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Literature DB >> 7567250

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

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.

Entities: Disease Species

Mesh：

Year: 1995 PMID： 7567250 DOI： 10.1007/BF02011114

Source DB: PubMed Journal: Pediatr Radiol ISSN： 0301-0449

10 in total

1. MR imaging of a hepatoma associated with Alagille syndrome.

Authors: E Ong; S M Williams; J C Anderson; P A Kaplan
Journal: J Comput Assist Tomogr Date: 1986 Nov-Dec Impact factor: 1.826

2. An unusual syndrome of widened medullary cavities of the metacarpals and phalanges, aortic calcification and abnormal dentition.

Authors: E B Singleton; D F Merten
Journal: Pediatr Radiol Date: 1973-03

3. Arteriohepatic dysplasia: familial pulmonary arterial stenosis with neonatal liver disease.

Authors: G H Watson; V Miller
Journal: Arch Dis Child Date: 1973-06 Impact factor: 3.791

4. Skeletal anomalies in Alagille's syndrome. Radiographic study in eighty cases.

Authors: F Brunelle; A Estrada; J P Dommergues; O Bernard; P Chaumont
Journal: Ann Radiol (Paris) Date: 1986

5. Syndrome of intrahepatic biliary dysgenesis and cardiovascular malformations.

Authors: R D Greenwood; A Rosenthal; A C Crocker; A S Nadas
Journal: Pediatrics Date: 1976-08 Impact factor: 7.124

6. Arteriohepatic dysplasia: a benign syndrome of intrahepatic cholestasis with multiple organ involvement.

Authors: C A Riely; E Cotlier; P S Jensen; G Klatskin
Journal: Ann Intern Med Date: 1979-10 Impact factor: 25.391

7. Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur.

Authors: D Alagille; M Odièvre; M Gautier; J P Dommergues
Journal: J Pediatr Date: 1975-01 Impact factor: 4.406

8. Long-term complications of arteriohepatic dysplasia.

Authors: S J Schwarzenberg; R M Grothe; H L Sharp; D C Snover; D Freese
Journal: Am J Med Date: 1992-08 Impact factor: 4.965

9. Thoracic aortic calcification in 3 children with candidiasis-endocrinopathy syndrome.

Authors: A Shikata; T Sugimoto; K Kosaka; T Tehara; S O Kido; H Matsuo; T Sawada; W E Berdon; H G Herrod; L Parvey
Journal: Pediatr Radiol Date: 1993

10. Arteriohepatic dysplasia: radiologic features of a new syndrome.

Authors: N S Rosenfield; M J Kelley; P S Jensen; E Cotlier; A T Rosenfield; C A Riely
Journal: AJR Am J Roentgenol Date: 1980-12 Impact factor: 3.959

10 in total

5 in total

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

1. MR imaging of a hepatoma associated with Alagille syndrome.

2. An unusual syndrome of widened medullary cavities of the metacarpals and phalanges, aortic calcification and abnormal dentition.

3. Arteriohepatic dysplasia: familial pulmonary arterial stenosis with neonatal liver disease.

4. Skeletal anomalies in Alagille's syndrome. Radiographic study in eighty cases.

5. Syndrome of intrahepatic biliary dysgenesis and cardiovascular malformations.

6. Arteriohepatic dysplasia: a benign syndrome of intrahepatic cholestasis with multiple organ involvement.

7. Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur.

8. Long-term complications of arteriohepatic dysplasia.

9. Thoracic aortic calcification in 3 children with candidiasis-endocrinopathy syndrome.

10. Arteriohepatic dysplasia: radiologic features of a new syndrome.

Review 1. Developmental signaling: does it bridge the gap between cilia dysfunction and renal cystogenesis?

2. Renal anomalies in Alagille syndrome: a disease-defining feature.

Review 3. Regulatory circuits controlling vascular cell calcification.

Review 4. Renal involvement and the role of Notch signalling in Alagille syndrome.

Review 5. Alagille syndrome: case report with bilateral radio-ulnar synostosis and a literature review.