Ligneous Conjunctivitis
[The case of Enea]
Achille Mauro Porcheddu 

congiuntivite lignea - conjuntivitis leńosa - ligneous conjonctivite - Hölzerne Bindehautentzündung -
De houtige blindvliesontsteking - ligneous conjunctivitis - conjuntivite ligneous - Houtgewassen conjunctivitis -

Ligneous Conjunctivitis: Translation [languages:24]

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Always seek advice from a qualified physician
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By Achille Mauro Porcheddu
[Enea's Father]
achille@budoniambiente.org

All Rights Reserved.


LLigneous conjunctivitis (McKusick 217090) is a rare form of chronic conjunctivitis characterized by the development of firm fibrin-rich, woody-like pseudomembraneous lesions mainly on the tarsal conjunctivae. Less frequently, similar lesions may occur on other mucous membranes of the body indicating that these manifestations are part of a systemic disease. Histopathological findings from affected humans and (plasminogen-deficient) mice indicate that wound healing, mainly of injured mucosal tissue, is impaired due to markedly decreased (plasmin-mediated) extracellular fibrinolysis. Pseudomembraneous lesions of the eyes and other mucosal tissue mainly contain clotted fibrin(ogen). Actually, systemic plasminogen deficiency has been linked to ligneous conjunctivitis in humans and mice. In one case, ligneous conjunctivitis has been induced by antifibrinolytic treatment with tranexamic acid. Further rare associated disorders of ligneous conjunctivitis are congenital occlusive hydrocephalus and juvenile colloid milium. This review outlines the historical background, clinical characteristics of ligneous conjunctivitis and its associated complications, histological abnormalities of pseudomembraneous lesions, inheritance, hemostasiologic and molecular genetic findings in affected patients, current treatment approaches, and the plasminogen-deficient mouse as an animal model.
(Surv Ophthalmol 48:369–388, 2003. 2003 by Elsevier Inc. All rights reserved.)

TThe prevalence of (heterozygous) type I plasminogen deficiency has been recently calculated as 0.26% (25 of 9,611 subjects) in a large epidemiological study in the United Kingdom.The theoretically predicted prevalence of homozygotes/compound-heterozygotes would therefore be in the range of 1.6 per 1,000,000 people. If we further assume that the majority of cases of ligneous conjunctivitis are due to homozygous or compound-heterozygous type I plasminogen deficiency (discussed later in the review), the prevalence of ligneous conjunctivitis may be roughly estimated in a similar range,that is, 1.6 × 10(-6), at least in European countries.
In countries where consanguinity is found more frequently (namely, Turkey and others) the prevalence of ligneous conjunctivitis may be higher. Further epidemiologic studies should be able to evaluate the correct frequency of distinct plasminogen gene mutations associated with ligneous conjunctivitis in different parts of the world.
Volker Schuster, MD,(1) and Stefan Seregard, MD, PhD(2)
(1)Department of Pediatrics, Leipzig University Medical School, Leipzig, Germany; and (2)Ophthalmic Pathology and Oncology Service, Saint Eriks Eye Hospital and Karolinska Institute, Stockholm, Sweden

Prevalence of the disease in the world (verified cases) under construction - update 2007

Ligneous Conjunctivitis: prevalence of the disease in the world (verified cases)

MANAGEMENT (Therapyes)

Ligneous Conjunctivitis: Management

Human Plasminogen Therapy - NEWS
The Human Plasminogen-Drops is the current therapy of ENEA

February 2008 - (EMEA) Human medicines - Orphan medicinal products
Human plasminogen, from Kedrion S.p.A, for treatment of ligneous conjunctivitis
Summaries of opinion on orphan designation
© 1995-2008 EMEA
The Human Plasminogen-Drops is the current therapy of ENEA

16/01/08 - (EMEA) (What's New Archive - January 2008)
Human plasminogen, from Kedrion S.p.A, for treatment of ligneous conjunctivitis
Pre-authorisation Evaluation of Medicines for Human Use
Document Date: London, 16 January 2008 - Doc.Ref.: EMEA/COMP/285792/2007
COMMITTEE FOR ORPHAN MEDICINAL PRODUCTS PUBLIC SUMMARY
OF POSITIVE OPINION FOR ORPHAN DESIGNATION OF
human plasminogen for the treatment of ligneous conjunctivitis [ Download: PDF Version (100 KB) ]
The Human Plasminogen-Drops is the current therapy of ENEA

