Prophylaxie de l’hémophilie en pédiatrie (notice n° 660318)
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| 000 -LEADER | |
|---|---|
| fixed length control field | 03565cam a2200325 4500500 |
| 005 - DATE AND TIME OF LATEST TRANSACTION | |
| control field | 20250121192215.0 |
| 041 ## - LANGUAGE CODE | |
| Language code of text/sound track or separate title | fre |
| 042 ## - AUTHENTICATION CODE | |
| Authentication code | dc |
| 100 10 - MAIN ENTRY--PERSONAL NAME | |
| Personal name | Harroche, Annie |
| Relator term | author |
| 245 00 - TITLE STATEMENT | |
| Title | Prophylaxie de l’hémophilie en pédiatrie |
| 260 ## - PUBLICATION, DISTRIBUTION, ETC. | |
| Date of publication, distribution, etc. | 2025.<br/> |
| 500 ## - GENERAL NOTE | |
| General note | 44 |
| 520 ## - SUMMARY, ETC. | |
| Summary, etc. | The aim of paediatric haemophilia prophylaxis is to prevent recurrent bleeding and its complications. Haemophilia, a genetic bleeding disorder, is divided into two main types: haemophilia A (factor VIII deficiency) and B (factor IX deficiency), affecting mainly boys. It is classified according to severity (severe, moderate or minor), and in severe forms manifests itself in childhood as joint bleeding (haemarthrosis) and haematomas. Prophylaxis, introduced early (ideally before the age of 2), aims to prevent irreversible joint damage and other serious complications such as intracranial haemorrhage. This may include regular injections of coagulation factors or, more recently, the use of emicizumab, a more practical subcutaneous treatment that transforms severe forms into minor ones. The latter is particularly suitable for young children and patients with venous difficulties. Patients with inhibitors (neutralising antibodies) require specific approaches, such as bypassing agents or emicizumab. Regular clinical and biological monitoring is crucial for assessing efficacy, adapting treatments and preventing complications. This strategy significantly improves patients’ quality of life. |
| 520 ## - SUMMARY, ETC. | |
| Summary, etc. | La prophylaxie de l’hémophilie pédiatrique vise à éviter les saignements récurrents et leurs complications. L’hémophilie, maladie génétique hémorragique, se divise en deux types principaux : l’hémophilie A (déficit en facteur VIII) et B (déficit en facteur IX), affectant surtout les garçons. Elle est classée selon sa sévérité (sévère, modérée ou mineure) et se manifeste dès l’enfance, dans les formes graves, par des saignements articulaires (hémarthroses) et des hématomes. La prophylaxie, introduite tôt (idéalement avant 2 ans), vise à prévenir les lésions articulaires irréversibles et d’autres complications graves comme les hémorragies intracrâniennes. Elle peut inclure des injections régulières de facteurs de coagulation ou, plus récemment, l’utilisation de traitements non substitutifs comme l'émicizumab, un traitement sous-cutané plus pratique, transformant les formes sévères en mineures. Ce dernier est particulièrement adapté aux jeunes enfants et aux patients ayant des difficultés veineuses. Les patients atteints d’inhibiteurs (anticorps neutralisants) nécessitent des approches spécifiques, comme les agents by-passants ou les traitements non substitutifs (émicizumab). La surveillance clinique et biologique régulière est cruciale pour évaluer l’efficacité, adapter les traitements et prévenir les complications. Cette stratégie améliore significativement la qualité de vie des patients. |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | émicizumab |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | hémarthroses |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | facteur VIII |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | Hémophilie A/B |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | facteur IX |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | agents by-passants |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | factor VIII |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | emicizumab |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | Haemophilia A/B |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | haemarthroses |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | bypassing agents |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | factor IX |
| 700 10 - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Meunier, Sandrine |
| Relator term | author |
| 786 0# - DATA SOURCE ENTRY | |
| Note | Hématologie | 30 | 6 | 2025-01-06 | p. 351-361 | 1264-7527 |
| 856 41 - ELECTRONIC LOCATION AND ACCESS | |
| Uniform Resource Identifier | <a href="https://shs.cairn.info/revue-hematologie-2024-6-page-351?lang=fr&redirect-ssocas=7080">https://shs.cairn.info/revue-hematologie-2024-6-page-351?lang=fr&redirect-ssocas=7080</a> |
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