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f dar I qbal

a
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Dr

MRCPCH Par t II

Pract ice
i ce Ex ams
Photographic Material
Data Interpretation
Grey Cases

Paediatric Exam Series

DATA
1.These are cardiac catheterisation data from a 4 month old boy :
Pressure
SVC
Right atrium

Saturation
60

13

63

76 / 14

87

17

93

LV

80 / 20

92

PA

74 / 36

88

Aorta

80 / 50

93

RV
Left atrium

What is the diagnosis ?

2.This is the result from a 3 week old baby :


TSH 35 Free T3 2 pmol/L Free T4 3 pmol/L
On further questioning , Mum says she was found to have a similar problem at birth and
is on treatment .
What are the possible causes for this result ?

3.These are the recordings from a difficult diabetics BM diary . He is currently on 2.5
U/kg/day of Insulin , 1.5 U/kg am and 1 U/kg pm , using a Mixtard 30/70 regime :
DATE

AM

PM

20.05.98

15.8

5.6

22.05.98

16.8

7.6

25.05.98

20.6

4.5

29.05.98

15.6

5.6

01.06.98

13.7

3.2

02.06.98

18.7

3.4

What further points in history will be needed to make the next decision regarding dosage
adjustment ?Name two.

4.This is the electrophoretic pattern of haemoglobin from a 10 year old boy with anaemia
:
Hb A1 - 85 % HbA2 - 7 % HbF - 8 %
What is the likely diagnosis ?

5.This is the arterial blood gas result from a 14 year old girl who presented with a history
of having an overdose of some undisclosed tablets :
pH 7.56 pCO2 2.7 pO2 15.4 HCO3 17.7
What is the diagnosis ?

6.A 4 day old ventilated neonate gained 100 gms in 24 hrs . His blood results showed
Na 128 K 4.5 Urea 4.5 Creatinine 87
What two further tests are needed to establish a diagnosis ?

7.These are the results from the CSF of a 4 year old boy with neck stiffness and fever :
Clear CSF , normal pressure , Microscopy - 200 WBC , 80 % mononuclear cells .
CSF protein 0.80 , sugar 3.4 , blood sugar 6.7 .
What is the likely diagnosis ?

8.These are the results from a 11 month old boy :


Calcium 2.3 mmol/L Phosphate 0.5 mmol/L ALP 2360 PTH 0. 6 (0.1 - 4.5) Urinary
phosphate elevated glucose - ve aminoacids - ve
What is the diagnosis ?

9.What is the diagnosis from this clinical situation ?


A 4 year old Nigerian boy went back to Africa for the first time since he was born, and
his mother ensured that he had taken all his vaccines and necessary medications on his
visit .
Blood results from Nigeria when he presented to a hospital for a specific complaint
showed :
Hb 5.4 WCC 6.7 Plt 364 Retics 12 %
What is the likely problem ?

10.A 10 month old boy had been vomiting for 24 hours and crying with what seemed like
abdominal pain . Now he was drowsy and 10 % dehydrated .
What immediate investigation is indicated ?

ANSWERS
1.This picture is suggestive of a VSD with a large L-R shunt and
pulmonary hypertension .
2.This result indicates hypothyroidism , with a high TSH and a low T3 and
T4 . The fact that Mum is also hypothyroid since birth should trigger the
thought of a dyshormonogenesis problem - enzymatic defects with thyroid
synthesis are rare , Pendred syndrome is a disorder with sensorineural
deafness and goitre .
3.The fact that this boy is on 2.5 U/kg/day of insulin , the morning
hyperglycaemia and the brittle state of his diabetes can be explained by
either the Somogyi phenomenon or Dawn phenomenon , provided other
simple causes like noncompliance at teatime are excluded. Somogyi
phenomenon is the rebound hyperglycaemia resulting from a hypo at
around 3 or 4 am . This is said to be the result of a massive surge in
counter-regulatory hormones . BMs done at these times can be helpful in
charting the course of the hyperglycaemia . History of early morning
waking up with headaches , sweating etc. can be elicited in many such
patients .
4.This is suggestive of a heterozygous Thalassaemia trait . In thalassaemia
major , there is virtually no HbA1 , and > 90 % is Hb F . In the trait , there
is a moderate rise in HbA2 and HbF , as is seen here .
5.Salicylate poisoning , with a respiratory alkalosis .
6.Serum and Urine osmolality is neede to prove the possible diagnosis of
SIADH in this child . The serum osmo will be low and the urine osmo
high in such cases .
7.Viral meningitis .
8.This picture is that of an Xlinked hypophosphataemic rickets .
9.It is quite likely this boy has G6PD deficiency and the antimalarials that
his mother has given him has triggered off an acute haemolysis .
10.Urinary glucose and ketones . The diagnosis of a DKA in this young
age is difficult and needs a high index of suspicion .

