What Is ARPKD/CHF?
Autosomal Recessive Polycystic Kidney Disease (ARPKD) is a rare
genetic disorder that affects approximately 1:6,000 to 1:40,000
persons in the general population, depending on the source of
reference. (There is a higher incidence in certain populations, i.e.
Finnish and Afrikaaner.) It is a chronic and progressive disease
that causes eventual kidney failure and liver abnormalities, hence
Congenital Hepatic Fibrosis (CHF). It is neither contagious, nor
affects intelligence. CHF has the potential to cause severe clinical
liver complications. The age spectrum for onset of symptoms is from
birth to adolescence, seldom adulthood. ARPKD is commonly diagnosed
early in life; approximately 50% are diagnosed prenatally.
There is no cure and early newborn death occurs up to 50% of the
time, most often not from kidney failure, but from pulmonary
hypoplasia (underdeveloped lungs). If ventilatory support is
offered and the newborn period is survived, then the chances of
survival increase to good. There is potential for an excellent
quality lifestyle with medical management.
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