Quadriceps strength, inter‐extremity difference (IED) and joint status in adult persons with severe haemophilia in different age stages

Abstract

Summary Quadriceps weakness seems to be a hallmark in adult persons with severe haemophilia (PWH). The purpose of this study was to compare PWH and non‐haemophilic controls in different age stages with reference to joint status and quadriceps strength. Further aims were to examine the extent of strength‐specific inter‐extremity‐difference (IED) and the prevalence of abnormal IED (AIED). A total of 106 adults with severe haemophilia (H) and 80 controls (C) had undergone an orthopaedic examination for classification of knee and ankle status using the WFH score. Quadriceps strength was evaluated unilaterally as well as bilaterally with a knee extensor device. Each group was divided into four age‐related subgroups (HA/CA: 18–29, HB/CB: 30–39, HC/CC: 40–49, HD/CD: 50–70; in years). H presented a worse knee and ankle status than C indicated by higher WFH scores (P < 0.01). Regarding the age‐matched subgroups only HB showed higher knee scores than CB (P < 0.05). The ankles were clinically more affected in HB‐HD compared with those in age‐matched controls (P < 0.05). H showed lower quadriceps strength than C (P < 0.05). In addition, all subgroups of H presented lower strength (HA: 10–17, HB: 19–23, HC: 35–36, HD: 53–61; in%, P < 0.05). IED was higher in H than in C [H: 12.0 (5.3/32.2) vs. C: 7.1 (2.9/10.9); Median (quartiles) in%, P < 0.001] and increased with age in H. We discovered an AIED in 35% of H. These findings highlight the importance for the early implementation of preventive and rehabilitative muscle training programmes in the comprehensive treatment of PWH.

The effect of resistance exercise on strength and safety outcome for people with haemophilia: A systematic review

“…Regarding strength performance, studies have shown reduced strength in certain muscle groups and patient groups: adults with haemophilia seem to have strength deficits in the upper and lower extremities as well as in the back muscles. This affects especially elderly adults, moderately to severely affected patients and those with a worse joint status . Studies on children and adolescents showed contradictory results which might be connected to the severity of the disease, the factor therapy, the joint affection and the method of examination.…”

Impact of joint status on contraction steadiness of m. quadriceps femoris in people with severe haemophilia

“…The percentage of IED in quadriceps strength was calculated as described earlier . In accordance with Sapega and Kannus , side‐to‐side imbalances of muscular strength greater than 20% were classified as AIED.…”

“…The weakness of the m. quadriceps femoris in particular is a frequently described problem in several studies . Besides this, also higher inter‐extremity differences (IED) of quadriceps strength were found in people with severe haemophilia (PWH) as well as a high prevalence for abnormal IED (AIED) . Another research revealed a linkage of such an AIED with significant alterations of the patellar tendon .…”

“…Repetitive bleeding induces a chronic joint destructive process called haemophilic arthropathy with chronic proliferative synovitis and cartilage destruction as the main components . Various studies have shown that adults with severe haemophilia exhibit considerably inferior capabilities in motor performance when compared with non‐haemophilic adults . The weakness of the m. quadriceps femoris in particular is a frequently described problem in several studies .…”

“…Various studies have shown that adults with severe haemophilia exhibit considerably inferior capabilities in motor performance when compared with non‐haemophilic adults . The weakness of the m. quadriceps femoris in particular is a frequently described problem in several studies . Besides this, also higher inter‐extremity differences (IED) of quadriceps strength were found in people with severe haemophilia (PWH) as well as a high prevalence for abnormal IED (AIED) .…”