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.

Pruritus

Pruritus is an itch or a sensation that makes a person want to scratch. This term refers to an abnormally increased disposition to experience pruritus.


Total: 10

                       


(per page)
PMID (PMCID)
28492167
 MALE Child
Management of cholestatic pruritus in children with Alagille syndrome: Case report and literature review.
Ben Ameur S, Chabchoub I, Telmoudi J, Belfitouri Y, Rebah O, Lacaille F, Aloulou H, Mehrzi A, Hachicha M.
Arch Pediatr. 2016;23(12):1247-1250.
In this report, we describe a child with Alagille syndrome who presented with severe pruritus and xanthomas as a consequence of severe hypercholesterolemia and discuss the treatment modalities.
28492167
 MALE Child
Management of cholestatic pruritus in children with Alagille syndrome: Case report and literature review.
Ben Ameur S, Chabchoub I, Telmoudi J, Belfitouri Y, Rebah O, Lacaille F, Aloulou H, Mehrzi A, Hachicha M.
Arch Pediatr. 2016;23(12):1247-1250.
Alagille syndrome causes intractable pruritus and disfiguring xanthomas because of retained bile acids and cholesterol.
28492167
 MALE Child
Management of cholestatic pruritus in children with Alagille syndrome: Case report and literature review.
Ben Ameur S, Chabchoub I, Telmoudi J, Belfitouri Y, Rebah O, Lacaille F, Aloulou H, Mehrzi A, Hachicha M.
Arch Pediatr. 2016;23(12):1247-1250.
Management of cholestatic pruritus in children with Alagille syndrome: Case report and literature review.
25333075
 MALE
Unusual clinical manifestations in a case of Alagille syndrome.
Gadkari R, Doshi B, Nayak C, Ghildiyal R, Madke B, Ghia D.
Pediatr Dermatol. 2014;31(5):599-602.
We report a case of Alagille syndrome in a 3-year-old boy with associated pruritus, follicular keratoses, palmar pits, and keratoderma-like changes over the dorsum of the hands.
23337010
 MALE
The association of Alagille syndrome and craniosynostosis.
Yilmaz S, Turhan T, Mutluer S, Aydogdu S.
Pediatr Neurol. 2013;48(2):146-8.
We report a child with Alagille syndrome and craniosynostosis who presented with pruritus, elevated liver enzymes, and suspected increased intracranial pressure.
22813814
 MALE Infant
Partial internal biliary diversion for Alagille syndrome: case report and review of the literature.
Sheflin-Findling S, Arnon R, Lee S, Chu J, Henderling F, Kerkar N, Iyer K.
J Pediatr Surg. 2012;47(7):1453-6.
Alagille syndrome is a hereditary disease resulting in chronic cholestasis and hypercholesterolemia that can lead to severe and intractable pruritus and disfiguring and debilitating xanthomas.
21925870
 FEMALE Adult
Alagille syndrome and pregnancy: anesthetic management for cesarean section.
Rahmoune FC, Bruyere M, Tecsy M, Benhamou D.
Int J Obstet Anesth. 2011;20(4):355-8.
She was diagnosed with Alagille syndrome soon after birth because of cholestasis and pruritus.
18191720
 FEMALE
Intense pruritus and failure to thrive in Alagille syndrome.
Schwartz R, Rehder K, Parsons DJ, Morrell DS.
J Am Acad Dermatol. 2008;58(2 Suppl):S9-11.
Intense pruritus and failure to thrive in Alagille syndrome.
16939864
 MALE Child
Pediatric median neuropathy due to pruritus in Alagille syndrome.
Voermans NC, van Alfen N, Tolboom JJ, Koetsveld AC, Sie LT.
Pediatr Neurol. 2006;35(3):216-9.
Pediatric median neuropathy due to pruritus in Alagille syndrome.
16939864
 MALE Child
Pediatric median neuropathy due to pruritus in Alagille syndrome.
Voermans NC, van Alfen N, Tolboom JJ, Koetsveld AC, Sie LT.
Pediatr Neurol. 2006;35(3):216-9.
To our knowledge, median neuropathy has never been associated with Alagille syndrome, although severe pruritus is considered a major symptom and many patients exhibit widespread scratching and rubbing.