Table 2.

Teaching points regarding Alagille syndrome

Chronic kidney disease is common in patients with Alagille syndrome, and 40% of patients have renal abnormalities.

There is a spectrum of histologic renal findings in Alagille syndrome, including renal dysplasia that likely results from mutations in the Notch signaling pathway.

Other renal histologic findings include mesangiolipidosis and bile cast nephropathy, highlighting the harmful effects of defects in the liver on the kidney