The collecting tubule carries urine from the nephrons, or the functional units of the kidney, to the ureters and bladder.
A cyst is a balloon-like widening of the tubule. Since there are many of these tiny tubes winding through the nephrons, multiple cysts mean the kidneys can become quite enlarged.
In a typical kidney, the collecting tubule fine-tunes the amount of water and acid in the tubular fluid so that the body retains an appropriate amount of water and eliminates excess amounts of acid. In ARPKD, the cystic collecting ducts cannot retrieve water efficiently, causing excessive urine production.
The majority of children with ARPKD experience progressive loss of kidney function. However, the age at which kidney failure develops varies greatly among patients. For reasons still unknown, the size of the kidneys does not necessarily correlate with the severity of the disease.
Prenatal ARPKD Symptoms
- Diminished amniotic fluid levels
- Enlarged kidneys
- Lung underdevelopment
- Lung functioning issues
Post-Birth ARPKD Symptoms
- Enlarged kidneys
- Breathing problems
- Excessive urine production
- Hypertension
- Growth problems
- Congenital hepatic fibrosis
- Enlarged spleen
- Low red and white blood cell count
- Low platelet counts
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