SELF-ASSESSMENT
Self-assessment
Questions
Case 1
1. A 14-year-old Nepalese boy presented to his GP with
a 6-week history of painful swelling with reduced range
of movement of the left shoulder. There was no history
of injury. He was noted to be of short stature, below the
0.4th centile, and reported that he had been intermit-
tently pyrexial, with night sweats. There was no rash
and there had been no previous episodes.
What are the THREE most likely diagnoses?
(a) Juvenile Idiopathic Arthritis (JIA)
(b) Leukaemia
(c) Staphylococcal osteomyelitis/Septic Arthritis (OM/
SA)
(d) Reactive Arthritis
(e) Trauma
(f) Osteosarcoma
(g) Foreign body
(h) Tuberculosis (TB)
2. Which THREE investigations would you request first?
(a) Chest X-ray
(b) MRI of the shoulder
(c) Full blood count/ESR/CRP
(d) Tuberculin skin test (TST) and interferon gamma
release assay (IGRA)
(e) Rheumatoid factor
(f) Serum ferritin
(g) X-ray of shoulder
(h) Joint aspiration for culture and microscopy
3. Two weeks later he re-presented with a discharging
sinus on the anterior aspect of his left shoulder. IGRA
was indeterminate. Further questioning reveals he has
also had back pain for several years, which is worsening.
Priya Sukhtankar MBChB, BSc, MRCPCH is Clinical Research Fellow and
ST6 in Paediatrics, at the NIHR Wellcome Trust Clinical Research
Facility, University of Southampton, University Hospital Southampton
NHS Foundation Trust, UK. Conflict of interest: none.
Marc Tebruegge DTM&H, DLSHTM, MRCPCH, MSc, MD is Clinical Lecturer, at
the NIHR Wellcome Trust Clinical Research Facility, University of
Southampton, University Hospital Southampton NHS Foundation
Trust; and Clinical and Experimental Sciences, Faculty of Medicine,
University of Southampton, UK. Conflict of interest: none.
Saul N Faust MRCPCH, PhD, FHEA is Consultant in Paediatric Infectious
Diseases, at the NIHR Wellcome Trust Clinical Research Facility,
University of Southampton, University Hospital Southampton NHS
Foundation Trust; Clinical and Experimental Sciences, Faculty of
Medicine, University of Southampton, UK. Conflict of interest: none.
PAEDIATRICS AND CHILD HEALTH 22:5
He was born in Nepal and moved to the UK 2 years ago.
He has a BCG scar, and his TST shows induration of 20
mm.
What THREE assessments/investigations would you
chose to do next?
(a) Chest and spine X-rays
(b) Sinogram
(c) Bone scintigram
(d) Swab sinus and send for acid fast bacilli test
(e) Neurological review
(f) HIV test
(g) MRI of shoulder and spine
(h) CT chest
(i) Repeat tuberculin skin test
4. Results of investigations are shown below (normal
ranges in parentheses):
Bloods: FBC: Hb 12.3 g/dL (13.0e17.0 g/dL), WCC 12.7 
109/L (4.0e11.0  109/litre), Neutrophils 7.2  109/litre
(2.0e7.5  109/L), Platelets 422  109/litre (150e400 
109/L); CRP 43 mg/L (less than 3 mg/L), ESR 23 mm/h (1
e19 mm/h).
Chest X-Ray (Figure 1).
Culture of pus from the sinus grew no organisms.
Repeat interferon gamma release assay (IGRA) was
positive.
Which ONE investigation would do you do next?
(a) Request orthopaedic review
(b) Request CT guided bone biopsy
(c) Bone marrow aspirate
(d) Investigate for immunodeficiency
(e) Induced sputum culture
MRI scan showed multifocal spinal disease.
On spinal examination a marked kyphoscoliosis was
noted. Neurological examination was normal. He was
referred to the orthopaedic team for assessment and
further investigation, and further imaging was requested
(Figure 2):
Following this scan a biopsy was taken from T12/L1 verte-
brae and sent for histology. The results were not suggestive
of malignancy, and culture for mycobacterium tuberculosis
was negative as was staining for acid-fast bacilli. Treatment
was started empirically on the basis of positive TST and
IGRA. He was discharged on quadruple therapy for 2
months and 2-drug therapy for a further 10 months.
