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.

Hypertension

The presence of chronic increased pressure in the systemic arterial system.


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PMID (PMCID)
30907355
 FEMALE Child
[Liver dysfunction for 8 years with hypertension for 1 week in an 8-year-old girl].
Kuang J, Zheng XR, Zhang GY, Wang X, Liu CT, Wu ML, Tang YJ.
Zhongguo Dang Dai Er Ke Za Zhi. 2019;21(3):282-286.
The rare disease Alagille syndrome should be considered when a child has refractory hypertension with the involvement of multiple systems, especially liver dysfunction with cholestasis as the main manifestation.
22923029
 FEMALE
Atherosclerosis causing recurrent catastrophic aortopulmonary shunt dehiscence in a patient with Alagille syndrome.
May L, Hanley FL, Connolly AJ, Reddy S.
Pediatr Cardiol. 2013;34(8):1945-8.
This report describes a 3 year-old girl with Alagille syndrome, hepatic cholestasis, systemic hypertension, hypercholesterolemia, hypertriglyceridemia, and tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/PA/MAPCAs).
23337010
 MALE
The association of Alagille syndrome and craniosynostosis.
Yilmaz S, Turhan T, Mutluer S, Aydogdu S.
Pediatr Neurol. 2013;48(2):146-8.
Craniosynostosis is an unusual but significant cause of increased intracranial hypertension in Alagille syndrome.
23337010
 MALE
The association of Alagille syndrome and craniosynostosis.
Yilmaz S, Turhan T, Mutluer S, Aydogdu S.
Pediatr Neurol. 2013;48(2):146-8.
Several mechanisms have been proposed to explain the mechanism of intracranial hypertension in Alagille syndrome.
20042397
 MIXED_SAMPLE Child
An unusual cause of hypertension and renal failure: a case series of a family with Alagille syndrome.
Shrivastava R, Williams A, Mikhail A, Roberts D, Richards M, Aithal V.
Nephrol Dial Transplant. 2010;25(5):1501-6.
An unusual cause of hypertension and renal failure: a case series of a family with Alagille syndrome.
16013021
 MALE Adult
Renal failure and hypertension in Alagille syndrome with a novel JAG1 mutation.
Harendza S, Hubner CA, Glaser C, Burdelski M, Thaiss F, Hansmann I, Gal A, Stahl RA.
J Nephrol. 2005;18(3):312-7.
Various renal abnormalities have also been associated with Alagille syndrome, whereas renal vascular hypertension combined with renal insufficiency has been reported in several cases.
16013021
 MALE Adult
Renal failure and hypertension in Alagille syndrome with a novel JAG1 mutation.
Harendza S, Hubner CA, Glaser C, Burdelski M, Thaiss F, Hansmann I, Gal A, Stahl RA.
J Nephrol. 2005;18(3):312-7.
Renal failure and hypertension in Alagille syndrome with a novel JAG1 mutation.
14768181
 MALE Adult
[Alagille syndrome and chronic renal failure].
Kaczynska A, Lewandowski J, Pruszczyk P, Gaciong Z.
Pol Arch Med Wewn. 2003;109(5):511-5.
We report 42-years old patient with hypertension, chronic renal failure and Alagille syndrome.