Correlation between Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score and Hemophilia Joint Health Score (HJHS) in patients with hemophilic arthropathy.

Marcel Prasetyo,Ratna Moniqa,Angela Tulaar,Joedo Prihartono,Stefanus Imanuel Setiawan

doi:10.1371/journal.pone.0248952

Abstract

Hemophilic arthropathy, a condition manifested as joint destruction due to spontaneous joint bleeding, is one complication of hemophiliac patients. Early detection and intervention may improve the outcome, in which ultrasonography can be an ideal modality with the introduction of HEAD-US (Hemophilia Early Arthropathy Detection with Ultrasound) protocol. Studies have shown US benefit in hemophiliac patients, including its potential as an alternative for the Hemophiliac Joint Health Score (HJHS) system. However, many of the studies were conducted in countries with better management of hemophilia using prophylaxis treatment. It is unclear whether HEAD-US has a correlation with HJHS in countries using episodic treatment only, like in Indonesia. This study aimed to explore the correlation between HEAD-US and HJHS in hemophiliac patients with joint problems in Indonesia. A cross-sectional correlation study between HEAD-US and HJHS was performed with primary data collected from 120 hemophilic patients. US examination was performed on elbow, knee and ankle joints using the HEAD-US scoring method by a musculoskeletal radiologist. HJHS examination was conducted by a trained physiotherapist and a medical rehabilitation specialist. All examiner is member of multidisciplinary Hemophiliac Management Team in Cipto Mangunkusumo General Hospital in Jakarta, Indonesia. The mean age of the participant was 9.3 (5-14) years old. The median score of HEAD-US was 8 (1-28) with most of the joint abnormalities found on the ankles. The median score of HJHS was 3 (0-35), with most joint abnormalities found on the knees. There was a moderate correlation between HEAD-US and HJHS score (p < 0.05, r = 0.65). HEAD-US shows a moderate correlation to HJHS in hemophiliac patients who received episodic treatment. HEAD-US can provide additional value in the anatomical evaluation of the joint and could be complementary to HJHS in assessing the joint status in hemophilic patient.

Highlights

Hemophilia is a genetically hereditary coagulopathy disease caused by factor VIII (Hemophilia A) or factor IX coagulation deficiency that occurs in 1 out of 10000 people [1,2,3]
This study aimed to explore the correlation between Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) and Hemophiliac Joint Health Score (HJHS) in hemophiliac patients with joint problems in Indonesia
There was a moderate correlation between HEAD-US and HJHS score (p < 0.05, r = 0.65)

Summary

Introduction

Hemophilia is a genetically hereditary coagulopathy disease caused by factor VIII (Hemophilia A) or factor IX (hemophilia B) coagulation deficiency that occurs in 1 out of 10000 people [1,2,3]. A study by Martinoli et al [12] has introduced a standardized, fast, repeatable, and simple US scoring protocol for hemophiliac joint called Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US). This standardized method can assess multiple joints simultaneously with an excellent inter-observer agreement. Detection and intervention may improve the outcome, in which ultrasonography can be an ideal modality with the introduction of HEAD-US (Hemophilia Early Arthropathy Detection with Ultrasound) protocol. Many of the studies were conducted in countries with better management of hemophilia using prophylaxis treatment It is unclear whether HEAD-US has a correlation with HJHS in countries using episodic treatment only, like in Indonesia.

Objectives

Methods

Results

Conclusion