0
March 1994, Vol 148, No. 3 >
Article | March 1994

Picture of the Month FREE

László Maródi, MD; Gyula Rigó, MD; Rita Káposzta, MD
Arch Pediatr Adolesc Med. 1994;148(3):287-288. doi:10.1001/archpedi.1994.02170030057013.
Text Size: A A A
Published online
Article
References
Comments

A 1½-YEAR-OLD with recurrent episodes of jaundice that were associated with intercurrent infections is pictured in Figure 1 and Figure 2. Conjugated hyperbilirubinemia and elevated serum cholesterol and triglyceride levels were present. A roentgenogram of his chest demonstrated abnormal vertebrae (Figure 3). His father has features of the same disorder and manifests an eye abnormality (Figure 4).

Denouement and Discussion 

Alagille Syndrome (Arteriohepatic Dysplasia) 

MANIFESTATIONS  Alagille syndrome is characterized by five major features: peculiar facies (95%), chronic cholestasis (91%), posterior embryotoxon of the eye (88%), butterflylike vertebral arch defects (87%), and peripheral pulmonary hypoplasia or stenosis (85%).1,2 The syndrome is one of many causes of intrahepatic cholestasis with paucity of the bile ducts, which include alpha1-antitrypsin deficiency, congenital rubella, and cytomegalovirus infections. The incidence is estimated to be one in 100 000 births.Features of the peculiar facies are a prominent forehead, hypertelorism with deep-set eyes, a

REFERENCES

1 +
Alagille D, Estrada A, Hadchouel M, Gautier M, Odievre M, Dommergues JP.  Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases . J Pediatr . 1987;;110:195-200.
2 +
Alagille D, Odievre M, Gautier M, Dommergues JP.  Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur . J Pediatr . 1975;;86:63-71.
3 +
Byrne JLB, Harrod MJE, Friedman JM, Howard-Peebles PN.  Del (20p) with manifestations of arteriohepatic dysplasia . Am J Med Genet . 1986;;24:673-678.

Figures

Tables

Interactive Graphics

Video

Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

1 +
Alagille D, Estrada A, Hadchouel M, Gautier M, Odievre M, Dommergues JP.  Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases . J Pediatr . 1987;;110:195-200.
2 +
Alagille D, Odievre M, Gautier M, Dommergues JP.  Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur . J Pediatr . 1975;;86:63-71.
3 +
Byrne JLB, Harrod MJE, Friedman JM, Howard-Peebles PN.  Del (20p) with manifestations of arteriohepatic dysplasia . Am J Med Genet . 1986;;24:673-678.

Correspondence

Submit a Letter
CME
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Clear Answers | Save For Later
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s “Cited By” API will populate this tab (http://www.crossref.org/citedby.html).
Compression-Only CPR: Pushing the Science Forward
Cone
AAM 2010;304:1493-1495.
All you need to read in the other general journals
BMJ 2010;341:c5893-c1494.
Submit a Comment

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

© 2013 American Medical Association. All Rights Reserved.
Powered bySilverchair Information Systems