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The hemostasis system in children with hereditary spherocytosis
Patients with hereditary spherocytosis (HS) are characterized by having an increased risk for thrombosis. An early manifestation of thrombotic complications can occur even in childhood, especially after surgery. Hypercoagulability can be associated with hemolytic crises. The aim of this study was to...
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| Published in: | Thrombosis research 2019-04, Vol.176, p.11-17 |
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| container_title | Thrombosis research |
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| creator | Seregina, E.A. Poletaev, A.V. Bondar, E.V. Vuimo, T.A. Ataullakhanov, F.I. Smetanina, N.S. |
| description | Patients with hereditary spherocytosis (HS) are characterized by having an increased risk for thrombosis. An early manifestation of thrombotic complications can occur even in childhood, especially after surgery. Hypercoagulability can be associated with hemolytic crises.
The aim of this study was to investigate the hemostatic state in children with HS using global hemostasis assays.
The hemostatic status of 62 children (38 boys and 24 girls; age range: 0.5 to 17 years) with HS during and without hemolytic crisis was assessed using clotting times (APTT, TT, and PR), fibrinogen and D-dimer levels, and global hemostasis, thromboelastography (TEG) and thrombodynamics (TD) assays. One hundred and two healthy children undergoing annual medical examination were enrolled as a control group.
TEG and TD parameters were increased in the children with HS compared to the control group (60 ± 5 mm vs. 53 ± 4 mm, p |
| doi_str_mv | 10.1016/j.thromres.2019.02.004 |
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The aim of this study was to investigate the hemostatic state in children with HS using global hemostasis assays.
The hemostatic status of 62 children (38 boys and 24 girls; age range: 0.5 to 17 years) with HS during and without hemolytic crisis was assessed using clotting times (APTT, TT, and PR), fibrinogen and D-dimer levels, and global hemostasis, thromboelastography (TEG) and thrombodynamics (TD) assays. One hundred and two healthy children undergoing annual medical examination were enrolled as a control group.
TEG and TD parameters were increased in the children with HS compared to the control group (60 ± 5 mm vs. 53 ± 4 mm, p < 0.05 for TEG maximum amplitude; 28 ± 3 μm/min vs. 24 ± 2 μm/min, p < 0.05 for TD clot growth rate), while APTT, TT and PR were not significantly different between the two groups. Patients with HS were divided into 2 groups: those during hemolytic crisis (28 patients) and those without hemolytic crisis (34 patients). TEG and TD parameters were increased in those during hemolytic crisis compared to the steady state HS group (62 ± 5 mm vs. 57 ± 4 mm, p < 0.05 for TEG maximum amplitude; 31 ± 4 μm/min vs. 26 ± 3 μm/min, p < 0.05 for TD clot growth rate). The D-dimer levels were increased in 4 HS patients, for whom the activation of blood clotting was noted. Fibrinogen levels were decreased in patients with HS compared to the control group (2.1 ± 0.4 mg/ml vs. 2.6 ± 0.4 mg/ml, p < 0.05). Other tests were within the reference ranges for both groups.
The global hemostasis tests TEG and TD revealed hypercoagulability in patients with HS. More dramatic changes were observed in patients experiencing a hemolytic crisis.
•Hereditary spherocytosis is a congenital disorder associated with hypecoagulability•Patients may have hemolytic crisis associated with thrombotic complications; however, thrombosis in children is rather rare•Conventional clotting times are not useful for diagnosing the coagulation system in patients with hereditary spherocytosis•The global tests thromboelastography and thrombodynamics revealed hypercoagulability in hereditary spherocytosis</description><identifier>ISSN: 0049-3848</identifier><identifier>EISSN: 1879-2472</identifier><identifier>DOI: 10.1016/j.thromres.2019.02.004</identifier><identifier>PMID: 30763822</identifier><language>eng</language><publisher>United States: Elsevier Ltd</publisher><subject>Adolescent ; Blood coagulation ; Child ; Child, Preschool ; Clotting times ; Female ; Fibrin Fibrinogen Degradation Products - analysis ; Fibrinogen - analysis ; Global hemostatic assays ; Hemostasis ; Hemostasis during hemolytic crisis in children ; Hemostasis in hemolytic anemia ; Hemostatic function ; Humans ; Hypercoagulability ; Infant ; Male ; Spherocytosis, Hereditary - blood ; Thrombelastography ; Thrombodynamics ; Thromboelastography ; Thrombophilia - blood</subject><ispartof>Thrombosis research, 2019-04, Vol.176, p.11-17</ispartof><rights>2019 Elsevier Ltd</rights><rights>Copyright © 2019 Elsevier Ltd. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c368t-670ec5bd4a190c277572df81822a4c98f5a3053444105faa81a87ef3593269fa3</citedby><cites>FETCH-LOGICAL-c368t-670ec5bd4a190c277572df81822a4c98f5a3053444105faa81a87ef3593269fa3</cites><orcidid>0000-0002-7534-3863</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/30763822$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Seregina, E.A.</creatorcontrib><creatorcontrib>Poletaev, A.V.</creatorcontrib><creatorcontrib>Bondar, E.V.</creatorcontrib><creatorcontrib>Vuimo, T.A.</creatorcontrib><creatorcontrib>Ataullakhanov, F.I.</creatorcontrib><creatorcontrib>Smetanina, N.S.</creatorcontrib><title>The hemostasis system in children with hereditary spherocytosis</title><title>Thrombosis research</title><addtitle>Thromb Res</addtitle><description>Patients with hereditary spherocytosis (HS) are characterized by having an increased risk for thrombosis. An early manifestation of thrombotic complications can occur even in childhood, especially after surgery. Hypercoagulability can be associated with hemolytic crises.
