title: Rare coagulation disorders: a retrospective analysis of 156 patients in Turkey.
abstract: OBJECTIVE
  To retrospectively evaluate the clinical findings, laboratory data, management, and outcome in a group ofTurkish children diagnosed with rare coagulation deficiencies (RCDs) between January 1999 and June 2009.
  
  
  METHODS
  The Turkish Society of Pediatric Hematology-Hemophilia-Thrombosis-Hemostasissubcommittee designed a Microsoft Excel-based questionnaire for standardized data collection and sent it to participatinginstitutions.
  
  
  RESULTS
  In total, 156 patients from 12 pediatric referral centers were included in the study. The cost common RCDswere as follows: FVII (n = 53 [34%]), FV (n = 24 [15.4%]), and FX (n = 23 [14.7%]) deficiency. The most common initialfinding in the patients was epistaxis, followed by ecchymosis, and gingival bleeding.
  
  
  CONCLUSIONS
  Initial symptoms were mucosal bleeding, and fresh frozen plasma (FFP) and tranexamic acid werethe most commonly used treatments. We think that prophylactic treatment used for hemophilia patients should beconsidered as an initial therapeutic option for patients with rare factor deficiencies and a severe clinical course, and forthose with a factor deficiency that can lead to severe bleeding.
authors.affiliation: Ondokuz Mayıs University, School of Medicine, Department of Pediatric Hematology, Samsun, Turkey.
authors.forename: Tunç
authors.initials: T
authors.lastname: Fışgın
authors.affiliation: Ege University, School of Medicine, Department of Pediatric Hematology, İzmir, Turkey.
authors.forename: Can
authors.initials: C
authors.lastname: Balkan
authors.affiliation: İstanbul University, Cerrahpaşa School of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey.
authors.forename: Tiraje
authors.initials: T
authors.lastname: Celkan
authors.affiliation: Çukurova University, School of Medicine, Department of Pediatric Hematology, Adana, Turkey.
authors.forename: Yurdanur
authors.initials: Y
authors.lastname: Kılınç
authors.affiliation: Tepecik Research and Education Hospital, Department of Pediatric Hematology, İzmir, Turkey.
authors.forename: Meral
authors.initials: M
authors.lastname: Türker
authors.affiliation: Göztepe Training and Research Hospital, Department of Pediatric Hematology, İstanbul, Turkey.
authors.forename: Cetin
authors.initials: C
authors.lastname: Timur
authors.affiliation: Gazi University, School of Medicine, Department of Pediatric Hematology, Ankara, Turkey.
authors.forename: Türkiz
authors.initials: T
authors.lastname: Gürsel
authors.affiliation: Gülhane Military Medical Academy, Department of Pediatric Hematology, Ankara, Turkey.
authors.forename: Emin
authors.initials: E
authors.lastname: Kürekçi
authors.affiliation: Ondokuz Mayıs University, School of Medicine, Department of Pediatric Hematology, Samsun, Turkey.
authors.forename: Feride
authors.initials: F
authors.lastname: Duru
authors.affiliation: Akdeniz University, School of Medicine, Department of Pediatric Hematology, Antalya, Turkey.
authors.forename: Alphan
authors.initials: A
authors.lastname: Küpesiz
authors.affiliation: Dr. Abdurahman Yurtarslan Oncology Training and Research Hospital, Department of Pediatric Hematology, Ankara, Turkey.
authors.forename: Lale
authors.initials: L
authors.lastname: Olcay
authors.affiliation: Dokuz Eylül University, School of Medicine, Department of Pediatric Hematology, İzmir, Turkey.
authors.forename: Sebnem
authors.initials: S
authors.lastname: Yılmaz
authors.affiliation: İnönü University, School of Medicine, Department of Pediatric Hematology, Malatya, Turkey.
authors.forename: Unsal
authors.initials: U
authors.lastname: Ozgen
authors.affiliation: Istanbul University, İstanbul School of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey.
authors.forename: Ayşegül
authors.initials: A
authors.lastname: Unüvar
authors.affiliation: Dokuz Eylül University, School of Medicine, Department of Pediatric Hematology, İzmir, Turkey.
authors.forename: Hale
authors.initials: H
authors.lastname: Oren
authors.affiliation: Ege University, School of Medicine, Department of Pediatric Hematology, İzmir, Turkey.
authors.forename: Kaan
authors.initials: K
authors.lastname: Kavaklı
keywords: Laboratory data; Rare coagulation deficiencies; clinical findings
references.citation: Haemophilia. 2008 Nov;14(6):1164-9
references.pmid: 19141156
references.citation: Blood Transfus. 2008 Sep;6 Suppl 2:s39-44
references.pmid: 19105509
references.citation: Haemophilia. 2009 Jan;15(1):267-80
references.pmid: 18976247
references.citation: Semin Thromb Hemost. 2009 Jun;35(4):407-15
references.pmid: 19598069
references.citation: Haemophilia. 2004 Sep;10(5):593-628
references.pmid: 15357789
references.citation: Surv Ophthalmol. 2003 Jul-Aug;48(4):369-88
references.pmid: 12850227
references.citation: Haemophilia. 2008 Nov;14(6):1201-8
references.pmid: 19141160
references.citation: Semin Thromb Hemost. 2009 Jun;35(4):349-55
references.pmid: 19598063
references.citation: Br J Haematol. 1998 Mar;100(4):773-6
references.pmid: 9531348
references.citation: Acta Paediatr. 1999 Mar;88(3):342-3
references.pmid: 10229050
references.citation: J Thromb Haemost. 2004 Feb;2(2):248-56
references.pmid: 14995986
references.citation: Blood Coagul Fibrinolysis. 2007 Jun;18(4):371-4
references.pmid: 17473580
references.citation: Haemophilia. 2008 Nov;14(6):1176-82
references.pmid: 19141158
references.citation: Thromb Haemost. 1999 Oct;82(4):1207-14
references.pmid: 10544899
references.citation: Semin Thromb Hemost. 2009 Jun;35(4):400-6
references.pmid: 19598068
references.citation: Haemophilia. 2008 Nov;14(6):1151-8
references.pmid: 19141154
references.citation: Haemophilia. 2004 May;10(3):271-5
references.pmid: 15086326
references.citation: Haemophilia. 2008 Nov;14(6):1261-8
references.pmid: 19141167
references.citation: Acta Neurochir (Wien). 1999;141(6):667-8
references.pmid: 10929735
references.citation: Haemophilia. 2008 Nov;14(6):1190-200
references.pmid: 19141159
references.citation: Haemophilia. 2008 Nov;14(6):1170-5
references.pmid: 19141157
references.citation: Haemophilia. 2002 May;8(3):308-21
references.pmid: 12010428