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Treatment of plasminogen deficiency patients with fresh frozen plasma
- Source :
- Pediatric bloodcancer. 65(2)
- Publication Year :
- 2017
-
Abstract
- Congenital plasminogen (Plg) deficiency leads to the development of ligneous membranes on mucosal surfaces. Here, we report our experience with local and intravenous fresh frozen plasma (FFP). We retrospectively reviewed medical files of 17 patients and their eight first-degree relatives. Conjunctivitis was the main complaint. Thirteen patients were treated both with intravenous and conjunctival FFP. Venous thrombosis did not develop in any. Genetic evaluation revealed heterogeneous mutations as well as polymorphisms. Diagnosis and treatment of Plg deficiency is challenging; topical and intravenous FFP may be an alternative treatment.
- Subjects :
- Male
medicine.medical_specialty
Blood Component Transfusion
030204 cardiovascular system & hematology
Gastroenterology
03 medical and health sciences
Plasma
0302 clinical medicine
Internal medicine
Ligneous conjunctivitis
Medicine
Humans
Ligneous gingivitis
Polymorphism, Genetic
business.industry
Genetic Diseases, Inborn
Infant, Newborn
Infant
Plasminogen
Hematology
medicine.disease
Conjunctivitis
Alternative treatment
Venous thrombosis
Oncology
Child, Preschool
Pediatrics, Perinatology and Child Health
Mutation
030221 ophthalmology & optometry
Female
Fresh frozen plasma
business
Plasminogen deficiency
Subjects
Details
- ISSN :
- 15455017
- Volume :
- 65
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- Pediatric bloodcancer
- Accession number :
- edsair.doi.dedup.....244cca7ad303ead29f013b6d4536e571