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Klin Monbl Augenheilkd 2000; 217(2): 100-108
DOI: 10.1055/s-2000-10392
KLINISCHE STUDIE

Georg Thieme Verlag Stuttgart · New York

Unilaterales oder asymmetrisches PEX-Syndrom? Eine elektronenmikroskopische Studie[1] [2]

Unilateral or asymmetric PEX syndrom? An electron microscopic studyThomas Hammer1,2 , Ursula Schlötzer-Schrehardt1 , Anselm Jünemann1
  • 1 Augenklinik mit Poliklinik der Friedrich-Alexander-Universität Erlangen-Nürnberg, Schwabachanlage 6, 91054 Erlangen (Direktor: Prof. Dr. G. O. H. Naumann)
  • 2 Klinik und Poliklinik für Augenheilkunde der Martin-Luther-Universität Halle-Wittenberg, Magdeburger Str. 8, 06097 Halle/S. (Direktor: Prof. Dr. G. I. W. Duncker)
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Publication Date:
31 December 2000 (online)

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Zusammenfassung

Hintergrund Das Pseudoexfoliations(PEX)-Syndrom, charakterisiert durch die Ablagerung eines abnormalen extrazellulären Materials in intra- und extraokulären Geweben, tritt klinisch sowohl unilateral als auch bilateral auf. Die generalisierte Natur dieses Matrixprozesses spricht jedoch gegen eine strikt einseitige okuläre Ausprägung. Ziel dieser Studie war daher eine detaillierte histopathologische Analyse scheinbar nicht-involvierter Partneraugen bei so genanntem „unilateralen” PEX-Syndrom.

Material und Methoden Für die elektronenmikroskopische Analyse standen 5 Spenderaugenpaare, bei denen spaltlampenmikroskopisch, makroskopisch und lichtmikroskopisch ein unilateraler PEX-Befund beschrieben wurde, sowie 6 normale Kontrollaugen zur Verfügung. Für die immunhistochemische Analyse auf licht- und elektronenmikroskopischer Ebene wurden Antikörper gegen LTBP-1 und HNK-1, zwei bekannte Marker für PEX-Ablagerungen, verwendet.

Ergebnisse Alle scheinbar nicht involvierten kontralateralen Augen zeigten ultrastrukturelle Veränderungen im Bereich der Iris, des Ziliarkörpers und des Trabekelwerkes. Diese Veränderungen umfassten die Ablagerung typischer PEX-Fibrillen auf Iris- und Ziliarepithel sowie im Bereich des Dilatators, die Ablagerung mikrofibrillärer Vorstufen in der Peripherie der Irisgefäße, degenerative Veränderungen des Irispigmentepithels und Dilatatormuskels, und vermehrte Akkumulation extrazellulärer Matrixkomponenten im Bereich der Irisgefäße, des Dilatators und des juxtakanalikulären Bindegewebes im Trabekelwerk. Immunhistochemisch konnten bei allen kontralateralen und PEX-Augen, nicht jedoch bei Kontrollaugen, LTBP-1- und HNK-1-positive Ablagerungen in der Peripherie der Irisgefäße und im Dilatatormuskel sowohl licht- als auch elektronenmikroskopisch identifiziert werden.

Schlussfolgerung Die Befunde an kontralateralen Augen unterstreichen die Auffassung, dass es sich beim PEX-Syndrom um eine generalisierte, grundsätzlich bilaterale Erkrankung mit klinisch asymmetrischer Ausprägung handelt. Dies sollte beim klinischen Managment der Patienten berücksichtigt werden.

Background The pseudoexfoliation (PEX) syndrome which is characterized by the accumulation of an abnormal extracellular material in intra- and extraocular tissues, clinically strictly manifests both unilateral and bilateral. However, the generalized nature of this matrix process does not support unilateral ocular manifestation. The aim of this study, therefore, was a detailed histopathological analysis of the apparently not involved fellow eyes in so-called “unilateral” PEX syndrome.

Material and Methods For transmission electron microscopy, 5 pairs of donor eyes with slitlamp-microscopic, macroscopic and light microscopic evidence of unilateral PEX syndrome and 6 normal control eyes were studied. For immunohistochemistry, light and electron microscopic antibodies against LTBP-1 and HNK-1, two well-known markers for PEX deposits, were used.

Results All apparently not involved contralateral eyes showed ultrastructural changes in the iris, in the ciliary body and in the trabecular meshwork. These changes include deposits of typical PEX fibrils on iris and ciliary epithelia as well as in the iris dilatator muscle, microfibrillar precursors in the periphery of iris vessels, degenerative changes of the iris pigment epithelium and of the dilatator muscle and an increased accumulation of extracellular matrix components around iris vessels, in the dilatator muscle and in the juxtacanalicular connective tissue of the trabecular meshwork. In all contralateral and PEX eyes, but not in the control eyes, LTBP-1 and HNK-1 positive deposits could be identified in the periphery of iris vessels and in the dilatator by light- and electron microscopic immunolabeling.

Conclusions The observed alterations in contralateral eyes in so-called “unilateral” PEX syndrome support the concept that the PEX syndrome is a generalized, basically bilateral disease which may present in a clinically asymmetric manifestation. This should be considered in the clinical management of these patients.

Schlüsselwörter

Pseudoexfoliations-Syndrom - PEX - Elektronenmikroskopie - LTBP-1 - HNK-1-Epitop - Immunhistochemie

Key words

Pseudoexfoliation syndrome - LTBP-1 - HNK-1 epitope - iris vessels - unilateral - electron microscopy - immunohistochemistry

1 Manuskript eingereicht am 2. 2. 00 und in der vorliegenden Form angenommen am 21. 2. 00.

2 Herrn Prof. Dr. Gottfried O. H. Naumann gewidmet.

Literatur

1 Manuskript eingereicht am 2. 2. 00 und in der vorliegenden Form angenommen am 21. 2. 00.

2 Herrn Prof. Dr. Gottfried O. H. Naumann gewidmet.

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