15/10/07 - (Kedrion)
Human plasminogen, from Kedrion S.p.A, for treatment of ligneous conjunctivitis
HUMAN PLASMINOGEN ACKNOWLEDGED AS ORPHAN DRUG
The European Commission, following the positive opinion of the European Medicines Agency (EMEA), has granted the designation of orphan drug to Human Plasminogen, made by Kedrion, in the form of eye drops used for the treatment of ligneous conjunctivitis.
The Human Plasminogen-Drops is the current therapy of ENEA

3/08/2007 (EUROPEAN COMMISSION)
Human plasminogen, from Kedrion S.p.A, for treatment of ligneous conjunctivitis
Register of designated Orphan Medicinal Products Human plasminogen
EU orphan designation number: EU/3/07/461
Active ingredient: Human plasminogen
Indication: Treatment of ligneous conjunctivitis
Sponsor: Kedrion S.p.A.
Decision: C(2007)3840 of 3/08/2007
Link:http://ec.europa.eu/enterprise/pharmaceuticals/register/o461.htm
The Human Plasminogen-Drops is the current therapy of ENEA

27 June 2007 (EMEA/COMP/276840/2007 Corr.)-
Human plasminogen, from Kedrion S.p.A, for treatment of ligneous conjunctivitis
EMEA -European Medicines Agency. Pre-authorisation Evaluation of Medicines for Human Use. PRESS RELEASE - Committee for Orphan Medicinal Products - June 2007 Meeting COMP Opinions for Orphan Medicinal Product Designation. The Committee adopted 10 positive opinions on orphan medicinal product designation during this meeting:... Human plasminogen, from Kedrion S.p.A, for treatment of ligneous conjunctivitis (review time:day 62)...
Download (PDF file): June 07 COMP Press Release
The Human Plasminogen-Drops is the current therapy of ENEA

Other documents

1 June 2006 to 31 May 2007 (Orphanews Europe-supported by the European Commission's)
Rare Diseases Task Force - Programme of Community Action on Rare Diseases - Contract 2004105
RDTF Scientific Secretariat - Second Annual Scientific - Report
[Download: Rare Diseases Task Force]
Project Leader: Dr Ségolčne Aymé, INSERM, Paris, France

June 13, 2005
Biological Therapeutics for Rare Plasma Protein Disorders Public Workshop [ Download: PDF Version (440 KB) ]
Lister Hill Center, Bldg. 38 A - National Institutes of Health - 8800 Rockville Pike - Bethesda, MD 20894

THE HEMOPHILIA BULLETIN (August 2005) [ Download: PDF Version (188 KB) ]
August, second issue of 2005. Carol K. Kasper, M.D.,
Orthopaedic Hospital, 2400 S. Flower St., Los Angeles, CA 90007;carolkasper@hotmail.com.
This publication may be copied for educational purposes.
We are grateful for the unrestricted support of this issue by Baxter-Advate®
(.......)
The development of several other products, for conditionsother than bleeding disorders, was instructive. Amy Shapiro described a rare and miserable condition, plasminogen deficiency, whichcauses ligneous conjunctivitis (a fibrous gluing together of the eye-lids leading to blindness) or ligneous cervicitis (causing infertility) orgingivitis or other involvement of mucous membranes. A fibrinolytic agent containing human plasminogen, Eminase®, is licensed in the USA but no longer marketed here. It‘s available in Europe. The manu-facturer decide that it was not feasible to update the biologics license application at the FDA when the market is so small. There is no concentrate of plasminogen alone. Dr. Shapiro has four affected patients. She showed a picture of a baby whose eyelids were sealed with crustylesions, and then a picture of the same child after treatment with Eminase®. The end-point is good: it‘s visible and obvious. Whole plasma also can be used but does not work as well, a situation reminiscent ofhemophilia.
(.......)


GeneCard for protein-coding PLG

GeneCard: developed at the Crown Human Genome Center & Weizmann Institute of Science

GeneCards® is an integrated database of human genes that includes automatically-mined genomic, proteomic and transcriptomic information, as well as orthologies, disease relationships, SNPs, gene expression, gene function, and service links for ordering assays and antibodies.


Other correlated documentations

" ASPECTOS EPIDEMIOLOGICOS DE LAS CONJUNTIVITIS EN NUESTRO MEDIO.": TESIS DOCTORAL - Autor: JORGE ALVAREZ MARÍN

TRABAJO PRESENTADO POR: JORGE ALVAREZ MARÍN para optar al grado de Doctor en Medicina
La Laguna, Diciembre de mil novecientos noventa y siete.



- - - THE MUSIC FOR ENEA - - -

Autori del sito

Achille Mauro Porcheddu
e Angela Maria Cossu
(Enea's parents)
Via Biella, 12
07026 OLBIA
Tel. 0789 64739
Cell. 347 7865073
Mail: EMAIL

page in construction - UPDATE 22 January 2010


Starded: 30 January 2009
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