5. Which of the following FOUR drugs are used in standard
quadruple therapy in the UK?
(a) Amoxicillin
(b) Ethambutol
(c) Isoniazid
(d) Moxifloxacin
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 SELF-ASSESSMENT

Figure 1 Chest X-ray

(e) Pyrazinamide 6. Which ONE of the following is most important?

(f) Rifabutin (a) Refer to social services
(g) Rifampicin (b) Refer family for TB screening
(h) Streptomycin (c) Inform school nurse
(d) Refer to dietician
This patient continued to be followed up in clinic for
(e) Monitor isoniazid level
a further year. Public health was notified. He required on-
going physiotherapy and orthotic input for a spinal brace. Case 2
M. tuberculosis specific PCR on biopsy samples A 7-year-old boy presents with fever and otalgia, wors-
confirmed spinal TB. ening over 1 week despite treatment with clarithromycin.
His temperature continued to spike to 39  C and he
developed a cough. He received 1 week of intravenous
antibiotic therapy in hospital for right sided pneumonia,
but made little improvement and was transferred to
tertiary centre.
On arrival he looked unwell and temperature on admis-
sion was 40  C. His heart rate was 150/min, blood pres-
sure 82/60 mmHg, respiratory rate 44/min, oxygen
saturation 91% in air. He had dullness to percussion on
the right side and reduced air entry on auscultation.

1. As the admitting doctor, which THREE of the following

are most important in initial management?
(a) Obtain intravenous access and give 20 ml/kg 0.9%
saline
(b) Start dopamine infusion at 10 mcg/kg/min
(c) Give 20 mg/kg paracetamol
(d) Apply high flow oxygen via facemask
(e) Contact anaesthetic team for intubation
(f) Insert chest drain
(g) Full blood count
(h) 50 mg/kg IV cefuroxime
Figure 2 Further imaging: bone scan. (i) Capillary blood gas

PAEDIATRICS AND CHILD HEALTH 22:5 212 Ó 2012 Elsevier Ltd. All rights reserved.
 SELF-ASSESSMENT
Figure 3 Chest X-ray on arrival at tertiary hospital.
His chest X-ray (Figure 3) and blood tests are shown
below.
Full blood count: Hb 9.4 g/dL (13.0e17.0 g/dL), WCC
10.9  109/L (4.0e11.0  109/L), Neutrophils 6.4  109/
L (2.0e7.5  109/L, Lymphocytes 4.3  109/L (1.5e4.0 
109/L).
CRP 169 mg/L (<3 mg/L).
Renal profile: Na 136 mmol/L (135e145 mmol/L), K4.6
(3.5e5.0 mmol/L, Urea 6.7 mmol/L (2.9e7.1 mmol/L),
Creatinine 50 mmol/L (35e95 mmol/L).
Blood cultures are pending.
2. What is your diagnosis? Choose ONE of the following:
(a) Interstitial pneumonia
(b) Pneumonia with pleural effusion
(c) Tuberculosis
(d) Lymphoma
(e) Pulmonary abscess
PAEDIATRICS AND CHILD HEALTH 22:5
3. What further investigation is most appropriate? Chose
ONE of the following:
(a) Ultrasound scan of chest
(b) CT scan of chest
(c) Bronchoscopy
(d) Sputum culture
(e) Pleural aspirate
4. What further management may be appropriate?
(a) Ventilation on PICU
(b) Treatment with 50 mg/kg intravenous cefuroxime
(c) Treatment with oral co-amoxiclav and observation
(d) Insert percutaneous chest drain
(e) Video Assisted Thoracoscopy
Pleural effusion was confirmed but was too small for drain
insertion. He was treated with cefuroxime. He initially
improved and was discharged home on oral co-amoxiclav.