f dar I qbal
a
S
.
s
Dr

MRCPCH Par t II

Pract ice
i ce Ex ams
Photographic Material
Data Interpretation
Grey Cases

Paediatric Exam Series

DATA
1. These are the CSF findings from a 6 year old who presented with inability to walk and then
ended up on a ventilator:
Clear fluid, Protein 2 g/L, glucose 4.5, blood sugar 5.4, microscopy 4 cells, lymphocytes 3, PMN
cells 1, culture negative.
What is the diagnosis?

2. These are cardiac catheterisation data from a 6 year old girl. What is the diagnosis?
Pressure
SVC
Right atrium

Saturation
75

76

65/3

76

95

LV

80 / 20

94

PA

25/10

74

Aorta

80 / 50

94

RV
Left atrium

3. These are the results from a 10 year old boy with meningococcaemia
Hb 10.4, WCC 23.4 neutrophils 20.4, Platelets 80. INR 2.5, APTT ratio 3.3, Fibrinogen 2.3, FDP
20,000
1. What is the diagnosis?
2. What treatment is indicated at this stage?

4. This 2 year old boy(bottom left corner) was found to have bowed legs and widened wrists at
the age of 8 months.

What is the diagnosis?

5. A 2 year old is seen in Clinic during a review for his congenital hypothyroidism. He is on 50
mcg of Thyroxine od. Last TSH 0.80, T4 578 nmol/L
What changes are needed to his treatment?

6. This is the growth chart of a 4 year old boy when he was an infant.

Currently, his height is just below the 0.4th centile. On examination he has some dysmorphic
features and the right side of his body is slightly larger compared to his left. What is the
diagnosis?

7. A 15 year old Nigerian boy with sickle cell disease has a water deprivation test performed:
Time
0 hr.
8 hr.
12 hr.
Drug A
13 hr.
16 hr.

Weight
50 kg
49 kg
48.4 kg
is given
48.2 kg
48 kg

Urine osmolality
100
200
250

Serum osmolality
290
315
320

240
250

325
331

1. What is Drug A?
2. What is the diagnosis?

8. These are immunoglobulin levels from a 9 month old baby boy:


IgG 0.5 g/L, IgM not detectable, IgA not detectable. What is the form of inheritance of this
disease?

9. Interpret this arterial blood gas result:


pH 7.34, pCO2 10.3, pO2 11.2, HCO3 32.3.
What abnormality does it show? What are the causes for this picture?

10. A 5 year old girl was being followed up for anaemia of autoimmune origin, and has had
multiple blood transfusions. Recently, she also started having bruising over her shins and
shoulders. Her blood picture is as follows:
Hb 8.5, WCC 12.4 neutr 8.7, Platelets 23. Reticulocytes 6%. Bilirubin 86 mol/L, conjugated 4.
Other LFTs normal.
What is the possible diagnosis?