The aim of this study was to investigate the hemostatic state in children with HS using global hemostasis assays.
The hemostatic status of 62 children (38 boys and 24 girls; age range: 0.5 to 17 years) with HS during and without hemolytic crisis was assessed using clotting times (APTT, TT, and PR), fibrinogen and D-dimer levels, and global hemostasis, thromboelastography (TEG) and thrombodynamics (TD) assays. One hundred and two healthy children undergoing annual medical examination were enrolled as a control group.
TEG and TD parameters were increased in the children with HS compared to the control group (60 ± 5 mm vs. 53 ± 4 mm, p < 0.05 for TEG maximum amplitude; 28 ± 3 μm/min vs. 24 ± 2 μm/min, p < 0.05 for TD clot growth rate), while APTT, TT and PR were not significantly different between the two groups. Patients with HS were divided into 2 groups: those during hemolytic crisis (28 patients) and those without hemolytic crisis (34 patients). TEG and TD parameters were increased in those during hemolytic crisis compared to the steady state HS group (62 ± 5 mm vs. 57 ± 4 mm, p < 0.05 for TEG maximum amplitude; 31 ± 4 μm/min vs. 26 ± 3 μm/min, p < 0.05 for TD clot growth rate). The D-dimer levels were increased in 4 HS patients, for whom the activation of blood clotting was noted. Fibrinogen levels were decreased in patients with HS compared to the control group (2.1 ± 0.4 mg/ml vs. 2.6 ± 0.4 mg/ml, p < 0.05). Other tests were within the reference ranges for both groups.
The global hemostasis tests TEG and TD revealed hypercoagulability in patients with HS. More dramatic changes were observed in patients experiencing a hemolytic crisis.
•Hereditary spherocytosis is a congenital disorder associated with hypecoagulability•Patients may have hemolytic crisis associated with thrombotic complications; however, thrombosis in children is rather rare•Conventional clotting times are not useful for diagnosing the coagulation system in patients with hereditary spherocytosis•The global tests thromboelastography and thrombodynamics revealed hypercoagulability in hereditary spherocytosis</description><subject>Adolescent</subject><subject>Blood coagulation</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Clotting times</subject><subject>Female</subject><subject>Fibrin Fibrinogen Degradation Products - analysis</subject><subject>Fibrinogen - analysis</subject><subject>Global hemostatic assays</subject><subject>Hemostasis</subject><subject>Hemostasis during hemolytic crisis in children</subject><subject>Hemostasis in hemolytic anemia</subject><subject>Hemostatic function</subject><subject>Humans</subject><subject>Hypercoagulability</subject><subject>Infant</subject><subject>Male</subject><subject>Spherocytosis, Hereditary - blood</subject><subject>Thrombelastography</subject><subject>Thrombodynamics</subject><subject>Thromboelastography</subject><subject>Thrombophilia - blood</subject><issn>0049-3848</issn><issn>1879-2472</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><recordid>eNqFkMtOwzAQRS0EoqXwC1WWbBL8SmyvACFeUiU2ZW25zkRxlUexXVD_Hldt2bIaeXTuzPggNCe4IJhUd-sitn7sPYSCYqIKTAuM-RmaEilUTrmg52iaOipnkssJugphjTERRJWXaMKwqJikdIruly1kLfRjiCa4kIVdiNBnbshs67raw5D9uNgmxEPtovG7LGzSY7S7OKbANbpoTBfg5lhn6PPlefn0li8-Xt-fHhe5ZZWMeSUw2HJVc0MUtlSIUtC6kSTdYLhVsikNwyXjnBNcNsZIYqSAhpWK0Uo1hs3Q7WHuxo9fWwhR9y5Y6DozwLgNmhLJiJRpbkKrA2r9GIKHRm-869PlmmC9l6fX-iRP7-VpTHVSlYLz447tqof6L3aylYCHAwDpp98OvA7WwWCTGQ826np0_-34BV6Pg_Y</recordid><startdate>201904</startdate><enddate>201904</enddate><creator>Seregina, E.A.</creator><creator>Poletaev, A.V.</creator><creator>Bondar, E.V.</creator><creator>Vuimo, T.A.</creator><creator>Ataullakhanov, F.I.</creator><creator>Smetanina, N.S.</creator><general>Elsevier Ltd</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-7534-3863</orcidid></search><sort><creationdate>201904</creationdate><title>The hemostasis system in children with hereditary spherocytosis</title><author>Seregina, E.A. ; Poletaev, A.V. ; Bondar, E.V. ; Vuimo, T.A. ; Ataullakhanov, F.I. ; Smetanina, N.S.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c368t-670ec5bd4a190c277572df81822a4c98f5a3053444105faa81a87ef3593269fa3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>Adolescent</topic><topic>Blood coagulation</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Clotting times</topic><topic>Female</topic><topic>Fibrin Fibrinogen Degradation Products - analysis</topic><topic>Fibrinogen - analysis</topic><topic>Global hemostatic assays</topic><topic>Hemostasis</topic><topic>Hemostasis during hemolytic crisis in children</topic><topic>Hemostasis in hemolytic anemia</topic><topic>Hemostatic function</topic><topic>Humans</topic><topic>Hypercoagulability</topic><topic>Infant</topic><topic>Male</topic><topic>Spherocytosis, Hereditary - blood</topic><topic>Thrombelastography</topic><topic>Thrombodynamics</topic><topic>Thromboelastography</topic><topic>Thrombophilia - blood</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Seregina, E.A.