title: Central venous thrombosis and thrombophilia in cystic fibrosis: A prospective study.
abstract: OBJECTIVE
  Catheter venous thrombosis may result in life-threatening embolic complications. Recently, a thrombophilic tendency was described in cystic fibrosis (CF), the significance of which remains unclear. The aims of this study were to (1) document the frequency of catheter venous thrombosis detected by colour-Doppler-ultrasound (Doppler-US), (2) assess genetic and acquired thrombophilia risk factors for catheter venous thrombosis and hypercoagulability status and (3) provide recommendations on laboratory screening when considering insertion of a totally implantable vascular access device (TIVAD) in CF patients.
  
  
  METHODS
  We designed a multicentre prospective study in patients selected at the time of catheter insertion. Doppler-US was scheduled at 1 and 6months after insertion and before insertion in case of a previous central line. Blood samplings were drawn at insertion and at 1 and 6months later.
  
  
  RESULTS
  One-hundred patients received a TIVAD and 90 completed the 6-month study. Prevalence of thrombophilia abnormalities and hypercoagulability was found in 50% of the cohorts. Conversely, catheter venous thrombosis frequency was low (6.6%).
  
  
  CONCLUSIONS
  Our data do not support biological screening at the time of a TIVAD insertion. We emphasise the contribution of a medical history of venous thromboembolism and prospective Doppler-US for identifying asymptomatic catheter venous thrombosis to select patients who may benefit from biological screening and possible anticoagulant therapy.
authors.affiliation: Assistance publique-Hôpitaux de Paris, Hôpital Robert Debré, Paediatric Gastroenterology and Respiratory Department, CF Centre, Université Paris 7, France. Electronic address: anne.munck@rdb.aphp.fr.
authors.forename: Anne
authors.initials: A
authors.lastname: Munck
authors.affiliation: Assistance publique-Hôpitaux de Paris, Hôpital Robert Debré, Imaging Department, Université Paris 7, France.
authors.forename: Ahmed
authors.initials: A
authors.lastname: Kheniche
authors.affiliation: Université Paris Diderot, Sorbonne Paris Cité, France; Inserm, CIE 5, Paris, France; Assistance publique-Hôpitaux de Paris, Hôpital Robert Debré, Clinical Epidemiology Unit, Paris, France.
authors.forename: Corinne
authors.initials: C
authors.lastname: Alberti
authors.affiliation: Assistance publique-Hôpitaux de Paris, Hôpital Cochin, CF Centre, Université Paris Descartes, Sorbonne Paris Cité, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Hubert
authors.affiliation: CF Centre, CHU Nord, Aix-Marseille Université, France.
authors.forename: Reynaud-Gaubert
authors.initials: RG
authors.lastname: Martine
authors.affiliation: Adult CF centre, Hospices Civils de Lyon, Groupe hospitalier Sud, Université de Lyon, France.
authors.forename: Raphaele
authors.initials: R
authors.lastname: Nove-Josserand
authors.affiliation: Pédiatrie, Pole Couple Enfants, CHU de Grenoble; INSERM U 823, Institut Albert Bonniot, Université Joseph Fournier, Grenoble, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Pin
authors.affiliation: CF centre, Hôpital d'enfants, Toulouse, France.
authors.forename: Francois
authors.initials: F
authors.lastname: Bremont
authors.affiliation: CF centre, Hôpital Arnaud de Villeneuve, Montpellier, France.
authors.forename: Raphael
authors.initials: R
authors.lastname: Chiron
authors.affiliation: Department of Pneumology, Rouen University Hospital, France.
authors.forename: Laure
authors.initials: L
authors.lastname: Couderc
authors.affiliation: CF centre, Hopital Jean Minjoz, Besançon, France.
authors.forename: Marie Laure
authors.initials: ML
authors.lastname: Dalphin
authors.affiliation: CF centre, Hôpital d' Angers, France.
authors.forename: Estelle
authors.initials: E
authors.lastname: Darviot
authors.affiliation: Assistance publique-Hôpitaux de Paris, Hôpital Robert Debré, Paediatric Gastroenterology and Respiratory Department, CF Centre, Université Paris 7, France.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Delaisi
authors.affiliation: Department of Pneumology, Rouen University Hospital, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Dominique
authors.affiliation: Adult CF centre, Hospices Civils de Lyon, Groupe hospitalier Sud, Université de Lyon, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Durieu
authors.affiliation: CF centre, Hôpital d'enfants du Bocage, Dijon, France.
authors.forename: Annelyse
authors.initials: A
authors.lastname: Fanton
authors.affiliation: CF centre, Hopital Pellegrin CIC 005, CHU Bordeaux, France.
authors.forename: Michael
authors.initials: M
authors.lastname: Fayon
authors.affiliation: Assistance publique-Hôpitaux de Paris, Hôpital Robert Debré, Paediatric Gastroenterology and Respiratory Department, CF Centre, Université Paris 7, France.
authors.forename: Michèle
authors.initials: M
authors.lastname: Gérardin
authors.affiliation: CF centre, Hôpital d' Angers, France.
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Giniès
authors.affiliation: CF centre, Hôpital de Clocheville, Tours, France.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Giraut
authors.affiliation: CF centre, Hôpital Foch, Suresnes, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Grenet
authors.affiliation: CF centre, Hôpital Robert Bisson, Lisieux, France.
authors.forename: Marcel
authors.initials: M
authors.lastname: Guillot
authors.affiliation: CF centre, Hôpital d'enfants du Bocage, Dijon, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Huet
authors.affiliation: Assistance publique-Hôpitaux de Paris, Hôpital Necker Enfants-Malades, CF centre, University Paris 5, France.
authors.forename: Muriel
authors.initials: M
authors.lastname: Le Bourgeois
authors.affiliation: CF centre, Hôpital Larrey, Toulouse, France.
authors.forename: Marlène
authors.initials: M
authors.lastname: Murris-Epin
authors.affiliation: CF centre, Centre Perharidy, Roscoff, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Ramel
authors.affiliation: CF centre, Hôpital de Lens, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Sardet
authors.affiliation: Assistance publique-Hôpitaux de Paris, Hôpital Necker Enfants-Malades, CF centre, University Paris 5, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Sermet-Gaudelus
authors.affiliation: CF centre, Hôpital Bretonneau, Tours, France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Varaigne
authors.affiliation: Hospices Civils de Lyon, Hopital Femme-Mère-Enfant, CF centre, Bron, France.
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Wanin
authors.affiliation: CF centre, Hôpital de Hautepierre, Strasbourg, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Weiss
authors.affiliation: Assistance publique-Hôpitaux de Paris, Hôpital Robert Debré, Biological Hematology Department, Université Paris 7, France.
authors.forename: Marie-Francoise
authors.initials: MF
authors.lastname: Hurtaud
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D002408:Catheters, Indwelling / Q000009:adverse effects*; D002648:Child; D015331:Cohort Studies; D015897:Comorbidity; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D005260:Female; D006801:Humans; D008297:Male; D015995:Prevalence; D011379:Prognosis; D011446:Prospective Studies; D012720:Severity of Illness Index; D017678:Sex Distribution; D019851:Thrombophilia / Q000097:blood / Q000453:epidemiology*; D018608:Ultrasonography, Doppler / Q000379:methods; D020246:Venous Thrombosis / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000209:etiology; D055815:Young Adult
keywords: Cystic fibrosis; Thrombophilia; Thrombosis