贝尼-苏夫大学医院儿童出血性疾病(血友病)的研究

Dalia Kamal, D. Ezzat, Rehab Lashin
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摘要

本研究的目的是研究贝尼-苏夫大学医院血友病儿童(年龄<18岁)的临床谱。在贝尼-苏夫大学医院儿科血液学诊所进行的一项横断面研究,为期12个月。在纳入的112例病例中,91%诊断为a型血友病,9%诊断为b型血友病。阳性家族史占46%。出现症状和诊断的平均年龄分别为1.7±1.4岁和3.8±3.8岁。)最常见的出血症状是瘀伤(86.5%),最严重的是颅内出血(9.6%)。血关节病占7.7%。结论:在其他方面正常的儿童中出现创伤后出血和牙龈出血应引起临床医生对血友病的评价。血友病分布在世界各地,根据疾病的严重程度有不同的表现。了解血友病在当地人群中的表现谱有助于早期诊断和管理规划。
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Study of bleeding disorders (hemophilia) in children in Beni-Suef university hospital
: The goal of this study is to study the clinical spectrum of hemophiliac children (age <18 years) in Beni-Suef university hospital. A cross-sectional study conducted in the pediatric hematology clinic of Beni-Suef university hospital, over a period of 12 months. Out of a total of 112 cases enrolled, 91% were diagnosed as hemophilia A. While (9%) were diagnosed as hemophilia B. Positive family history was found in (46%) of cases. The mean age of onset of symptoms and diagnosis was 1.7 ± 1.4 and 3.8 ± 3.8 years, respectively. ), the most common bleeding symptom was bruises (86.5), the most serious was intracranial hemorrhage (9.6%). While haemarthrosis was in (7.7%).Conclusion: Occurrence of posttraumatic bleeds and gum bleeds in an otherwise normal child should warn the clinician for evaluation of hemophilia. Hemophilias are distributed worldwide and have heterogeneous presentation depending upon the disease severity. Knowledge of the spectrum of presentation of hemophilia in the local population helps in early diagnosis and planning of management.
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