Successful treatment of two brothers with congenital afibrinogenemia for splenic rupture using heat- and solvent detergent-treated fibrinogen concentrates.
OBJECTIVE
This report describes life-threatening spontaneous splenic rupture in two brothers with congenital afibrinogenemia.


METHODS
Two brothers, aged 11 and 14 years, had intra-abdominal bleeding due to splenic rupture confirmed by ECHO ultrasonography and computed tomography scans.


RESULTS
Splenectomy was performed after administration of heat- and solvent-detergent treated fibrinogen concentrates.


CONCLUSIONS
Hemostatic treatment for splenic rupture using heat- and solvent detergent-treated fibrinogen concentrates was effective. Careful attention must be paid to the risk of splenic rupture during the growth spurt in physically active children with this rare coagulation disorder.
Shima
M
M
Department of Pediatrics, Nara Medical University, Japan.
Tanaka
I
I
Sawamoto
Y
Y
Kanehiro
H
H
Matsuo
N
N
Nishimura
A
A
Giddings
J C
JC
Yoshioka
A
A
D000293:Adolescent; D000347:Afibrinogenemia / Q000150:complications* / Q000235:genetics / Q000628:therapy*; D000925:Anticoagulants / Q000737:chemistry / Q000627:therapeutic use*; D002648:Child; D003902:Detergents; D005340:Fibrinogen / Q000737:chemistry / Q000627:therapeutic use*; D005500:Follow-Up Studies; D006358:Hot Temperature; D006801:Humans; D008297:Male; D012997:Solvents; D013161:Splenic Rupture / Q000000981:diagnostic imaging / Q000209:etiology / Q000628:therapy*; D014057:Tomography, X-Ray Computed
D000925:Anticoagulants; D003902:Detergents; D012997:Solvents; D005340:Fibrinogen