Distribution of haemophilia in the French Basque country

Bauduer, Frédéric; Degioanni, Anna; Ducout, Louis; Scribans, Cédric; Dutour, Olivier

doi:10.1046/j.1365-2516.2002.00659.x

Cited by 8 publications

(11 citation statements)

References 19 publications

(27 reference statements)

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“…1:12 300 or 1:5600 males. These data are consistent with data from other European countries [6][7][8]. The ratio of haemophilia A to B is 6.2:1, very similar as in Greece (6.6:1) [7] and higher than in Sweden and Scotland (4:1 and 3:1 respectively) [8,9].…”

Section: Discussionsupporting

confidence: 90%

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Haemophilia in Poland

et al. 2006

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The primary aim of this study was to analyse the data on 2269 haemophilic patients in the Polish National Register of Inherited Bleeding Disorders -- 1953 haemophilia A patients and 316 haemophilia B patients. Haemophilia A occurred in 1512 families, haemophilia B in 240 families. In the majority of haemophilia A and B cases severe haemophilia prevailed (59.7% and 56.6% respectively). In about 50% of haemophilic patients, no family history of bleeding diathesis was reported. For haemophilia A patients the mean age was 30.9 years and for haemophilia B patients, 29.2 years. Prevalence of haemophilia in Poland is approximately 1:12 300 inhabitants (1:5600 males). The second aim was to describe the orthopaedic status of severe haemophilia patients and to relate this status to the type of replacement therapy they received prior to the study. Ninety-two severe haemophilia patients (median age 26.0 years) were enrolled in the study. Right and left knee, elbow and ankle joints were evaluated clinically using the Gilbert scale. X-ray examinations were evaluated according to the Pettersson scale. Knee joints proved to be most affected. Eighty-four patients (91.3%) reported pain. Only one scored 0 on the Gilbert scale, another on the Pettersson scale. Thirty-seven per cent of patients used orthopaedic equipment, either occasionally or constantly. Twenty-five per cent had a history of orthopaedic surgery. Thirty-eight per cent were unemployed with some form of social subvention. On-demand treatment was applied. None of the patients received primary prophylaxis. The mean consumption of clotting factor concentrates was 68 054 IU per patient during the 12 months prior to the current study. These results indicate that in Poland all severe haemophilia patients above 20 years are affected by haemophilic arthropathy.

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Section: Discussionsupporting

confidence: 90%

“…Age at the start of the replacement therapy -mean ± SD; median (range) 2.5 ± 2.1; 2(1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14) Age at the shift from FFP/cryoprecipitate to CFC -mean ± SD; median (range)13.8 ± 4.3; 13.5 (1-24) Age at the start of home therapy* -mean ± SD; median (range)…”

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confidence: 99%

Haemophilia in Poland

et al. 2006

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“…A non-representative sample occurs when the respondents from the sample do not match the desired target population. Some countries sampled a subset of its population to estimate prevalence [9,[53][54][55]79,81] while others are old studies, and the prevalence reported for the 1970s may not represent the observed prevalence today [14,55,[62][63][64][65][66]77,78,80,81]. A survey nonresponse occurs when a respondent does not respond to a survey request.…”

Section: Discussionmentioning

confidence: 99%

A study of variations in the reported haemophilia A prevalence around the world

et al. 2009

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The objectives of this paper were to study the reported haemophilia A prevalence (per 100 000 males) on a country-by-country basis and address the following: Does the reported prevalence of haemophilia A vary by national economies? We collected prevalence data for 106 countries from the World Federation of Hemophilia (WFH) annual global surveys and the literature. We found that the reported haemophilia A prevalence varied considerably among countries, even among the wealthiest of countries. The prevalence (per 100 000 males) for high income countries was 12.8 +/- 6.0 (mean +/- SD) whereas it was 6.6 +/- 4.8 for the rest of the world. Within a country, there was a strong trend of increasing prevalence over time--the prevalence for Canada ranged from 10.2 in 1989 to 14.2 in 2008 (R = 0.94 and P < 0.001) and for the United Kingdom it ranged from 9.3 in 1974 to 21.6 in 2006 (R = 0.94 and P < 0.001). Prevalence data reported from the WFH compared well with prevalence data from the literature. Patient registries generally provided the highest quality of prevalence data. The lack of accurate country-specific prevalence data has constrained planning efforts for the treatment and care of people with haemophilia A. With improved information, healthcare agencies can assess budgetary needs to develop better diagnostic and treatment facilities for affected patients and families and work to ensure adequate supplies of factor VIII concentrates for treatment. In addition, this information can help manufacturers plan the production of concentrates and prevent future shortages.

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“…In our series, most of the patients with vWD suffered from bruise/haematoma, and many had joint deformities from haemarthrosis, as they were of type 3 vWD. The severity distribution of haemophilia in our series (severe/moderate/mild: 45.1/41.9/13.0) is similar to Korea (54.8/36.5/8.7) but quite different from Sweden (29.6/16/54.4) [6], and the likely explanation may be the mild ones were not suspected to have a coagulation disorder (with mild bleeding symptoms) until a serious bleeding has occurred in the less developed countries.…”

Section: Discussionmentioning

confidence: 99%

Retrospective analysis of 1312 patients with haemophilia and related disorders in a single Chinese institute

Zhang

Zhao

et al. 2003

Haemophilia

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With 1.3 billion people, China has the largest population in the world, and therefore has the largest population of persons with haemophilia (PWH). As there is no national registry for haemophilia, it is difficult to ascertain how many PWH have actually been diagnosed. Between January 1983 and June 2002, 1312 patients with coagulation disorders were referred to our hospital, and 1190 patients were evaluable. Among them, 1069 (89.8%) patients had haemophilia, 68 had vWD, 20 had factor XI deficiency, 10 had acquired factor VIII inhibitor and 23 had other coagulation disorders. Of the 1069 PWH, 14.7% were unclassified, 38.4% severe, 35.7% moderate and 11.1% mild. If the unclassified cases were excluded, 45.1% were severe, 41.9% moderate and 13.0% mild. Twenty-nine of the 68 vWD patients had vWF:Ag <5%, and subcategorized as type 3 vWD. Because vWF multimer analysis was not performed in our centre, the remaining vWD patients were not subdivided.

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Distribution of haemophilia in the French Basque country

Cited by 8 publications

References 19 publications

Haemophilia in Poland

Haemophilia in Poland

A study of variations in the reported haemophilia A prevalence around the world

Retrospective analysis of 1312 patients with haemophilia and related disorders in a single Chinese institute

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