PB2442 BRONCHOSCOPIC TREATMENT WITH FRESH FROZEN PLASMA AND TISSUE PLASMINOGEN ACTIVATOR FOR A CHILD WITH TYPE 1 PLASMINOGEN DEFICIENCY

Abstract

Background:Congenital plasminogen deficiency is a genetic disease with autosomal recessive inheritance pattern. Fibrous plaques that accumulate in the mucosal surfaces of oral cavity, eye and respiratory tract.Aims:We aimed to report the first bronchoscopic usage of fresh frozen plasma (FFP) and tissue plasminogen activator (t‐PA) for respiratory complications of a child with plasminogen deficiency.Methods:A child with plasminogen deficiency treated with FFP, t‐PA is reported.Results:A one‐year‐old male patient was admitted to the hospital for 1 week with cough and increased respiratory distress. Antibiotic therapy was started for bronchopneumonia.From his medical history it was learned that he had lingous conjunctivitis, in addition a ventriculo‐peritoneal shunt was inserted due to hydrocephalus in the antenatal period. His parents were cousins. The patient had significant paracardiac infiltrations, linear infiltrations in the parenchyma of the chest x ray. The patient was thought to have plasminogen deficiency by clinical findings and his plasminogen level was found to be 14% (N: 70‐130%).Type 1 plasminogen deficiency and related pulmonary involvement were considered. Bronchoscopy was performed. There were fibrin deposits in the trachea and bronchi. Recurrent pulmonary lavage was performed with FFP and t‐PA. Fibrin plugs occluding the bronchial cuffs were decreased and some of them disappeared.The patient recovered from respiratory distress that required high flow oxygen therapy, and discharged. Homozygous frameshift mutation in the PLG gene was detected.Summary/Conclusion:In this case, it has been firstly reported that total pulmonary lavage treatment with FFP and t‐PA can be a useful treatment method for paitents with plasminogen deficiency in the English literature.