{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,3]],"date-time":"2025-09-03T12:22:12Z","timestamp":1756902132557,"version":"3.44.0"},"reference-count":13,"publisher":"The Pediatric Pharmacy Association","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023]]},"abstract":"Fibrinogen deficiencies in neonates can lead to bleeding complications. In this report, we describe a case of congenital afibrinogenemia in a newborn with critical pulmonary stenosis who presented with bilateral cephalohematomas after an uncomplicated delivery. The initial use of cryoprecipitate was followed by administration of fibrinogen concentrate. We estimated a half-life of 24 to 48 hours with the concentrate product. This patient received fibrinogen replacement and had a subsequent successful cardiac repair. The drug's shorter half-life in this neonate contrasts with prior reports of longer half-life in older patients and is important to note in treating future neonatal patients with this diagnosis.<\/jats:p>","DOI":"10.5863\/1551-6776-28.3.268","type":"journal-article","created":{"date-parts":[[2023,6,8]],"date-time":"2023-06-08T02:00:28Z","timestamp":1686189628000},"page":"268-271","source":"Crossref","is-referenced-by-count":0,"title":["Treatment of Congenital Afibrinogenemia in a Neonate With Critical Pulmonary Stenosis"],"prefix":"10.5863","volume":"28","author":[{"given":"Priya","family":"Parikh","sequence":"first","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology (PP, TLL), UCSF Benioff Children's Hospital, San Francisco, CA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kimvi","family":"Diep","sequence":"additional","affiliation":[{"name":"Department of Pharmaceutical Services, Department of Clinical Pharmacy (KD), UCSF Benioff Children's Hospital, San Francisco, CA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vinod","family":"Balasa","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology (VB), Valley Children's Hospital, San Francisco, CA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tiffany L.","family":"Lucas","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology (PP, TLL), UCSF Benioff Children's Hospital, San Francisco, CA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"3883","reference":[{"issue":"suppl 4","key":"i2331-348X-28-3-268-b1","series-title":"Haemophilia","first-page":"148","article-title":"Rare bleeding disorders","volume":"18","year":"2012","unstructured":"Peyvandi\n F,\n \n \n Bolton-Maggs\n PHB,\n \n \n Batorova\n A,\n \n \n De Moerloose\n P.\n \n \n Rare bleeding disorders.\n\t\t\t\t\tHaemophilia.\n\t\t\t\t\t2012;\n\t\t\t\t\t18(\n\t\t\t\t\tsuppl 4):\n\t\t\t\t\t148\u2013\n\t\t\t\t\t153."},{"key":"i2331-348X-28-3-268-b2","series-title":"Blood","first-page":"1879","article-title":"Activation of multiple cryptic donor splice sites by the common congenital afibrinogenemia mutation, FGA IVS4 + 1 G-->T","volume":"97","year":"2001","unstructured":"Attanasio\n C,\n \n \n de Moerloose\n P,\n \n \n Antonarakis\n SE,\n \n \n .\n\t\t\t\t\tActivation of multiple cryptic donor splice sites by the common congenital afibrinogenemia mutation, FGA IVS4 + 1 G-->T.\n\t\t\t\t\tBlood.\n\t\t\t\t\t2001;\n\t\t\t\t\t97:\n\t\t\t\t\t1879\u2013\n\t\t\t\t\t1881."},{"issue":"12","key":"i2331-348X-28-3-268-b3","series-title":"Am J Hematol","first-page":"928","article-title":"Laboratory testing for fibrinogen abnormalities","volume":"83","year":"2008","unstructured":"Verhovsek\n M,\n \n \n Moffat\n KA,\n \n \n Hayward\n CPM.\n \n \n Laboratory testing for fibrinogen abnormalities.\n\t\t\t\t\tAm J Hematol.\n\t\t\t\t\t2008;\n\t\t\t\t\t83(\n\t\t\t\t\t12):\n\t\t\t\t\t928\u2013\n\t\t\t\t\t931."},{"year":"2011","series-title":"Standards for Blood Banks and Transfusion Services","key":"i2331-348X-28-3-268-b4","unstructured":"American Association of Blood Banks.\n \n \n Standards for Blood Banks and Transfusion Services\n .\n\t\t\t\t\t27th ed.\n\t\t\t\t\tAbe-Books;\n\t\t\t\t\t2011."},{"issue":"3","key":"i2331-348X-28-3-268-b5","series-title":"Transfus Med Rev","first-page":"177","article-title":"Cryoprecipitate: the current state of knowledge","volume":"23","year":"2009","unstructured":"Callum\n JL,\n \n \n Karkouti\n K,\n \n \n Lin\n Y.\n \n \n Cryoprecipitate: the current state of knowledge.\n\t\t\t\t\tTransfus Med Rev.\n\t\t\t\t\t2009;\n\t\t\t\t\t23(\n\t\t\t\t\t3):\n\t\t\t\t\t177\u2013\n\t\t\t\t\t188."},{"issue":"10","key":"i2331-348X-28-3-268-b6","series-title":"QJM","first-page":"711","article-title":"Fibrinogen: biochemistry, epidemiology and determinants","volume":"96","year":"2003","unstructured":"Kamath\n S,\n \n \n Lip\n GYH.\n \n \n Fibrinogen: biochemistry, epidemiology and determinants.