Document Detail


Premature birth, respiratory distress, intracerebral hemorrhage, and silvery-gray hair: differential diagnosis of the 3 types of Griscelli syndrome.
MedLine Citation:
PMID:  20661159     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
SUMMARY: A preterm neonate, born to consanguineous parents, presented with respiratory distress, intracerebral hemorrhage, and a silvery-gray sheen of the hair and eyelashes. Griscelli syndrome (GS) type 3 was diagnosed after the detection of a novel homozygous mutation of the melanophilin gene. Thus, only the hypopigmentation, but not the patient's other clinical features, were attributable to this form of GS. Differential diagnosis of the various forms of GS must be performed as early as possible as GS2 is associated with a life threatening but curable immune disorder.
Authors:
Eman Al-Idrissi; Geyhad ElGhazali; Mofarah Alzahrani; Gaël Ménasché; Jana Pachlopnik Schmid; Geneviève de Saint Basile
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of pediatric hematology/oncology     Volume:  32     ISSN:  1536-3678     ISO Abbreviation:  J. Pediatr. Hematol. Oncol.     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-08-06     Completed Date:  2010-08-20     Revised Date:  2011-10-06    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  494-6     Citation Subset:  IM    
Affiliation:
Department of Immunology, King Fahad Medical City, Riyadh, Kingdom of Saudi Arabia.
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MeSH Terms
Descriptor/Qualifier:
Adaptor Proteins, Signal Transducing / genetics*
Cerebral Hemorrhage / genetics*
Child, Preschool
Diagnosis, Differential
Humans
Infant, Newborn
Infant, Premature
Piebaldism / genetics*,  physiopathology
Premature Birth
Respiratory Distress Syndrome, Newborn / genetics*
Syndrome
Chemical
Reg. No./Substance:
0/Adaptor Proteins, Signal Transducing; 0/MLPH protein, human
Comments/Corrections
Erratum In:
J Pediatr Hematol Oncol. 2011 May;33(4):319

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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