Massive Recurrent Post-Tonsillectomy Bleedings Revealing a Transient Factor XIII deficiency in a 10-year-old boy. A case report.

Publication date: Available online 28 May 2019Source: International Journal of Pediatrics and Adolescent MedicineAuthor(s): M. Jankovic, M.L. Choucair, B. Hallak, E. Hernandez, M. Russo, J. Llor, S. Kayemba-Kay’sAbstractA previously healthy 10-year-old boy was hospitalized for a left cervical abscess associated with massive tonsillar hypertrophy. He underwent abscess drainage and bilateral tonsillectomy. At H36 post-surgery, he presented with tonsillar hemorrhage requiring surgical revision. Hemorrhage relapsed 2 days later, with a total of 7 episodes, 5 of which required surgical revisions. Laboratory investigations were normal except for a markedly low factor XIII (FXIII) activity at 7%. After administration of a single dose of 40 IU/kg plasma-derived FXIII (Fibrogammin®) I.V., the bleeding stopped with no further recurrence. FXIII activity gradually normalized (75%) at 6 weeks, confirming the transient character of factor XIII deficiency.Severe congenital FXIII deficiency (FXIIID) (<1%) is very rare (1:2’000’000 births), whereas partial congenital deficiency and/or acquired deficiency may be more frequent but likely underreported. Acquired FXIIID may result from impaired synthesis (liver failure) or increased consumption (surgery, sepsis, leukemia, Henoch-Schönlein, inflammatory bowel disease, stroke, disseminated intravascular coagulation). FXIII replacement in form of fresh frozen plasma (FFP) or plasma-derived FXIII may be necessary for the presence of bleed...
Source: International Journal of Pediatrics and Adolescent Medicine - Category: Pediatrics Source Type: research