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Plasma Kallikrein Deficiency

MedGen UID:
1387546
Concept ID:
C4331013
Disease or Syndrome

Definition

A coagulation disorder characterized by the partial or complete absence of plasma-type kallikrein activity in the blood. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPlasma Kallikrein Deficiency

Professional guidelines

PubMed

Caballero T
J Investig Allergol Clin Immunol 2021 Feb;31(1):1-16. doi: 10.18176/jiaci.0653. PMID: 33602658
Wu MA
Expert Opin Biol Ther 2019 Dec;19(12):1233-1245. Epub 2019 Nov 4 doi: 10.1080/14712598.2019.1685490. PMID: 31657963
Cicardi M, Suffritti C, Perego F, Caccia S
J Investig Allergol Clin Immunol 2016;26(4):212-21; quiz two pages after page 221. doi: 10.18176/jiaci.0087. PMID: 27470642

Recent clinical studies

Etiology

Kiani-Alikhan S, Gower R, Craig T, Wedner HJ, Kinaciyan T, Aygören-Pürsün E, Banerji A, Bernstein JA, Anderson J, Collis P, Johnston DT, Desai B, Tomita D, Gagnon R, Tachdjian R, Soteres DF, Farkas H, Caballero T, McNeil D, Jacobs J, Lumry WR; APeX-2 investigators
J Allergy Clin Immunol Pract 2024 Mar;12(3):733-743.e10. Epub 2023 Dec 18 doi: 10.1016/j.jaip.2023.12.019. PMID: 38122865
Mailer RK, Kuta P, Renné T
Hamostaseologie 2022 Feb;42(1):65-72. Epub 2022 Feb 23 doi: 10.1055/a-1717-7958. PMID: 35196732
Zuraw B, Lumry WR, Johnston DT, Aygören-Pürsün E, Banerji A, Bernstein JA, Christiansen SC, Jacobs JS, Sitz KV, Gower RG, Gagnon R, Wedner HJ, Kinaciyan T, Hakl R, Hanzlíková J, Anderson JT, McNeil DL, Fritz SB, Yang WH, Tachdjian R, Busse PJ, Craig TJ, Li HH, Farkas H, Best JM, Clemons D, Cornpropst M, Dobo SM, Iocca HA, Kargl D, Nagy E, Murray SC, Collis P, Sheridan WP, Maurer M, Riedl MA
J Allergy Clin Immunol 2021 Jul;148(1):164-172.e9. Epub 2020 Oct 21 doi: 10.1016/j.jaci.2020.10.015. PMID: 33098856
Wu MA
Expert Opin Biol Ther 2019 Dec;19(12):1233-1245. Epub 2019 Nov 4 doi: 10.1080/14712598.2019.1685490. PMID: 31657963
Girolami A, Scarparo P, Candeo N, Lombardi AM
Expert Rev Hematol 2010 Dec;3(6):685-95. doi: 10.1586/ehm.10.69. PMID: 21091145

Diagnosis

Patel G, Pongracic JA
Allergy Asthma Proc 2019 Nov 1;40(6):441-445. doi: 10.2500/aap.2019.40.4267. PMID: 31690390
Cicardi M, Suffritti C, Perego F, Caccia S
J Investig Allergol Clin Immunol 2016;26(4):212-21; quiz two pages after page 221. doi: 10.18176/jiaci.0087. PMID: 27470642
Hepner M, Karlaftis V
Methods Mol Biol 2013;992:355-64. doi: 10.1007/978-1-62703-339-8_28. PMID: 23546728
Girolami A, Scarparo P, Candeo N, Lombardi AM
Expert Rev Hematol 2010 Dec;3(6):685-95. doi: 10.1586/ehm.10.69. PMID: 21091145
Bracho FA
Curr Opin Hematol 2005 Nov;12(6):493-8. doi: 10.1097/01.moo.0000179805.57486.4e. PMID: 16217168

