OC 01.2 - Assessment of hemophilic joints in a low resourced area using clinical tools: the effect of different prophylaxis types

Saturday, June 22, 2024

13:15 – 13:30 ICT

Room: Ballroom B1

Co-Author(s)

ILHAM YOUSSRY

Professor of Pediatrics and Head of the Pediatric Hematology and BMT unit, Cairo University
Faculty of Medicine, Cairo University
Cairo, Al Qahirah, Egypt
RE

Rania El-Taweel

Assistant lecturer of pediatrics
Cairo University
Cairo, Al Qahirah, Egypt
hS

hadeel Seif

profrssor of Radiology
Cairo University
Cairo, Al Qahirah, Egypt
RS

Rania Sami

professor of clinical pathology
Cairo University
Cairo, Al Qahirah, Egypt
ET

Ebtehal Taha

physiotherapy
NCUCH
Cairo, Al Qahirah, Egypt
KO

Karim Okasha

orthopedic surgeon
Cairo University
Cairo, Al Qahirah, Egypt

Presenting Author(s)

Assad haffar, Master of Science

Medical director
World Federation of hemophilia (WFH)
Montreal, Quebec, Canada

Co-Author(s)

MA

Marwa Abd Elhady

Assistant professor of Pediatrics
Cairo University
Cairo, Al Qahirah, Egypt

Background: Hemophilic arthropathy (HA) is the most frequent complication in people with hemophilia (PWH). MRI is considered the gold standard to assess early joint changes, however, there are limitations for its frequent use, especially in the low resourced areas

Aims: primary aim; to compare clinical, functional, laboratory and ultrasonographic joint scores with MRI scores to determine an accurate, and accessible alternative to MRI.
Secondary aim; to identify the effect of various replacement therapies on the degree of joint affection.

Methods: Fifty PWH, aged < 20 years, with at least one affected joint, with or without inhibitors and receiving either on demand treatment or secondary prophylaxis treatment were included, table (1). All participants had joint assessment by clinical HJHS 2.1, functional FISH, HEAD-US and MRI DENVER scores. Also, serum COMP level was assessed by ELISA technique for the PWH and 50 healthy subjects as control.

Results: The HJHS 2.1 score had a significant positive correlation with ABR (annualized bleeding rate), HEAD-US and the MRI DENVER scores, table (2). The FISH score had a significant negative correlation with the ABR, HJHS 2.1, HEAD-US and MRI DENVER scores, table (2). The serum COMP level was comparable between the PWH and the controls. The HEAD-US score had a significant positive correlation with the MRI score, table (2). The US score correlated with the MRI score in detecting synovial hypertrophy and intraarticular effusion, p< 0.001 for both. All of the joints’ scores for the PWH on Emicizumab prophylaxis showed significant lower HJHS 2.1 and MRI DENVER scores but higher FISH score than the joints scores of the patients receiving other types of prophylaxis, p-value < 0.001 for all.

Conclusion(s): The clinico-functional joints assessment scores (HJHS 2.1, FISH) and ABR were objective tools that correlated significantly with the HEAD-US and the Denver MRI scores. Emicizumab prophylaxis led to better joint status.