Ligneous conjunctivitis, hydrocephalus and protracted respiration infections in a patient with plasminogen deficit

Česká verzia

Authors: J. Čižmár; J. Behúnová; Ľ. Podracká
Authors place of work: I. Klinika detí a dorastu LF UPJŠ a DFN, Košice, prednosta prof. MUDr. Ľ. Podracká, CSc.
Published in the journal: Čes-slov Pediat 2012; 67 (1): 38-42.
Category: Case Report

Summary

We report a rare case of an infant with internal hydrocephalus, pseudomembranous conjunctivitis and recurrent respiratory infections associated with plasminogen deficiency. Both, systemic and topical treatment were ineffective. Repeated attempts on ventriculoperitoneal shunting had to be performed due to non-inflammatory obstruction of drainage system. Pseudomembranous conjunctivitis was the most prominent clinical finding. Bilateral thick yellow fibrin membranes on the palpebral surfaces were present. Suspicion of defective fibrinolysis was supported by histological finding of „pure“ fibrin from the conjunctivas coating membranes. Severely reduced activity of plasminogen (14.4%, norm 75–150%) was confirmed.

Plasminogen deficit type I is a rare systemic disease characterized by a marked defect of extracellular fibrinolysis that leads to the formation of ligneous (fibrin rich) pseudomembranes at mucosae. Most common clinical manifestation is ligneous conjunctivitis, although pseudomembranes can also form in respiratory tract, gastrointestinal tract etc. Diagnosis of hypoplasminogenemia is based on typical clinical manifestation and evidence of quantitative or qualitative defect of plasminogen activity. Treatment of ligneous conjunctivitis is intricate; topical plasminogen concentrate holds promise. In systemic affection, replacement therapy with human plasminogen can be efficient.

Key words:
plasminogen deficit, ligneous conjunctivitis, pseudomembranes, congenital hydrocephalus

Zdroje

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