ABSTRACT
Purpose: Hemophilia is a hereditary coagulation disorder characterized by acute hemorrhages into the musculoskeletal system, leading eventually to arthropathy and disability. Chronic inflammation of the synovial membrane arises as a result of frequent joint hemorrhage. Proteolytic enzymes in the blood and cartilage cause deterioration after that, and joint space narrows. Chronic hemophilic arthropathy develops as a result of these unfavorable developments, which occur more quickly, especially in the target joints. Balance is a process that allows us to maintain our orientation in three-dimensional space while also regulating our body posture to avoid falling. After the central nervous system evaluates deep stimuli from sensory, visual, and auditory receptors, movement of the corresponding muscle groups is delivered. Methods: The goal of this study was to investigate how impairment to deep sensory receptors (proprioception) in the arthropathic joint structure affected hemophiliacs' balance. The study comprised 34 patients with hemophilic arthropathy, and 34 age and weight matched healthy volunteers. Results: When balance tests of patients with hemophilic arthropathy were compared to healthy controls, hemophiliacs had a greater risk of falling. As the degree of arthropathy increased, so did the risk of falling and balance test values in individuals with hemophilic arthropathy. Conclusions: Treatment and coagulation factor prophylaxis to prevent the onset of arthropathy will improve patients' quality of life and reduce morbidity associated with frequent falls and bleeding. Supplementary Information: The online version contains supplementary material available at 10.1007/s12288-022-01526-0.
ABSTRACT
We hypothesized that the individual hematological response to chemo/ radiotherapy may be used as a parameter to assess the degree of myeloablation and probability of transplant-related events. This study included 58 pediatric patients who underwent allogeneic hematopoietic stem cell transplantation (HSCT). White blood cell (WBC) ratio (pre-conditioning WBC: day 0 WBC), day 0 WBC count, and WBC nadir day were used as potential indicators of myeloablation. The association between WBC kinetics and clinical result of HSCT was investigated. There was a positive correlation between WBC ratio and the date of engraftment. A positive correlation was noted between day 0 WBC count and engraftment day. There was a negative correlation between WBC nadir day and engraftment day. WBC nadir day was lower in patients with acute graft-versus-host disease (GVHD) than in cases without acute GVHD. Among patients who had fever during the conditioning regimen, the WBC ratio was higher, day 0 WBC count was lower, and WBC nadir day was lower in patients who developed >5 days of fever between day 0 and day +30. The present preliminary study suggests that WBC kinetics may be used as a measure of initial hematological response to the conditioning regimen and perhaps in determining the degree of myeloablation.
