Step 2 Advice

Step 2 Qmax: Question of the Week

For this week’s quiz, we’ve pulled a pediatric question from our Step 2 Qmax library. Let’s see if you can determine the cardiac issue associated with this boy’s condition. And, as always, merit points go to those who can name that condition.

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9_20_18 - 21467.4

8 replies »

  1. C)DCM associated with DMD
    DUCHENNE Muscular Dystrophy (DMD)
    Clinical signs depicted here are
    -GOWER sign +
    -reduced mobility
    -pseudohypertrophy of calf
    -X linked recessive inheritance higher affection in males
    -(most common) cause of death —> cardiorespiratory problems

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    • This patient’s condition is not associated with hypertrophic cardiomyopathy. An example of hypertrophic cardiomyopathy in infants is Pompe disease, which can be associated with weak muscles in the arms and legs in the setting of facial hypotonia, macroglossia, and tongue weakness.

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  2. The correct answer is C. This patient is presenting with signs and symptoms suggestive of Duchenne muscular dystrophy (DMD). DMD is present at birth but typically does not begin to manifest until age 3-5 years. DMD classically affects the proximal musculature, so patients will have difficulty climbing stairs. This patient is displaying a positive Gower’s sign. Patients with proximal muscle weakness will roll to the prone position and use their arms to climb up their legs, as opposed to using truncal muscles to get into a sitting position and then thigh muscles to rise to standing. Patients with DMD develop hyperlordosis and scoliosis because of weakness of truncal muscles. The chest deformity, along with muscle weakness, impairs respiratory function, and patients often require tracheostomy. Calves are enlarged due to pseudohypertrophy as shown in the image. Creatine kinase is elevated, indicating a primary muscle disorder as opposed to a neurologic disorder.

    Death commonly occurs in the third decade due to respiratory failure or cardiomyopathy, typically dilated type. DMD is inherited in an X-linked recessive fashion. Clues in the family history include a maternal uncle who died of respiratory failure in his 20s and normal, unaffected females.

    To learn more, go to First Aid for the USMLE Step 2 CK, 9/e, and review pages 413-414 and 535.

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