Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood
Background Previous natural history studies have advanced the understanding of sickle cell disease (SCD), but generally have not included sufficient lifespan data or investigation of the role of genetics in clinical outcomes, and have often occurred before the widespread use of disease‐modifying the...
Gespeichert in:
Veröffentlicht in: | Pediatric blood & cancer 2018-09, Vol.65 (9), p.e27228-n/a |
---|---|
Hauptverfasser: | , , , , , , , , , , , , , , , , , , , , , , |
Format: | Artikel |
Sprache: | eng |
Schlagworte: | |
Online-Zugang: | Volltext |
Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
container_end_page | n/a |
---|---|
container_issue | 9 |
container_start_page | e27228 |
container_title | Pediatric blood & cancer |
container_volume | 65 |
creator | Hankins, Jane S. Estepp, Jeremie H. Hodges, Jason R. Villavicencio, Martha A. Robison, Leslie L. Weiss, Mitchell J. Kang, Guolian Schreiber, Jane E. Porter, Jerlym S. Kaste, Sue C. Saving, Kay L. Bryant, Paulette C. Deyo, Jeffrey E. Nottage, Kerri A. King, Allison A. Brandow, Amanda M. Lebensburger, Jeffrey D. Adesina, Oyebimpe Chou, Stella T. Zemel, Babette S. Smeltzer, Matthew P. Wang, Winfred C. Gurney, James G. |
description | Background
Previous natural history studies have advanced the understanding of sickle cell disease (SCD), but generally have not included sufficient lifespan data or investigation of the role of genetics in clinical outcomes, and have often occurred before the widespread use of disease‐modifying therapies, such as hydroxyurea and chronic erythrocyte transfusions. To further advance knowledge of SCD, St. Jude Children's Research Hospital established the Sickle Cell Clinical Research and Intervention Program (SCCRIP), to conduct research in a clinically evaluated cohort of individuals with SCD across their lifetime.
Procedures
Initiated in 2014, the SCCRIP study prospectively recruits patients diagnosed with SCD and includes retrospective and longitudinal collection of clinical, neurocognitive, geospatial, psychosocial, and health outcomes data. Biological samples are banked for future genomics and proteomics studies. The organizational structure of SCCRIP is based upon organ/system‐specific working groups and is opened to the research community for partnerships.
Results
As of August 2017, 1,044 (92.3% of eligible) patients with SCD have enrolled in the study (860 children and 184 adults), with 11,915 person‐years of observation. Population demographics included mean age at last visit of 11.3 years (range 0.7–30.1), 49.8% females, 57.7% treated with hydroxyurea, 8.5% treated with monthly transfusions, and 62.9% hemoglobin (Hb) SS or HbSB0‐thalassemia, 25.7% HbSC, 8.4% HbsB+‐Thalassemia, 1.7% HbS/HPFH, and 1.2% other.
Conclusions
The SCCRIP cohort will provide a rich resource for the conduct of high impact multidisciplinary research in SCD. |
doi_str_mv | 10.1002/pbc.27228 |
format | Article |
fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_2045275094</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2073801572</sourcerecordid><originalsourceid>FETCH-LOGICAL-c3538-5a8de3336896dcc6e3bc4c8e87b72eb372f16978625354f3554618535d2f22d33</originalsourceid><addsrcrecordid>eNp1kcFu1DAQhiMEoqVw4AWQJS7tYVvHjmOHW4mgrFSpqxbOkWNPui6OvbUd0L4PD4rTlB6QeprR6JtPv_QXxfsSn5YYk7Ndr04JJ0S8KA5LVrEVwyV_-bTj5qB4E-NdRmvMxOvigDS84XVVHRZ_boz6aQG1YC1qrXFGSYuuIYIMaouk02jtEoRf4JLxDm2Cvw1yRMc3bXu93px8QufImgHiTjqk_NaHhGKa9B4NPqC4yNUs1yY7I6DdbIAYZ9sQ_IjSNh9BG5mCUflZ3gIyLnkk9WTT1nv9tng1SBvh3eM8Kn58_fK9_ba6vLpYt-eXK0UZFSsmhQZKaS2aWitVA-1VpQQI3nMCPeVkKOuGi5owyqqBMlbVpci7JgMhmtKj4njx5oz3E8TUjSbO4aUDP8WO4IoRznBTZfTjf-idn4LL6TLFqcAl4yRTJwulgo8xwNDtghll2Hcl7ubqulxd91BdZj88Gqd-BP1E_usqA2cL8NtY2D9v6jaf20X5Fxs3otw</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2073801572</pqid></control><display><type>article</type><title>Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood</title><source>MEDLINE</source><source>Wiley Online Library Journals Frontfile Complete</source><creator>Hankins, Jane S. ; Estepp, Jeremie H. ; Hodges, Jason R. ; Villavicencio, Martha A. ; Robison, Leslie L. ; Weiss, Mitchell J. ; Kang, Guolian ; Schreiber, Jane E. ; Porter, Jerlym S. ; Kaste, Sue C. ; Saving, Kay L. ; Bryant, Paulette C. ; Deyo, Jeffrey E. ; Nottage, Kerri A. ; King, Allison A. ; Brandow, Amanda M. ; Lebensburger, Jeffrey D. ; Adesina, Oyebimpe ; Chou, Stella T. ; Zemel, Babette S. ; Smeltzer, Matthew P. ; Wang, Winfred C. ; Gurney, James G.</creator><creatorcontrib>Hankins, Jane S. ; Estepp, Jeremie H. ; Hodges, Jason R. ; Villavicencio, Martha A. ; Robison, Leslie L. ; Weiss, Mitchell J. ; Kang, Guolian ; Schreiber, Jane E. ; Porter, Jerlym S. ; Kaste, Sue C. ; Saving, Kay L. ; Bryant, Paulette C. ; Deyo, Jeffrey E. ; Nottage, Kerri A. ; King, Allison A. ; Brandow, Amanda M. ; Lebensburger, Jeffrey D. ; Adesina, Oyebimpe ; Chou, Stella T. ; Zemel, Babette S. ; Smeltzer, Matthew P. ; Wang, Winfred C. ; Gurney, James G.</creatorcontrib><description>Background
Previous natural history studies have advanced the understanding of sickle cell disease (SCD), but generally have not included sufficient lifespan data or investigation of the role of genetics in clinical outcomes, and have often occurred before the widespread use of disease‐modifying therapies, such as hydroxyurea and chronic erythrocyte transfusions. To further advance knowledge of SCD, St. Jude Children's Research Hospital established the Sickle Cell Clinical Research and Intervention Program (SCCRIP), to conduct research in a clinically evaluated cohort of individuals with SCD across their lifetime.
Procedures
Initiated in 2014, the SCCRIP study prospectively recruits patients diagnosed with SCD and includes retrospective and longitudinal collection of clinical, neurocognitive, geospatial, psychosocial, and health outcomes data. Biological samples are banked for future genomics and proteomics studies. The organizational structure of SCCRIP is based upon organ/system‐specific working groups and is opened to the research community for partnerships.
Results
As of August 2017, 1,044 (92.3% of eligible) patients with SCD have enrolled in the study (860 children and 184 adults), with 11,915 person‐years of observation. Population demographics included mean age at last visit of 11.3 years (range 0.7–30.1), 49.8% females, 57.7% treated with hydroxyurea, 8.5% treated with monthly transfusions, and 62.9% hemoglobin (Hb) SS or HbSB0‐thalassemia, 25.7% HbSC, 8.4% HbsB+‐Thalassemia, 1.7% HbS/HPFH, and 1.2% other.
