Kessi Eric Michel Charlemagne Junior, Maslouhi Kaoutar, Guelzim Yousra, Belkouchi Lina, Allali Nazik, Chat Latifa, El Haddad Siham
Université Mohammed V de Rabat, Rabat, Morocco.
Hopital Ibn Sina, Rabat, Morocco.
Glob Pediatr Health. 2024 Oct 3;11:2333794X241280116. doi: 10.1177/2333794X241280116. eCollection 2024.
Hemophilia is a congenital coagulopathy characterized by a deficiency of coagulation factors and the development of haematomas and haemarthrosis, either spontaneously or after minor trauma. Recurrent joint hemorrhage in hemophilia patients leads to progressive and degenerative arthropathy, which affects around 90% of patients with severe disease and contributes significantly to disease morbidity. Positive diagnosis is based on biology. Imaging, particularly MRI, plays an essential role in assessing the evolution and complications, especially osteoarticular complications. We report 2 cases of severe hemophilia A, who presented with almost identical clinical and radiological symptoms. The patients developed severe arthropathy with a course marked by recurrences of haemarthrosis.
血友病是一种先天性凝血病，其特征是凝血因子缺乏，可自发或在轻微创伤后出现血肿和关节积血。血友病患者反复的关节出血会导致进行性和退行性关节病，约90%的重症患者会受其影响，且这是导致疾病致残的重要因素。确诊基于生物学检查。影像学检查，尤其是磁共振成像（MRI），在评估病情进展及并发症，特别是骨关节并发症方面起着至关重要的作用。我们报告了2例重度甲型血友病患者，他们表现出几乎相同的临床和放射学症状。这些患者均发展为严重的关节病，病程以关节积血反复发作为特征。
