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Vaso-Occlusive Crisis in Sickle Cell-Hemoglobin C Disease

MedGen UID:
1793693
•Concept ID:
C5557252
•
Disease or Syndrome
Synonyms: Vaso-Occlusive Crisis in Hemoglobin SC Disease; Vaso-Occlusive Crisis of Hemoglobin SC Disease; Vaso-Occlusive Crisis of Sickle Cell-Hemoglobin C Disease

Definition

Sickle cell-hemoglobin C disease exacerbated by sudden pain caused by sickled erythrocytes impeding blood flow within a vessel. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVaso-Occlusive Crisis in Sickle Cell-Hemoglobin C Disease

Professional guidelines

PubMed

Treatment with recombinant ADAMTS13, alleviates hypoxia/reoxygenation-induced pathologies in a mouse model of human sickle cell disease.
Rossato P, Glantschnig H, Canneva F, Schuster M, Coulibaly S, Schrenk G, Voelkel D, Dockal M, Plaimauer B, Rottensteiner H, Gritsch H, Federti E, Matte A, De Franceschi L, Scheiflinger F, Hoellriegl W
J Thromb Haemost 2023 Feb;21(2):269-275. Epub 2022 Dec 22 doi: 10.1016/j.jtha.2022.10.016. PMID: 36700507
Clinical management of sickle cell hemoglobinopathies during pregnancy.
McLaughlin BN, Martin RW, Morrison JC
Clin Perinatol 1985 Oct;12(3):585-97. PMID: 3902313

Recent clinical studies

Etiology

Rheology of red blood cells in patients with HbC disease.
Lemonne N, Billaud M, Waltz X, Romana M, Hierso R, Etienne-Julan M, Connes P
Clin Hemorheol Microcirc 2016;61(4):571-7. doi: 10.3233/CH-141906. PMID: 25335812
Vasoactive factors in sickle cell disease: in vitro evidence for endothelin-1-mediated vasoconstriction.
Ergul S, Brunson CY, Hutchinson J, Tawfik A, Kutlar A, Webb RC, Ergul A
Am J Hematol 2004 Jul;76(3):245-51. doi: 10.1002/ajh.20107. PMID: 15224360
The increasing prevalence of childhood sickle-cell disease in Ireland.
McMahon C, Callaghan CO, O'Brien D, Smith OP
Ir J Med Sci 2001 Jul-Sep;170(3):183-5. doi: 10.1007/BF03173886. PMID: 12120971
Acute chest syndrome in adults with sickle cell disease.
Maitre B, Habibi A, Roudot-Thoraval F, Bachir D, Belghiti DD, Galacteros F, Godeau B
Chest 2000 May;117(5):1386-92. doi: 10.1378/chest.117.5.1386. PMID: 10807826
Sickle cell disease and infection.
Onwubalili JK
J Infect 1983 Jul;7(1):2-20. doi: 10.1016/s0163-4453(83)90863-0. PMID: 6313809

Diagnosis

Pediatric sickle cell retinopathy: correlation with clinical factors.
Rosenberg JB, Hutcheson KA
J AAPOS 2011 Feb;15(1):49-53. doi: 10.1016/j.jaapos.2010.11.014. PMID: 21397806
The increasing prevalence of childhood sickle-cell disease in Ireland.
McMahon C, Callaghan CO, O'Brien D, Smith OP
Ir J Med Sci 2001 Jul-Sep;170(3):183-5. doi: 10.1007/BF03173886. PMID: 12120971
Acute chest syndrome in adults with sickle cell disease.
Maitre B, Habibi A, Roudot-Thoraval F, Bachir D, Belghiti DD, Galacteros F, Godeau B
Chest 2000 May;117(5):1386-92. doi: 10.1378/chest.117.5.1386. PMID: 10807826
Thrombotic thrombocytopenic purpura associated with sickle cell-hemoglobin C disease.
Chinowsky MS
South Med J 1994 Nov;87(11):1168-71. doi: 10.1097/00007611-199411000-00025. PMID: 7973909
Clinical management of sickle cell hemoglobinopathies during pregnancy.
McLaughlin BN, Martin RW, Morrison JC
Clin Perinatol 1985 Oct;12(3):585-97. PMID: 3902313

