According to the 1998 USRDS, the annual incidence of pediatric ESRD is 1,087, or 13 per million.1 Many causes of pediatric ESRD are unique (Table 13B.1). Alport's syndrome is the association of nephropathy, deafness, and cataracts. Infantile polycystic kidney disease (ARPKD) is autosomal recessive, occurs one in 10,000 to one in 40,000 live births, and involves both kidneys and liver. Adult or autosomal dominant polycystic kidney disease (AKPDK) occurs one in 250 live births, but only 10% present in the first two decades of life. Hemolytic uremic syndrome (HUS) is a disease of infancy characterized by microangiopathic hemolytic anemia, renal cortical necrosis, renal failure, and thrombocytopenia. IgA nephropa-thy (Berger's disease) presents as a respiratory illness, hematuria, and glomerular mesangial IgA deposits. Henoch Schonlein syndrome (HSP) is characterized by purpuric skin lesions, abdominal pain, arthralgia, and renal dysfunction. Cysti-nosis is a rare, autosomal recessive, lysosomal storage disease that leads to the accumulation of intracellular cystine, cell death, and eventual end organ failure. Renal transplantation is appropriate for all diseases listed in Table 12B.1.
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