{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,9]],"date-time":"2026-04-09T04:55:26Z","timestamp":1775710526269,"version":"3.50.1"},"reference-count":25,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2026,4,7]],"date-time":"2026-04-07T00:00:00Z","timestamp":1775520000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003196","name":"Italian Ministry of Health","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Severe congenital Protein C deficiency (SCPCD) is a rare autosomal recessive thrombophilia that typically presents in the neonatal period with early-onset life-threatening thrombotic complications. We report the case of a female infant who presented at birth with digital ischemic necrosis and laboratory evidence of consumptive coagulopathy consistent with neonatal purpura fulminans. Severe Protein C deficiency was confirmed by markedly reduced Protein C activity (&lt;0.03 IU\/mL) and compound heterozygous variants in the PROC gene. After initial stabilization and intermittent intravenous Protein C replacement, pharmacokinetic assessment showed marked peak\u2013trough variability. Continuous subcutaneous infusion of Protein C concentrate was therefore initiated using a programmable insulin pump in combination with oral anticoagulation. This strategy achieved stable Protein C activity levels, allowed progressive reduction of the weight-adjusted replacement dose, and enabled removal of the central venous catheter. Continuous subcutaneous infusion of Protein C concentrate via an insulin pump, combined with oral anticoagulation, may represent a feasible long-term therapeutic option in selected patients with SCPCD.<\/jats:p>","DOI":"10.3390\/children13040515","type":"journal-article","created":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T07:25:12Z","timestamp":1775633112000},"page":"515","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Management of Severe Congenital Protein C Deficiency with Continuous Subcutaneous Infusion via Insulin Pump: A Pediatric Case Report"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-9668-5127","authenticated-orcid":false,"given":"Angelo","family":"Gentile","sequence":"first","affiliation":[{"name":"Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, 16132 Genoa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-9889-5409","authenticated-orcid":false,"given":"Giordano","family":"Spacco","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, 16132 Genoa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5280-6319","authenticated-orcid":false,"given":"Nicola","family":"Minuto","sequence":"additional","affiliation":[{"name":"Pediatric Clinic and Endocrinology Unit, Department of Medical and Pediatric Sciences, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9879-7051","authenticated-orcid":false,"given":"Vera","family":"Morsellino","sequence":"additional","affiliation":[{"name":"Home Care Service, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2888-4122","authenticated-orcid":false,"given":"Sandro","family":"Dallorso","sequence":"additional","affiliation":[{"name":"Home Care Service, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8078-8402","authenticated-orcid":false,"given":"Angelo Claudio","family":"Molinari","sequence":"additional","affiliation":[{"name":"Hemostasis and Thrombosis Center, Department of Hematology and Oncology, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7183-5238","authenticated-orcid":false,"given":"Mohamad","family":"Maghnie","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, 16132 Genoa, Italy"},{"name":"Pediatric Clinic and Endocrinology Unit, Department of Medical and Pediatric Sciences, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9166-1079","authenticated-orcid":false,"given":"Marta","family":"Bassi","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, 16132 Genoa, Italy"},{"name":"Pediatric Clinic and Endocrinology Unit, Department of Medical and Pediatric Sciences, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6671-4167","authenticated-orcid":false,"given":"Laura","family":"Banov","sequence":"additional","affiliation":[{"name":"Hemostasis and Thrombosis Center, Department of Hematology and Oncology, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,4,7]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1055\/s-2003-42586","article-title":"Protein C pathway in infants and children","volume":"29","year":"2003","journal-title":"Semin. 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