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type=\u0022text\/css\u0022 rel=\u0022stylesheet\u0022 href=\u0022\/\/d282kpwvnogo5m.cloudfront.net\/sites\/default\/files\/advagg_css\/css__ce2QY63WIanKyr8eSq7eavr1XQRRmFD6ZSmwpyJi8lM__zXwFqpqmxrZOXXcd_TpBQpjuELbmIP9wBR5UuTDWAO4__YJWWMMdfCJuAFm5cUEp88OsodhO3ZA-2lzRfoBsSlk4.css\u0022 media=\u0022all\u0022 \/\u003E\n\u003Clink rel=\u0027stylesheet\u0027 type=\u0027text\/css\u0027 href=\u0027\/sites\/all\/modules\/contrib\/panels\/plugins\/layouts\/onecol\/onecol.css\u0027 \/\u003E\u003C\/head\u003E\u003Cbody\u003E\u003Cdiv class=\u0022panels-ajax-tab-panel panels-ajax-tab-panel-sageoa-tab-art\u0022\u003E\u003Cdiv class=\u0022panel-display panel-1col clearfix\u0022 \u003E\n  \u003Cdiv class=\u0022panel-panel panel-col\u0022\u003E\n    \u003Cdiv\u003E\u003Cdiv class=\u0022panel-pane pane-highwire-markup\u0022 \u003E\n  \n      \n  \n  \u003Cdiv class=\u0022pane-content\u0022\u003E\n    \u003Cdiv class=\u0022highwire-markup\u0022\u003E\u003Cdiv xmlns=\u0022http:\/\/www.w3.org\/1999\/xhtml\u0022 id=\u0022content-block-markup\u0022 xmlns:xhtml=\u0022http:\/\/www.w3.org\/1999\/xhtml\u0022\u003E\u003Cdiv class=\u0022article fulltext-view \u0022\u003E\u003Cspan class=\u0022highwire-journal-article-marker-start\u0022\u003E\u003C\/span\u003E\u003Cdiv class=\u0022section abstract\u0022 id=\u0022abstract-1\u0022\u003E\u003Ch2\u003ESummary\u003C\/h2\u003E\u003Cp id=\u0022p-1\u0022\u003EThe Kids B-LONG study was conducted to evaluate the pharmacokinetics, safety, and efficacy of recombinant human coagulation factor IX Fc fusion protein (rFIXFc) in pediatric subjects with hemophilia B. rFIXFc is considered safe and efficacious for prophylaxis and the control of acute bleeding in children age \u0026lt;\u200512 years.\u003C\/p\u003E\u003C\/div\u003E\u003Cul class=\u0022kwd-group\u0022\u003E\u003Cli class=\u0022kwd\u0022\u003Epediatric\u003C\/li\u003E\u003Cli class=\u0022kwd\u0022\u003Ehemophilia B\u003C\/li\u003E\u003Cli class=\u0022kwd\u0022\u003Efactor IX\u003C\/li\u003E\u003Cli class=\u0022kwd\u0022\u003Erecombinant human coagulation factor IX Fc fusion protein\u003C\/li\u003E\u003Cli class=\u0022kwd\u0022\u003Ehemophilia treatment\u003C\/li\u003E\u003Cli class=\u0022kwd\u0022\u003Ehematology clinical trials\u003C\/li\u003E\u003C\/ul\u003E\u003Cdiv class=\u0022section\u0022 id=\u0022sec-1\u0022\u003E\u003Cp id=\u0022p-2\u0022\u003EThe Kids B-LONG study [\u003Ca class=\u0022external-ref external-ref-type-clintrialgov\u0022 href=\u0022\/lookup\/external-ref?link_type=CLINTRIALGOV\u0026amp;access_num=NCT01440946\u0026amp;atom=%2Fspmdc%2F15%2F21%2F6.2.atom\u0022\u003ENCT01440946\u003C\/a\u003E] was conducted to evaluate the pharmacokinetics, safety, and efficacy of recombinant human coagulation factor IX Fc fusion protein (rFIXFc) in previously treated pediatric subjects with hemophilia B. Kathelijn Fischer, MD, PhD, University Medical Center Utrecht, Utrecht, The Netherlands, provided a summary of the Kids B-LONG study results.\u003C\/p\u003E\u003Cp id=\u0022p-3\u0022\u003EEligible subjects were boys aged \u0026lt;\u200512 years with \u2264\u20052 IU\/dL endogenous factor IX (FIX) and with \u2265\u200550 previous exposure days (EDs) to FIX without a history of FIX inhibitor. The study schematic is illustrated in \u003Ca id=\u0022xref-fig-1-1\u0022 class=\u0022xref-fig\u0022 href=\u0022#F1\u0022\u003EFigure 1\u003C\/a\u003E. All subjects had pharmacokinetic assessment with FIX 50 IU\/kg and then with rFIXc 50 IU\/kg before this study. Then, all received weekly prophylaxis with rFIXFc and were followed for 50 EDs. Surgery was allowed after 3 EDs.