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HK J Paediatr (new series) 2002;7
1. Please use pencil to shade the correct box for the answer sheet (see loose leaf page).
2. Send back the answer sheet to the Hong Kong College of Paediatricians for the award of 4 CME points for those with
The following statements are true / false
(A) Non-ketotic Hyperglycinaemia: A Case Report
5. Which of the following is incorrect concerning NMDA
and Review on Treatment
receptor and its antagonist?:a. NMDA receptors are present only in the cerebral
1. The mode of inheritance of non-ketotic hyperglycinaemia
b. Excessive stimulation of NMDA receptors is thought
to be related to the "positive" symptoms of NKH like
c. Dextromethorphan and ketamine are examples of
d. Usefulness of dextromethorphan in seizure control has
been documented in case control studies only.
2. Which of the following is not a clinical feature of NKH?:
e. Dextromethorphan does not improve the long term
a. Family history of unexplained baby death.
developmental outcome of patients with NKH.
b. Transient hyperglycinaemia which resolved after
(B) Vitamin K Deficiency Bleeding Revisited
d. Burst suppression pattern in electroencephalogram.
e. Marked elevation of glycine level in cerebrospinal
1. Which of the following is not an effective mode of vitamin
K supplementation in the newborn infant?:a. Intramuscular injection of vitamin K at birth.
3. Which of the following is incorrect about Glycine
b. Oral administration of vitamin K at birth.
a. GCS is found inside the mitochondria.
b. GCS consists of 4 components of protein, namely
2. Which of the following approach in the use of vitamin K
c. Defects of the GCS result in depletion of glycine in
in the newborn has not been supported by clinical
d. Different mutations of GCS run in different ethnic
a. Giving vitamin K to newborns of "high-risk"
e. Different mutations of GCS run in different affected
b. Giving a single dose of vitamin K intramuscularly at
c. Giving a single dose of vitamin K orally followed by
4. Which of the methods of decreasing glycine level is
small daily doses up to 3 months old.
d. Giving a single dose of vitamin K orally followed by
repeated doses at weekly or monthly intervals up to 3
c. Peritoneal dialysis.
d. Folic acid.
e. High dose benzoate.
3. Which of the following statements concerning the side
(D) Water Intoxication in a 7-month Infant
effects of vitamin K has been reported in the literature?:a. The use of intramuscular vitamin K at birth increases
1. Causes of hyponatremia include the followings:
the chance of cancer in subsequent years.
b. The association of intramuscular vitamin K and
subsequent childhood cancers has been definitely
c. Syndrome of inappropriate anti-diuretic hormone.
c. The occurrence of haemolysis after vitamin K
administration is only limited to the use of water-soluble preparations at high doses.
2. Treatment of acute symptomatic hyponatremia includes:
d. Use of the Konakion MM preparation by the oral route
does not lead to high serum levels of vitamin K as
compared with the intramuscular route.
4. The use of oral vitamin K prophylaxis against vitamin K
deficiency bleeding is met with the following problemsexcept:
3. Rapid correction of chronic hyponatremia causes:
b. Variable absorption in infants with cholestasis.
c. Increased incidenc of classic vitamin K deficiency
bleeding compared with the intramuscular route.
d. Increased incidence of late vitamin K deficiency
bleeding compared with the intramuscular route.
4. In chronic hyponstremia, the serum sodium level should
5. When vitamin K prophylaxis for vitamin K deficiency
bleeding by the oral route is used at the time of birth, the
a. Follow with the administration of subsequent doses at
the appropriate times according to recommended
schedules in exclusively breast fed babies.
b. Watch out for clinical signs of bleeding.
c. Understand and accept the small but definite risk of
5. Treatment of acute gastroenteritis in infants include:
a. Exclusive free water replacement.
b. Oral rehydration solution.
c. Diluted formula.
d. Stoppage of breast feeding.
(C) An Adolescent Chinese Boy with Behçet's
(E) Evaluation of Paediatric Epilepsy Care
Concerning Behçet's disease:1. The incidence of HLA-B51 is significantly higher among
1. In the text, which of the following is not considered as a
2. Recurrent oral ulceration is the most common symptom.
3. Arthritis is typically erosive and can be disabling.
4. Colchicine is ineffective in treatment of muco-cutaneous
5. Patients with pulmonary artery aneurysms have a bad
e. Adequate trial of first line antiepileptic drug.
2. Which of the following fails in the evaluation?
2. The average 1 year and 3 years graft survival rate,
c. Recording advice about possible drug adverse effects.
d. 80-85% & 70-75%.
e. 75-80% & 65-70%.
3. Which of the following reasons for requesting EEG are
3. The immunosuppressive drug combination most
iv. Cyclosporin A.
v. T cell antibody preparation.
4. What is the percentage of appropriate EEG requests in
c. Steroid, Azathioprinei and Cyclosporin A.
d. Steroid, Mycophenolate mofetil and Cyclosporin A.
e. Steroid, Azathioprine, Cyclosporin A and T cell
5. What are the recommendations made in this survey in
4. The following statement concerning acute rejection is
a. Better provision and documentation of parental
a. Acute rejection predisposes to chronic rejection.
education considering side effects of medication.
b. Non-compliance is an important cause of acute
b. Practical advice and recommendation in the role of
c. MMF can reduce acute rejection rate.
c. With-holding neuroimaging in evaluation seizures.
d. T cell antibody is a form of treatment for acute
e. Lower acute rejection rate occurs with prednisolone,
azathioprine and cyclosporin A combination therapythan prednisolone, MMF and cyclosporin A
(F) 10 Years’ Experience of Paediatric Kidney
Transplantation at a Paediatric Nephrology
1. The most important obstacle to long-term graft survival
is:a. Acute rejection.
b. Chronic rejection.
e. Recurrence of the original renal disease.
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