Author(s):
Salih khudhair Abdullah, Asmaa Mohammed Khaleel, Khalid Satam Sultan
Email(s):
Salihaljubory95@gmail.com
DOI:
10.52711/2231-5691.2021.00016 
Address:
Salih khudhair Abdullah¹*, Asmaa Mohammed Khaleel², Khalid Satam Sultan³
¹Subspeciality in Clinical Haematology, Ibn-Sena Teaching Hospital, Mosul, Iraq.
²Chief General Physician, Hematology Lab Unit, Ibn-Sena Teaching Hospital, Mosul, Iraq.
³Chief General Physician, Lab Units, Ibn-Alatheer Children Hospital, Mosul, Iraq.
*Corresponding Author
Published In:
Volume - 11,
Issue - 2,
Year - 2021
ABSTRACT:
Background: Hemophilia is a recessive mutation in X-linked chromosome. Hemophilia A is characterized by a deficiency of clotting factor F-VIII. Hemophilia B is characterized by a deficiency of clotting factor F-IX. Fibrin Stabilizer is a deficiency of F-XIII. Alexander's disease is a deficiency of clotting factor F-VII. Von Willebrand disease is a deficiency of clotting factor VWF. Afibrinogenemia is a deficiency of clotting factor F-I. Aim: This study amid to find out prevalence of deficiency clotting factors in Nineveh province. Methods: This research was conducted at Ibn-Sina Teaching Hospital. Staco special kits were used to determine factors under the study. Results: 365 out of 829 total patients have been detected deficiency in one or more of different types of factors. The most prevalence of deficiency factors in Nineveh are F-VIII, FIX and VWF. Infected males are more than females. The ages between 1-20 years and blood groups (A?, B?, and O?) are most prevalent. Conclusions: It is necessary to monitor patients during the initial disease, follow it up, and use effective treatment methods to limit the increased number of cases. Moreover, it is necessary to follow up on the family's genetic history to avoid new infections.
Cite this article:
Salih khudhair Abdullah, Asmaa Mohammed Khaleel, Khalid Satam Sultan. The Most Prominent Hemophilia Clotting Factors in the Province of Nineveh. Asian Journal of Pharmaceutical Research. 2021; 11(2):85-1. doi: 10.52711/2231-5691.2021.00016
Cite(Electronic):
Salih khudhair Abdullah, Asmaa Mohammed Khaleel, Khalid Satam Sultan. The Most Prominent Hemophilia Clotting Factors in the Province of Nineveh. Asian Journal of Pharmaceutical Research. 2021; 11(2):85-1. doi: 10.52711/2231-5691.2021.00016 Available on: https://asianjpr.com/AbstractView.aspx?PID=2021-11-2-2
REFERECES:
1. Fijnvandraat, K, Cnossen, M, Leebeek, F, and Peters M. Diagnosis and management of haemophilia. BMJ. 2012; 344: e2707 doi: 10.1136/bmj.e2707.
2. Keeling D, Tait C, Makris M. 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. Haemophilia 2008; 14: 671-84.
3. Hermans C, Altisent C, Batorova A, Chambost H, De Moerloose P, Karafoulidou A, et al. Replacement therapy for invasive procedures in patients with haemophilia: literature review, European survey and recommendations. Haemophili.a 2009; 15: 639-58.
4. Centers for Disease Control and Prevention. Hemophilia A mutation project. www.cdc. gov/ncbddd/hemophilia/champs.html.
5. White GC, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J. Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost. 2001; 85: 560.
6. Srivastava A, You SK, Ayob Y, Chuansumrit A, de Bosch N, Perez Bianco R, et al. Hemophilia treatment in developing countries: products and protocols. Semin Thromb Hemost. 2005; 31: 495-500.
7. Teitel JM, Barnard D, Israels S, Lillicrap D, Poon MC, Sek J. Home management of haemophilia. Haemophilia 2004; 10: 118-3.
8. Colman, W. Hemostasis and thrombosis: Basic Principles and clinical Practice. Baltimore: Lippincott Williams and Wilkins. 2012; 1578 p.
9. Orlova, N, Kovnir, S, Vorobiev, I, Gabibov, A, and Vorobiev A. Blood Clotting Factor VIII: From Evolution to Therapy. Acta Nature. 2013; 5: 2(17), 19-39. DOI: 10.32607/20758251-2013-5-2-19-39.
10. White, GC, Rosendaal, F, Aledort, LM, et al. Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost. 2001; 85(3): 560.
11. Scharrer, I, Becker, T. Products used to treat hemophilia: evolution of treatment for hemophilia A and B. In: Lee CA, Berntorp EE, Hoots WK, editors. Textbook of hemophilia. Blackwell Publishing Ltd; Malden, MA, USA: 2005. p. 131-5.
12. National Hemophilia Foundation. History of bleeding disorders. Available from: www. hemophilia.org/NHFWeb/MainPgs/ MainNHF.aspx?menuid=178&contentid=6 [Last accessed 23 June 2012].
