A Successful Treatment Approach in 2 Patients With Type 1 Plasminogen Deficiency: Intratracheal Application of Fresh Frozen Plasma and Tissue Plasminogen Activator.

Autor: Al S; Departments of Pediatric Allergy and Immunology., Ulusoy O; Pediatric Surgery, Dokuz Eylul University Medical Faculty, İzmir., Asilsoy S; Departments of Pediatric Allergy and Immunology., Uzuner N; Departments of Pediatric Allergy and Immunology., Kangalli O; Departments of Pediatric Allergy and Immunology., Atay O; Departments of Pediatric Allergy and Immunology., Odaman Al I; Department of Pediatric Hematology and Oncology, Istanbul Medipol University, Medical Faculty, Istanbul, Turkey., Ates O; Pediatric Surgery, Dokuz Eylul University Medical Faculty, İzmir.
Jazyk: angličtina
Zdroj: Journal of pediatric hematology/oncology [J Pediatr Hematol Oncol] 2023 Oct 01; Vol. 45 (7), pp. 423-426. Date of Electronic Publication: 2023 Jun 22.
DOI: 10.1097/MPH.0000000000002698
Abstrakt: Background: Respiratory system involvement is common in congenital plasminogen deficiency. Although many treatment approaches have been tried, there is still no definitive treatment for respiratory system involvement.
Observations: We report 2 congenital plasminogen deficiency cases, who presented with severe respiratory symptoms, for whom a novel treatment modality was tried. After intravenous administration of FFP (fresh frozen plasma), tissue plasminogen activator and FFP were administered intratracheally, and respiratory system findings improved.
Conclusions: Intratracheal administration of tissue plasminogen activator and FFP is an available treatment modality for patients with lung involvement. Fibrin plaques should be carefully removed and new lesion formation should be prevented.
Competing Interests: The authors declare no conflict of interest.
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Databáze: MEDLINE