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Original Article
Brief Report
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Volume 339:1679-1686 December 3, 1998 Number 23
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Therapy with a Purified Plasminogen Concentrate in an Infant with Ligneous Conjunctivitis and Homozygous Plasminogen Deficiency
Dorothee Schott, M.D., Carl-Erik Dempfle, M.D., Peter Beck, M.D., Andreas Liermann, M.D., Anita Mohr-Pennert, M.D., Michael Goldner, M.D., Peter Mehlem, M.D., Hiroyuki Azuma, M.D., Volker Schuster, M.D., Anne-Marie Mingers, M.D., Hans Peter Schwarz, M.D., Michael Dieter Kramer, M.D., Hans Liesenhoff, M.D., and Karl Heinz Niessen

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Ligneous conjunctivitis is a rare disease characterized by acute or chronic recurrent conjunctivitis in which the conjunctival membranes acquire a wood-like consistency, due primarily to deposits of fibrin.1,2 Corneal involvement and chronic obstruction of the eye may lead to blindness. The disease is frequently associated with nasopharyngitis, tracheobronchial obstruction, otitis media, vulvovaginitis, and defective wound healing.2,3,4,5,6,7,8,9 Pseudomembranous conjunctivitis was first described in 1847 by Bouisson,10 and the term "conjunctivitis lignosa" was introduced by Borel in 1933.11 More than 100 cases have been reported in the literature, but no satisfactory treatment has yet been found. The results of therapy with hyaluronidase . . . [Full Text of this Article]

Case Report

Methods

Histologic Findings

Analysis of Mutations

Direct Detection of Mutations

Laboratory Analyses

Lys-Plasminogen

Results

Histologic Evaluation

Molecular Genetic Findings

Restriction-Fragment–Length Analysis

Recovery and Half-Life of Lys-Plasminogen

Effect of Lys-Plasminogen on Measures of Fibrinolysis and Coagulation

Clinical Course

Discussion


Source Information

From the Department of Pediatrics (D.S., P.B., M.G., P.M.), the First Department of Medicine (C.-E.D.), and the Department of Ophthalmology (A.L.), Klinikum Mannheim, University of Heidelberg, Mannheim, Germany; the Hyland-Immuno Division of Baxter Healthcare, Heidelberg, Germany (A.M.-P.), and Vienna, Austria (H.P.S.); the First Department of Internal Medicine, University of Tokushima, Tokushima, Japan (H.A.); the Department of Pediatrics, University of Wuerzburg, Wuerzburg, Germany (V.S., A.-M.M.); and the Department of Immunology, University of Heidelberg, Heidelberg, Germany (M.D.K.). Other authors were Hans Liesenhoff, M.D., Department of Ophthalmology, and Karl Heinz Niessen, M.D., Department of Pediatrics, Klinikum Mannheim, University of Heidelberg, Mannheim, Germany.Presented in part at the 41st Annual Meeting of the Gesellschaft für Thrombose- und Hämostaseforschung, Vienna, Austria, February 22–26, 1997, and at the 39th Annual Meeting of the American Society of Hematology, San Diego, Calif., December 5, 1997.

Address reprint requests to Dr. Schott at the Klinikum Mannheim Kinderklinik, Theodor-Kutzer-Ufer 1-3, D-68167 Mannheim, Germany.

References


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