Duchenne muscular dystrophy treatment steroids

Duchenne was convinced that the "truth" of his pathognomic experiments could only be effectively rendered by photography, the subject's expressions being too fleeting to be drawn or painted. "Only photography," he writes, "as truthful as a mirror, could attain such desirable perfection." [14] He worked with a talented, young photographer, Adrien Tournachon , (the brother of Felix Nadar ), and also taught himself the art in order to document his experiments. [15] From an art-historical point of view, the Mechanism of Human Physiognomy was the first publication on the expression of human emotions to be illustrated with actual photographs. Photography had only recently been invented, and there was a widespread belief that this was a medium that could capture the "truth" of any situation in a way that other mediums were unable to do.

Patients with Becker muscular dystrophy have an abnormality in dystrophin, but unlike patients with Duchenne muscular dystrophy, some dystrophin is present.

Muscular dystrophies, of which Duchenne and Becker are two types, are characterized by progressive skeletal muscle weakness, defects in muscle proteins, and the death of muscle cells and tissue. Because Becker muscular dystrophy has dystrophin (albeit abnormal), the progression of muscle weakness is slower, with the diagnosis typically made after age 8 years. Similar to patients with Duchenne muscular dystrophy, patients with Becker muscular dystrophy have pseudohypertrophy of the calves, markedly increased creatine kinase levels, and X-linked transmission of the condition.

Incorrect Answers:
Answer 2:This answer choice describes Spinal muscular atrophy. These patients have proximal muscle weakness, but the onset of weakness occurs earlier in childhood. These patients also have absent deep tendon reflexes and fasciculations, but pseudohypertrophy is absent and creatine kinase levels are normal.
Answer 3: This answer choice describes Emery-Dreifuss dystrophy. Patients with Emery-Dreifuss dystrophy may have a similar clinical picture to Becker’s muscular dystrophy, but pseudohypertrophy is absent and creatine kinase levels are only mildly elevated. In addition, neck extension, elbow flexion, and ankle equinus contractures develop at an early age.
Answer 4: This answer choice describes Guillain-Barre syndrome. This is a condition associated with results from postinfectious demyelination of the peripheral nerves. These patients have the acute onset of weakness, hypotonia, and areflexia; creatine kinase levels are normal.
Answer 5: This answer choice describes Duchenne muscular condition is primarily differentiated from Becker's muscular dystrophy by the complete absence of dystrophin, and a more severe disease course.

Mercier et al. (2013) reviewed the features of 26 female carriers of pathogenic mutations in the DMD gene who were referred for symptoms related to the disorder before 17 years of age. Five had a Duchenne-like phenotype with loss of ambulation before age 15 years, 13 had a Becker-like phenotype with muscle weakness but persistence of ambulation after age 15 years, and 8 had exercise intolerance. Initial symptoms included significant muscle weakness (88%), mostly affecting the lower limbs, or exercise intolerance (27%). Cardiac dysfunction was present in 19%, and cognitive impairment in 27%. Cognitive impairment was associated with mutations in the distal part of the gene. Muscle biopsy showed dystrophic changes in 83% and mosaic immunostaining for dystrophin in 81%. The X-chromosome inactivation pattern was biased in 62% of cases. Mercier et al. (2013) concluded that carrier females may have significant symptoms of the disorder.

Duchenne muscular dystrophy treatment steroids

duchenne muscular dystrophy treatment steroids

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