Intestinal lymphangiectasia in a patient with Zellweger cerebrohepatorenal syndrome.

Erdem, G; Oran, O; Kotiloglu, E; Kale, G; Topçu, M; Renda, Y

Erdem, G; Oran, O; Kotiloglu, E; Kale, G; Topçu, M; Renda, Y.

Affiliation

Erdem G; Department of Pediatrics, Hacettepe University Children's Hospital, Ankara, Turkey.

Am J Med Genet ; 58(2): 152-4, 1995 Aug 28.

Article in En | MEDLINE | ID: mdl-8533807

ABSTRACT

ABSTRACT

Zellweger cerebrohepatorenal syndrome (ZWCHRS) is an autosomal-recessive disease, characterized by the absence or profound deficiency of peroxisomes. We report a case of ZWCHRS with intestinal lymphangiectasia, observed as an autopsy finding. This combination is previously unreported.

Subject(s)

Lymphangiectasis, Intestinal/complications; Zellweger Syndrome/complications; Female; Humans; Infant, Newborn; Lymphangiectasis, Intestinal/genetics; Lymphangiectasis, Intestinal/pathology; Zellweger Syndrome/genetics

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PubMed Links

Database: MEDLINE Main subject: Zellweger Syndrome / Lymphangiectasis, Intestinal Limits: Female / Humans / Newborn Language: En Year: 1995 Type: Article

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PubMed Links

Database: MEDLINE Main subject: Zellweger Syndrome / Lymphangiectasis, Intestinal Limits: Female / Humans / Newborn Language: En Year: 1995 Type: Article