{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,29]],"date-time":"2025-11-29T20:27:53Z","timestamp":1764448073869,"version":"3.46.0"},"reference-count":10,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,6,26]],"date-time":"2024-06-26T00:00:00Z","timestamp":1719360000000},"content-version":"vor","delay-in-days":177,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatrics International"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Regular prophylaxis with activated prothrombin complex concentrates (aPCCs) is effective in adult patients with hemophilia with inhibitors; however, data in children are scarce.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This was a single\u2010center retrospective study at Saitama Children's Medical Center. Patients with severe and moderate hemophilia with inhibitors aged &lt;15\u2009years at the start of aPCCs prophylaxis were included. Medical records were retrospectively reviewed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We treated nine pediatric patients with hemophilia with inhibitors (median age, 1.9\u2009years; age range, 1.3\u201312.9\u2009years; inhibitor titers before treatment with aPCCs, 5.9\u201369\u2009BU\/mL) using prophylactic aPCCs (doses, 50\u2013100\u2009U\/kg; 2\u20133 times\/week). The median prophylactic period was 13\u2009months (range: 5\u201331\u2009months). The median annualized bleeding rate (ABR) during prophylactic treatment with aPCCs was 2 (range, 0\u201317). In four patients, ABR was reduced by 19%\u2013100% with prophylactic aPCCs compared to on\u2010demand aPCCs. An adverse effect of treatment was that a patient with hemophilia B developed nephrotic syndrome 34\u2009months after starting regular prophylaxis with aPCCs.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Regular prophylactic aPCCs reduced the ABR even in younger children with hemophilia A and B. Serious adverse events include nephrotic syndrome, which requires caution.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ped.15774","type":"journal-article","created":{"date-parts":[[2024,6,26]],"date-time":"2024-06-26T10:32:24Z","timestamp":1719397944000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Regular prophylaxis with activated prothrombin complex concentrates in pediatric hemophilia"],"prefix":"10.1111","volume":"66","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9065-5550","authenticated-orcid":false,"given":"Ayumi","family":"Horiguchi","sequence":"first","affiliation":[{"name":"Department of Hematology\/Oncology Saitama Children's Medical Center  Saitama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3001-2240","authenticated-orcid":false,"given":"Yuki","family":"Arakawa","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology Saitama Children's Medical Center  Saitama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5090-8568","authenticated-orcid":false,"given":"Yuichi","family":"Mitani","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology Saitama Children's Medical Center  Saitama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kiyotaka","family":"Isobe","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology Saitama Children's Medical Center  Saitama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Makiko","family":"Mori","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology Saitama Children's Medical Center  Saitama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2568-0877","authenticated-orcid":false,"given":"Kohei","family":"Fukuoka","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology Saitama Children's Medical Center  Saitama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0771-3551","authenticated-orcid":false,"given":"Koichi","family":"Oshima","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology Saitama Children's Medical Center  Saitama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katsuyoshi","family":"Koh","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology Saitama Children's Medical Center  Saitama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,6,26]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12246"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1104435"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/MBC.0000000000000525"},{"key":"e_1_2_9_5_1","first-page":"266a","article-title":"Factor VIII inhibitor bypass activity (FEIBA) for prophylaxis during immune tolerance induction (ITI) in patients with high\u2010responding inhibitors","volume":"96","author":"Krenz W","year":"2000","journal-title":"Blood"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2007.01442.x"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.01980.x"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02116.x"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12602"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.28474"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14471"}],"container-title":["Pediatrics International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ped.15774","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,29]],"date-time":"2025-11-29T20:24:33Z","timestamp":1764447873000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ped.15774"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1]]},"references-count":10,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2024,1]]}},"alternative-id":["10.1111\/ped.15774"],"URL":"https:\/\/doi.org\/10.1111\/ped.15774","archive":["Portico"],"relation":{},"ISSN":["1328-8067","1442-200X"],"issn-type":[{"type":"print","value":"1328-8067"},{"type":"electronic","value":"1442-200X"}],"subject":[],"published":{"date-parts":[[2024,1]]},"assertion":[{"value":"2023-09-12","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-03-26","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-06-26","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e15774"}}