Increased Erythrocytic Osmotic Fragility in Pseudohypertrophic Muscular Dystrophy
- Edwin R. Fisher, MD;
- Emmanuel Silvestri;
- John W. Vester, MD;
- Sean Nolan, MD;
- Usman Ahmad, MD;
- Thaddeus S. Danowski, MD
Since this article does not have an abstract, we have provided the first 150 words of the full text.
Excerpt
THE DEMONSTRATION by scanning electron microscopy1 that erythrocytes in patients with pseudohypertrophic muscular dystrophy (PHMD) have an irregular, crenated-like surface prompted us to investigate the fragility of these erythrocytes, since crenation represents an early stage in the development of osmotic fragility.2 Our studies indicate that the susceptibility of erythrocytes to hemolysis in hypotonic saline is increased in 85% of patients with PHMD.
Materials and Methods Venous blood (0.1 ml) from 94 patients with PHMD and 19 healthy, aging, and sex-matched controls was added to hypotonic saline for the detection of osmotic fragility of erythrocytes. The concentrations of saline are indicated in the Table. The percent of hemolysis of erythrocytes was determined by spectrophotometry.
The diagnosis of PHMD was based in each instance on muscle weakness on recording ergometry, increased calf size, and increased serum creatine phosphokinase levels. In addition, electromyography (41 patients), motor nerve conduction velocity (40 patients),
Footnotes
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Reprint requests to Director of Laboratories, Shadyside Hospital, 5230 Centre Ave, Pittsburgh, PA 15232 (Dr Fisher).
