Mirza Asif Baig, Anas M. Khan, Ameen D. Bakhsh, Thamer Ali Aljohani, Ahmed al Mutairi, Afrah S. Alharbi, Abrar Aljohani, Ahmed A. Alenezi, Abdulrahim A, Ayshah M. Mostafa, Ghadeer aljohani, Zaraah sofiyani, Mona altarqyi, Fayza Ahmed
{"title":"确定儿科患者中具有特定 VWF:RCof/ VWF:Ag 比率的各种 VWD 亚型的发病率","authors":"Mirza Asif Baig, Anas M. Khan, Ameen D. Bakhsh, Thamer Ali Aljohani, Ahmed al Mutairi, Afrah S. Alharbi, Abrar Aljohani, Ahmed A. Alenezi, Abdulrahim A, Ayshah M. Mostafa, Ghadeer aljohani, Zaraah sofiyani, Mona altarqyi, Fayza Ahmed","doi":"10.36348/sjpm.2023.v08i11.001","DOIUrl":null,"url":null,"abstract":"Background: VWD is the most common inherited bleeding disorder characterized by defects in the concentration, structure, or function VWF. There are three main types of VWD that differ according to the degree of disease severity and inheritance pattern (Type 1, Type 2A, 2B, 2M, 2N and Type 3). Methodology: This is 05 years study (Feb 2016–Jan 2021) conducted in hematology section. In present study, amongst total inherited bleeding disorders, Incidence of VWD is 27%, Hemophilia (50%), inherited platelet disorders (14%) and remainder are undiagnosed. Amongst VWD, Type 1 VWD is the most common subtype studied, comprising 66% of total cases. Type 2 & 3 VWD comprised 23% and 10% of total cases showing good correlation with the literature and other studies. Discussion: The European cross-sectional study yielded a population-based estimate of 0.05 per 100,000 for type 3 VWD. Estimates by VWD type from Europe and Western Pacific reported higher prevalence estimates for type 1 disease than type 2 or type 3: 2.7–7.2 per 100,000 for type 1 VWD, 0.8–2.5 per 100,000 for type 2, and 0.1–0.3 per 100,000 for type 3. Conclusion: VWD usually presents with mild bleeding symptoms (except in type 3 VWD) the diagnosis is often delayed. Prompt diagnosis and management can help to avoid potentially life-threatening bleeding events and unnecessary exposure to blood products.","PeriodicalId":407259,"journal":{"name":"Saudi Journal of Pathology and Microbiology","volume":"43 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2023-11-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"To Determine the Incidence of Various Subtypes of VWD with Specified VWF:RCof/ VWF:Ag Ratio’s In Pediatric Patients\",\"authors\":\"Mirza Asif Baig, Anas M. Khan, Ameen D. Bakhsh, Thamer Ali Aljohani, Ahmed al Mutairi, Afrah S. Alharbi, Abrar Aljohani, Ahmed A. Alenezi, Abdulrahim A, Ayshah M. Mostafa, Ghadeer aljohani, Zaraah sofiyani, Mona altarqyi, Fayza Ahmed\",\"doi\":\"10.36348/sjpm.2023.v08i11.001\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Background: VWD is the most common inherited bleeding disorder characterized by defects in the concentration, structure, or function VWF. There are three main types of VWD that differ according to the degree of disease severity and inheritance pattern (Type 1, Type 2A, 2B, 2M, 2N and Type 3). Methodology: This is 05 years study (Feb 2016–Jan 2021) conducted in hematology section. In present study, amongst total inherited bleeding disorders, Incidence of VWD is 27%, Hemophilia (50%), inherited platelet disorders (14%) and remainder are undiagnosed. Amongst VWD, Type 1 VWD is the most common subtype studied, comprising 66% of total cases. Type 2 & 3 VWD comprised 23% and 10% of total cases showing good correlation with the literature and other studies. Discussion: The European cross-sectional study yielded a population-based estimate of 0.05 per 100,000 for type 3 VWD. Estimates by VWD type from Europe and Western Pacific reported higher prevalence estimates for type 1 disease than type 2 or type 3: 2.7–7.2 per 100,000 for type 1 VWD, 0.8–2.5 per 100,000 for type 2, and 0.1–0.3 per 100,000 for type 3. Conclusion: VWD usually presents with mild bleeding symptoms (except in type 3 VWD) the diagnosis is often delayed. Prompt diagnosis and management can help to avoid potentially life-threatening bleeding events and unnecessary exposure to blood products.\",\"PeriodicalId\":407259,\"journal\":{\"name\":\"Saudi Journal of Pathology and Microbiology\",\"volume\":\"43 1\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2023-11-10\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Saudi Journal of Pathology and Microbiology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.36348/sjpm.2023.v08i11.001\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Saudi Journal of Pathology and Microbiology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.36348/sjpm.2023.v08i11.001","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
To Determine the Incidence of Various Subtypes of VWD with Specified VWF:RCof/ VWF:Ag Ratio’s In Pediatric Patients
Background: VWD is the most common inherited bleeding disorder characterized by defects in the concentration, structure, or function VWF. There are three main types of VWD that differ according to the degree of disease severity and inheritance pattern (Type 1, Type 2A, 2B, 2M, 2N and Type 3). Methodology: This is 05 years study (Feb 2016–Jan 2021) conducted in hematology section. In present study, amongst total inherited bleeding disorders, Incidence of VWD is 27%, Hemophilia (50%), inherited platelet disorders (14%) and remainder are undiagnosed. Amongst VWD, Type 1 VWD is the most common subtype studied, comprising 66% of total cases. Type 2 & 3 VWD comprised 23% and 10% of total cases showing good correlation with the literature and other studies. Discussion: The European cross-sectional study yielded a population-based estimate of 0.05 per 100,000 for type 3 VWD. Estimates by VWD type from Europe and Western Pacific reported higher prevalence estimates for type 1 disease than type 2 or type 3: 2.7–7.2 per 100,000 for type 1 VWD, 0.8–2.5 per 100,000 for type 2, and 0.1–0.3 per 100,000 for type 3. Conclusion: VWD usually presents with mild bleeding symptoms (except in type 3 VWD) the diagnosis is often delayed. Prompt diagnosis and management can help to avoid potentially life-threatening bleeding events and unnecessary exposure to blood products.