e338
LETTERS TO THE EDITORS
suitable for performing aortic PTA, due to the larger introducer sheath (9 Fr vs. 6–7 Fr) required. In addition, the radial approach has inherent technical challenges, including smaller vessel size, arterial spasm and tortuosity involving the radial and subclavian arteries. In an observational study comparing coarctation repair by balloon angioplasty with or without stenting, a residual pressure gradient ≤10 mmHg immediately after the procedure was shown to be potentially predictive of favourable treatment outcomes [9]. FEIBA administered before and for 2 weeks after angioplasty was effective in preventing bleeding events. This concurs with the well-documented efficacy of FEIBA in haemophilia A [7]. According to a recent analysis, the haemostatic efficacy was rated as ‘excellent’ or ‘good’ in 75–100% of surgical interventions performed in haemophilic patients with FVIII inhibitors [7]. Similar results were obtained from analysis of the SURF international registry of surgery in haemophilia patients with antibodies, in which 91.4% of surgical procedures had good or excellent haemostasis. Our case shows that aortic coarctation was successfully and safely repaired by endovascular stent implantation, in the presence of FEIBA, in an adult patient
References 1 Kempton CL, White GC II. How we treat a hemophilia A patient with a factor VIII inhibitor. Blood 2009; 113: 11–7. 2 Hay CR. The epidemiology of factor VIII inhibitors. Haemophilia 2006; 12(Suppl. 6): 23–8; discussion 8-9. 3 Tjønnfjord GE, Holme PA. Factor eight inhibitor bypass activity (FEIBA) in the management of bleeds in hemophilia patients with high-titer inhibitors. Vasc Health Risk Manag 2007; 3: 527– 31.
with severe haemophilia A and high-titre inhibitors. The availability of effective haemostatic agents is likely to further expand the number of procedures that can be safely performed in patients with haemophilia and inhibitors.
Acknowledgements The authors thank Lorenza Lanini and Mary Hines for providing editorial assistance on behalf of in Science Communications, Springer Healthcare. This medical writing assistance was funded by Baxter.
Author contributions Gaetano Giuffrida designed the research study and wrote the paper. Rita Lombardo and Nunziatina Laura Parrinello collected and analysed the data and wrote the paper. Anna Maria Triolo analysed the data. Maria Rocca Cingari, Ernesto Di Francesco, Gian Paolo Ussia, Antonio Andrea Arcidiacono and Salvatore Scandurra contributed essential reagents or tools. Francesco Di Raimondo wrote the paper.
Disclosures The authors stated that they had no interests which might be perceived as posing a conflict or bias.
4 Konkle BA. The aging patient with hemophilia. Am J Hematol 2012; 87(Suppl. 1): S27–32. 5 Kulkarni R. Comprehensive care of the patient with haemophilia and inhibitors undergoing surgery: practical aspects. Haemophilia 2013; 19: 2–10. 6 Rangarajan S, Yee TT, Wilde J. Experience of four UK comprehensive care centres using FEIBAâ for surgeries in patients with inhibitors. Haemophilia 2011; 17: 28–34. 7 Rangarajan S, Austin S, Goddard NJ et al. Consensus recommendations for the use of FEIBAâ in haemophilia A patients with inhibitors undergoing elective orthopaedic
and non-orthopaedic surgery. Haemophilia 2013; 19: 294–303. 8 Aledort LM. Comparative thrombotic event incidence after infusion of recombinant factor VIIa versus factor VIII inhibitor bypass activity. J Thromb Haemost 2004; 2: 1700–8. 9 Zabal C, Attie F, Rosas M et al. The adult patient with native coarctation of the aorta: balloon angioplasty or primary stenting? Heart 2003; 89: 77–83. 10 Coppola A, Tagliaferri A, Franchini M. The management of cardiovascular diseases in patients with hemophilia. Semin Thromb Hemost 2010; 36: 91–102.