\u003C\/p\u003E\u003Cdiv id=\u0022F1\u0022 class=\u0022fig pos-float  odd\u0022\u003E\u003Cdiv class=\u0022highwire-figure\u0022\u003E\u003Cdiv class=\u0022fig-inline-img-wrapper\u0022\u003E\u003Cdiv class=\u0022fig-inline-img\u0022\u003E\u003Ca href=\u0022http:\/\/d282kpwvnogo5m.cloudfront.net\/content\/spmdc\/15\/21\/6.2\/F1.large.jpg?width=800\u0026amp;height=600\u0026amp;carousel=1\u0022 title=\u0022Kids B-LONG Study DesignED, exposure day; FIX, factor IX; PK, pharmacokinetic; rFIXFc, recombinant factor IX Fc fusion protein.aA 96-hour washout period with no FIX treatment was required prior to administration of prestudy FIX, 28\u0026#x2005;\u0026#xB1;\u0026#x2005;7 d prior to rFIXFc dosing at baseline, and prior to administration of rFIXFc on day 1; in younger children and subjects who required a second washout attempt, a 72-hour washout period was permitted.Reproduced with permission from K Fischer, MD, PhD.\u0022 class=\u0022fragment-images colorbox-load\u0022 rel=\u0022gallery-fragment-images-1175764432\u0022 data-figure-caption=\u0022\u0026amp;lt;div xmlns=\u0026amp;quot;http:\/\/www.w3.org\/1999\/xhtml\u0026amp;quot;\u0026amp;gt;Kids B-LONG Study DesignED, exposure day; FIX, factor IX; PK, pharmacokinetic; rFIXFc, recombinant factor IX Fc fusion protein.\u0026amp;lt;sup\u0026amp;gt;a\u0026amp;lt;\/sup\u0026amp;gt;A 96-hour washout period with no FIX treatment was required prior to administration of prestudy FIX, 28\u0026#x2005;\u0026#xB1;\u0026#x2005;7 d prior to rFIXFc dosing at baseline, and prior to administration of rFIXFc on day 1; in younger children and subjects who required a second washout attempt, a 72-hour washout period was permitted.Reproduced with permission from K Fischer, MD, PhD.\u0026amp;lt;\/div\u0026amp;gt;\u0022 data-icon-position=\u0022\u0022 data-hide-link-title=\u00220\u0022\u003E\u003Cimg class=\u0022fragment-image\u0022 alt=\u0022Figure 1.\u0022 src=\u0022http:\/\/d282kpwvnogo5m.cloudfront.net\/content\/spmdc\/15\/21\/6.2\/F1.medium.gif\u0022\/\u003E\u003C\/a\u003E\u003C\/div\u003E\u003C\/div\u003E\u003Cul class=\u0022highwire-figure-links inline\u0022\u003E\u003Cli class=\u00220 first\u0022\u003E\u003Ca href=\u0022http:\/\/d282kpwvnogo5m.cloudfront.net\/content\/spmdc\/15\/21\/6.2\/F1.large.jpg?download=true\u0022 class=\u0022highwire-figure-link highwire-figure-link-download\u0022 title=\u0022Download Figure 1.\u0022 data-icon-position=\u0022\u0022 data-hide-link-title=\u00220\u0022\u003EDownload figure\u003C\/a\u003E\u003C\/li\u003E\u003Cli class=\u00221\u0022\u003E\u003Ca href=\u0022http:\/\/d282kpwvnogo5m.cloudfront.net\/content\/spmdc\/15\/21\/6.2\/F1.large.jpg\u0022 class=\u0022highwire-figure-link highwire-figure-link-newtab\u0022 target=\u0022_blank\u0022 data-icon-position=\u0022\u0022 data-hide-link-title=\u00220\u0022\u003EOpen in new tab\u003C\/a\u003E\u003C\/li\u003E\u003Cli class=\u00222 last\u0022\u003E\u003Ca href=\u0022\/highwire\/powerpoint\/16841\u0022 class=\u0022highwire-figure-link highwire-figure-link-ppt\u0022 data-icon-position=\u0022\u0022 data-hide-link-title=\u00220\u0022\u003EDownload powerpoint\u003C\/a\u003E\u003C\/li\u003E\u003C\/ul\u003E\u003C\/div\u003E\u003Cdiv class=\u0022fig-caption\u0022 xmlns:xhtml=\u0022http:\/\/www.w3.org\/1999\/xhtml\u0022\u003E\u003Cspan class=\u0022fig-label\u0022\u003EFigure 1.\u003C\/span\u003E \u003Cp id=\u0022p-4\u0022 class=\u0022first-child\u0022\u003EKids B-LONG Study Design\u003C\/p\u003E\u003Cp id=\u0022p-5\u0022\u003EED, exposure day; FIX, factor IX; PK, pharmacokinetic; rFIXFc, recombinant factor IX Fc fusion protein.\u003C\/p\u003E\u003Cp id=\u0022p-6\u0022\u003E\u003Csup\u003Ea\u003C\/sup\u003EA 96-hour washout period with no FIX treatment was required prior to administration of prestudy FIX, 28\u2005\u00b1\u20057 d prior to rFIXFc dosing at baseline, and prior to administration of rFIXFc on day 1; in younger children and subjects who required a second washout attempt, a 72-hour washout period was permitted.\u003C\/p\u003E\u003Cp id=\u0022p-7\u0022\u003EReproduced with permission from K Fischer, MD, PhD.