Spontaneous splenic rupture in a patient with congenital afibrinogenemia

被引：14

作者：

Arcagok, Baran Cengiz ^{[1
]}

Ozdemir, Nihal ^{[1
]}

Tekin, Ayse ^{[1
]}

Ozcan, Rahsan ^{[2
]}

Elicevik, Mehmet ^{[2
]}

Senyuz, Osman Faruk ^{[2
]}

Cam, Halit ^{[1
]}

Celkan, Tiraje ^{[1
]}

机构：

[1] Istanbul Univ Cerrahpasa, Tip Fak, Cocuk Sagligi & Hastaliklari Anabilim Dali, Istanbul, Turkey

[2] Istanbul Univ Cerrahpasa, Tip Fak, Cocuk Cerrahisi Anabilim Dali, Istanbul, Turkey

来源：

TURK PEDIATRI ARSIVI-TURKISH ARCHIVES OF PEDIATRICS | 2014年 / 49卷 / 03期

关键词：

Spleen rupture; congenital afibrinogenemia; bleeding disorder;

D O I：

10.5152/tpa.2014.1070

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Afibrinogenemia is a rare bleeding disorder which is observed with an incidence of 1: 1 000 000. It is an autosomal recessive disease and occurs as a result of mutation in one of the three genes which code the three polypeptide chains of fibrinogen. Basic clinical findings include spontaneous bleeding, bleeding after minor trauma or due to surgery. Splenic rupture in afibrinogenemia has been reported only in 6 cases so far. In this article, we present a 15-year old congenital afibrinogenemia patient with spontaneous splenic rupture.

引用

页码：247 / 249

页数：3

共 11 条

[1] The rare coagulation disorders - review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation [J].

Bolton-Maggs, PHB ;

Perry, DJ ;

Chalmers, EA ;

Parapia, LA ;

Wilde, JT ;

Williams, MD ;

Collins, PW ;

Kitchen, S ;

Dolan, G ;

Mumford, AD .

HAEMOPHILIA, 2004, 10 (05) :593-628

[2] SPLENIC RUPTURE IN AFIBRINOGENEMIA - CONSERVATIVE VERSUS SURGICAL-MANAGEMENT [J].

EHMANN, WC ;

ALMONDHIRY, H .

AMERICAN JOURNAL OF MEDICINE, 1995, 99 (04) :444-444

[3] CONGENITAL AFIBRINOGENEMIA AND SPLENIC RUPTURE [J].

EHMANN, WC ;

ALMONDHIRY, H .

AMERICAN JOURNAL OF MEDICINE, 1994, 96 (01) :92-94

[4]

Erdem F, 2004, AUTD, V36, P89

[5]

FRIED K, 1980, CLIN GENET, V17, P223

[6] Congenital Afibrinogenemia presenting as antenatal intracranial bleed: a case report [J].

Hariharan, Gopakumar ;

Ramachandran, Sivji ;

Parapurath, Rajiv .

ITALIAN JOURNAL OF PEDIATRICS, 2010, 36

[7] Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders - A United Kingdom Haemophilia Center Doctors' Organisation (UKHCDO) guideline - Approved by the British Committee for Standards in Haematology [J].

Keeling, D. ;

Tait, C. ;

Makris, M. .

HAEMOPHILIA, 2008, 14 (04) :671-684

[8]

Lak M, 1999, BRIT J HAEMATOL, V107, P204

[9] Recessively inherited coagulation disorders [J].

Mannucci, PM ;

Duga, S ;

Peyvandi, F .

BLOOD, 2004, 104 (05) :1243-1252

[10] Successful treatment of two brothers with congenital afibrinogenemia for splenic rupture using heat- and solvent detergent-treated fibrinogen concentrates [J].

Shima, M ;

Tanaka, I ;

Sawamoto, Y ;

Kanehiro, H ;

Matsuo, N ;

Nishimura, A ;

Giddings, JC ;

Yoshioka, A .

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, 1997, 19 (05) :462-465

← 1 2 →