Part 1.

A 5-year-old Indian boy presented with fever, cough, rapid breathing, and pallor and left sided
abdominal pain since 4 days ago. He was also less active, only lying down at home and refusing any
physical activity. On examination, he was lethargic and pale. His temperature was 39.5° Celsius,
respiratory rate of 38 breaths per minute, pulse rate of 122 beats per minute with a blood pressure of
92/67mmHg. Lung examination revealed bilateral crepitations. On abdominal examination, the liver was
enlarged, measuring 3cm from the right costal margin and a tender and enlarged spleen measuring 7cm
from the left costal margin. A chest X-ray showed Interstitial infiltrates bilaterally.

The blood investigations are as follows:

Hemoglobin : 5.5g/dL
White blood cell (WBC) : 17 x 109/L
Neutrophil 69%
Lymphocytes 25%
Platelet : 70 x 109/L
Reticulocyte count : 14%
Lactate dehydrogenase : 440 IU/L
Mycoplasma Pneumoniae IgM titre : 1:640
Full blood picture : presence of sickle shaped red cells, no platelet
clumping.
Urine for hemoglobin : negative

Interpret the investigation results (4M)

Anaemia with reticulocytosis (0.5) with no hemoglobinuria, only slightly raised LDH – indicating non-
hemolytic type anaemia (1). There is also associated thrombocytopenia, FBP showing signs of sickle cell
disease(0.5), pointing towards splenic sequestration(1). Anti-mycoplasma IgM titre is elevated (0.5),
indicating a recent/ongoing mycoplasma infection(0.5).

Name ONE (1) investigation and its expected result to confirm the underlying diagnosis (1M)
Hemoglobin electrophoresis (0.5) – predominance of HbS / elevated HbS (0.5)

State the FULL diagnosis (3M)

Acute splenic sequestration (2) precipitated by Atypical Pneumonia / Mycoplasma pneumoniae
pneumonia (0.5) with underlying Sickle cell disease (0.5)

Name FOUR (4) complications that may arise from the underlying disease (4M)
Blindness
DVT / Pulmonary embolisme
Pain crisis
Acute chest syndrome
Ischemic stroke
Hand-foot syndrome (Dactylitis)
Priapism
Symptomatic anemia / failure to thrive / delayed puberty – (0.5max)

Part 2.

A 5-year-old Indian boy presented with fever, cough, rapid breathing, pallor and left sided abdominal
pain since 4 days ago. He also was less active, only lying down at home and refusing any physical activity.
On examination, he was lethargic and pale. His temperature was 39.5°Celsius, respiratory rate of 38
breaths per minute, pulse rate of 122 beats per minute with a blood pressure of 92/67mmHg. Lung
examination revealed bilateral crepitations. On abdominal examination, the liver was enlarged,
measuring 4cm from the right costal margin and a tender and enlarged spleen measuring 6cm from the
left costal margin. Peripheral examination also revealed swelling and redness of all fingers. A chest X-ray
showed Interstitial infiltrates bilaterally. He was diagnosed with acute splenic sequestration precipitated
by atypical pneumonia with underlying sickle cell disease. He was promptly treated and discharged after
6 days. The patient was subsequently seen on a 4 monthly basis at the clinic. At 8 years old, during
follow up, an abdominal examination showed hepatomegaly, measuring 5cm from the right costal
margin with no palpable spleen and resonant Traube’s space. A full blood picture showed the following:

Hb: 8.9g/dL
Total WBC: 11.3 x 109/L
Platelet: 852 x 109/L
Presence of Howell-Jolly bodies, numerous platelets, target cells, Heinz bodies

Interpret the investigation results (2M)

There is anemia due to underlying sickle cell disease. Presence of thrombocytosis (1M) and HJ bodies
denotes hyposplenia (1M).

State the diagnosis (1M)

Autosplenectomy (1) due to sickle cell disease

Outline the management at this point (10M)

Start anti-platelet therapy – Aspirin (1) and watch out for signs of veno-occlusive complications (1)

Antibiotic prophylaxis, (1) +( 1 of either Oral Pen or IM Pen mentioned)

Start Oral penicillin 250mg BD daily(1) or IM Benzathine Penicillin 25mg/kg monthly (1)

Appropriate immunization, (1) + (1Max if mention any of the following vaccine)

Pnemococcal (1) and Meningococcal (1) vaccination given, or HiB (1) vaccine if has not received before
Patient should receive yearly influenza vaccination(1)
Aggressive management of suspected infection (1) – Total 2 marks
Suspected infection must be urgently and promptly evaluated. Blood, urine and relevant body fluids sent
for culture and patient is initiated on appropriate intravenous broad spectrum antibiotics (1).

Parent education (1) – Total 2marks

Risks must be explained to all caretakers because they are an integral part of the management team.
Seeking medical advice at the first sign of illness is crucial (1)

SMEQ

Part 1

A 7-year-old Malay girl presented to the emergency department with progressively worsening shortness
of breath for 1 week. She had been having malaise, weight loss of approximately 5 kgs, reduced appetite
since 2 months ago, with a peculiar behavior of avoiding sun exposure. On examination, she was pale
and lethargic, with a respiratory rate of 34 breaths per minute, blood pressure of pulse rate of 115 beats
per minute, temperature of 37.3deg Celsius. On cardiovascular examination, the apex beat was situated
at the left 5th intercostal space lateral to the left mid-clavicular line, with muffled heart sounds.

1. State the most likely diagnosis (4M)

Systemic Lupus Erythematosus(2) with pericardial effusion(2)

2. List 4 Investigations to aid in your diagnosis and their expected findings (8M)
- Full blood count / Picture (1)– Showing normochromic normocytic anemia, thrombocytopenia,
leukopenia or lymphopenia (1)
- Anti-nuclear antibody (1) is positive with a raised titre (1)
- Positive Anti-Sm / Anti-phospholipid antibody (anti-cardiolipin/+ve lupus anticoagulant / false
positive fluorescent treponemal antibody absorption test (maximum 2)
- Anti-double stranded DNA (1) is positive with raised titre (1)
- C3 and C4 (1) – both low denoting hypocomplementemia (1)
- Coomb’s test – positive in the absence of hemolysis
- Urinalysis(1) showing proteinuria (1 or) with protein 3+, or cellular cast(1)
- 24 hour Urine for protein(1)  > 0.5 grams per day (1)
3. Echocardiogram(1) – showing pericardial effusion (1)

Part 2

Blood investigations was positive for Anti-nuclear and anti-double-stranded DNA antibodies with low C 3
and C4 levels. Echocardiogram revealed massive pericardial effusion with cardiac tamponade. A
diagnosis of systemic lupus erythematosus was made.

List 4 other physical findings that are related to her condition (8M)
Malar rash / Discoid rash (2M)
Alopecia / Oral ulcers (2M)
Neurology – Mononeuritis / peripheral neuropathy (2M if mention any)
Respiratory – Pleural effusion (2M)
Musculoskeletal – Tender, swollen joint (arthritis) (2M)

Outline the acute management for this patient (5M)

Provide supplemental oxygen therapy
Admit and monitor vital signs / IO in an intensive care unit setting
Consult with paediatric cardiologist regarding pericardiocentesis
Start with oral prednisolone 2mg/kg/day in divided doses
Consultation with a pediatric rheumatologist / immunologist