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UPDATE: 22 January 2010

THERAPIES

Therapies for the treatment of Care Ligneous Conjunctivitis and their effectiveness


Therapies and the effectiveness


Systemic medication: plasminogen replacement

Reported Therapeutic Options
Topical therapies
- Plasminogen
- Fresh frozen plasma
- Excision
- Transplant of amniotic membranes
- Cyclosporin
- Corticosteroids
- Heparin
- Hyaluronidase
- α-chymotrypsin
- Azothioprine
- Plasmin

Systemic therapies

- Plasminogen
- Fresh frozen plasma
- Excision
- Transplant of amniotic membranes
- Cyclosporin
- Corticosteroids
- Heparin
- Hyaluronidase
- α-chymotrypsin
- Azothioprine
- Plasmin

Legend:
Personal efficacy rating

Source: Biological Therapeutics for Rare Plasma Protein Disorders June 13, 2005
Rare Bleeding Disorders: The Physician’s Perspective On Therapeutic Needs
Amy D. Shapiro, M.D. - Medical Director - Indiana Hemophilia & Thrombosis Center - Indianapolis, Indiana


Download del documento integrale della conferenza ( amy.pdf )


Management of Ligneous Conjunctivitis

THE PLASMINOGEN DROPS by KEDRION S.p.a. - This is the current therapy of ENEA
[this site: Human Plasminogen Drops, the Therapy by Enea's parents]
Long-term efficacy of surgical removal of pseudomembranes in a child
with ligneous conjunctivitis treated with plasminogen eyedrops [Plasminogen-Drops].

Roberto Caputo[1]; Neri Pucci[2]; Francesca Mori[2]; Jacopo Secci[1]; Elio Novembre[2]; Riccardo Frosini[3]
[1]Pediatric Ophthalmology Unit A, Meyer Children’s Hospital, Florence, Italy;
[2]Allergology Unit A, Meyer Children’s Hospital, Florence, Italy;
[3]Pediatric Ophthalmology Unit, Careggi Hospital, Florence, Italy

Download official document: Thromb Haemost 2008; 100: 1196–1198
Source:
http://www.schattauer.de/index.php?id=1882&no_cache=1&tx_ivabonnenten_pi5[download]=1&tx_ivabonnenten_pi5[artikel]=23991 (Deleted)

Topical hyaluronidase (1.5 mg/ml) alone or in combination and alpha-chymotrypsin (0.2 mg/ml).

Topical cromolyn (disodium cromoglycate)

Combination of corticosteroids and cyclosporine A (2%; 20 mg/ml), an immunosuppressive drug

Topical cyclosporine A (0.1%; 1 mg/ml solution) in combination with fluorometholon (0.1%)

Systemic use of azathioprine, an immunosuppressive drug

DeCock et al have initiated a new approach in 17 patients with ligneous conjunctivitis. Treatment consists of surgical excision of pseudomembranes and immediate, perioperative topical treatment with intensive (every 30 to 60 min) standard Heparin(1,000 or 5,000 units/ml) in combination with topical corticosteroids (0.5% or 1% prednisolone and/or 0.1% dexamethasone), and, in 12 patients, with alpha-chymotrypsin (2,500 or 5,000 units/ml).
Overall, ligneous conjunctivitis in 13 (76.4%) of the 17 patients was successfully controlled.
Trigger factors for recurrences in 3 patients were shown to be early treatment withdrawal or systemic febrile illness. In this series, the potential benefit of alpha-chymotrypsin was not clearly shown. Therefore, the combination of heparin and corticosteroids seems to be logical: Heparin accelerates the activity of antithrombin III, which itself inhibits thrombin formation and activity, thereby blocking the conversion of fibrinogen to fibrin. Heparin also neutralizes factor Xa, preventing formation of thrombin from prothrombin. Moreover, topical corticosteroids reduces conjunctival inflammation.

Combination the antiproliferative agent mitomycin C

Triple combination of topical corticosteroids, heparin, and cyclosporine A (2%)

Topical treatment with a (not further characterized)fibrinolytic agent (fibrinolysin) (initially eye drops with a concentration of 5,000 units/ml over 15 minutes, and later 1,000 units/ml every hour) in a child with ligneous conjunctivitis softened the consistency of pseudomembranes, decreased the viscosity of the serofibrinous discharge and delayed the time for membrane recurrence from initially 3–6 days without treatment to 3–6 weeks following therapy.

