Prevalence of Factor VIII Inhibitors in Patients with Hemophilia A in Brazil

Rieger, Alexandre; Roisenberg, Israel

doi:10.1055/s-0037-1614506

Cited by 4 publications

(6 citation statements)

References 7 publications

(8 reference statements)

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“…2 (NB, the data from the UKHCDO report have been omitted, as they are included in the study by Rizza [8]). Only two studies reporting on more than 1000 patients gave figures of >10% [12,13].…”

Section: Studies Of the Prevalence Of Inhibitors Including Registry mentioning

confidence: 99%

“…The study by Reiger [13] is reported as a letter. Data were derived both from reference centres where inhibitor tests were performed routinely, and also from patients treated elsewhere whose plasma samples were sent to the reference centres for testing.…”

Section: Studies Of the Prevalence Of Inhibitors Including Registry mentioning

confidence: 99%

“…6 4 or 5 7 3 and 6 8 limit 7 to yr¼2000-2002 9 limit 7 to topic reviews cochrane 10 limit 7 to article reviews best evidence 11 limit 7 to article reviews dare 12 7 and review.pt. 13…”

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confidence: 99%

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The epidemiology of inhibitors in haemophilia A: a systematic review

2003

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This paper emphasizes the importance of distinguishing between the prevalence, incidence and cumulative incidence of inhibitors in haemophilia A. Incidence and cumulative incidence data will include patients with transient inhibitors or whose inhibitors have been eliminated by treatment. As these will not be included in prevalence data, prevalence studies will tend to give rise to lower figures than incidence studies. As a result, the most accurate estimates of the true risk of inhibitor development comes from prospective studies of newly diagnosed haemophiliacs who are tested regularly for the presence of inhibitors. This paper reports a systematic review of the best available evidence relating to the epidemiology of inhibitors in haemophilia A. Cohort studies, registry data reporting incidence or prevalence of inhibitors in patients with haemophilia A, and prospective studies of factor VIII (FVIII) in the treatment of previously untreated patients which reported the development of inhibitors as an outcome, were included in the review. The overall prevalence of inhibitors in unselected haemophiliac populations was found to be 5-7%. The cumulative risk of inhibitor development varied (0-39%). Incidence and prevalence were substantially higher in patients with severe haemophilia. Studies of patients using a single plasma-derived FVIII (pdFVIII) preparation reported lower inhibitor incidence than those using multiple pdFVIII preparations or single recombinant FVIII preparations. Incidence data should be used to estimate the likely demand for treatments aimed at eliminating inhibitors, whereas the best estimates of the overall burden to the National Health Service (NHS) of treating bleeding episodes in patients with continuing inhibitors will come from prevalence studies.

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Section: Studies Of the Prevalence Of Inhibitors Including Registry mentioning

confidence: 99%

Section: Studies Of the Prevalence Of Inhibitors Including Registry mentioning

confidence: 99%

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The epidemiology of inhibitors in haemophilia A: a systematic review

2003

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“…During his academic career, Israel investigated a series of pathological conditions and was also interested in the development of new laboratory techniques. Details are given in the Selected List of his publications, below, which can be summarized as follows: (a) Hemophilias A and B (numbers 2,6,9,11,13,16,19,20,44)…”

Section: Researchmentioning

confidence: 99%

Israel Roisenberg (1934-2013): a life dedicated to science

Salzano

2014

Genet. Mol. Biol.

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Historians of science have continuously searched for the factors which determine why certain persons achieve proeminence in the area. Of prime importance, of course, is their biological constitution; only persons with a given degree of intelligence can reach success. But there are many additional factors which are also important, including work drive, persistence, as well as appropriate family and academic environments. This combination of factors led to Israel Roisenberg's brilliant career, and the main events of his life are listed in Table 1. He was born in Porto Alegre on July 31, 1934, where he lived all his life, except for varied periods of time in São Paulo and outside Brazil. After primary and secondary education (the latter in a public school, Colégio Júlio de Castilhos), he was approved in the Federal University of Rio Grande do Sul (UFRGS) entrance exam for the Biological Sciences Career in 1958. Soon he developed an interest in research, and as a consequence I invited him to work as an undergraduate student in our laboratory. He graduated in 1961 and soon afterwards received a contract as a Biologist at UFRGS. Our PostGraduate Program was just starting at the time, but he also immediately enrolled as a Ph.D. student.

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“…1, but both may increase the risk of formation of anti‐FVIII antibodies that interfere with treatment [1–5]. As part of a long‐term project we have been conducting for more than three decades now (selected references in [6–9]) on different aspects of haemorrhagic diseases in our population, this report presents data on the prevalence of these two inversions in severe haemophilia A patients from southern Brazil and their association with the presence of anti‐FVIII inhibitors. Two questions of general interest were asked: (i) do the frequencies of inv.…”

Section: Introductionmentioning

confidence: 99%

Introns 1 and 22 inversions and factor VIII inhibitors in patients with severe haemophilia A in southern Brazil

et al. 2009

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A total of 107 unrelated severe haemophilia A patients living in the southern Brazilian state of Rio Grande do Sul were studied in relation to the prevalence of inversions present in introns 22 and 1 and a subsample of them (95) tested for the presence of Factor VIII inhibitors. These data were then incorporated with those from 15 other countries and 3871 patients. The frequencies of these two inversions show a remarkable homogeneity in series collected in different continents, from people with diverse ethnic extraction. The prevalence of inhibitors among patients with inversion 22, on the other hand, varies widely (5-51%; seven countries, 1482 patients), the value observed by us being the highest. The importance of obtaining data from patients throughout the world to clarify the aetiology of this important complicating factor in the therapeutics of the disease is emphasized.

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Prevalence of Factor VIII Inhibitors in Patients with Hemophilia A in Brazil

Cited by 4 publications

References 7 publications

The epidemiology of inhibitors in haemophilia A: a systematic review

The epidemiology of inhibitors in haemophilia A: a systematic review

Israel Roisenberg (1934-2013): a life dedicated to science

Introns 1 and 22 inversions and factor VIII inhibitors in patients with severe haemophilia A in southern Brazil

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