</creatorcontrib><creatorcontrib>Poletaev, A.V.</creatorcontrib><creatorcontrib>Bondar, E.V.</creatorcontrib><creatorcontrib>Vuimo, T.A.</creatorcontrib><creatorcontrib>Ataullakhanov, F.I.</creatorcontrib><creatorcontrib>Smetanina, N.S.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Thrombosis research</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Seregina, E.A.</au><au>Poletaev, A.V.</au><au>Bondar, E.V.</au><au>Vuimo, T.A.</au><au>Ataullakhanov, F.I.</au><au>Smetanina, N.S.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>The hemostasis system in children with hereditary spherocytosis</atitle><jtitle>Thrombosis research</jtitle><addtitle>Thromb Res</addtitle><date>2019-04</date><risdate>2019</risdate><volume>176</volume><spage>11</spage><epage>17</epage><pages>11-17</pages><issn>0049-3848</issn><eissn>1879-2472</eissn><abstract>Patients with hereditary spherocytosis (HS) are characterized by having an increased risk for thrombosis. An early manifestation of thrombotic complications can occur even in childhood, especially after surgery. Hypercoagulability can be associated with hemolytic crises.
The aim of this study was to investigate the hemostatic state in children with HS using global hemostasis assays.
The hemostatic status of 62 children (38 boys and 24 girls; age range: 0.5 to 17 years) with HS during and without hemolytic crisis was assessed using clotting times (APTT, TT, and PR), fibrinogen and D-dimer levels, and global hemostasis, thromboelastography (TEG) and thrombodynamics (TD) assays. One hundred and two healthy children undergoing annual medical examination were enrolled as a control group.
TEG and TD parameters were increased in the children with HS compared to the control group (60 ± 5 mm vs. 53 ± 4 mm, p < 0.05 for TEG maximum amplitude; 28 ± 3 μm/min vs. 24 ± 2 μm/min, p < 0.05 for TD clot growth rate), while APTT, TT and PR were not significantly different between the two groups. Patients with HS were divided into 2 groups: those during hemolytic crisis (28 patients) and those without hemolytic crisis (34 patients). TEG and TD parameters were increased in those during hemolytic crisis compared to the steady state HS group (62 ± 5 mm vs. 57 ± 4 mm, p < 0.05 for TEG maximum amplitude; 31 ± 4 μm/min vs. 26 ± 3 μm/min, p < 0.05 for TD clot growth rate). The D-dimer levels were increased in 4 HS patients, for whom the activation of blood clotting was noted. Fibrinogen levels were decreased in patients with HS compared to the control group (2.1 ± 0.4 mg/ml vs. 2.6 ± 0.4 mg/ml, p < 0.05). Other tests were within the reference ranges for both groups.
The global hemostasis tests TEG and TD revealed hypercoagulability in patients with HS. More dramatic changes were observed in patients experiencing a hemolytic crisis.
•Hereditary spherocytosis is a congenital disorder associated with hypecoagulability•Patients may have hemolytic crisis associated with thrombotic complications; however, thrombosis in children is rather rare•Conventional clotting times are not useful for diagnosing the coagulation system in patients with hereditary spherocytosis•The global tests thromboelastography and thrombodynamics revealed hypercoagulability in hereditary spherocytosis</abstract><cop>United States</cop><pub>Elsevier Ltd</pub><pmid>30763822</pmid><doi>10.1016/j.thromres.2019.02.004</doi><tpages>7</tpages><orcidid>https://orcid.org/0000-0002-7534-3863</orcidid></addata></record> |
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| subjects | Adolescent Blood coagulation Child Child, Preschool Clotting times Female Fibrin Fibrinogen Degradation Products - analysis Fibrinogen - analysis Global hemostatic assays Hemostasis Hemostasis during hemolytic crisis in children Hemostasis in hemolytic anemia Hemostatic function Humans Hypercoagulability Infant Male Spherocytosis, Hereditary - blood Thrombelastography Thrombodynamics Thromboelastography Thrombophilia - blood |
| title | The hemostasis system in children with hereditary spherocytosis |
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