Conclusions
The SCCRIP cohort will provide a rich resource for the conduct of high impact multidisciplinary research in SCD.</description><identifier>ISSN: 1545-5009</identifier><identifier>EISSN: 1545-5017</identifier><identifier>DOI: 10.1002/pbc.27228</identifier><identifier>PMID: 29797644</identifier><language>eng</language><publisher>United States: Wiley Subscription Services, Inc</publisher><subject>Adolescent ; Adult ; Anemia, Sickle Cell - genetics ; Anemia, Sickle Cell - mortality ; Anemia, Sickle Cell - therapy ; Biological properties ; Biological samples ; Biological Specimen Banks - organization & administration ; Blood Transfusion ; Body Fluids ; Child ; Child, Preschool ; Children ; Cognition ; Cohort analysis ; Demographics ; Demography ; Disease Progression ; disease‐modifying therapy ; Female ; Females ; Follow-Up Studies ; Genetics ; Genomics ; Genotype ; Hematology ; Hemoglobin ; Hemoglobinopathies - genetics ; Humans ; Hydroxyurea ; Hydroxyurea - therapeutic use ; Infant ; Informed Consent ; Life span ; Longevity ; Longitudinal Studies ; Male ; Multidisciplinary research ; natural history ; Oncology ; Organizational structure ; Partnerships ; Patient Selection ; Pediatrics ; Prospective Studies ; Proteomics ; Research Design ; Sampling Studies ; sickle cell anemia ; Sickle cell disease ; Thalassemia ; United States - epidemiology</subject><ispartof>Pediatric blood & cancer, 2018-09, Vol.65 (9), p.e27228-n/a</ispartof><rights>2018 Wiley Periodicals, Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3538-5a8de3336896dcc6e3bc4c8e87b72eb372f16978625354f3554618535d2f22d33</citedby><cites>FETCH-LOGICAL-c3538-5a8de3336896dcc6e3bc4c8e87b72eb372f16978625354f3554618535d2f22d33</cites><orcidid>0000-0003-4439-7321</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fpbc.27228$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fpbc.27228$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29797644$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Hankins, Jane S.</creatorcontrib><creatorcontrib>Estepp, Jeremie H.</creatorcontrib><creatorcontrib>Hodges, Jason R.</creatorcontrib><creatorcontrib>Villavicencio, Martha A.</creatorcontrib><creatorcontrib>Robison, Leslie L.</creatorcontrib><creatorcontrib>Weiss, Mitchell J.</creatorcontrib><creatorcontrib>Kang, Guolian</creatorcontrib><creatorcontrib>Schreiber, Jane E.</creatorcontrib><creatorcontrib>Porter, Jerlym S.</creatorcontrib><creatorcontrib>Kaste, Sue C.</creatorcontrib><creatorcontrib>Saving, Kay L.</creatorcontrib><creatorcontrib>Bryant, Paulette C.</creatorcontrib><creatorcontrib>Deyo, Jeffrey E.</creatorcontrib><creatorcontrib>Nottage, Kerri A.</creatorcontrib><creatorcontrib>King, Allison A.</creatorcontrib><creatorcontrib>Brandow, Amanda M.</creatorcontrib><creatorcontrib>Lebensburger, Jeffrey D.</creatorcontrib><creatorcontrib>Adesina, Oyebimpe</creatorcontrib><creatorcontrib>Chou, Stella T.</creatorcontrib><creatorcontrib>Zemel, Babette S.</creatorcontrib><creatorcontrib>Smeltzer, Matthew P.</creatorcontrib><creatorcontrib>Wang, Winfred C.</creatorcontrib><creatorcontrib>Gurney, James G.</creatorcontrib><title>Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood</title><title>Pediatric blood & cancer</title><addtitle>Pediatr Blood Cancer</addtitle><description>Background
Previous natural history studies have advanced the understanding of sickle cell disease (SCD), but generally have not included sufficient lifespan data or investigation of the role of genetics in clinical outcomes, and have often occurred before the widespread use of disease‐modifying therapies, such as hydroxyurea and chronic erythrocyte transfusions. To further advance knowledge of SCD, St. Jude Children's Research Hospital established the Sickle Cell Clinical Research and Intervention Program (SCCRIP), to conduct research in a clinically evaluated cohort of individuals with SCD across their lifetime.
Procedures
Initiated in 2014, the SCCRIP study prospectively recruits patients diagnosed with SCD and includes retrospective and longitudinal collection of clinical, neurocognitive, geospatial, psychosocial, and health outcomes data. Biological samples are banked for future genomics and proteomics studies. The organizational structure of SCCRIP is based upon organ/system‐specific working groups and is opened to the research community for partnerships.
Results
As of August 2017, 1,044 (92.3% of eligible) patients with SCD have enrolled in the study (860 children and 184 adults), with 11,915 person‐years of observation. Population demographics included mean age at last visit of 11.3 years (range 0.7–30.1), 49.8% females, 57.7% treated with hydroxyurea, 8.5% treated with monthly transfusions, and 62.9% hemoglobin (Hb) SS or HbSB0‐thalassemia, 25.7% HbSC, 8.4% HbsB+‐Thalassemia, 1.7% HbS/HPFH, and 1.2% other.
