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.

Peripheral pulmonary artery stenosis

Stenosis of a peripheral branch of the pulmonary artery.


合計: 8

                       


（表示件数）
PMID (PMCID)
30160645
 MALE Infant
Successful living donor liver transplantation after stent implantation in a patient with Alagille syndrome and severe bilateral pulmonary artery stenosis.
Akagi K, Tanaka T, Baba S.
Cardiol Young. 2018;28(12):1465-1467.
Here, we report successful living donor liver transplantation after bilateral pulmonary artery stent implantation in a patient with Alagille syndrome, severe bilateral peripheral pulmonary artery stenosis, and extremely high main pulmonary artery and right ventricle pressures.
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.
Alagille syndrome, also known as arteriohepatic dysplasia, is a multisystem autosomal dominant disorder characterized by chronic cholestasis due to a paucity of intrahepatic bile ducts, peripheral pulmonary artery stenosis with associated vertebral, ocular, and renal anomalies, and characteristic facies.
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.
Alagille syndrome, also known as arteriohepatic dysplasia, is a multisystem autosomal dominant disorder characterized by chronic cholestasis due to a paucity of intrahepatic bile ducts, peripheral pulmonary artery stenosis with associated vertebral, ocular, and renal anomalies, and characteristic facies.
22927862
(3425039)
 OTHER
Williams-Beuren's Syndrome: A Case Report.
Zamani H, Babazadeh K, Fattahi S, Mokhtari-Esbuie F.
Case Rep Med. 2012;2012:585726.
Peripheral pulmonary artery stenosis is associated with Noonan syndrome, Alagille syndrome, Cutis laxa, Ehler-Danlos syndrome, and Silver-Russel syndrome.
20382546
 MALE Adult
Multiple abdominal vascular anomalies in a patient with Alagille syndrome.
Kayhan A, Ilkhchoui Y, Venu N, Jensen DM, Oto A.
J Vasc Interv Radiol. 2010;21(6):937-40.
The most common vascular manifestation of Alagille syndrome is peripheral pulmonary artery stenosis.
16499598
 MALE Infant
Severe peripheral pulmonary artery stenosis is not a contraindication to liver transplantation in Alagille syndrome.
Ozcay F, Varan B, Tokel K, Cetin I, Dalgic A, Haberal M.
Pediatr Transplant. 2006;10(1):108-11.
We described a case of Alagille syndrome with severe peripheral pulmonary artery stenosis and very high right ventricular pressure that underwent successful living-related liver transplantation without any peri-operative and mid-term postoperative complication because of this cardiac malformation.
9566070
 FEMALE Infant, Newborn
Balloon pulmonary valvuloplasty and stent implantation. For peripheral pulmonary artery stenosis in Alagille syndrome.
Saidi AS, Kovalchin JP, Fisher DJ, Ferry GD, Grifka RG.
Tex Heart Inst J. 1998;25(1):79-82.
For peripheral pulmonary artery stenosis in Alagille syndrome.
7825452
 FEMALE Child
Alagille syndrome with a spontaneous appearance of the interlobular bile ducts.
Fujisawa T, Kage M, Ushijima K, Kimura A, Ono E, Kato H.
Acta Paediatr Jpn. 1994;36(5):506-9.
We report a rare case of a 10 year old girl diagnosed as having Alagille syndrome on the basis of a characteristic face, posterior embryotoxon, cholestasis, peripheral pulmonary artery stenosis and the absence of interlobular bile ducts in a liver biopsy at 1.5 years of age.