Clinical Course and Treatment of Ligneous Conjunctivitis in Children With Plasminogen Deficiency

• Status: Not yet recruiting
• Trial end date: 2024-12-31
• Target enrollment: 0
• Participant gender: All

Summary

Congenital plasminogen deficiency causes impaired wound healing and growth of pseudomembranous lesions over multiple parts of the body. The most common lesions involve eyes and are known as Ligneous conjunctivitis. These can cause scarring of the sclera, vision loss and even blindness. These pseudomembranous lesions are recur after surgical excisions, administration of intra-ocular cyclosporine, autologous serum drops or corticosteroids.

Clinical data shows that these growths do not worsen and do not recur after administration of plasminogen (either as concentrate or as plasma) in the eyes, locally or intravenously.

As plasminogen is not available as concentrate, we are using aliquoted allogenic plasma provided by Canadian Blood Services for intra-ocular application. These will be applied to eyes multiple times a day for a period of 6 weeks to 6 months depending on disease severity and patient response. These may be used again if ligneous conjunctivitis recurs.

The patient will be followed for a period of 2 years at least. All serious adverse events will be reported to Canadian Blood Services and Health Canada as appropriate.

• Phase: N/A
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: University of Saskatchewan
• Collaborator: Canadian Blood Services

Criteria

Inclusion Criteria:

• Patients under the age of 18 years

• Diagnosis of plasminogen deficiency (Definition: clinical presence of pseudomembranous lesions and serum plasminogen level < 50%)

• Ocular involvement (Ligneous Conjunctivitis) due to plasminogen deficiency

Exclusion Criteria:

• Patient is 18 years or older

• Patients with no plasminogen deficiency

• Patients with no ocular manifestations of plasminogen deficiency