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Duchenne muscular dystrophy (DMD) is a severe recessive x-linked form of muscular dystrophy that is characterized by rapid progession of muscle degeneration, eventually leading to loss in ambulation, paralysis, and death. This affliction affects one in 3500 males, making it the most prevalent of muscular dystrophies. In general, males are only afflicted, though females can be carriers. The disorder is caused by a mutation in the gene DMD, located in humans on the X chromosome. The DMD gene codes for the protein dystrophin, an important structural component within muscle tissue. Dystrophin provides structural stability to the dystroglycan complex (DGC), located on the cell membrane.
Treatment
# Intravenous administration of glucose and insulin, which promotes movement of potassium from the extracellular space back into the cells.
# Intravenous calcium to temporarily protect the heart and muscles from the effects of hyperkalemia.
# Sodium bicarbonate administration to counteract acidosis and to promote movement of potassium from the extracellular space back into the cells.
# Diuretic administration to decrease the total potassium stores through increasing potassium excretion in the urine. It is important to note that most diuretics increase kidney excretion of potassium. Only the potassium-sparing diuretics mentioned above decrease kidney excretion of potassium.
# Medications that stimulate beta-2 adrenergic receptors, such as albuterol and epinephrine, have also been used to drive potassium back into cells.
# Medications known as cation-exchange resins, which bind potassium and lead to its excretion via the gastrointestinal tract.
# Dialysis, particularly if other measures have failed