Key result
Advanced Duchenne muscular dystrophy is linked to ~23% lower muscle blood flow versus controls.
Why the study?
Muscle blood flow and capillary diffusion capacity in Duchenne muscular dystrophy patients compared to controls were not well characterized to understand ischemic contributions.
Does muscle blood flow, capillary diffusion capacity, and capillary morphology differ between patients with Duchenne muscular dystrophy and age-matched controls?
Case-Control (n=13)
Does muscle blood flow, capillary diffusion capacity, and capillary morphology differ between patients with Duchenne muscular dystrophy and age-matched controls?
The study demonstrates altered capillary morphology and reduced muscle blood flow in advanced Duchenne muscular dystrophy, but the findings do not support a primary ischemic etiology for the disease.
Muscle blood flow (MBF) and capillary diffusion capacity (CDC) were determined in 8 patients with Duchenne muscular dystrophy and 5 age-matched control males by measuring the simultaneous clearance of 133Xe and 131I-. MBF was significantly decreased in older patients with severe dystrophy: 35.1 +/- 5.8 ml/100 g/min in 4 advanced Duchenne patients and 45.8 +/- 6.5 ml/100 g/min in controls (p less than 0.005). MBF in 4 early cases did not differ from controls (44.0 +/- 7.9 ml/100 g/min). There was no significant difference in CDC in Duchenne patients and controls. No structural abnormalities in muscle microvasculature were found by light microscopy. In the electron microscope, the Duchenne basement membranes had duplicated or multiple layers of electron-dense material in 63.9% of muscle capillaries while only 6.6% of the control capillaries showed this. These results do not support an ischemic etiology of Duchenne muscular dystrophy.
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Leinonen et al. (2008) conducted a case-control in Duchenne muscular dystrophy (n=13). Duchenne muscular dystrophy vs. Age-matched control males was evaluated on Muscle blood flow (MBF) (p=<0.005). Advanced Duchenne muscular dystrophy was associated with significantly decreased muscle blood flow compared to controls (35.1 vs 45.8 ml/100 g/min; p<0.005), not supporting an ischemic etiology.
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