ANSWERS
1. Guillain-Barre syndrome.
2. Pulmonary stenosis.
3. DIC. FFP is needed.
4. X linked hypophosphataemic rickets.
5. He seems to be on too much thyroxine. Reduce the dose!
6. Russell Silver syndrome.
7. Drug A is DDAVP. There is no response to the drug. This is Nephrogenic Diabetes insipidus.
8. This is X linked agammaglobulinaemia.
9. This is compensated respiratory acidosis. This may be a exprem baby on oxygen with CLD or
a baby with obstructive sleep apnoea.
10. Evan's syndrome - autoimmune anaemia and thrombocytopaenia.

f dar I qbal
a
S
.
s
Dr

MRCPCH Par t II

Pract ice
i ce Ex ams
Photographic Material
Data Interpretation
Grey Cases

Paediatric Exam Series

DATA
1. A 12 hour old neonate is on 150 ml/kg/24 hr of 15% dextrose.
Results include blood glucose level of 2.1 mmol/L, Na 135, K 4.3, Urea 3.5, Creatinine 67
micromol/L, Insulin 45 mU/L (normal 3-20).
What is the most likely diagnosis?
2. These are the results from a 15 month old with a 2 week history of being miserable,
clingy and excessively hungry:
pH 7.02, pCO2 2.45, pO2 12.85, HCO3 7.8, Base deficit 25 mmol/L.
What is the most likely diagnosis?
3. Results from a 5 year old boy on chemotherapy for ALL who presented with acute
respiratory distress:
Hb 10.6, WCC 0.6 neutrophils 0.1, Platelets 128. Na 127, K 4.3, Urea 13.6, Creatinine 150. Ca
2.45, Phosphate 1.1.
Total protein 54, Alb 36, Bilirubin 26, AST 387, ALP 473, gamma GT 176.
Chest X ray: bilateral patchy infiltrates.
What is the most likely organism causing the above changes?
4. A 6 week old ventilated preterm infant on prolonged TPN for NEC has the following
results:
Hb 12.4, WCC 13.6 neutrophils 4.5, Platelets 146.
Na 134, K 4.6, Urea 7.6, Creat 87, Total protein 40 g/L, albumin 26, Total bilirubin 186 mol/L
conjugated fraction 78, AST 267, ALP 1463, GGT 357.
Ca 2.3, PO4 0.9
What could explain these changes?
5. These are arterial blood gas results from a 25 week gestation neonate on HFOV
(ventilator set at 10 Hz, MAP 14, amplitude 36, FIO2 0.50).
pH 7.34, pO2 4.7, pCO2 5.8, HCO3 20.4
What is the next appropriate step in the ventilatory management?

6. What is this investigation? Diagnosis.

7. The hepatitis serology of a mother on the postnatal ward is:


HepBsAg positive, HepBeAg negative, HepBeAb positive.
What is the immunisation policy for this neonate against hepB?
8. Results from a previously well 10 year old boy who came in to Casualty collapsed:
Serum Na 117 mmol/L, K 6.5, Urea 11.5, Creatinine 67, Ca 2.99, PO4 1.8.
What is the likely diagnosis?
9. A 6 week old neonate on CPAP has had an acute deterioration with worsening blood
gases:
pH 7.21, pCO2 10.7, pO2 8.9, HCO3 28.3
Nasopharyngeal aspirate: Ureaplasma urealyticum
What is the treatment?
10. A 10 year old boy presents with facial swelling and oliguria. Results:
Hb 12.3 g/dL, WCC 13.5, Platelets 265
Na 135, K 4.6, Urea 13.5, Creat 46. Urine dipstik protein 4+, blood 3+, nitrites negative,
leucocytes negative.
Serum C3 0.3 (1.1-1.7). ASLO titre 200 IU/L
What is the diagnosis?

