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重度甲型和乙型血友病关节病诊断、评估及分类中MRI、超声检查与功能独立性评分方法的比较

A comparison between MRI, sonography and Functional Independence Score in Haemophilia methods in diagnosis, evaluation and classification of arthropathy in severe haemophilia A and B.

作者信息

Tasbihi Mandana, Pishdad Parisa, Haghpanah Sezaneh, Ardeshiri Rezvan, Tavoosi Hakimeh, Karimi Mehran

机构信息

aHematology Research Center bMedical Imaging Research Center, Department of Radiology, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.

出版信息

Blood Coagul Fibrinolysis. 2016 Mar;27(2):131-5. doi: 10.1097/MBC.0000000000000376.

Abstract

Evaluation of joints in children with haemophilia is important in detecting abnormalities, staging their severity and following the effects of treatment. The aim of this study is to evaluate the correlation of FISH score (Functional Independence Score in Haemophilia) with the scores obtained by MRI and sonography for the diagnosis, evaluation and classification of arthropathy in severe haemophilia. In this cross-sectional study on 25 severe haemophilia patients, FISH, MRI and sonography procedures were performed in the elbow or knee joint. All patients' information, including age, type of haemophilia, affected joint, scores of MRI, sonography and FISH, dose of factor consumed, weight and prophylaxis protocol were collected and analysed. Among the 25 patients (age range of 11-70 years), 22 patients were haemophilia A and three patients were haemophilia B. Affected joints were right knee in 12 patients, left knee in nine and right elbow in four. There was only a statistically significant negative correlation between FISH and MRI Additive (A) scale (rs = -0.537, P = 0.006). Considering cartilage loss domain (related MRI A scale: 13-20), 20 patients (80%) were classified in this group with FISH scores ranged from 17 to 22. On the basis of our results, FISH scores in severe haemophilia patients were negatively correlated with MRI A scale. Also, it seems that a FISH score less than 22 could be considered as loss of cartilage; however, due to the small number of our patients, it needs further assessment in different populations.

摘要

评估血友病患儿的关节对于检测异常、确定其严重程度分期以及跟踪治疗效果非常重要。本研究的目的是评估血友病功能独立性评分(FISH评分)与通过MRI和超声检查所获得的分数之间的相关性,以用于重度血友病关节病的诊断、评估和分类。在这项针对25例重度血友病患者的横断面研究中,对肘关节或膝关节进行了FISH、MRI和超声检查。收集并分析了所有患者的信息,包括年龄、血友病类型、受累关节、MRI、超声和FISH评分、凝血因子使用剂量、体重和预防方案。在这25例患者(年龄范围为11 - 70岁)中,22例为A型血友病,3例为B型血友病。受累关节中,12例为右膝,9例为左膝,4例为右肘。FISH评分与MRI相加(A)量表之间仅存在统计学上显著的负相关(rs = -0.537,P = 0.006)。考虑软骨损伤领域(相关MRI A量表:13 - 20),20例患者(80%)被归为此组,其FISH评分范围为17至22。根据我们的结果,重度血友病患者的FISH评分与MRI A量表呈负相关。此外,似乎FISH评分低于22可被视为软骨损伤;然而,由于我们的患者数量较少,需要在不同人群中进行进一步评估。

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