In spite of this he continued to spike high temperatures,
213 Ó 2012 Elsevier Ltd. All rights reserved.
 SELF-ASSESSMENT
and re-presented 1 week later. Repeat chest X-ray showed
worsening pneumonia and pleural effusion and he was
started on intravenous meropenem and vancomycin.
On further questioning he had a past medical history of
perianal abscess requiring surgical drainage aged 5 years,
but nothing else of note.
5. What investigations would you do next? Choose THREE:
(a) Pleural fluid culture and histology
(b) Repeat ultrasound scan of chest
(c) CT scan of chest
(d) Bronchoscopy
(e) Immunoglobulin levels, specific response to vacci-
nations and T-cell subsets
(f) Mantoux test
(g) Echocardiogram
(h) Spirometry
(i) Ultrasound scan of abdomen
He continued to spike high temperatures. Blood results
showed normal immunoglobulin levels, normal antibody
response to diphtheria and tetanus vaccinations, normal
T-cell subsets, but abnormal neutrophil oxidative burst by
flow cytometry.
6. What is your diagnosis? Choose one
(a) Leucocyte adhesion defect
(b) Chronic granulomatous disease
(c) Severe combined immunodeficiency
(d) Normal immune function, with acute phase reaction
(e) WiskotteAldrich Syndrome
Loculated pleural effusion was confirmed on repeat
imaging and was successfully drained. Two further weeks
of IV cefuroxime were completed and the child recovered
fully from his pneumonia. Follow up continues.
7. What follow up is appropriate at his next clinic
appointment? Choose three
(a) Refer for bone marrow transplant
(b) 13-valent pneumococcal vaccination
(c) Monthly MRSA swabs
(d) Prophylactic itraconazole and co-trimoxazole
(e) Repeat neutrophil oxidative burst flow cytometry
(f) Monthly immunoglobulin replacement
(g) Screening of parents and siblings for CGD carrier
status/disease
(h) Nebulised colomycin
(i) Repeat chest X-ray
Answers
Case 1
1. C, F, H
The duration of illness, bony pain and intermittent
pyrexia are consistent with a diagnosis of osteosarcoma,
which should be excluded. Non-tuberculous osteomye-
litis/septic arthritis (OA/SA), should be considered in
view of fever and joint pain and stiffness. This is most
commonly caused by Staphylococcus aureus in those
more than 6 years old. Tuberculosis as a cause of OA/SA
PAEDIATRICS AND CHILD HEALTH 22:5
should be considered given the history of nights sweats,
short stature and ethnicity. Leukaemia and JIA are also
important differentials. JIA is the most common cause of
monoarthritis in this age group.
2. C, D, E
Initial investigations should include basic blood tests with
inflammatory markers. X-ray of the shoulder is important
to exclude trauma, and is an inexpensive and quick
imaging test to look for bony abnormalities. Since this
child is from a high TB prevalence country, has short
stature and an history of night sweats it is also important
to exclude tuberculosis, so IGRA and tuberculin skin test
should be performed.
3. A, D, G
Indeterminate IGRA is not uncommon and does not
convey any information regarding TB infection status.
This should be repeated, as the history is highly suspi-
cious of TB. TST induration of greater than 15 mm in
a child with BCG vaccination is suggestive of TB
infection.
Chest and spine X-ray are indicated as chronic back pain
is a worrying feature suggestive of tuberculous osteo-
myelitis of the spine, which may result in vertebral
collapse and neurological compromise. This should be
followed up with MRI of the spine and shoulder.
As the sinus is now discharging culture and microscopy
of pus is indicated.
4. B
Bone scintigraphy shows a significant lesion in the
thoracic spine. So far pus swab and blood culture has
been sterile. Whilst the most likely cause is TB, other
infections and malignancy have not yet been excluded.
CT guided biopsy of the spinal lesion is indicated and
samples should be sent for TB culture and microscopy for
acid fast bacilli as well as histology. In this case the
diagnosis was made on PCR. Culture and microscopy
were negative.
