title: Barriers to primary prophylaxis in haemophilic children: the issue of the venous access.
abstract: Modem treatment for hemophilic children is based on prophylaxis and immune tolerance induction (ITI). Both treatment regimens are based on frequent infusions at early ages, therefore an adequate venous access is essential. Peripheral veins represent the best option, however, different solutions, as central venous access devices (CVADs) and arteriovenous fistulae (AVFs), can be adopted if needed. CVADs have been used in hemophiliacs, however their survival is affected by infectious complications. Among CVADs, fully implantable devices are usually preferred to external lines due to a lower infectious risk. The limited survival of CVADs may have a relevant impact on treatment outcome, especially in case of ITI where treatment interruptions are counterproductive. To overcome such drawbacks, internal AVF has been considered as an alternative option owing to a lower rate of infectious complications. Moreover, AVF is easy to use in the home setting and well accepted by children. Possible complications not preventing AVF use are postoperative hematoma and transient symptoms of distal ischemia; one case of symptomatic thrombosis has been reported so far. Long-term complications include loss of patency, aneurysmatic dilatation and, rarely, limb dysmetria and a regular follow-up is mandatory to allow early remedial intervention. Surgical dismantlement of AVF is recommended as soon as transition to peripheral veins is possible.
authors.affiliation: Centro Emofilia e Trombosi Angelo Bianchi Bonomi, Fondazione IRCCS Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Università degli Studi di Milano, Milano, Italia. hemophilia_ctr@policlinico.mi.it
authors.forename: Elena
authors.initials: E
authors.lastname: Santagostino
authors.forename: Maria Elisa
authors.initials: ME
authors.lastname: Mancuso
mesh_terms: D001166:Arteriovenous Shunt, Surgical / Q000009:adverse effects; D016470:Bacteremia / Q000209:etiology / Q000517:prevention & control; D002405:Catheterization, Central Venous / Q000009:adverse effects; D002406:Catheterization, Peripheral / Q000009:adverse effects; D002408:Catheters, Indwelling / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D020878:Device Removal; D004866:Equipment Contamination; D005164:Factor IX / Q000008:administration & dosage* / Q000627:therapeutic use; D005169:Factor VIII / Q000008:administration & dosage* / Q000627:therapeutic use; D006467:Hemophilia A / Q000150:complications / Q000188:drug therapy*; D002836:Hemophilia B / Q000150:complications / Q000188:drug therapy*; D006470:Hemorrhage / Q000209:etiology / Q000517:prevention & control*; D006801:Humans; D007223:Infant; D017053:Infection Control; D015918:Infusion Pumps, Implantable; D007262:Infusions, Intravenous; D011322:Primary Prevention; D012306:Risk; D014654:Vascular Patency
chemical_list: D005169:Factor VIII; D005164:Factor IX