title: Successful use of recombinant factor VIIa in a child with Schoenlein-Henoch purpura presenting with compartment syndrome and severe factor XIII deficiency.
abstract: In this article, we present a 7-year-old boy with Schoenlein-Henoch purpura (HSP) presented with compartment syndrome and factor XIII deficiency and treated with recombinant factor VIIa and fasciotomy. Treatment decisions for patients with HSP presenting with compartment syndrome should be made on a case-by-case basis. Factor XIII deficiency should be in mind in these patients. The use of recombinant factor VIIa might be effective and well tolerated for treating hemorrhage in patients with HSP and compartment syndrome. Surgical treatment should be preferred in patients with compartment syndrome. However, in patients who have a coagulation defect, the first priority is to correct the clotting deficiency. The use of recombinant factor VIIa is a treatment option for children who develop compartment syndrome due to a coagulation defect.
authors.affiliation: Department of Pediatric Hematology, Ankara Training and Research Hospital, Ankara, Turkey. alioglub@gmail.com
authors.forename: Bulent
authors.initials: B
authors.lastname: Alioglu
authors.forename: M Hakan
authors.initials: MH
authors.lastname: Ozsoy
authors.forename: Esra
authors.initials: E
authors.lastname: Tapci
authors.forename: Sirma
authors.initials: S
authors.lastname: Karamercan
authors.forename: Pinar I
authors.initials: PI
authors.lastname: Agras
authors.forename: Yildiz
authors.initials: Y
authors.lastname: Dallar
mesh_terms: D001780:Blood Coagulation Tests; D016063:Blood Loss, Surgical / Q000517:prevention & control; D002648:Child; D003131:Combined Modality Therapy; D003161:Compartment Syndromes / Q000209:etiology* / Q000601:surgery; D019299:Decompression, Surgical; D005169:Factor VIII / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use*; D005176:Factor XIII / Q000378:metabolism; D005177:Factor XIII Deficiency / Q000209:etiology*; D000071938:Fasciotomy; D005340:Fibrinogen / Q000627:therapeutic use; D006230:Hand Injuries / Q000150:complications; D006470:Hemorrhage / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D011695:IgA Vasculitis / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D011300:Preoperative Care; D011994:Recombinant Proteins / Q000627:therapeutic use; D062666:Vascular Access Devices / Q000009:adverse effects
chemical_list: D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; C026912:cryoprecipitate coagulum; D005169:Factor VIII; D005340:Fibrinogen; D005176:Factor XIII; C103587:recombinant FVIIa; D015942:Factor VIIa; D008775:Methylprednisolone