Conclusions
The SCCRIP cohort will provide a rich resource for the conduct of high impact multidisciplinary research in SCD.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Anemia, Sickle Cell - genetics</subject><subject>Anemia, Sickle Cell - mortality</subject><subject>Anemia, Sickle Cell - therapy</subject><subject>Biological properties</subject><subject>Biological samples</subject><subject>Biological Specimen Banks - organization & administration</subject><subject>Blood Transfusion</subject><subject>Body Fluids</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Children</subject><subject>Cognition</subject><subject>Cohort analysis</subject><subject>Demographics</subject><subject>Demography</subject><subject>Disease Progression</subject><subject>disease‐modifying therapy</subject><subject>Female</subject><subject>Females</subject><subject>Follow-Up Studies</subject><subject>Genetics</subject><subject>Genomics</subject><subject>Genotype</subject><subject>Hematology</subject><subject>Hemoglobin</subject><subject>Hemoglobinopathies - genetics</subject><subject>Humans</subject><subject>Hydroxyurea</subject><subject>Hydroxyurea - therapeutic use</subject><subject>Infant</subject><subject>Informed Consent</subject><subject>Life span</subject><subject>Longevity</subject><subject>Longitudinal Studies</subject><subject>Male</subject><subject>Multidisciplinary research</subject><subject>natural history</subject><subject>Oncology</subject><subject>Organizational structure</subject><subject>Partnerships</subject><subject>Patient Selection</subject><subject>Pediatrics</subject><subject>Prospective Studies</subject><subject>Proteomics</subject><subject>Research Design</subject><subject>Sampling Studies</subject><subject>sickle cell anemia</subject><subject>Sickle cell disease</subject><subject>Thalassemia</subject><subject>United States - epidemiology</subject><issn>1545-5009</issn><issn>1545-5017</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kcFu1DAQhiMEoqVw4AWQJS7tYVvHjmOHW4mgrFSpqxbOkWNPui6OvbUd0L4PD4rTlB6QeprR6JtPv_QXxfsSn5YYk7Ndr04JJ0S8KA5LVrEVwyV_-bTj5qB4E-NdRmvMxOvigDS84XVVHRZ_boz6aQG1YC1qrXFGSYuuIYIMaouk02jtEoRf4JLxDm2Cvw1yRMc3bXu93px8QufImgHiTjqk_NaHhGKa9B4NPqC4yNUs1yY7I6DdbIAYZ9sQ_IjSNh9BG5mCUflZ3gIyLnkk9WTT1nv9tng1SBvh3eM8Kn58_fK9_ba6vLpYt-eXK0UZFSsmhQZKaS2aWitVA-1VpQQI3nMCPeVkKOuGi5owyqqBMlbVpci7JgMhmtKj4njx5oz3E8TUjSbO4aUDP8WO4IoRznBTZfTjf-idn4LL6TLFqcAl4yRTJwulgo8xwNDtghll2Hcl7ubqulxd91BdZj88Gqd-BP1E_usqA2cL8NtY2D9v6jaf20X5Fxs3otw</recordid><startdate>201809</startdate><enddate>201809</enddate><creator>Hankins, Jane S.</creator><creator>Estepp, Jeremie H.</creator><creator>Hodges, Jason R.</creator><creator>Villavicencio, Martha A.</creator><creator>Robison, Leslie L.</creator><creator>Weiss, Mitchell J.</creator><creator>Kang, Guolian</creator><creator>Schreiber, Jane E.</creator><creator>Porter, Jerlym S.</creator><creator>Kaste, Sue C.</creator><creator>Saving, Kay L.</creator><creator>Bryant, Paulette C.</creator><creator>Deyo, Jeffrey E.</creator><creator>Nottage, Kerri A.</creator><creator>King, Allison A.</creator><creator>Brandow, Amanda M.</creator><creator>Lebensburger, Jeffrey D.</creator><creator>Adesina, Oyebimpe</creator><creator>Chou, Stella T.</creator><creator>Zemel, Babette S.</creator><creator>Smeltzer, Matthew P.</creator><creator>Wang, Winfred C.</creator><creator>Gurney, James G.</creator><general>Wiley Subscription Services, Inc</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>7T5</scope><scope>7TK</scope><scope>7TO</scope><scope>8FD</scope><scope>FR3</scope><scope>H94</scope><scope>K9.</scope><scope>P64</scope><scope>RC3</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0003-4439-7321</orcidid></search><sort><creationdate>201809</creationdate><title>Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood</title><author>Hankins, Jane S. ; Estepp, Jeremie H. ; Hodges, Jason R. ; Villavicencio, Martha A. ; Robison, Leslie L. ; Weiss, Mitchell J. ; Kang, Guolian ; Schreiber, Jane E. ; Porter, Jerlym S. ; Kaste, Sue C. ; Saving, Kay L. ; Bryant, Paulette C. ; Deyo, Jeffrey E. ; Nottage, Kerri A. ; King, Allison A. ; Brandow, Amanda M. ; Lebensburger, Jeffrey D. ; Adesina, Oyebimpe ; Chou, Stella T. ; Zemel, Babette S. ; Smeltzer, Matthew P. ; Wang, Winfred C. ; Gurney, James G.