. NCLEX-RN® is a registered trademark of the National Council of State Boards of Nursing, Inc. Test names and other trademarks are the property of the respective trademark holders. None of the trademark holders are endorsed by nor affiliated with Osmosis or this website.