ANSWERS
1. This baby is on nearly 15 mg/kg/min of glucose (a commonly used index of glucose needs).
Do the calculations yourself! The plasma insulin at the time of low blood sugar is high, reflecting
that the cause of the hypoglycemia is a high insulin level. Causes include an infant of a diabetic
mother, nesidioblastosis, islet cell adenoma, exogenous insulin administration,
panhypopituitarism etc.. However, the very high levels of insulin and the raised glucose needs
upto 15 mg/kg/min indicate that nesidioblastosis or islet cell adenoma (less commonly) are the
diagnoses to consider.
2. Diabetic ketoacidosis.
3. Legionella. The renal failure, hepatic injury, low Na and PO4 with the characteristic setting of
an immunocompromised child put it at the top of the list. Other organisms like Pneumocystis and
CMV may cause the lung picture as described but do not fit all the data. Treatment is with
erythromycin and rifampicin.
4. TPN induced liver injury & cholestasis and metabolic bone disease of prematurity can both
explain the data. The low PO4 is a result of inadequate PO4 in the TPN and causes osteopenia
and elevation of ALP as in rickets. Prevention/treatment is with phosphate supplementation. The
role of alphacalcidiol is controversial. TPN cholestasis may be a result of Intralipid induced
biliary damage, but virtually all components of TPN have been implicated in damage caused by
long-term TPN.
5. In a hypoxic infant, the first step is always to raise the FIO2. Increasing the MAP will also
improve oxygenation on HFOV.
6. This is a dynamic renal isotope scan to assess excretion (DTPA or MAG3). The diagnosis is a
scarred right kidney with virtually no function and a normal left side. The sharp upslope and then
the gradual fall as seen in the left is normal. On the rt. there is failure of the isotope to be taken
up by the kidney and excreted and therefore a steady line.
7. Presence of maternal HepBsAg necessitates the infant being immunised with the vaccine. The
presence of anti e antibodies suggests a strong maternal immunity and the least likelihood of
infectivity, supported by the absence of HepBeAg. This newborn only needs the vaccine, not the
immunoglobulin.
8. Hypoadrenalism. This is most likely an adrenal crisis with a low Na and high K. The high urea
suggests intravascular dehydration.
9. Erythromycin is the antibiotic of choice for Ureaplasma infection which has been implicated
in the development/worsening of CLD in preterms.
10. This picture suggests both a nephrotic and a nephritic component to the illness. The low C3
suggests against minimal change disease as a cause of the nephrosis.

f dar I qbal
a
S
.
s
Dr

MRCPCH Par t II

Pract ice
i ce Ex ams
Photographic Material
Data Interpretation
Grey Cases

Paediatric Exam Series

DATA
1. List two findings in this ECG from a 4 year old boy.

2. These are the results from a 6 month old with failure to thrive:
ABG: pH 7.23, pCO2 4.2, pO2 14.5, HCO3 17.5
Na 136, K 3.3, Urea 3.5, Cl 118, Ca 2.3
What is the most likely diagnosis?
3. Does this child have cystic fibrosis?
Sweat Na from left arm 49 mmol/L, sweat amount 60 mg
Sweat Na from right arm 30 mmol/L, sweat amount 88 mg.
4. These are results from a standard ACTH stimulation test in a 10 year old boy with
suspected Addison's disease:
0800 hrs. Baseline bloods
30 min after ACTH
60 min after ACTH
What is the conclusion of this test?

Cortisol level in serum


100 nmol/L
160 nmol/L
210 nmol/L

5. These are the results from a 8 year old with sickle cell disease:
Initial blood results: Hb electrophoresis showed Hb A1 85%, Hb F 4%, Hb S 10 %.
Substance A was given regularly for 6 months.
After 6 months, Hb A1 85%, Hb F 10%, Hb S 4%.
What could be substance A?
6. What could be the reason for this infant to not gain weight?

7. These are the results from a 2 day old premature neonate (24 weeks gestation):
Na 150, K 5.4, Urea 8.7, Creatinine 90, Chloride 103, Ca 2.45
Serum osmolality 320, urine osmolality 500
What appropriate management is needed at this stage?
8. A 2 month old floppy infant had these results:
Creatine kinase 400 IU
Nerve conduction study normal, EMG showed fibrillation potentials, muscle biopsy showed
groups of giant type I fibres within fascicles of atrophic fibres.

What is the diagnosis?


9. This is the antibody profile of a 4 year old girl with arthritis:
Rh Factor -ve, SS1 -ve, SS2 -ve, ANA +, HLA B27 -ve, anti ds DNA -ve.
What is the likely diagnosis?
10. A 10 year old with hereditary spherocytosis recovering from a recent haemolytic crisis
shows the following in his blood picture:
Hb 9.9, WCC 4.4, Plat 140. MCV 108, reticulocytes 2.5%, no nucleated red blood cells.
Occasional hypersegmented neutrophil seen.
What is the cause for this picture?

