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.

Nephritis

The presence of inflammation affecting the kidney.


Total: 2

                       


(per page)
PMID (PMCID)
1482647
 MALE Child
IgA nephritis in a patient with Alagille syndrome and a transplanted liver.
Gilboa N, Hopp L, Agostini RM.
Pediatr Nephrol. 1992;6(6):559-61.
The present report describes a patient with Alagille syndrome who presented with hematuria and IgA nephritis 7 years after an orthotopic liver transplantation and immunosuppression.
1482647
 MALE Child
IgA nephritis in a patient with Alagille syndrome and a transplanted liver.
Gilboa N, Hopp L, Agostini RM.
Pediatr Nephrol. 1992;6(6):559-61.
This patient suggests that glomerular lipidosis is not an inherent feature of the Alagille syndrome, and that IgA nephritis may develop in spite of ongoing immunosuppressive treatment.