Pediatric Clinics of North America - CIPERJ

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Table 1 (continued ) Factor VIII (or factor IX) concentrates useful in treatment of alloantibody and autoantibody inhibitor-related bleeding Product name (manufacturer) Viral inactivation method Dosage Recombinant factor VIIa (genetic engineered) NovoSeven (Novo Nordisk, Inc.) (stabilized in mannitol; bovine calf serum used in culture medium) FEIBA-VH (Baxter Immuno, Inc.) (human plasma derived) Affinity chromatography Solvent/detergent (TNPB/polysorbate 80) Vapor heated (10 h, 60 C, 190 mbar plus 1 h, 80 C, 375 mbar) 90-mg/kg intravenous bolus every 2–3 h until bleeding ceases (larger dosing regimens are experimental but may be useful in refractory bleeding). This product is the treatment of choice for individuals who have allofactor IX antibody inhibitors and anaphylaxis or renal disease associated with the use of factor IX containing concentrates. 50–100 IU/kg not to exceed 200 IU/kg/24 h (for factor VIII and IX inhibitors) Porcine plasma-derived factor VIII concentrate Hyate C (Ibsen Biomeasure, Inc.) No longer available O50 IU/mg (for factor VIII inhibitors only) Adapted from Kessler CM. New perspectives in hemophilia treatment. Hematology Am Soc Hematol Educ Program 2005;429–35; with permission. 362 RODRIGUEZ & HOOTS
ADVANCES IN HEMOPHILIA 363 prophylaxis can prevent this subclinical bleeding from producing even minimal arthropathy years later. Timing of prophylaxis The question of when to start primary prophylaxis has been a subject of controversy among hemophilia caregivers worldwide. To date, there is no consensus. Several studies show that children who have hemophilia and few or no joint bleeds [5–8] who are started on prophylaxis early (mean age of 3 years) exhibit a better musculoskeletal outcome. Progression of joint arthropathy, even after starting prophylaxis, is described in patients who have at least five joint bleeds occurring at the same or different joints [5–8]. In a study published by Astermark and colleagues [5], the only significant predictor for development of hemophilic arthropathy was the age of patients when prophylaxis was started. Using the Pettersson score, a scoring system that allows radiologic evaluation of the joints in patients who have hemophilia, Fischer and colleagues [9] described the Pettersson scores increasing by 8% each year that prophylaxis was postponed after the first occurrence of hemarthrosis. These studies show that irreversible joint damage may follow after a few joint bleeds and that even early prophylaxis may not abrogate that process completely. Therefore, worldwide recommendations to start prophylaxis before joint damage are favored to promote joint integrity, ideally before 3 years of age. Secondary prophylaxis Patients who have preexisting joint disease and who experience frequent acute hemarthroses may be treated with periodic use of factor concentrates for a short or long period of time to curtail bleeding recurrence. This approach is known as secondary prophylaxis and is used commonly to minimize bleeding frequency and lessen the progression of joint disease. Even though secondary prophylaxis cannot reverse the changes of chronic arthropathy, it may be beneficial by reducing frequency of bleeding, hospital admissions, and lost days from school or work and by decreasing damage progression. The use of secondary prophylaxis versus on-demand therapy has been the subject of various studies done in children and adults who have severe hemophilia [10–12]. In summary, the results indicate that patients treated with secondary prophylaxis have decreased number of joint bleeds at the expense of higher clotting factor consumption. A recent study, however, does not confirm the higher cost of this approach. A long-term outcome study (follow-up of 22 years) published by Fischer and colleagues [13] compared the costs of prophylaxis (primary and secondary) versus secondary prophylaxis alone versus on-demand therapy in patients who had severe hemophilia. In this study, short-term prophylaxis
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Pediatr Clin N Am 55 (2008) xiii-xi
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Pediatr Clin N Am 55 (2008) 287-304
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DECISION ANALYSIS IN PEDIATRIC HEMA
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Nietert et al [30] Treatment of sic
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VENOUS THROMBOEMBOLISM IN CHILDREN
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VENOUS THROMBOEMBOLISM IN CHILDREN
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412 BLANCHETTE & BOLTON-MAGGS with
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414 BLANCHETTE & BOLTON-MAGGS react
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416 BLANCHETTE & BOLTON-MAGGS [16]
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422 FASANO & LUBAN of storage and t
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424 FASANO & LUBAN Leukocyte reduct
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426 FASANO & LUBAN RBCs is decrease
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Table 1 Blood component characteris
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430 FASANO & LUBAN Alloimmunization
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432 FASANO & LUBAN of individual un
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434 FASANO & LUBAN patients have an
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436 FASANO & LUBAN adults; therefor
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438 FASANO & LUBAN [21]. Meta-analy
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440 FASANO & LUBAN Pretransfusion m
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442 FASANO & LUBAN [55,56]. This is
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444 FASANO & LUBAN [20] Roseff SD,
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THALASSEMIA UPDATE 449 hypochromia
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THALASSEMIA UPDATE 451 Fig. 1. Chan
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THALASSEMIA UPDATE 453 delivery of
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THALASSEMIA UPDATE 455 thromboses i
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THALASSEMIA UPDATE 457 On the basis
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THALASSEMIA UPDATE 459 [31] Kan YW,
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ORAL IRON CHELATORS 463 large iron-
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ORAL IRON CHELATORS 465 of 50 child
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ORAL IRON CHELATORS 467 Table 2 Com
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ORAL IRON CHELATORS 469 use in Nort
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ORAL IRON CHELATORS 471 needed to d
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ORAL IRON CHELATORS 473 in liver fi
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ORAL IRON CHELATORS 475 in the lowe
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ORAL IRON CHELATORS 477 Other oral
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ORAL IRON CHELATORS 479 [12] Borgna
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ORAL IRON CHELATORS 481 [55] Wonke
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HYDROXYUREA FOR CHILDREN WITH SICKL
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Fig. 1. Guideline for initiating, m
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504 TRACY & RICE congenital spheroc
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506 TRACY & RICE and antibodies aga
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508 TRACY & RICE limited splenic vo
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510 TRACY & RICE constitutes the pi
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512 TRACY & RICE German experience
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514 TRACY & RICE Table 1 Effect of
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516 TRACY & RICE decreased bilirubi
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518 TRACY & RICE [13] Bader-Meunier
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Pediatric Clinics of North America - CIPERJ

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