Document Detail

Ocular histopathology and ultrastructure of Morquio syndrome (systemic mucopolysaccharidosis IV A).
MedLine Citation:
PMID:  2119328     Owner:  NLM     Status:  MEDLINE    
Morquio syndrome (mucopolysaccharidosis IV) is a hereditary lysosomal storage disease characterized by dwarfism, spondyloepiphyseal and dental abnormalities, corneal opacification, and normal intelligence. We report the light and electron microscopic features of two patients with mucopolysaccharidosis type IV A (MPS IV A). Variable degrees of mucopolysaccharide deposition were seen in tissue surveyed by light microscopy. Transmission electron microscopy demonstrated fibrillogranular and multimembranous membrane-bound inclusions distributed primarily in the cornea and trabecular meshwork, to a milder degree in the conjunctiva and sclera, and rarely in the retinal pigment epithelium. Our findings indicate that the accumulation of mucopolysaccharide in MPS IV A occurs primarily in the structures of the anterior segment.
M Iwamoto; Y Nawa; I H Maumenee; J Young-Ramsaran; R Matalon; W R Green
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv für klinische und experimentelle Ophthalmologie     Volume:  228     ISSN:  0721-832X     ISO Abbreviation:  Graefes Arch. Clin. Exp. Ophthalmol.     Publication Date:  1990  
Date Detail:
Created Date:  1990-10-22     Completed Date:  1990-10-22     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8205248     Medline TA:  Graefes Arch Clin Exp Ophthalmol     Country:  GERMANY, WEST    
Other Details:
Languages:  eng     Pagination:  342-9     Citation Subset:  IM    
Wilmer Ophthalmological Institute, Baltimore, MD.
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MeSH Terms
Anterior Eye Segment / ultrastructure
Cells, Cultured
Conjunctiva / ultrastructure
Cornea / ultrastructure
Eye / ultrastructure*
Fibroblasts / enzymology
Mucopolysaccharidosis IV / pathology*
Trabecular Meshwork / ultrastructure

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

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