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Abstract: Objective　To learn about the characteristics of early joint lesions in children with hemophilia at different developmental stages after clotting factor replacement therapy. Methods　A total of 39 children with hemophilia at the age of 3 to 18 years who had received clotting factor replacement therapy for more than a year in Guangzhou Women and Children’s Medical Center from May 2020 to September 2022 were analyzed retrospectively. According to the activity level of coagulation factors，the severity of hemophilia was divided into mild（n=10），moderate（n=13）and severe（n=16）. All patients were divided into three groups according to age：infant group（3-6 years old，n=17），child group（7-12 years old，n=16），juvenile group（13-18 years old，n=6）. Ultrasound and hemophilia joint health score 2.1（HJHS 2.1）were applied to detect the health status of elbow，knee and ankle which had no joint bleeding in the one year before the experiment. Ultrasonic detection was used to obtain the maximum values of joint effusion and synovial thickness， and at the same time，investigate whether there was blood flow signal and cartilage loss in the joint or not. The positive rates（score≥1）of swelling，duration of swelling，muscle atrophy，crepitus on motion，flexion loss，extension loss，joint pain and muscle strength were obtained by HJHS 2.1. Chi-square test and rank-sum test were used to analyze the joint health of hemophilia children with different severity and age. Results　Knee had the highest positive rate in crepitus on motion（8% for elbow，59.74% for knee and 3.89% for ankle）in joints with different severity. The thickening of synovial membrane was most obvious in ankle ［elbow：1.50 （1.20，1.80）mm；knee：0 （0，1.20）mm；ankle：2.40 （1.80，3.05）mm］，and the difference was statistically significant（P<0.05）. Juvenile group had the most obvious synovial thickening in elbow，knee and ankle，elbow effusion，and crepitus on motion in knee，the differences being statistically significant（all P<0.05）. Ankle had the highest proportion of blood flow signal（12.99%）. The indicators of swelling，duration of swelling，muscle atrophy，extension loss and muscle strength were moderately or weakly correlated. Conclusion　The joints of children with hemophilia after clotting factor replacement therapy who have no joint bleeding have some impair manifestations in the joints such as crepitus on motion and synovial thickening ，with the joints in the lower extremities being the most obvious. Some joints may have small amount of bleeding. The joint lesions become more severe with age.

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