Griscelli syndrome: Report of Three Cases

The clinical features of three children with Griscelli syndrome and autopsy findings of two are presented. The patients were 5 years, 9 months, and 3 months old, respectively. Clinical features included partial albinism, hepatosplenomegaly, and various neurological symptoms. Light and electron micro...

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Veröffentlicht in:Fetal and pediatric pathology 1995, Vol.15 (2), p.309-319
Hauptverfasser: Gö ü, Safiye, Topçu, Meral, Küçükali, Türkan, Akçören, Zuhal, Berkel, Izzet, Ersoy, Figen, Günay, Meral, Saatçi, I il
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Sprache:eng
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Zusammenfassung:The clinical features of three children with Griscelli syndrome and autopsy findings of two are presented. The patients were 5 years, 9 months, and 3 months old, respectively. Clinical features included partial albinism, hepatosplenomegaly, and various neurological symptoms. Light and electron microscopic studies of the skin were compatible with Griscelli syndrome. Postmortem examination of the viscera and central nervous system revealed lymphohistiocytic infiltration with erythrophagocytosis. Bilateral diffuse involvement of the central nervous system, cranial nerve, and spinal cord was detected in both cases.
ISSN:1551-3815
1077-1042
1551-3823
DOI:10.3109/15513819509026966