13. Gouw, SC, Van den Berg, HM, Fischer K, et al. Intensity of factor VIII treatment and inhibitor development in children with severe hemophilia A: the RODIN study. Blood 2013; 121(20): 4046-55.
14. Mannucci, PM. Plasma-derived versus recombinant factor VIII concentrates for the treatment of haemophilia A: plasma-derived is better. Blood Transfus 2010; 8(4): 288-91.
15. Franchini, M, Tagliaferri, A, Mengoli C, Cruciani, M. Cumulative inhibitor incidence in previously untreated patients with severe hemophilia A treated with plasma-derived versus recombinant factor VIII concentrates: a critical systematic review. Crit Rev Oncol Hematol 2012; 81(1): 82-93.
16. Plug, I, Van der Bom, JG, Peters, M, et al. Thirty years of hemophilia treatment in the Netherlands, 1972-2001. Blood 2004; 104(12): 3494-500.
17. Anwar, R, Miloszewski, KJA, Markham, AF. Identification of a large deletion, spanning exons 4 to 11 of the human factor XIIIA gene, in a factor XIII deficient family. Blood. 1998; 91(1): 149-53.
18. Board PG, Lesososky MS, Miloszewski KJ. Factor XIII: Inherited and Acquired Deficiency. Blood Reviews. 1993; 7: 229-242.
19. Orphanet Portal for Rare Diseases and Orphan Drugs. Congenital factor XIII deficiency. 2012. www.orpha.net/consor/cgi-bin/ OC_Exp.php?lng=EN&Expert=331.
20. Hsieh, L, and Nugent, D. Factor XIII deficiency. Haemophilia. 2008; 14: 1190–1200. DOI: 10.1111/j.1365-2516.2008.01857.x.
21. Amesse C, Lacroix S, Lupien G. La déficience en facteur VII: Unemaladie héréditaire de la coagulation. 2nd. Ed., 2013.
22. Graff-Radford, J, Schwartz, K, Gavrilova, RH, Lachance, DH, Kumar, N. Neuroimaging and clinical features in type II (late-onset) Alexander disease. Neurology. 2014; 7; 82(1): 49-56. doi: 10.1212/01.wnl.0000438230.33223.bc.
23. Zang, L, Wang, J, Jiang, Y, Gu, Q, Gao, Z, Yang, et al. Follow-up study of 22 Chinese children with Alexander disease and analysis of parental origin of de novo GFAP mutations. J Hum Genet. 2013 Apr; 58(4): 183-8. Doi: 10.1038/jhg.2012.152.
24. Gorospe, JR, Maletkovic, J. Alexander disease and megalencephalic leukoencephalopathy with subcortical cysts: leukodystrophies arising from astrocyte dysfunction. Ment Retard Dev Disabil Res Rev. 2006; 12(2): 113-22.
25. Bharati, K, and Prashanth, U. Von Willebrand Disease: An Overview. Ind J Pharma Sci. 2011; 1: 7-17.
26. Favaloro, EJ. Collagen binding assay for von Willebrand factor (VWF: CBA): Detection of von Willebrand disease (VWD), and the discrimination of VWD subtypes, depends on collagen source. Thromb Haemost. 2000; 83: 127-35.
27. Chen YB, Zieve D. Congenital afibrinogenemia. MedlinePlus. 2015; Jan. 27. http://www.nlm.nih.gov/medlineplus/ency/article/ 001313.htm.
28. Abolghasemi H, Shahverdi E. Umbilical bleeding: a presenting feature for congenital afibrinogenemia. Blood Coagul Fibrinolysis. 2015; 26(7): 834-5. https://www.ncbi.nlm.nih.gov/pubmed/ 26407137.
29. Neerman-Arbez M, Moerloose P, and Casini A. Laboratory and Genetic Investigation of Mutations Accounting for Congenital Fibrinogen Disorders. Semin Thromb Hemost. 2016; 42(4): 356-65.
30. Keeling, D, Tait, C, Makris, M. Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders. A United Kingdom.
31. Richards, M, Williams, M, Chalmers, E, Liesner, R, Collins, P, Vidler, V, et al. A United Kingdom Haemophilia Centre Doctors’ Organization guideline approved by the British Committee for Standards in Haematology: guideline on the use of prophylactic factor VIII concentrate in children and adults with severe haemophilia A. Br J Haematol 2010; 149: 498-507.
32. Chalmers, E, Williams, M, Brennand, J, Liesner, R, Collins, P, Richards, M. Guideline on the management of haemophilia in the fetus and neonate. Br J Haematol 2011; 154: 208-15.
33. Lee, CA, Chi, C, Pavord, SR, Bolton-Maggs PH, Pollard D, Hinchcliffe-Wood A, et al. The obstetric and gynaecological management of women with inherited bleeding disorders-review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization. Haemophilia 2006; 12: 301-36.
34. Budde, U, Pieconka, A, Will, K, Schneppenheim R. Laboratory testing for von Willebrand disease: Contribution of multimer analysis to diagnosis and classification. Semin Thromb Haemost. 2006; 32: 514-21.