Ullrich congenital muscular dystrophy (UCMD) is characterized by congenital weakness, proximal joint contractures, and hyperlaxity of distal joints. UCMD is basically due to a defect in extra cellular matrix protein, collagen type VI. A 37-year-old woman who cannot walk independently visited our outpatient clinic. She had orthopedic deformities (scoliosis, joint contractures, and distal joint hyperlaxity), difficulty of respiration, and many skin keloids. Her hip computed tomography showed diffuse fatty infiltration and the 'central shadow' sign in thigh muscles. From the clinical information suggesting collagen type VI related muscle disorder, UCMD was highly considered.
Citations
To examine the effect of the posture of immobilization upon the tensile properties in injured Achilles tendon of rat for an initial period of immobilization.
Forty-two Sprague-Dawley rats were used in the present study. Eighteen rats received a total tenotomy of the right Achilles tendon to mimic total rupture and were divided into three groups comprising of 6 rats each. Ankles of group A were immobilized at 60° of plantarflexion. Ankles of group B were immobilized at neutral position. Whereas, those of group C were immobilized at 60° of dorsiflexion. Other 18 rats received hemitenotomy to mimic partial rupture and were divided into three groups. The remaining 6 rats were kept free as control. After 14 days, we dissected the tendons and analyzed maximum force, stiffness, and energy uptake during pulling of the tendons until they ruptured. The tendons of 6 rats in each group and control were reserved for histology. Picrosirius staining was done for the analysis of collagen organization.
In total tenotomy, tensile properties were significantly different between the control and the intervention groups (p<0.05). Group C showed relatively higher values than the groups A and B with respect to tensile properties (p>0.05). In partial tenotomy, tensile properties were significantly different between the control and the intervention groups (p<0.05). Group C showed significantly higher value than other intervention groups in terms of maximum force and energy uptake (p<0.05). The semiquantitative histologic grading scores were assigned for collagen organization. The scores for dorsiflexion posture were higher than the ones for plantarflexion.
Dorsiflexion posture in partial ruptured Achilles tendon showed better functional recovery than other immobilized postures. In total ruptured case, the tensile properties showed increasing tendency in dorsiflexion posture.
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To investigate whether the cartilage regenerative effects of intra-aricular platelet-rich plasma (PRP) are different, according to the severity of osteoarthritis (OA), in a collagenase-induced knee OA rabbit model.
New Zealand white rabbits (N=21) were randomly divided into three groups. Three different doses (0.25 mg, group 1; 0.5 mg, group 2; and 1.0 mg, group 3) of collagenase were injected twice into both knees of each group under an ultrasound guidance. The mean platelet concentration of the PRP fraction was 2,664±970×103/µl and was enriched 8.2-times, compared with the whole blood. PRP (0.3 ml) was injected into the left knee and saline (0.3 ml) into the right knee at 4 weeks, and macroscopic and histological scores of both injected knees were evaluated at 9 weeks after the first collagenase injection.
Macroscopic and histological scores of group 3 were significantly higher than those of group 1 and 2 (p<0.05). Macroscopic and histological scores of the PRP-injected knees were significantly lower than those of the saline-injected knees, in all groups (p<0.05). Differences of gross morphologic and histologic scores between saline- and PRP-injected knees in group 3 were significantly higher than those in group 1 and 2 (p<0.05).
Intra-articular PRP injection influences cartilage regeneration in all severities of rabbit knee OA, and the cartilage regenerative power of PRP injection in moderate knee OA was greater than that in mild or very mild OA. A large preclinical trial is needed to establish the validity of our study.
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Objective: Ultrasound has been therapeutically applied for pain control in rheumatoid arthritis although little physiologic effects of sonication on rheumatoid tissue were known. This investigation was conducted to determine the effects of sonication on the cell proliferation and matrix metalloproteinase (MMP) production of cultured fibroblast like synoviocytes (FLS) derived from synovial tissues of rheumatoid arthritis.
Method: Pulsed ultrasound (1.0 MHZ, 20 msec on, 80 msec off) with varying intensities (0, 0.1, 0.25, 0.5, 0.75, 1.0 W/cm2) was applied to experimental cell groups growing as monolayers in culture plates for varying durations (0, 30, 90, 180 seconds) in the presence and absence of interleukin-1β (IL-1β).
Results: There were no significant differences in thymidine incorporation between 0, 30, 90 and 180 second sonication groups with 0.5 W/cm2 after 1 day and 2 days. There were no significant differences in thymidine incorporation between 0, 0.1, 0.25, 0.5, 0.75, 1.0 W/cm2 sonication groups 1 day and 2 days after 90 second sonication. There were significant increase in MMP-1 (p=0.025) and MMP-3 production (p=0.000) of FLS after sonication in the absence of IL-1β but there were no significant differences in MMP-1 and MMP-3 production in the presence of IL-1β. And MMP-1 and MMP-3 production were increased significantly in the presence of IL-1β but not than in the absence of IL-1β.
Conclusion: While comparisons made between a limited number of FLS cell lines must be open to question, the overall consistency of the findings suggest sonication with nonthermal effect is not the contraindication in rheumatoid arthritis treatment but further study is needed in vivo in animal and in clinical studies.
Objective: To assess the significance of anti-type I collagen antibody titer in estimating cumulative trauma and predicting the presence of occupational low back pain.
Method: Under the hypothesis that cumulative trauma on the spine will expose collagen and stimulate the formation of auto-antibody, we measured the serum anti-type I collagen antibody titers (IgM and IgG) in 408 male workers of a metal welding and manufacturing company. The antibody titers were measured in duplicates by ELISA. Statistical analysis was done to compare the titers according to occupational profiles (type of occupation and duration of employment) and clinical profiles (occurrence of low back pain, duration of low back pain and clinical impression).
Results: The anti-type I collagen IgG antibody titers were significantly increased in labor workers (n=357) in comparison with office workers (n=51)(p<0.05). Among the labor workers both IgM and IgG antibody titers were increased in the low back pain group (n=50) though it did not reach statistical significance (p-value of IgM antibody titers=0.07). IgM and IgG antibody titers were increased in the chronic low back pain group (≥3 months)(n=8).
Conclusion: These data suggest that anti-type I collagen IgM and IgG antibody may be useful in predicting the presence of occupational low back pain and estimating cumulative trauma, respectively.