Abstract

Introduction
Haemophilia A is an X-linked congenital bleeding disorder caused by mutations in coagulation factor VIII gene (F8) which result in absent, deficient, or defective factor VIII protein (FVIII). The range of clinical severity is proportional to the plasma level of FVIII. A FVIII level of less than 1% produces severe disease. Patients with severe disease have spontaneous bleeding in joints, soft tissues, and vital organs as well as exaggerated bleeding following trauma or surgery. Preventive replacement therapy (with plasma-derived or recombinant FVIII) has increased the life expectancy of subjects with haemophilia A, but this complex pathology requires expert clinical management by a multidisciplinary team (paediatrician, haematologist, orthopaedist, etc.)1,2. Cardiac surgery in infants with haemophilia can be a particularly challenging problem because significant coagulation abnormalities may occur during and after surgery. Careful pre-operative planning and meticulous post-operative monitoring of FVIII replacement therapy are essential for maintaining optimal haemostasis. Although considerable advances have been made in the management of haemophilia, there is a lack of guidelines pertaining to cardiac surgery in children.

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Authors

Giuseppe Lassandro - Paediatric Section, Department of Biomedicine and Human Oncology, “A. Moro” University, Bari

Gabriele Scalzo - Department of Paediatric Cardiac Surgery, “Giovanni XXIII” Paediatric Hospital, Bari

Viviana V. Palmieri - Paediatric Section, Department of Biomedicine and Human Oncology, “A. Moro” University, Bari

Ugo Vairo - Department of Paediatric Cardiology, “Giovanni XXIII” Paediatric Hospital, Bari

Leonardo Milella - Department of Paediatry Anaesthesia and Reanimation, Cardiac Surgery Intensive Care, Operating Rooms, “Giovanni XXIII” Paediatric Hospital, Bari

Giuseppe Scrascia - Department of Paediatric Cardiac Surgery, “Giovanni XXIII” Paediatric Hospital, Bari

Maria E. Mancuso - Department of Internal Medicine and Dermatology, “Angelo Bianchi Bonomi” Haemophilia and Thrombosis Centre, IRCCS Maggiore Hospital, “Mangiagalli and Regina Elena” Foundation and University of Milan, Milan, Italy

Paola Giordano - Paediatric Section, Department of Biomedicine and Human Oncology, “A. Moro” University, Bari

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