5. B, C, E, G
Standard treatment of tuberculosis is with quadruple
therapy for 2 months with rifampicin, isoniazid, pyr-
azinamide and ethambutol, followed by a further
4 months of rifampicin and isoniazid. If exposure to
multi-drug resistant (MDR) TB is suspected this would
require modification. (See NICE guideline for tubercu-
losis. http://publications.nice.org.uk/tuberculosis-cg117)
Tuberculous osteomyelitis is uncommon, but should be
suspected in patients from countries where TB is
endemic, especially if there is a history of night sweats.
Many cases of osteoarticular tuberculosis have no history
of pulmonary TB. 50% of all osteoarticular TB affects the
spine, particularly in adolescents. These patients are at
risk of vertebral collapse and spinal cord compression
causing paraplegia so orthopaedic input and orthotic
support is essential.1,2
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 SELF-ASSESSMENT
Case 2
1. A, D, G
Despite antibiotic therapy at the referring hospital this
child has arrived hypotensive, tachypnoeic and tachy-
cardic. Facemask oxygen, fluid resuscitation and repeat
full blood count and inflammatory markers are appro-
priate. The patient should then be reassessed. It may also
be important to modify antibiotic therapy.
2. B
The CRP is 169 mg/dL although the WCC is normal. This
child is also anaemic with haemoglobin of 94 g/dL. The
chest X-ray shows right middle lobe pneumonia with
a pleural effusion.
3. A
Ultrasound scan will allow the depth and nature of the
effusion to be assessed without exposing the child to ionizing
radiation. This allows the team to assess whether the effusion
requires drainage. Bronchoscopy, pleural aspirate and
sputum culture are unlikely to be of value at this stage.
4. B
At this stage there has been no organism identified and
there is persistent pyrexia. It is appropriate to continue
high dose broad spectrum intravenous antibiotic therapy.
The child is otherwise clinically stable and does not
require PICU admission. A chest drain may be necessary,
but the effusion requires ultrasound assessment and is
not currently causing any lung collapse.
5. B, C, E
This is now the 3rd presentation with pyrexia and
shortness of breath despite adequate antibiotic therapy. It
is likely that the effusion has re-accumulated, or extended
and ultrasound scan should be repeated to assess this. CT
scan of the chest is also of benefit to assess the effusion
and pneumonia.
PAEDIATRICS AND CHILD HEALTH 22:5
In view of the unusual course of pneumonia, and previous
admission with perianal abscess, immune deficiency
should be considered. This child had immune response to
vaccinations tested, as well as lymphocyte subsets, and
neutrophil burst tested by flow cytometry. Immunoglob-
ulin and complement levels were also checked.
6. B
The neutrophil burst test is expressed as a percentage and
this child had low percentage initially, followed by absent
neutrophil burst on repeat. This gives a diagnosis of
chronic granulomatous disease, which is a deficiency in
one of the five subunits of the phagocyte NADPH oxidase
molecule which generates reactive oxidase species in
response to infection.
7. B, D, G
CGD is a disorder of the NADPH oxidase system causing
phagocyte dysfunction. Children with CGD are at risk of
catalase-positive bacterial and fungal infection, and
abscesses. The 13-valent pneumococcal vaccination
greatly reduces the risk of pneumococcal infection.
Prophylactic co-trimoxazole provides additional protec-
tion against catalase-positive bacteria, and fluconazole
prevents aspergillus and yeast infections.
CGD is X-linked and the family should be screened for
carrier or disease status.3
REFERENCES
1 De Vuyst D, Vanhoenacker F, Gielen J, Bernaerts A, De Schepper AM.
Imaging features of musculoskeletal tuberculosis. Eur Radiol
2003; 13: 1809e19.
2 Teo HE, Peh WC. Skeletal tuberculosis in children. Pediatr
Radiol 2004; 34: 853e60.
3 Heyworth PG, Cross AR, Curnutte JT. Chronic granulomatous
disease. Curr opinion Immunology 2003; 15: 578e84.
215 Ó 2012 Elsevier Ltd. All rights reserved.