\u003C\/p\u003E\u003Cdiv class=\u0022sb-div caption-clear\u0022\u003E\u003C\/div\u003E\u003C\/div\u003E\u003C\/div\u003E\u003Cp id=\u0022p-8\u0022\u003EThirty subjects were enrolled in the study; 15 patients were \u0026lt;\u20056 years old, and 15 were between 6 and 12 years. All subjects were treated with FIX prophylaxis before entering the study, and 77% were receiving FIX \u2265\u20052 times per week. Twenty-seven subjects (90%) completed the study, with a median study duration of 49.4 weeks. A total of 24 subjects (80%) had at least 50 EDs of rFIXFc.\u003C\/p\u003E\u003Cp id=\u0022p-9\u0022\u003EPharmacokinetic analyses indicated that rFIXFc had an increased half-life and reduced clearance in children compared with FIX products administered in the earlier phase of pharmacokinetic assessment. Also, incremental recovery with rFIXFc was similar to or slightly better than that with FIX.\u003C\/p\u003E\u003Cp id=\u0022p-10\u0022\u003ENo inhibitors or anti-rFIXFc antibodies developed in study subjects. The majority of subjects (86.7%) had \u2265\u20051 adverse event, the most common being nasopharyngitis (23.3%) and fall (20.0%). One mild nonserious adverse event of decreased appetite was considered to be related to study treatment; all other adverse events were considered unrelated to rFIXFc. Eleven serious adverse events occurred in 4 subjects during the study; none was considered related to the study drug. There were no discontinuations from the study because of adverse events.\u003C\/p\u003E\u003Cp id=\u0022p-11\u0022\u003ENo changes were made to the dosing interval in 97% of subjects. The median prophylactic dose was 59.4 IU\/kg\/wk (interquartile range [IQR], 53.0 to 64.8) in subjects aged \u0026lt;\u20056 years and 57.8 IU\/kg\/wk (IQR, 51.7 to 65.0) in subjects aged 6 to \u0026lt;\u200512 years.\u003C\/p\u003E\u003Cp id=\u0022p-12\u0022\u003EThirty-three percent of subjects had no bleeding episodes, and there were no joint bleeds in 63% of subjects. The overall median annualized bleeding rate was 2.0 (IQR, 0.0 to 3.1). In acute bleeding episodes, 75.0% were controlled with 1 infusion, and 91.7% were controlled with 1 or 2 infusions (median dose per infusion, 63.5 IU\/kg [IQR, 48.9 to 99.4]). After the first infusions, 88.7% had an excellent or good response. Although no major surgeries were performed with rFIXFc, 3 minor surgeries were performed in 2 subjects. Excellent hemostatic response was achieved in all surgical cases. Physical activity remained the same or increased in 77% of study subjects. In her concluding remarks, Dr Fischer said she believes these data support the potential for extended-interval dosing with low bleeding rates in this population.\u003C\/p\u003E\u003C\/div\u003E\u003Cul class=\u0022copyright-statement\u0022\u003E\u003Cli class=\u0022fn\u0022 id=\u0022copyright-statement-1\u0022\u003E\u00a9 2015 SAGE Publications\u003C\/li\u003E\u003C\/ul\u003E\u003Cspan class=\u0022highwire-journal-article-marker-end\u0022\u003E\u003C\/span\u003E\u003C\/div\u003E\u003Cspan id=\u0022related-urls\u0022\u003E\u003C\/span\u003E\u003C\/div\u003E\u003Ca href=\u0022http:\/\/mdc.sagepub.com\/content\/15\/21\/6.2.abstract\u0022 class=\u0022hw-link hw-link-article-abstract\u0022 data-icon-position=\u0022\u0022 data-hide-link-title=\u00220\u0022\u003EView Summary\u003C\/a\u003E\u003C\/div\u003E  \u003C\/div\u003E\n\n  \n  \u003C\/div\u003E\n\u003C\/div\u003E\n  \u003C\/div\u003E\n\u003C\/div\u003E\n\u003C\/div\u003E\u003Cscript type=\u0022text\/javascript\u0022 src=\u0022http:\/\/mdc.sagepub.com\/sites\/all\/modules\/highwire\/highwire\/plugins\/highwire_markup_process\/js\/highwire_figures.js?nzlga1\u0022\u003E\u003C\/script\u003E\n\u003Cscript type=\u0022text\/javascript\u0022 src=\u0022http:\/\/mdc.sagepub.com\/sites\/all\/modules\/highwire\/highwire\/plugins\/highwire_markup_process\/js\/highwire_openurl.js?nzlga1\u0022\u003E\u003C\/script\u003E\n\u003C\/body\u003E\u003C\/html\u003E"}