ANSWERS
1. Left axis (~ -30) and left ventricular hypertrophy by voltage criteria (S > R in V1, tall R in V46). Note that the T waves in V4-6 are upright. This indicates a diastolic overload of the left
ventricle. In systolic overload, there is evidence of strain in the form of depressed T waves in the
left precordial leads.
2. This is consistent with a renal tubular acidosis with normal anion gap. This may be type I or II.
3. Cannot say. The minimum amount of sweat needed is 100 mg. An elevated sweat Na > 60 is
diagnostic of CF. Bprderline values of 50-60 need repeat samples. A positive test is always
repeated to confirm the diagnosis.
4. This is a positive ACTH stimulation test. Peak values > 500 and/or an increment of 200 is
accepted as normal. Basal Peak serum cortisol at 0800 hrs < 100 is very diagnostic of Adrenal
insufficiency. (Ref: Arch Dis Child April 1999 Vol 80(4) p:330-332)
5. Hydroxyurea. This has been used to raise the Hb F levels to prevent severe sickling crises.
6. Coeliac disease is a strong possibility. The onset of weight loss corresponds to weaning
period. Inadequate weaning is also a possibility.
7. Increase the fluids. This is suggestive of hypernatremic dehydration secondary to the preterm
infant's highly permeable skin and high insensible water loss.
8. Spinal muscular atrophy type I. The answer is incomplete without the type (based on the age).
9. Pauciarticular type I juvenile chronic arthritis.
10. Inadequate folate and/or B12 causing megaloblastic anemia. When there is a high demand in
times of high bone marrow activity (as in this recovering marrow), there is a shortage of folate
unless there is an adequate amount in the diet or in supplements.

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Dr

MRCPCH Par t II

Pract ice
i ce Ex ams
Photographic Material
Data Interpretation
Grey Cases

Paediatric Exam Series

PHOTOS
1. What investigation is being done?

2. What is the diagnosis in this 2 year old boy?

3. What is the treatment for these lesions?

4. What is the diagnosis?

5. This 3 year old boy became unwell within a few hours. What is the likely diagnosis?

6. What investigation is being done in this picture? What is the diagnosis?

7. This is a cranial ultrasound of a neonate. What is the diagnosis? What is the prognosis?

8. What is the prognosis for this newborn infant?

9. What is the cause for this child's hand being deformed?

10. What is the diagnosis? What are the associated abnormalities?

ANSWERS
1. This is a DMSA scan - a radioisotope scan of the kidneys to assess the function of the kidney.
Scars, poorly perfused areas, and split function can be assessed.
2. This is a left sided torsion of the testicle. Note the discolouration of that hemiscrotum.
Management is surgical exploration and orchidectomy if the testis is not viable. Orchidopexy of
the other side is recommended. Colour Doppler flow studies for the testicular artery flow is a
form of non-invasive testing that diagnoses torted testes.
3. This is erythema nodosum. No specific treatment - just analgesia and treatment of the
underlying cause.
4. Urticaria of the face. This was secondary to nut allergy.
5. Meningococcal disease.
6. MRI scan of the brain. There is gross dilatation of the ventricular system - hydrocephalus.
7. Cystic PVL. The cysts are obvious around the ventricles. The neurological outcome is almost
always predominated by spastic diplegia. Other problems including visual impairment and
hearing loss may be associated.
8. Excellent. This is just a ventouse delivery and the associated chignon of the head (swelling of
the scalp).
9. Amniotic bands.
10. This is agenesis of corpus callosum. Note the distorted anatomy and the lack of the white
band of corpus callosum above the midline. Associations include Trisomy 8 and 18 and Aicardi
syndrome (mental retardation, seizures, colobomas of the iris and retinal anomalies with butterfly
vertebrae). Isolated defects of the corpus may not present clinically at all.

ar I qba
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f
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Dr

MRCPCH Par t II

Pract ice
i ce Ex ams
Photographic Material
Data Interpretation
Grey Cases

Paediatric Exam Series

PHOTOS
1. This child was extremely sick at presentation. What are the first three steps to be taken in the
emergncy room ?

