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Abnormal factor IX activity

MedGen UID:
1765773
Concept ID:
C5421579
Finding
HPO: HP:0033062

Definition

Abnormal activity of coagulation factor IX. Factor IX, which itself is activated by factor Xa or factor VIIa to form factor IXa, activates factor X into factor Xa. [from HPO]

Professional guidelines

PubMed

Kempton CL, Harvey RD 3rd, Roberts HR
Cardiovasc Hematol Agents Med Chem 2006 Oct;4(4):319-34. doi: 10.2174/187152506778520745. PMID: 17073609
Staehler M, Rohrmann K, Haseke N, Stief CG, Siebels M
Curr Drug Targets 2005 Nov;6(7):835-46. doi: 10.2174/138945005774574498. PMID: 16305462
Lusher JM
Am J Obstet Gynecol 1996 Sep;175(3 Pt 2):778-83. doi: 10.1016/s0002-9378(96)80084-6. PMID: 8828561

Recent clinical studies

Etiology

Puetz J, Hu B
Pediatr Blood Cancer 2023 Nov;70(11):e30644. Epub 2023 Aug 28 doi: 10.1002/pbc.30644. PMID: 37638815
De Graef D, Ligezka AN, Rezents J, Mazza GL, Preston G, Schwartz K, Krzysciak W, Lam C, Edmondson AC, Johnsen C, Kozicz T, Morava E
Mol Genet Metab 2023 Jun;139(2):107606. Epub 2023 May 9 doi: 10.1016/j.ymgme.2023.107606. PMID: 37224763Free PMC Article
Hernández-Zamora E, Rodríguez-Olivas AO, Rosales-Cruz E, Galicia-Alvarado MA, Zavala-Hernández C, Reyes-Maldonado E
Clin Appl Thromb Hemost 2023 Jan-Dec;29:10760296221151166. doi: 10.1177/10760296221151166. PMID: 36650707Free PMC Article
Böhm JK, Schaeben V, Schäfer N, Güting H, Lefering R, Thorn S, Schöchl H, Zipperle J, Grottke O, Rossaint R, Stanworth S, Curry N, Maegele M; CENTER-TBI Participants and Investigators
Neurocrit Care 2022 Jun;36(3):927-941. Epub 2021 Dec 16 doi: 10.1007/s12028-021-01400-3. PMID: 34918214Free PMC Article
Winter WE, Flax SD, Harris NS
Lab Med 2017 Nov 8;48(4):295-313. doi: 10.1093/labmed/lmx050. PMID: 29126301

Diagnosis

Hernández-Zamora E, Rodríguez-Olivas AO, Rosales-Cruz E, Galicia-Alvarado MA, Zavala-Hernández C, Reyes-Maldonado E
Clin Appl Thromb Hemost 2023 Jan-Dec;29:10760296221151166. doi: 10.1177/10760296221151166. PMID: 36650707Free PMC Article
Xiang K, Yanshan H, Chunmei Z, Minmin G, Yan W, Xiaojia Y
Exp Biol Med (Maywood) 2022 Sep;247(17):1501-1517. Epub 2022 Jul 26 doi: 10.1177/15353702221110642. PMID: 35880886Free PMC Article
Winter WE, Flax SD, Harris NS
Lab Med 2017 Nov 8;48(4):295-313. doi: 10.1093/labmed/lmx050. PMID: 29126301
Andrews RK, Berndt MC
Semin Thromb Hemost 2013 Sep;39(6):656-62. Epub 2013 Aug 8 doi: 10.1055/s-0033-1353390. PMID: 23929303
Green D
Ann Clin Lab Sci 1980 Mar-Apr;10(2):123-7. PMID: 6770741

Therapy

De Graef D, Ligezka AN, Rezents J, Mazza GL, Preston G, Schwartz K, Krzysciak W, Lam C, Edmondson AC, Johnsen C, Kozicz T, Morava E
Mol Genet Metab 2023 Jun;139(2):107606. Epub 2023 May 9 doi: 10.1016/j.ymgme.2023.107606. PMID: 37224763Free PMC Article
Franchini M
Int J Hematol 2006 Feb;83(2):126-38. doi: 10.1532/IJH97.E0517. PMID: 16513530
Staehler M, Rohrmann K, Haseke N, Stief CG, Siebels M
Curr Drug Targets 2005 Nov;6(7):835-46. doi: 10.2174/138945005774574498. PMID: 16305462
Franchini M, Zaffanello M, Veneri D
Thromb Haemost 2005 Jun;93(6):1027-35. doi: 10.1160/TH05-01-0032. PMID: 15968384
Sallah S
Ann Hematol 1997 Jul-Aug;75(1-2):1-7. doi: 10.1007/s002770050305. PMID: 9322677

Prognosis

Puetz J, Hu B
Pediatr Blood Cancer 2023 Nov;70(11):e30644. Epub 2023 Aug 28 doi: 10.1002/pbc.30644. PMID: 37638815
Kunitake RC, Howard BM, Kornblith LZ, Christie SA, Conroy AS, Cohen MJ, Callcut RA
J Trauma Acute Care Surg 2017 Feb;82(2):302-308. doi: 10.1097/TA.0000000000001313. PMID: 27906868Free PMC Article
Wolberg AS, Aleman MM, Leiderman K, Machlus KR
Anesth Analg 2012 Feb;114(2):275-85. Epub 2011 Nov 21 doi: 10.1213/ANE.0b013e31823a088c. PMID: 22104070Free PMC Article
Girolami A, Scandellari R, Scapin M, Vettore S
Vitam Horm 2008;78:281-374. doi: 10.1016/S0083-6729(07)00014-3. PMID: 18374200
McGraw RA, Davis LM, Lundblad RL, Stafford DW, Roberts HR
Clin Haematol 1985 Jun;14(2):359-83. PMID: 3899439

Clinical prediction guides

Puetz J, Hu B
Pediatr Blood Cancer 2023 Nov;70(11):e30644. Epub 2023 Aug 28 doi: 10.1002/pbc.30644. PMID: 37638815
Wada H, Shiraki K, Matsumoto T, Shimpo H, Shimaoka M
Ann Lab Med 2023 Nov 1;43(6):531-538. Epub 2023 Jun 30 doi: 10.3343/alm.2023.43.6.531. PMID: 37387486Free PMC Article
Böhm JK, Schaeben V, Schäfer N, Güting H, Lefering R, Thorn S, Schöchl H, Zipperle J, Grottke O, Rossaint R, Stanworth S, Curry N, Maegele M; CENTER-TBI Participants and Investigators
Neurocrit Care 2022 Jun;36(3):927-941. Epub 2021 Dec 16 doi: 10.1007/s12028-021-01400-3. PMID: 34918214Free PMC Article
Wolberg AS, Aleman MM, Leiderman K, Machlus KR
Anesth Analg 2012 Feb;114(2):275-85. Epub 2011 Nov 21 doi: 10.1213/ANE.0b013e31823a088c. PMID: 22104070Free PMC Article
Green D
Ann Clin Lab Sci 1980 Mar-Apr;10(2):123-7. PMID: 6770741

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