Therapy

Hemorheological risk factors of acute chest syndrome and painful vaso-occlusive crisis in children with sickle cell disease.
Lamarre Y, Romana M, Waltz X, Lalanne-Mistrih ML, Tressières B, Divialle-Doumdo L, Hardy-Dessources MD, Vent-Schmidt J, Petras M, Broquere C, Maillard F, Tarer V, Etienne-Julan M, Connes P
Haematologica 2012 Nov;97(11):1641-7. Epub 2012 Jun 11 doi: 10.3324/haematol.2012.066670. PMID: 22689686Free PMC Article
Immunosuppressive therapy for kidney transplant prevents vaso-occlusive crisis in a haemoglobin SC disease patient.
Chies JA, Dresch C, Cruz MS, da Silva G, Barros E, Bittar C, Friedrisch J, Silla LM
Med Hypotheses 2005;64(1):174-6. doi: 10.1016/j.mehy.2004.05.004. PMID: 15533637
A collaborative, double-blind randomized study of cetiedil citrate in sickle cell crisis.
Benjamin LJ, Berkowitz LR, Orringer E, Mankad VN, Prasad AS, Lewkow LM, Chillar RK, Peterson CM
Blood 1986 May;67(5):1442-7. PMID: 3516257

Prognosis

Vasoactive factors in sickle cell disease: in vitro evidence for endothelin-1-mediated vasoconstriction.
Ergul S, Brunson CY, Hutchinson J, Tawfik A, Kutlar A, Webb RC, Ergul A
Am J Hematol 2004 Jul;76(3):245-51. doi: 10.1002/ajh.20107. PMID: 15224360
The increasing prevalence of childhood sickle-cell disease in Ireland.
McMahon C, Callaghan CO, O'Brien D, Smith OP
Ir J Med Sci 2001 Jul-Sep;170(3):183-5. doi: 10.1007/BF03173886. PMID: 12120971
Acute chest syndrome in adults with sickle cell disease.
Maitre B, Habibi A, Roudot-Thoraval F, Bachir D, Belghiti DD, Galacteros F, Godeau B
Chest 2000 May;117(5):1386-92. doi: 10.1378/chest.117.5.1386. PMID: 10807826
A collaborative, double-blind randomized study of cetiedil citrate in sickle cell crisis.
Benjamin LJ, Berkowitz LR, Orringer E, Mankad VN, Prasad AS, Lewkow LM, Chillar RK, Peterson CM
Blood 1986 May;67(5):1442-7. PMID: 3516257
Clinical management of sickle cell hemoglobinopathies during pregnancy.
McLaughlin BN, Martin RW, Morrison JC
Clin Perinatol 1985 Oct;12(3):585-97. PMID: 3902313

Clinical prediction guides

Rheology of red blood cells in patients with HbC disease.
Lemonne N, Billaud M, Waltz X, Romana M, Hierso R, Etienne-Julan M, Connes P
Clin Hemorheol Microcirc 2016;61(4):571-7. doi: 10.3233/CH-141906. PMID: 25335812
The increasing prevalence of childhood sickle-cell disease in Ireland.
McMahon C, Callaghan CO, O'Brien D, Smith OP
Ir J Med Sci 2001 Jul-Sep;170(3):183-5. doi: 10.1007/BF03173886. PMID: 12120971
Disturbance of plasma and platelet thrombospondin levels in sickle cell disease.
Browne PV, Mosher DF, Steinberg MH, Hebbel RP
Am J Hematol 1996 Apr;51(4):296-301. doi: 10.1002/(SICI)1096-8652(199604)51:4<296::AID-AJH8>3.0.CO;2-R. PMID: 8602630
A collaborative, double-blind randomized study of cetiedil citrate in sickle cell crisis.
Benjamin LJ, Berkowitz LR, Orringer E, Mankad VN, Prasad AS, Lewkow LM, Chillar RK, Peterson CM
Blood 1986 May;67(5):1442-7. PMID: 3516257
Clinical management of sickle cell hemoglobinopathies during pregnancy.
McLaughlin BN, Martin RW, Morrison JC
Clin Perinatol 1985 Oct;12(3):585-97. PMID: 3902313

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