Recently, Watts et al for the first time successfully treated 3 children with ligneous conjunctivitis with a topical plasminogen concentrate (approximately 1mg/ml) prepared from fresh frozen plasma: Initially,the plasminogen drops were applicated every 2 hours for 3–4 weeks and then reduced to four times a day. After 12 months pseudomembranes had completely resolved in 2 of the 3 children or were minimal in the first case.
Effective Treatment of Ligneous Conjunctivitis With Topical Plasminogen
American Journal of Ophthalmology - April 2002 (Volume 133, Number 4)
Watts P, Suresh P, Mezer E, et al.
American Journal of Ophthalmology. 2002;133(4):451-455
Abstract: http://www.medscape.com/viewarticle/432748_3

This latest research is based on the current therapy of ENEA
[this site: Human Plasminogen Drops, the Therapy]
Download official document: Thromb Haemost 2008; 100: 1196–1198
Long-term efficacy of surgical removal of pseudomembranes in a child
with ligneous conjunctivitis treated with plasminogen eyedrops [Plasminogen-Drops].

Roberto Caputo[1]; Neri Pucci[2]; Francesca Mori[2]; Jacopo Secci[1]; Elio Novembre[2]; Riccardo Frosini[3]
[1]Pediatric Ophthalmology Unit A, Meyer Children’s Hospital, Florence, Italy;
[2]Allergology Unit A, Meyer Children’s Hospital, Florence, Italy;
[3]Pediatric Ophthalmology Unit, Careggi Hospital, Florence, Italy
Source: http://www.schattauer.de/index.php?id=1882&no_cache=1&tx_ivabonnenten_pi5[download]=1&tx_ivabonnenten_pi5[artikel]=23991 (Deleted)

= = = = = = = = = = = = = = = = = = = = = = = = = = = =
By Achille Mauro Porcheddu - Enea's father 22/01/2010
= = = = = = = = = = = = = = = = = = = = = = = = = = = =



CONCLUSION
(Source: LIGNEOUS CONJUNCTIVITIS - SURVEY OF OPHTHALMOLOGY VOLUME 48 • NUMBER 4 • JULY–AUGUST 2003 -)

potential useful approach an initial topical application of a fibrinolytic agent, such as a plasminogen concentrate, possibly in combination with a plasminogen activator (uPA or tPA) which may soften the fibrin-rich pseudomembranes and facilitate removal. To prevent regrowth of pseudomembranes, lesions should be intensively locally treated with heparin and corticosteroids, as described by DeCock et al,29 until wound healing is completed and no further regrowth of pseudomembranes is observed. If this procedure is not sufficient, long-term application of topical cyclosporine A, in combination with topical steroids, should be considered. Further studies are needed to evaluate the optimal treatment approach.
Replacement therapy with a retarded plasminogen preparation, liver transplantation, or gene therapy are potential therapeutic options for the future.

Source: LIGNEOUS CONJUNCTIVITIS - SURVEY OF OPHTHALMOLOGY VOLUME 48 • NUMBER 4 • JULY–AUGUST 2003 -
Volker Schuster(1), MD, and Stefan Seregard(2), MD,
(1) Department of Pediatrics, Leipzig University Medical School, Leipzig, Germany; (2) Ophthalmic Pathology and Oncology Service, Saint Eriks Eye Hospital and Karolinska Institute, Stockholm, Sweden





Clinical Characteristics of 106 Patients with Conjunctivitis Ligneous
SEX
Female 60
Male 43
Not indicate 1
Ratio F/M 1,39
LOCATION
Unilateral 50/106
Bilateral 56/106
Palpebral conjunctiva 90/106
Bulbar conjunctiva 23/106
Cornea 30/106
Concurrent upper respiratory 30/160 (a)
Vaginal or cervical pseudomembranes 5/58 (a)
Maximum duration of disease (yrs) 44
Spontaneous resolution 7/104
Severe visual impairment 13/104
Occlusive hydro-cephalus 16/43
(a)The percentage may be higher since concomitant infectionor affection of the genital tract, respectively, may nothave been noted or not been reported.

Source: LIGNEOUS CONJUNCTIVITIS - SURVEY OF OPHTHALMOLOGY VOLUME 48 • NUMBER 4 • JULY–AUGUST 2003 -
Volker Schuster(1), MD, and Stefan Seregard(2), MD,
(1) Department of Pediatrics, Leipzig University Medical School, Leipzig, Germany; (2) Ophthalmic Pathology and Oncology Service, Saint Eriks Eye Hospital and Karolinska Institute, Stockholm, Sweden

© Achille Mauro Porcheddu 2004 ~ 2008 - Enea's Case - achille@budoniambiente.org