Alagille-Watson syndrome; arteriohepatic dysplasia; AWS; Cholestasis with peripheral pulmonary stenosis; Intrahepatic bile duct hypoplasia; Syndromal form of the "paucity of interlobular bile ducts (PILBD)"; Syndromatic hepatic ductular hypoplasia; Watson-Alagille Syndrome
HistoryThis section has been translated automatically.
Watson and Miller, 1973; Alagille, 1975
DefinitionThis section has been translated automatically.
Rare syndrome with the following symptoms:
- Chronic cholestasis with pruritus (100% of patients)
- Typical facies (85%)
- Hypoplasia or stenosis of the pulmonary artery (84%)
- Butterfly deformity of the vertebral arches (63%)
- Posterior embryotoxone (76%)
- Xanthome (28%).
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EtiopathogenesisThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
- Integument: Pruritus, xanthomatosis ( xanthomas are observed on palms and fingers, elbows, groin and popliteal fossa and buttocks ).
- Extracutaneous manifestations: Cholestasis within the first 3 months of life (normal colored or acholic stools). Hepatomegaly is always present. Bilirubin levels are not excessive (70-120 µmol/l), but extremely high cholesterol and lipid levels are found. Typical facies with prominent forehead, sunken eyes and mild hypertelorism and a small prominent chin. The so-called "posterior embryotoxon" is a white line 1-2 mm above the limbus corneae, which is found in 10% of all healthy people and 10% of all patients with cholestasis.
TherapyThis section has been translated automatically.
Treatment of the underlying disease by paediatricians, internists and surgeons.
External therapyThis section has been translated automatically.
Symptomatic local therapy of the pruritus, s.a. Pruritus, hepatic.
Radiation therapyThis section has been translated automatically.
If necessary, test with mild UVB irradiation.
Internal therapyThis section has been translated automatically.
- Colestyramine (e.g. Colestyr of ct) 4-16 g/day p.o. For children, weight-adapted dose: adult dose x kg bw of the child divided by 70.
- Trial with non-sedating oral antihistamines such as levocetirizine (e.g. Xusal) 1 time / day 1 tbl. p.o, desloratadine (e.g. Aerius) 1 time/day 1 tbl. p.o. or sedative antihistamines such as clemastine (e.g. Tavegil) 2 time/day 1 tbl., dimetinden (e.g. Fenistil) 2 time/day 1 tbl. or hydroxyzine (e.g. Atarax) 1-3 tbl/day
Progression/forecastThis section has been translated automatically.
The prognosis of alagille syndrome, i.e. the syndromal form of biliary hypoplasia, is significantly better than that of other forms of chronic intrahepatic childhood cholestasis
LiteratureThis section has been translated automatically.
- Alagille D et al (1975) Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur. J Pediatr 86: 63-71
- Alagille D (1988) Paucity of Interlobular Bile Ducts. In: Waldschmidt J, Charissi G, Schier F (eds.) Cholestasis in Neonates. Zuckerschwerdt publishing house, Munich, S. 33-46
- Dufour JF, Pratt DS (2001) Alagille syndrome with colonic polyposis. At J Gastroenterol 96: 2775-2777
- Garcia MA et al (2005) Alagille syndrome: cutaneous manifestations in 38 children. Pediatric dermatol 22: 11-14
- Krantz ID et al (2002) Alagille syndrome: chipping away at the tip of the iceberg. At J Med Genet 112: 160-162
- Lykavieris P et al (2003) Bleeding tendency in children with Alagille syndrome. Pediatrics 111: 167-170
- Odievre M et al (1981) Long-term prognosis for infants with intrahepatic cholestatis and patent extrahepatic biliary tract. Arch Dis Child 56: 373
- Watson GH, Miller V (1973) Arteriohepatic dysplasia. Familial pulmonary artery stenosis with neonatal liver disease. Arch Dis Child 48: 459-466
Outgoing links (13)Antihistamines, systemic; Clemastine; Desloratadine; Dimetinden; Haydu-cheney syndrome; Hydroxycin; Levocetirizine; Notch signaling pathway; Pruritus; Pruritus hepatic; ... Show all
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