Medullary Sponge Kidney also known as Cacchi-Ricci disease.
Medullary sponge kidney is a congenital disorder of the kidneys characterized by a cystic dilatation of the collecting tubules in one or both kidneys. MSK affects about 1 person per 5,000 to 20,000 people in the United States. The internal part of the kidney is called the medulla, while the outer region is known as the cortex. The cortex and medulla include about one million tiny units called nephrons. Each nephron is made up of a very little filter (glomerulus) that is attached to a tubule. As blood passes through the nephron, fluid and waste products are filtered out.
Normally most of the fluid is returned to the blood, while the waste products are accumulated in any surplus fluid and flow down to the bladder as urine. In a person with medullary sponge kidney, cysts reason the collecting tubule to become abnormally wide, which makes the drainage of urine slow and inefficient. Waste products such as excess calcium build up in the kidneys. Kidney stones may form within kidney tissue. Sometimes, kidney stones can form inside the cysts. Medullary Sponge Kidney caused by MSK includes hematuria, or blood in urine; kidney stones; and urinary tract infections (UTIs).
These problems do not generally appear until the ages of 30 to 40. Medullary sponge kidney causes no symptoms most of the time, but a person with the disorder is prone to developing painful kidney stones, blood in the urine, and kidney infections. Infection causes fever; back and flank pain; cloudy, frequent, and burning urine; and general discomfort. Stones cause pain in the flank or groin as they pass. They usually cause some bleeding. Symptoms occur primarily in adults aged 20-50 years; however, infants as young as 2 years. There is no cure for medullary sponge kidney. Medications to discourage the formation of kidney stones.
Prophylactic antibiotics may help patients with medullary sponge kidney and recurrent UTIs. Sometimes a thiazide diuretic or amiloride is recommended. Stones may need to be surgically removed. Stone removal may require a procedure called lithotripsy, which uses sound waves to break stones into sand-like particles. The particles can then pass easily through the urinary tract with the flow of urine. Drinking large amounts of fluid every day to discourage the formation of kidney stones Prevention is better than cure. Avoiding non-steroidal anti-inflammatory (NSAIDs) medications.
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