Oral health status among 6- to 12-year-old haemophilic children–An educational intervention study K . R . G A D D A M , S . N U V V U L A , S . N I R M A L A and R . K A M A T H A M Paedodontics and Preventive dentistry, Narayana Dental College, Nellore, Andhrapradesh, India
Correspondence: Kumar Raja Gaddam, Paedodontics and Preventive dentistry, Narayana Dental College, Nellore, Andhrapradesh, India. Tel.: +919866495595; fax: +918673272627; e-mail: [email protected] Accepted after revision 9 March 2014 DOI: 10.1111/hae.12434 Haemophilia (2014), 20, e336--e358
Haemophilia is the most common inherited bleeding disorder characterized by a lifelong defect in clotting mechanism. It is classified as haemophilia A and B, based on the deficiency of factor VIII and IX respectively; further as mild (5–25%), moderate (1–5%) and severe (
LETTERS TO THE EDITORS
suitable for performing aortic PTA, due to the larger introducer sheath (9 Fr vs. 6–7 Fr) required. In addition, the radial approach has inherent technical challenges, including smaller vessel size, arterial spasm and tortuosity involving the radial and subclavian arteries. In an observational study comparing coarctation repair by balloon angioplasty with or without stenting, a residual pressure gradient ≤10 mmHg immediately after the procedure was shown to be potentially predictive of favourable treatment outcomes [9]. FEIBA administered before and for 2 weeks after angioplasty was effective in preventing bleeding events. This concurs with the well-documented efficacy of FEIBA in haemophilia A [7]. According to a recent analysis, the haemostatic efficacy was rated as ‘excellent’ or ‘good’ in 75–100% of surgical interventions performed in haemophilic patients with FVIII inhibitors [7]. Similar results were obtained from analysis of the SURF international registry of surgery in haemophilia patients with antibodies, in which 91.4% of surgical procedures had good or excellent haemostasis. Our case shows that aortic coarctation was successfully and safely repaired by endovascular stent implantation, in the presence of FEIBA, in an adult patient
References 1 Kempton CL, White GC II. How we treat a hemophilia A patient with a factor VIII inhibitor. Blood 2009; 113: 11–7. 2 Hay CR. The epidemiology of factor VIII inhibitors. Haemophilia 2006; 12(Suppl. 6): 23–8; discussion 8-9. 3 Tjønnfjord GE, Holme PA. Factor eight inhibitor bypass activity (FEIBA) in the management of bleeds in hemophilia patients with high-titer inhibitors. Vasc Health Risk Manag 2007; 3: 527– 31.
with severe haemophilia A and high-titre inhibitors. The availability of effective haemostatic agents is likely to further expand the number of procedures that can be safely performed in patients with haemophilia and inhibitors.
Acknowledgements The authors thank Lorenza Lanini and Mary Hines for providing editorial assistance on behalf of in Science Communications, Springer Healthcare. This medical writing assistance was funded by Baxter.
Author contributions Gaetano Giuffrida designed the research study and wrote the paper. Rita Lombardo and Nunziatina Laura Parrinello collected and analysed the data and wrote the paper. Anna Maria Triolo analysed the data. Maria Rocca Cingari, Ernesto Di Francesco, Gian Paolo Ussia, Antonio Andrea Arcidiacono and Salvatore Scandurra contributed essential reagents or tools. Francesco Di Raimondo wrote the paper.
Disclosures The authors stated that they had no interests which might be perceived as posing a conflict or bias.
4 Konkle BA. The aging patient with hemophilia. Am J Hematol 2012; 87(Suppl. 1): S27–32. 5 Kulkarni R. Comprehensive care of the patient with haemophilia and inhibitors undergoing surgery: practical aspects. Haemophilia 2013; 19: 2–10. 6 Rangarajan S, Yee TT, Wilde J. Experience of four UK comprehensive care centres using FEIBAâ for surgeries in patients with inhibitors. Haemophilia 2011; 17: 28–34. 7 Rangarajan S, Austin S, Goddard NJ et al. Consensus recommendations for the use of FEIBAâ in haemophilia A patients with inhibitors undergoing elective orthopaedic
and non-orthopaedic surgery. Haemophilia 2013; 19: 294–303. 8 Aledort LM. Comparative thrombotic event incidence after infusion of recombinant factor VIIa versus factor VIII inhibitor bypass activity. J Thromb Haemost 2004; 2: 1700–8. 9 Zabal C, Attie F, Rosas M et al. The adult patient with native coarctation of the aorta: balloon angioplasty or primary stenting? Heart 2003; 89: 77–83. 10 Coppola A, Tagliaferri A, Franchini M. The management of cardiovascular diseases in patients with hemophilia. Semin Thromb Hemost 2010; 36: 91–102.
Oral health status among 6- to 12-year-old haemophilic children–An educational intervention study K . R . G A D D A M , S . N U V V U L A , S . N I R M A L A and R . K A M A T H A M Paedodontics and Preventive dentistry, Narayana Dental College, Nellore, Andhrapradesh, India
Correspondence: Kumar Raja Gaddam, Paedodontics and Preventive dentistry, Narayana Dental College, Nellore, Andhrapradesh, India. Tel.: +919866495595; fax: +918673272627; e-mail: [email protected] Accepted after revision 9 March 2014 DOI: 10.1111/hae.12434 Haemophilia (2014), 20, e336--e358
Haemophilia is the most common inherited bleeding disorder characterized by a lifelong defect in clotting mechanism. It is classified as haemophilia A and B, based on the deficiency of factor VIII and IX respectively; further as mild (5–25%), moderate (1–5%) and severe (