\n\t\t\t\t\tQJM.\n\t\t\t\t\t2003;\n\t\t\t\t\t96(\n\t\t\t\t\t10):\n\t\t\t\t\t711\u2013\n\t\t\t\t\t729."},{"key":"i2331-348X-28-3-268-b7","unstructured":"RiaSTAP (fibrinogen concentrate [Human]) [prescribing Information].\n\t\t\t\t\tCSL Behring GmbH, Marburg, Germany, US License No. 1765. Accessed February 23, 2022. https:\/\/www.riastap.com\/professional\/prescribing-information.aspx","article-title":"RiaSTAP (fibrinogen concentrate [Human]) [prescribing Information]"},{"issue":"5","key":"i2331-348X-28-3-268-b8","series-title":"Blood Coagul Fibrinolysis","first-page":"527","article-title":"Congenital afibrinogenemia in a new born: a rare cause for bleeding","volume":"25","year":"2014","unstructured":"Kaur\n M,\n \n \n Kumar\n N,\n \n \n Bose\n SK,\n \n \n .\n\t\t\t\t\tCongenital afibrinogenemia in a new born: a rare cause for bleeding.\n\t\t\t\t\tBlood Coagul Fibrinolysis.\n\t\t\t\t\t2014;\n\t\t\t\t\t25(\n\t\t\t\t\t5):\n\t\t\t\t\t527\u2013\n\t\t\t\t\t529."},{"issue":"12","key":"i2331-348X-28-3-268-b9","series-title":"J Thromb Haemost","first-page":"2064","article-title":"Pharmacokinetics and safety of fibrinogen concentrate","volume":"7","year":"2009","unstructured":"Manco-Johnson\n MJ,\n \n \n Dimichele\n D,\n \n \n Castaman\n G,\n \n \n .\n\t\t\t\t\tPharmacokinetics and safety of fibrinogen concentrate.\n\t\t\t\t\tJ Thromb Haemost.\n\t\t\t\t\t2009;\n\t\t\t\t\t7(\n\t\t\t\t\t12):\n\t\t\t\t\t2064\u2013\n\t\t\t\t\t2069."},{"issue":"7","key":"i2331-348X-28-3-268-b10","series-title":"Ann Pharmacother","first-page":"1145","article-title":"Treatment of congenital afibrinogenemia in a premature neonate","volume":"42","year":"2008","unstructured":"Toledano\n A,\n \n \n Lachassinne\n E,\n \n \n Roumegoux\n C,\n \n \n .\n\t\t\t\t\tTreatment of congenital afibrinogenemia in a premature neonate.\n\t\t\t\t\tAnn Pharmacother.\n\t\t\t\t\t2008;\n\t\t\t\t\t42(\n\t\t\t\t\t7):\n\t\t\t\t\t1145\u2013\n\t\t\t\t\t1146."},{"issue":"2","key":"i2331-348X-28-3-268-b11","series-title":"Haemophilia","first-page":"283","article-title":"Efficacy and safety of fibrinogen concentrate for on-demand treatment of bleeding and surgical prophylaxis in paediatric patients with congenital fibrinogen deficiency","volume":"27","year":"2021","unstructured":"Khayat\n C,\n \n \n Lohade\n S,\n \n \n .\n\t\t\t\t\tEfficacy and safety of fibrinogen concentrate for on-demand treatment of bleeding and surgical prophylaxis in paediatric patients with congenital fibrinogen deficiency.\n\t\t\t\t\tHaemophilia.\n\t\t\t\t\t2021;\n\t\t\t\t\t27(\n\t\t\t\t\t2):\n\t\t\t\t\t283\u2013\n\t\t\t\t\t292."},{"key":"i2331-348X-28-3-268-b12","unstructured":"Fibryga (fibrinogen concentrate [Human]) [summary of product characteristics].\n\t\t\t\t\tCSL Behring GmbH, Marburg, Germany, US License No. 1765. Accessed October 23, 2021. https:\/\/www.octapharma.com\/api\/download\/x\/57e9a23bee\/fibryga-pm-en.pdf","article-title":"Fibryga (fibrinogen concentrate [Human]) [summary of product characteristics]"},{"key":"i2331-348X-28-3-268-b13","series-title":"J Thromb Haemost","first-page":"1634","article-title":"Incidence of bleeding symptoms in 100 patients with inherited afibrinogenemia or hypofibrinogenemia","volume":"4","year":"2006","unstructured":"Peyvandi\n F,\n \n \n Haertel\n S,\n \n \n Knaub\n S,\n \n \n Mannucci\n PM.\n \n \n Incidence of bleeding symptoms in 100 patients with inherited afibrinogenemia or hypofibrinogenemia.\n\t\t\t\t\tJ Thromb Haemost.\n\t\t\t\t\t2006;\n\t\t\t\t\t4:\n\t\t\t\t\t1634\u2013\n\t\t\t\t\t1637."}],"container-title":["The Journal of Pediatric Pharmacology and Therapeutics"],"original-title":[],"link":[{"URL":"https:\/\/jppt.kglmeridian.com\/view\/journals\/jppt\/28\/3\/article-p268.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/meridian.allenpress.com\/jppt\/article-pdf\/28\/3\/268\/3229061\/i2331-348x-28-3-268.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/jppt.kglmeridian.com\/downloadpdf\/journals\/jppt\/28\/3\/article-p268.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/jppt.kglmeridian.com\/downloadpdf\/journals\/jppt\/28\/3\/article-p268.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,5,16]],"date-time":"2025-05-16T00:49:16Z","timestamp":1747356556000},"score":1,"resource":{"primary":{"URL":"https:\/\/jppt.kglmeridian.com\/view\/journals\/jppt\/28\/3\/article-p268.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023]]},"references-count":13,"journal-issue":{"issue":"3"},"URL":"https:\/\/doi.org\/10.5863\/1551-6776-28.3.268","relation":{},"ISSN":["2331-348X","1551-6776"],"issn-type":[{"type":"electronic","value":"2331-348X"},{"type":"print","value":"1551-6776"}],"subject":[],"published":{"date-parts":[[2023]]}}}