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CT显示一名患有阿拉吉列综合征的青少年存在主动脉钙化和肾囊肿。

Aortic calcification and renal cysts demonstrated by CT in a teenager with Alagille syndrome.

作者信息

Pombo F, Isla C, Gayol A, Bargiela A

机构信息

Department of Radiology, Hospital Juan Canalejo, La Coruña, Spain.

出版信息

Pediatr Radiol. 1995;25(4):314-5. doi: 10.1007/BF02011114.

DOI:10.1007/BF02011114
PMID:7567250
Abstract

Alagille syndrome, or arteriohepatic dysplasia, is a disorder characterized by paucity of intrahepatic bile ducts, peculiar facies and skeletal anomalies. We report a typical case of this syndrome in an 18-year-old girl, in whom abdominal CT showed bilateral renal cysts and aortic wall calcification, findings unreported in the radiological literature.

摘要

阿拉吉耶综合征，又称动脉肝发育不良，是一种以肝内胆管稀少、特殊面容和骨骼异常为特征的疾病。我们报告一名18岁女孩患此综合征的典型病例，其腹部CT显示双侧肾囊肿和主动脉壁钙化，这些发现放射学文献中未报道过。

相似文献

1
Aortic calcification and renal cysts demonstrated by CT in a teenager with Alagille syndrome.CT显示一名患有阿拉吉列综合征的青少年存在主动脉钙化和肾囊肿。
Pediatr Radiol. 1995;25(4):314-5. doi: 10.1007/BF02011114.
2
Not only Alagille syndrome. Syndromic paucity of interlobular bile ducts secondary to HNF1β deficiency: a case report and literature review.不仅是 Alagille 综合征。HNF1β 缺陷导致的肝内胆小管发育不良综合征：病例报告及文献复习。
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[Alagille syndrome and chronic renal failure].[阿拉吉列综合征与慢性肾衰竭]
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Unusual clinical manifestations in a case of Alagille syndrome.一例阿拉吉列综合征的不寻常临床表现。
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Splenic hamartomas in Alagille syndrome: case report and literature review.阿拉吉耶综合征中的脾错构瘤：病例报告及文献综述
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引用本文的文献

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Kidney and vascular involvement in Alagille syndrome.阿拉吉耶综合征中的肾脏和血管受累情况。
Pediatr Nephrol. 2025 Apr;40(4):891-899. doi: 10.1007/s00467-024-06562-8. Epub 2024 Oct 24.
2
Diseases of bile duct in children.儿童胆管疾病。
World J Gastroenterol. 2024 Mar 7;30(9):1043-1072. doi: 10.3748/wjg.v30.i9.1043.
3
Developmental signaling: does it bridge the gap between cilia dysfunction and renal cystogenesis?发育信号传导：它能弥合纤毛功能障碍与肾囊肿发生之间的差距吗？

本文引用的文献

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Thoracic aortic calcification in 3 children with candidiasis-endocrinopathy syndrome.
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Renal anomalies in Alagille syndrome: a disease-defining feature.Alagille 综合征的肾脏异常：一种具有疾病定义特征的表现。
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Alagille syndrome: case report with bilateral radio-ulnar synostosis and a literature review.阿拉吉列综合征：伴有双侧桡尺骨融合的病例报告及文献综述
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Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur.肝内小胆管发育不全，伴有特殊面容、脊柱畸形、身体、智力及性发育迟缓以及心脏杂音。
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10
Arteriohepatic dysplasia: a benign syndrome of intrahepatic cholestasis with multiple organ involvement.动脉肝发育不良：一种伴有多器官受累的肝内胆汁淤积良性综合征。
Ann Intern Med. 1979 Oct;91(4):520-7. doi: 10.7326/0003-4819-91-4-520.