{"title":"致色测定 :准确诊断严重血友病 A 的重要诊断工具","authors":"Goutham Krishna, Hitha Diljith, Abdul Majeed","doi":"10.32677/ijch.v11i5.4681","DOIUrl":null,"url":null,"abstract":"Haemophilia A is a genetic disorder characterized by the absence or reduced activity of factor VIII (FVIII), a crucial protein for blood clotting. The severity of Haemophilia A is directly related to the level of FVIII activity in the blood. The conventional one-stage clotting assay is the most commonly used diagnostic tool due to its simplicity and widespread availability. However, in cases of mild or moderate Haemophilia A, there can be significant disparities between results obtained from one-stage and two-stage assays. These discrepancies can result in normal FVIII levels being reported by the one-stage assay, potentially leading to a missed diagnosis or underestimation of the patient's bleeding risk. We present a case of a 18-year-old male diagnosed with severe Haemophilia A. The patient’s medical history included multiple severe bleeding episodes, including spontaneous haemarthrosis and prolonged bleeding following minor injuries. Despite these symptoms, the initial diagnostic approach using the one-stage assay suggested normal FVIII activity, which could have led to inadequate treatment and management strategies. Findings in Chromogenic Assay were crucial in correctly identifying the severe nature of the disorder, thus underscoring the importance of using multiple diagnostic tools to confirm FVIII activity levels. This case highlights the role of the chromogenic assay in the accurate diagnosis of Haemophilia A, particularly in patients where clinical symptoms do not match with one-stage assay results. This case emphasizes the need for heightened awareness among clinicians regarding the limitations of the one-stage assay and the benefits of incorporating the chromogenic assay into routine diagnostic practice for Haemophilia A.","PeriodicalId":13441,"journal":{"name":"Indian Journal of Child Health","volume":"3 9","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2024-08-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Chromogenic Assay : A Critical Diagnostic Tool for Accurate Diagnosis of Severe Hemophilia A\",\"authors\":\"Goutham Krishna, Hitha Diljith, Abdul Majeed\",\"doi\":\"10.32677/ijch.v11i5.4681\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Haemophilia A is a genetic disorder characterized by the absence or reduced activity of factor VIII (FVIII), a crucial protein for blood clotting. The severity of Haemophilia A is directly related to the level of FVIII activity in the blood. The conventional one-stage clotting assay is the most commonly used diagnostic tool due to its simplicity and widespread availability. However, in cases of mild or moderate Haemophilia A, there can be significant disparities between results obtained from one-stage and two-stage assays. These discrepancies can result in normal FVIII levels being reported by the one-stage assay, potentially leading to a missed diagnosis or underestimation of the patient's bleeding risk. We present a case of a 18-year-old male diagnosed with severe Haemophilia A. The patient’s medical history included multiple severe bleeding episodes, including spontaneous haemarthrosis and prolonged bleeding following minor injuries. Despite these symptoms, the initial diagnostic approach using the one-stage assay suggested normal FVIII activity, which could have led to inadequate treatment and management strategies. Findings in Chromogenic Assay were crucial in correctly identifying the severe nature of the disorder, thus underscoring the importance of using multiple diagnostic tools to confirm FVIII activity levels. This case highlights the role of the chromogenic assay in the accurate diagnosis of Haemophilia A, particularly in patients where clinical symptoms do not match with one-stage assay results. This case emphasizes the need for heightened awareness among clinicians regarding the limitations of the one-stage assay and the benefits of incorporating the chromogenic assay into routine diagnostic practice for Haemophilia A.\",\"PeriodicalId\":13441,\"journal\":{\"name\":\"Indian Journal of Child Health\",\"volume\":\"3 9\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-08-09\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Indian Journal of Child Health\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.32677/ijch.v11i5.4681\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Indian Journal of Child Health","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.32677/ijch.v11i5.4681","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
摘要
血友病 A 是一种遗传性疾病,其特征是缺乏第八因子(FVIII)或第八因子活性降低,而第八因子是凝血的关键蛋白。血友病 A 的严重程度与血液中 FVIII 的活性水平直接相关。传统的单阶段凝血测定法因其简单易行和广泛使用而成为最常用的诊断工具。然而,在轻度或中度血友病 A 的病例中,单阶段检测和双阶段检测得出的结果可能存在显著差异。这些差异可能导致单阶段检测报告的 FVIII 水平正常,从而可能导致漏诊或低估患者的出血风险。我们介绍了一例被诊断为重度血友病 A 的 18 岁男性患者的病例。患者的病史包括多次严重出血,包括自发性血崩和轻伤后长时间出血。尽管出现了这些症状,但使用单阶段测定法进行的初步诊断显示 FVIII 活性正常,这可能导致治疗和管理策略不当。色原测定的结果对于正确识别该疾病的严重性质至关重要,因此强调了使用多种诊断工具确认 FVIII 活性水平的重要性。本病例强调了色原测定在准确诊断甲型血友病中的作用,尤其是在临床症状与单阶段测定结果不相符的患者中。本病例强调了临床医生需要提高对单级测定局限性的认识,以及将显色测定纳入血友病 A 常规诊断实践的益处。
Chromogenic Assay : A Critical Diagnostic Tool for Accurate Diagnosis of Severe Hemophilia A
Haemophilia A is a genetic disorder characterized by the absence or reduced activity of factor VIII (FVIII), a crucial protein for blood clotting. The severity of Haemophilia A is directly related to the level of FVIII activity in the blood. The conventional one-stage clotting assay is the most commonly used diagnostic tool due to its simplicity and widespread availability. However, in cases of mild or moderate Haemophilia A, there can be significant disparities between results obtained from one-stage and two-stage assays. These discrepancies can result in normal FVIII levels being reported by the one-stage assay, potentially leading to a missed diagnosis or underestimation of the patient's bleeding risk. We present a case of a 18-year-old male diagnosed with severe Haemophilia A. The patient’s medical history included multiple severe bleeding episodes, including spontaneous haemarthrosis and prolonged bleeding following minor injuries. Despite these symptoms, the initial diagnostic approach using the one-stage assay suggested normal FVIII activity, which could have led to inadequate treatment and management strategies. Findings in Chromogenic Assay were crucial in correctly identifying the severe nature of the disorder, thus underscoring the importance of using multiple diagnostic tools to confirm FVIII activity levels. This case highlights the role of the chromogenic assay in the accurate diagnosis of Haemophilia A, particularly in patients where clinical symptoms do not match with one-stage assay results. This case emphasizes the need for heightened awareness among clinicians regarding the limitations of the one-stage assay and the benefits of incorporating the chromogenic assay into routine diagnostic practice for Haemophilia A.