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3538-5a8de3336896dcc6e3bc4c8e87b72eb372f16978625354f3554618535d2f22d33</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Anemia, Sickle Cell - genetics</topic><topic>Anemia, Sickle Cell - mortality</topic><topic>Anemia, Sickle Cell - therapy</topic><topic>Biological properties</topic><topic>Biological samples</topic><topic>Biological Specimen Banks - organization & administration</topic><topic>Blood Transfusion</topic><topic>Body Fluids</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Children</topic><topic>Cognition</topic><topic>Cohort analysis</topic><topic>Demographics</topic><topic>Demography</topic><topic>Disease Progression</topic><topic>disease‐modifying therapy</topic><topic>Female</topic><topic>Females</topic><topic>Follow-Up Studies</topic><topic>Genetics</topic><topic>Genomics</topic><topic>Genotype</topic><topic>Hematology</topic><topic>Hemoglobin</topic><topic>Hemoglobinopathies - genetics</topic><topic>Humans</topic><topic>Hydroxyurea</topic><topic>Hydroxyurea - therapeutic use</topic><topic>Infant</topic><topic>Informed Consent</topic><topic>Life span</topic><topic>Longevity</topic><topic>Longitudinal Studies</topic><topic>Male</topic><topic>Multidisciplinary research</topic><topic>natural history</topic><topic>Oncology</topic><topic>Organizational structure</topic><topic>Partnerships</topic><topic>Patient Selection</topic><topic>Pediatrics</topic><topic>Prospective Studies</topic><topic>Proteomics</topic><topic>Research Design</topic><topic>Sampling Studies</topic><topic>sickle cell anemia</topic><topic>Sickle cell disease</topic><topic>Thalassemia</topic><topic>United States - epidemiology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Hankins, Jane S.</creatorcontrib><creatorcontrib>Estepp, Jeremie H.</creatorcontrib><creatorcontrib>Hodges, Jason R.</creatorcontrib><creatorcontrib>Villavicencio, Martha A.</creatorcontrib><creatorcontrib>Robison, Leslie L.</creatorcontrib><creatorcontrib>Weiss, Mitchell J.</creatorcontrib><creatorcontrib>Kang, Guolian</creatorcontrib><creatorcontrib>Schreiber, Jane E.</creatorcontrib><creatorcontrib>Porter, Jerlym S.</creatorcontrib><creatorcontrib>Kaste, Sue C.</creatorcontrib><creatorcontrib>Saving, Kay L.</creatorcontrib><creatorcontrib>Bryant, Paulette C.</creatorcontrib><creatorcontrib>Deyo, Jeffrey E.</creatorcontrib><creatorcontrib>Nottage, Kerri A.</creatorcontrib><creatorcontrib>King, Allison A.</creatorcontrib><creatorcontrib>Brandow, Amanda M.</creatorcontrib><creatorcontrib>Lebensburger, Jeffrey D.</creatorcontrib><creatorcontrib>Adesina, Oyebimpe</creatorcontrib><creatorcontrib>Chou, Stella T.</creatorcontrib><creatorcontrib>Zemel, Babette S.</creatorcontrib><creatorcontrib>Smeltzer, Matthew P.</creatorcontrib><creatorcontrib>Wang, Winfred C.</creatorcontrib><creatorcontrib>Gurney, James G.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Oncogenes and Growth Factors Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric blood & cancer</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Hankins, Jane S.</au><au>Estepp, Jeremie H.</au><au>Hodges, Jason R.</au><au>Villavicencio, Martha A.</au><au>Robison, Leslie L.</au><au>Weiss, Mitchell J.</au><au>Kang, Guolian</au><au>Schreiber, Jane E.</au><au>Porter, Jerlym S.</au><au>Kaste, Sue C.</au><au>Saving, Kay L.</au><au>Bryant, Paulette C.</au><au>Deyo, Jeffrey E.</au><au>Nottage, Kerri A.</au><au>King, Allison A.</au><au>Brandow, Amanda M.</au><au>Lebensburger, Jeffrey D.</au><au>Adesina, Oyebimpe</au><au>Chou, Stella T.</au><au>Zemel, Babette S.</au><au>Smeltzer, Matthew P.</au><au>Wang, Winfred C.</au><au>Gurney, James G.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood</atitle><jtitle>Pediatric blood & cancer</jtitle><addtitle>Pediatr Blood Cancer</addtitle><date>2018-09</date><risdate>2018</risdate><volume>65</volume><issue>9</issue><spage>e27228</spage><epage>n/a</epage><pages>e27228-n/a</pages><issn>1545-5009</issn><eissn>1545-5017</eissn><abstract>Background
Previous natural history studies have advanced the understanding of sickle cell disease (SCD), but generally have not included sufficient lifespan data or investigation of the role of genetics in clinical outcomes, and have often occurred before the widespread use of disease‐modifying therapies, such as hydroxyurea and chronic erythrocyte transfusions. To further advance knowledge of SCD, St. Jude Children's Research Hospital established the Sickle Cell Clinical Research and Intervention Program (SCCRIP), to conduct research in a clinically evaluated cohort of individuals with SCD across their lifetime.
