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

Al S., ULUSOY O., Asilsoy S., Uzuner N., Kangalli O., Atay O., ...Daha Fazla

Journal of pediatric hematology/oncology, cilt.45, sa.7, ss.423-426, 2023 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 45 Sayı: 7
  • Basım Tarihi: 2023
  • Doi Numarası: 10.1097/mph.0000000000002698
  • Dergi Adı: Journal of pediatric hematology/oncology
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, MEDLINE
  • Sayfa Sayıları: ss.423-426
  • İstanbul Medipol Üniversitesi Adresli: Evet

Özet

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.