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.

Biliary atresia

Atresia of the biliary tree.


合計: 4

                       


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PMID (PMCID)
26847548
 FEMALE Infant, Newborn
Neonatal Jaundice with Splenomegaly: Not a Common Pick.
Gotti G, Marseglia A, De Giacomo C, Iascone M, Sonzogni A, D'Antiga L.
Fetal Pediatr Pathol. 2016;35(2):108-11.
The most common conditions causing cholestatic jaundice in infants are biliary atresia, neonatal hepatitis, and Alagille syndrome.
10400102
 MIXED_SAMPLE Infant
Percutaneous endoscopic gastrostomy for continuous feeding in children with chronic cholestasis.
Duche M, Habes D, Lababidi A, Chardot C, Wenz J, Bernard O.
J Pediatr Gastroenterol Nutr. 1999;29(1):42-5.
Percutaneous endoscopic gastrostomy was performed in five children (age range, 20 months to 13 years) with severe cholestasis (Alagille syndrome in four; biliary atresia in one) and severe malnutrition (mean weight, -2.6 standard deviations; mean height, -2.7 standard deviations) who were awaiting liver transplantation.
2240136
 MIXED_SAMPLE Infant, Newborn
Ocular pathologic features of arteriohepatic dysplasia (Alagille's syndrome).
Johnson BL.
Am J Ophthalmol. 1990;110(5):504-12.
Arteriohepatic dysplasia (Alagille's syndrome), an autosomal dominant condition involving jaundice caused by a developmental scarcity of intrahepatic bile ducts, has characteristic cardiovascular, skeletal, facial, and ocular features that distinguish it from extrahepatic biliary atresia and an array of other neonatal intrahepatic cholestatic disorders.
3912072
 MIXED_SAMPLE Infant
[Liver transplantation in children].
Otte JB, de Hemptine B, Moulin D, Veyckemans F, Carlier MA, Buts JP, Claus D, Reynaert M, de Ville de Goyet J, Rahier J, et al..
Chir Pediatr. 1985;26(5):261-73.
The indications for liver transplantation in children are end-stage liver diseases consisting of a) cholestatic diseases among which the most frequent is biliary atresia after unsuccessful Kasai procedure followed by familial cholestasis (Byler syndrome) and the paucity of the intrahepatic bile ducts of the syndromatic (Alagille syndrome) or non syndromatic type.