Alagille syndrome

A rare syndrome variably characterized by chronic cholestasis due to paucity of intrahepatic bile ducts, peripheral pulmonary artery stenosis, vertebrae segmentation anomalies, characteristic facies, posterior embryotoxon/anterior segment abnormalities, pigmentary retinopathy, and dysplastic kidneys.

Proteinuria

Increased levels of protein in the urine.


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PMID (PMCID)
20091053
 FEMALE
Glomerular basement membrane lipidosis in Alagille syndrome.
Davis J, Griffiths R, Larkin K, Rozansky D, Troxell M.
Pediatr Nephrol. 2010;25(6):1181-4.
We report a 5-year-old girl with Alagille syndrome who presented with persistent subnephrotic proteinuria and renal tubular acidosis.