Therapy

Kiani-Alikhan S, Gower R, Craig T, Wedner HJ, Kinaciyan T, Aygören-Pürsün E, Banerji A, Bernstein JA, Anderson J, Collis P, Johnston DT, Desai B, Tomita D, Gagnon R, Tachdjian R, Soteres DF, Farkas H, Caballero T, McNeil D, Jacobs J, Lumry WR; APeX-2 investigators
J Allergy Clin Immunol Pract 2024 Mar;12(3):733-743.e10. Epub 2023 Dec 18 doi: 10.1016/j.jaip.2023.12.019. PMID: 38122865
Mailer RK, Kuta P, Renné T
Hamostaseologie 2022 Feb;42(1):65-72. Epub 2022 Feb 23 doi: 10.1055/a-1717-7958. PMID: 35196732
Caballero T
J Investig Allergol Clin Immunol 2021 Feb;31(1):1-16. doi: 10.18176/jiaci.0653. PMID: 33602658
Zuraw B, Lumry WR, Johnston DT, Aygören-Pürsün E, Banerji A, Bernstein JA, Christiansen SC, Jacobs JS, Sitz KV, Gower RG, Gagnon R, Wedner HJ, Kinaciyan T, Hakl R, Hanzlíková J, Anderson JT, McNeil DL, Fritz SB, Yang WH, Tachdjian R, Busse PJ, Craig TJ, Li HH, Farkas H, Best JM, Clemons D, Cornpropst M, Dobo SM, Iocca HA, Kargl D, Nagy E, Murray SC, Collis P, Sheridan WP, Maurer M, Riedl MA
J Allergy Clin Immunol 2021 Jul;148(1):164-172.e9. Epub 2020 Oct 21 doi: 10.1016/j.jaci.2020.10.015. PMID: 33098856
Kaplan AP
Chem Immunol Allergy 2014;100:140-7. Epub 2014 May 22 doi: 10.1159/000358619. PMID: 24925394

Prognosis

Veronez CL, Christiansen SC, Smith TD, Riedl MA, Zuraw BL
Allergy Asthma Proc 2021 Nov 1;42(6):506-514. doi: 10.2500/aap.2021.42.210083. PMID: 34871158Free PMC Article
Kajdácsi E, Jandrasics Z, Veszeli N, Makó V, Koncz A, Gulyás D, Köhalmi KV, Temesszentandrási G, Cervenak L, Gál P, Dobó J, de Maat S, Maas C, Farkas H, Varga L
Front Immunol 2020;11:794. Epub 2020 May 5 doi: 10.3389/fimmu.2020.00794. PMID: 32431708Free PMC Article
Longhurst H, Farkas H
Expert Opin Biol Ther 2020 May;20(5):493-501. Epub 2020 Mar 19 doi: 10.1080/14712598.2020.1724280. PMID: 31994957
Girolami A, Scarparo P, Candeo N, Lombardi AM
Expert Rev Hematol 2010 Dec;3(6):685-95. doi: 10.1586/ehm.10.69. PMID: 21091145
Ebo DG, Verweij MM, De Knop KJ, Hagendorens MM, Bridts CH, De Clerck LS, Stevens WJ
Paediatr Drugs 2010 Aug 1;12(4):257-68. doi: 10.2165/11532590-000000000-00000. PMID: 20593909

Clinical prediction guides

Kiani-Alikhan S, Gower R, Craig T, Wedner HJ, Kinaciyan T, Aygören-Pürsün E, Banerji A, Bernstein JA, Anderson J, Collis P, Johnston DT, Desai B, Tomita D, Gagnon R, Tachdjian R, Soteres DF, Farkas H, Caballero T, McNeil D, Jacobs J, Lumry WR; APeX-2 investigators
J Allergy Clin Immunol Pract 2024 Mar;12(3):733-743.e10. Epub 2023 Dec 18 doi: 10.1016/j.jaip.2023.12.019. PMID: 38122865
Gramstad OR, Schjalm C, Mollnes TE, Nielsen EW
Clin Exp Immunol 2023 Dec 12;214(2):170-181. doi: 10.1093/cei/uxad091. PMID: 37561062Free PMC Article
Miyata T, Horiuchi T
Allergol Int 2023 Jul;72(3):375-384. Epub 2023 May 9 doi: 10.1016/j.alit.2023.04.004. PMID: 37169642
Zuraw B, Lumry WR, Johnston DT, Aygören-Pürsün E, Banerji A, Bernstein JA, Christiansen SC, Jacobs JS, Sitz KV, Gower RG, Gagnon R, Wedner HJ, Kinaciyan T, Hakl R, Hanzlíková J, Anderson JT, McNeil DL, Fritz SB, Yang WH, Tachdjian R, Busse PJ, Craig TJ, Li HH, Farkas H, Best JM, Clemons D, Cornpropst M, Dobo SM, Iocca HA, Kargl D, Nagy E, Murray SC, Collis P, Sheridan WP, Maurer M, Riedl MA
J Allergy Clin Immunol 2021 Jul;148(1):164-172.e9. Epub 2020 Oct 21 doi: 10.1016/j.jaci.2020.10.015. PMID: 33098856
Gailani D, Gruber A
Arterioscler Thromb Vasc Biol 2016 Jul;36(7):1316-22. Epub 2016 May 12 doi: 10.1161/ATVBAHA.116.306925. PMID: 27174099Free PMC Article

Recent systematic reviews

Girolami A, Rolland C, Sexton D, Vardi M, Bernstein JA
Allergy Asthma Proc 2020 Jan 17;41(1):10-18. doi: 10.2500/aap.2020.41.190005. PMID: 31888778

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