Clinical Challenge

Case 1

Joko, 14 years of age, is a competitive long-distance runner. He completed his last marathon race in under 2.5 hours 10 months ago. However, in the past few weeks, he has noticed excessive shortness of breath with even minimal training. Joko does not have asthma and has not had any trauma or signs of infection. He presents to your office with his mother Raya for advice. Assessment reveals a systolic murmur and leads you to consider hypertrophic cardiomyopathy (HCM). You order a number of investigations to further assess this possibility.

Question 1

Which one of the following is the least common clinical presentation of HCM in an adolescent?

1. Exertional dyspnoea
2. Atrial fibrillation
3. Cardiac failure
4. Supraventricular tachycardia

Question 2

Which one of the following is the least advisable investigation for a general practitioner to request for an adolescent with cardiovascular symptoms and a systolic cardiac murmur?

1. 12-lead electrocardiogram (ECG)
2. Resting echocardiogram
3. 24-hour Holter monitoring
4. Cardiac magnetic resonance imaging

Question 3

Raya is worried Joko may have a familial heart condition, which could affect her other children as well. Which one of the following is the most common inherited cardiac condition?

1. Hypertrophic cardiomyopathy
2. Ventricular septal defect
3. Atrial septal defect
4. Accessory conduction pathways

Question 4

Raya is worried about the risk of sudden cardiac death (SCD) in HCM. Which of the following is not a recognised risk factor for sudden death in HCM?

1. Family history of SCD
2. Atrial fibrillation
3. Unexplained syncope
4. Documented non-sustained ventricular tachycardia

Case continued

Joko begins ongoing care with a paediatric cardiologist. Management is predominantly centred around lifestyle change. In the meantime, Joko needs to have some dental work done. He has no allergies.

Question 5

The correct advice for dental prophylaxis is:

1. Not required
2. Single dose of amoxicillin at time of procedure
3. Long-term benzathene penicillin
4. Single dose of cephalexin at time of procedure

Question 6

Over time, Joko will be transitioning from paediatric to adult care. Which one of the following is not a key element for a successful transition process?

1. Coordination
2. Delaying referral to adult services until late as possible
3. Incorporating the young person’s views
4. Early introduction of consultation with adolescent without parent

Question 7

Raya is quite worried about Joko’s adherence to treatment plans. Which one of the following is least likely to affect Joko’s compliance?’

1. Forgetfulness
2. Opinions of peers
3. Fear of disease complications
4. Conflict with parents

Question 8

Which one of the following has the best evidence for improving treatment compliance in this situation?

1. Education
2. Behaviour modification
3. Psychological interventions
4. Parenteral discipline

Case 2

Georgina is a mother of four children. The oldest is Arthur who developed ulcerative colitis at the age of 22 years. Georgina presents today with her youngest child, Elissa, aged 13 years, who has been experiencing episodic abdominal pain and loose bowels. Georgina is very worried that Elissa may also have inflammatory bowel disease (IBD), possibly Crohn’s disease.

Question 9

Compared with adults, the severity of IBD in children is:

1. More
2. Less
3. Equal
4. Unpredictable

Question 10

Additional problems that children with IBD experience include all of the following except:

1. Growth failure
2. Early onset puberty
3. Poor bone density
4. Psychosocial impact at a vulnerable period of life.

Question 11

Which of the following is one of the most common presenting symptoms in paediatric Crohn’s disease?

1. Lethargy
2. Anorexia
3. Delay in linear growth
4. Diarrhoea

Case continued

Georgina recalls how complex it was to diagnose ulcerative colitis in Arthur, as his presentation was somewhat atypical.

Question 12

What percentage of children have normal biochemistry at diagnosis of IBD?

1. <5%
2. 10%
3. 20%
4. >20%

Question 13

If Ellisa does have Crohn’s disease, which one of the following is the usual choice for induction therapy?

1. Azathioprine
2. Sulphasalazine
3. Prednisolone
4. Methotrexate

Question 14

Which one of the following is the most accurate marker to monitor disease progression in Crohn’s disease?

1. Urine calprotectin
2. Faecal calprotectin
3. Serum calprotectin
4. Abdominal pain

Case 3

Jennifer, 12 years of age, has presented with her mother after experiencing worsening joint pains and a rash over the past three months. You suspect Jennifer may have a rheumatic disease. You decide to perform a HEADDS (home, education, activities, drugs, sexuality, suicide) assessment.

Question 15

Which one of the following is not part of the HEADDS assessment?

1. Cardiovascular examination
2. Sexuality
3. Education
4. Activities

Question 16

You have heard that the pGALS is a useful screening tool for the examination of young people with musculoskeletal complaints. Which one of the following is not part of the pGALS assessment?

1. Gait
2. Arms
3. Legs
4. Neurological