2. What is the diagnosis?

3. This child is seen in Casualty. What are the initial two steps to be taken after taking history
and examining?

4. What abnormality can you spot on this chest Xray? What are the diagnostic possibilities?
Name two.

5. What are the possible causes of this appearance?

6. What is the diagnosis?

7. What steps can be taken to prevent this condition?

8. This 8 week old infant presented with breathlessness for the past 2 weeks, gradually increasing
and now causing poor feeding. What abnormalities are seen? Name two. What is the diagnosis?

9. What organisms cause this picture?

10. What two abnormalities can be seen on this picture?

ANSWERS
1. The same first three steps to be taken in any emergency room - airway, breathing, circulation.
This is the picture of a child with meningococcal sepsis. As soon as iv access is obtained the
priority is fluid resuscitation and antibiotics. Im penicillin is advised whenever the GP has
suspicion of the disease, followed by urgent referral.
2. Sacrococcygeal teratoma.
3. This is highly suggestive of child abuse, with the characteristic hot plate injury on the
buttocks. Admit the child and contact the Social services on-call team, alert the Consultant oncall and never forget the medical management of the condition.
4. There is a fusiform mass on the clavicle towards the medial side. This is an old fracture of the
clavicle with callus. Is this post-traumatic or child abuse?
5. Frostbite, Ischaemia and thrombosis.
6. Severe candidal dermatitis of the nappy area.
7. Prevention of anencephaly is by means of pre-conceptional and antenatal administration of
folic acid to prevent neural tube defects.
8. Hyperinflated left side of the lung, and mediastinal shift to the right. This is congenital lobar
emphysema of the left upper lobe. This is not a tension pneumothorax because lung markings are
seen in the left lung and the length of the history is too long to be true for a pneumothorax.
9. This is impetigo. Staphylococci and Streptococci are common causes of impetigo.
10. Hickman line seen in the rt. atrium (central venous line) and a rt. humeral prosthesis. This is
an oncology patient with a Hickman line for chemotherapy and ease of sampling bloods.

ar I qba
d
f
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l s
Dr

MRCPCH Par t II

Pract ice
i ce Ex ams
Photographic Material
Data Interpretation
Grey Cases

Paediatric Exam Series

PHOTOS
1. What investigation is being done? What is the diagnosis?

2. What treatment modalities are available for this lesion?

3. Name three conditions that are associated with this picture?

4. What is the diagnosis?

5. This 12 year old boy had a severe headache for 12 hours and a fever of 40 C at admission.
What is the likely diagnosis?

6. What investigation is being done in this picture?

7. This is a renal ultrasound of a neonate following some concerns on his antenatal ultrasound.
What is the diagnosis?

8. What investigation is being performed? What is the abnormal finding in this picture?

9. What is the cause for this child's hand being deformed?

10. What is the commonest inheritance pattern of this problem?

ANSWERS
1. This is a barium enema. This demonstrates the "claw sign". The diagnosis is acute
intussusception. Common in infants, presents with sudden onset colicky abdominal pain,
vomiting, passing "red currant" jelly stools and examination reveals a pale child with intermittent
colic, maybe a lump in the abdomen and blood on per rectal exam. Can be acute, recurrent or
chronic. Can be ileocolic (common), ileocolocolic, colocolic and ileoileal types. Diagnostic
investigations would include abdominal xray, ultrasound and a barium enema. Reduction is
achieved by the enema itself - either barium or pneumatic.
2. Excision and skin grafting. This is a giant pigmented naevus.
3. Streptococcal infection, inflammatory bowel disease, TB, leprosy, sulphonamides. This is
erythema nodosum.
4. Rickets with wideneing of the wrists.
5. Meningococcal disease with meningitis.
6. Blood gas analysis. Samples can be arterial, capillary or venous. Some machines will do
electrolytes and glucose as well.
7. Multicystic kidney. These kidneys are usually dysplastic and non-functional. Assessment of
the other kidney's function is essential by doing a MCUG to explore VUR.
8. Micturating cystogram. Bladder diverticulum.
9. Amniotic bands.
10. AR. This is ambiguous genitalia. Commonest cause is a virilised female in adrenogenital
syndrome. Commonest defect is 21 hydroxylase deficiency.

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