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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container_title Fetal and pediatric pathology
container_volume 15
creator Gö ü, Safiye
Topçu, Meral
Küçükali, Türkan
Akçören, Zuhal
Berkel, Izzet
Ersoy, Figen
Günay, Meral
Saatçi, I il
description 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.
doi_str_mv 10.3109/15513819509026966
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source MEDLINE; Taylor & Francis Medical Library - CRKN; Access via Taylor & Francis
subjects Brain Diseases - pathology
Child, Preschool
Consanguinity
Female
Griscelli syndrome
hemophagocytosis
Histiocytosis, Non-Langerhans-Cell - pathology
Humans
Infant
lymphohistiocytosis
Male
Microscopy, Electron
partial albinism
Piebaldism - pathology
Splenomegaly - pathology
Syndrome
title Griscelli syndrome: Report of Three Cases
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