| An atypical ultrastructural pattern in Fabry's disease: a study on its nature and incidence in 7 cases. | |
| | |
MedLine Citation:
|
PMID: 2126406 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
This is a report on a stored lipid atypical ultrastructural pattern in skin samples of Fabry's disease expressed exclusively in the endothelium. The pattern consisted of intersecting short crescentic tightly packed membranes with a periodicity identical to that in classical ultrastructural variants. At low magnification the lysosomal aggregates of the material resembled "sunbursts" or aggregates of densely packed squirming villus-like structures. According to results of ultrastructural, lipid, and lectin histochemical analyses including analysis of the patients' blood groups, it could be concluded that it is just a variant physical state of the otherwise typical Fabry lipid. Its origin could be attributed to impeded formation (or maintenance) of larger lipid lamellae. It was found in great amounts in skin capillaries in 2 cases, and rarely in 5 additional cases. Knowledge of this atypical ultrastructural pattern is of practical significance because it could, if prevalent, cause diagnostic problems. |
| | |
Authors:
|
M Elleder; J Ledvinová; F Vosmík; J Zeman; D Stejskal; A Lageron |
Related Documents
:
|
21888566 - The first report of bilateral retropharyngeal lymph node metastasis from papillary thyr... 128476 - Occupational cobalt and chromium dermatitis in an offset printing factory. 23569386 - Syringocystadenoma papilliferum in the right lower abdomen: a case report and review of... 1178096 - Spinal neurinomas.--a comprehensive clinical and statistical study on 47 cases. 1552426 - Infantile gynecomastia with bloody nipple discharge. 14991956 - Definitive palliation for neoplastic colonic obstruction using enteral stents: personal... |
Publication Detail:
|
Type: Journal Article |
Journal Detail:
|
Title: Ultrastructural pathology Volume: 14 ISSN: 0191-3123 ISO Abbreviation: Ultrastruct Pathol Publication Date: 1990 Nov-Dec |
Date Detail:
|
Created Date: 1991-03-14 Completed Date: 1991-03-14 Revised Date: 2009-06-26 |
Medline Journal Info:
|
Nlm Unique ID: 8002867 Medline TA: Ultrastruct Pathol Country: UNITED STATES |
Other Details:
|
Languages: eng Pagination: 467-74 Citation Subset: IM |
Affiliation:
|
1st Institute of Pathology, School of Medicine, Charles University, Paris, France. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adolescent Adult Fabry Disease / metabolism, pathology* Glycolipids / metabolism Histocytochemistry Humans Lectins Lipid Metabolism Microscopy, Electron Retrospective Studies Skin / enzymology, metabolism, ultrastructure* |
| Chemical | |
Reg. No./Substance:
|
0/Glycolipids; 0/Lectins |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: The comparative analysis of the mutational variability and instability of the thi, Bd strains induce...
Next Document: Killing of Drosophila larvae by the fields of an electrohydraulic lithotripter.