Procedures
Initiated in 2014, the SCCRIP study prospectively recruits patients diagnosed with SCD and includes retrospective and longitudinal collection of clinical, neurocognitive, geospatial, psychosocial, and health outcomes data. Biological samples are banked for future genomics and proteomics studies. The organizational structure of SCCRIP is based upon organ/system‐specific working groups and is opened to the research community for partnerships.
Results
As of August 2017, 1,044 (92.3% of eligible) patients with SCD have enrolled in the study (860 children and 184 adults), with 11,915 person‐years of observation. Population demographics included mean age at last visit of 11.3 years (range 0.7–30.1), 49.8% females, 57.7% treated with hydroxyurea, 8.5% treated with monthly transfusions, and 62.9% hemoglobin (Hb) SS or HbSB0‐thalassemia, 25.7% HbSC, 8.4% HbsB+‐Thalassemia, 1.7% HbS/HPFH, and 1.2% other.
Conclusions
The SCCRIP cohort will provide a rich resource for the conduct of high impact multidisciplinary research in SCD.</abstract><cop>United States</cop><pub>Wiley Subscription Services, Inc</pub><pmid>29797644</pmid><doi>10.1002/pbc.27228</doi><tpages>12</tpages><orcidid>https://orcid.org/0000-0003-4439-7321</orcidid></addata></record> |
fulltext | fulltext |
identifier | ISSN: 1545-5009 |
ispartof | Pediatric blood & cancer, 2018-09, Vol.65 (9), p.e27228-n/a |
issn | 1545-5009 1545-5017 |
language | eng |
recordid | cdi_proquest_miscellaneous_2045275094 |
source | MEDLINE; Wiley Online Library Journals Frontfile Complete |
subjects | Adolescent Adult Anemia, Sickle Cell - genetics Anemia, Sickle Cell - mortality Anemia, Sickle Cell - therapy Biological properties Biological samples Biological Specimen Banks - organization & administration Blood Transfusion Body Fluids Child Child, Preschool Children Cognition Cohort analysis Demographics Demography Disease Progression disease‐modifying therapy Female Females Follow-Up Studies Genetics Genomics Genotype Hematology Hemoglobin Hemoglobinopathies - genetics Humans Hydroxyurea Hydroxyurea - therapeutic use Infant Informed Consent Life span Longevity Longitudinal Studies Male Multidisciplinary research natural history Oncology Organizational structure Partnerships Patient Selection Pediatrics Prospective Studies Proteomics Research Design Sampling Studies sickle cell anemia Sickle cell disease Thalassemia United States - epidemiology |
title | Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood |
url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-04T03%3A21%3A40IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Sickle%20Cell%20Clinical%20Research%20and%20Intervention%20Program%20(SCCRIP):%20A%20lifespan%20cohort%20study%20for%20sickle%20cell%20disease%20progression%20from%20the%20pediatric%20stage%20into%20adulthood&rft.jtitle=Pediatric%20blood%20&%20cancer&rft.au=Hankins,%20Jane%20S.&rft.date=2018-09&rft.volume=65&rft.issue=9&rft.spage=e27228&rft.epage=n/a&rft.pages=e27228-n/a&rft.issn=1545-5009&rft.eissn=1545-5017&rft_id=info:doi/10.1002/pbc.27228&rft_dat=%3Cproquest_cross%3E2073801572%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2073801572&rft_id=info:pmid/29797644&rfr_iscdi=true |