title: Continuous subcutaneous infusion of protein C concentrate using an insulin pump in a newborn with congenital protein C deficiency.
abstract: We describe the case of a newborn presenting with multicystic encephalomalacy, hydrocephalus and bilateral hemovitreous. An underlying coagulation disorder was suspected and laboratory tests revealed severe protein C deficiency. At 25 days of life, after the appearance of purpura fulminans, replacement therapy with intravenous protein C concentrate (Ceprotin; Baxter, Vienna, Austria) was started.Due to difficulties in getting peripheral venous access and to repeated loss of the venous access, continuous subcutaneous infusion of protein C was started with an insulin pump (VEO 754; Medtronic, Minneapolis, Minnesota, USA), normally adopted in patients with type 1 diabetes mellitus. Protein C values increased into the normal range and the resolution of the purpuric skin lesion was achieved. Chronic prophylaxis with low-molecular-weight heparin failed and, due to cutaneous and cerebral recrudescence, replacement therapy with the pump was started again. The insulin pump allowed us to reduce the number of injections per day and to deal with the difficulties in getting peripheral venous access, permitting medical and paramedical staff an easier management of the therapy. The dosing schedule could be easily adapted with the insulin pump and the continuous subcutaneous administration of small amounts of protein C concentrate prevented fluctuation in trough levels of protein C. This is the first reported case of a novel, successful use of an insulin pump in an extremely rare disease, to administer a drug different from insulin, which needs to be further analyzed, underlining the importance of a multidisciplinary team approach in order to provide effective and efficient care in high-complexity diseases.
authors: aTuscany Regional Centre of Pediatric Diabetes, Meyer Children's Hospital bUniversity of Florence cNeonatal Intensive Care Unit, Medical and Surgical Feto-Neonatal Department, Meyer Children's Hospital, Florence, Italy.
authors: Barbara
authors: B
authors: Piccini
authors: Laura
authors: L
authors: Capirchio
authors: Lorenzo
authors: L
authors: Lenzi
authors: Monica
authors: M
authors: Guasti
authors: Giulia
authors: G
authors: Braccesi
authors: Cecilia
authors: C
authors: Bresci
authors: Emilio
authors: E
authors: Casalini
authors: Patrizio
authors: P
authors: Fiorini
authors: Elisabetta
authors: E
authors: Agostini
authors: Sonia
authors: S
authors: Toni
mesh_terms: D005260:Female; D006801:Humans; D007231:Infant, Newborn; D055104:Infusions, Subcutaneous; D007332:Insulin Infusion Systems; D011486:Protein C / Q000008:administration & dosage / Q000627:therapeutic use*; D020151:Protein C Deficiency / Q000188:drug therapy*
chemical_list: D011486:Protein C