CPSP Highlights

Do you know the answers?

1. Health surveillance is a cycle with three stages. The first and second stages are ‘data acquisition/collection’ and ‘data analysis and interpretation’, respectively. What is the third stage and why is it the most important stage of surveillance?

2. What is the gold standard measure for preventing hemorrhagic disease of the newborn?

3. Which chromosomal defect causes Prader-Willi syndrome?

4. What is the principal cause of acute renal failure in Canadian children?

5. Two provinces, British Columbia and Ontario, have implemented prenatal screening for Smith-Lemli-Opitz syndrome. What is the benefit of the prenatal screening?

6. One of the conditions under surveillance by the Canadian Paediatric Surveillance Program (CPSP) contributes to Canada’s commitment to the World Health Organization’s global eradication initiative. What is the CPSP condition and what disease has been targeted for global eradication?

7. The management of a child with CHARGE association/syndrome requires the paediatrician to assume the role of coordinator to bring about a multidisciplinary approach. Name other health-care professionals who would assist in caring for a child with CHARGE association/syndrome.

8. Although rare, congenital rubella syndrome still occurs. Name three at-risk groups identified through the CPSP.

9. Healthy children infected with varicella are at risk of developing a serious secondary infection/complication at the site of a vesicle? What is the secondary infection?

10. Surveillance has shown that vitamin D deficiency rickets is more frequent in Canada than expected. Name the at-risk population needing targeted public health prevention.

11. Severe hyperbilirubinemia of the newborn can be secondary to glucose-6-phosphatase dehydrogenase deficiency. How is it inherited, and what percentage of the worlds population is affected by this disorder? Which groups are more at risk?

CPSP quiz – Answers

1. The third stage of the health surveillance cycle is ‘communication of information for action’. Results from the Canadian Paediatric Surveillance Program (CPSP) studies are communicated to decision makers, such as the governments (local, provincial and federal) and advocacy groups like the Canadian Paediatric Society (and its respective subcommittees). Decisions by these agencies have changed clinical and public health practices.

2. A single intramuscular dose of vitamin K, 0.5 mg (birth weight of 1500 g or less) or 1.0 mg (birth weight greater than 1500 g), remains the gold standard for the prevention of classic and late hemorrhagic disease of the newborn.

3. Prader-Willi syndrome is caused by abnormalities of chromosome 15, including paternal interstitial deletion in 70% of cases, maternal uniparental disomy in the majority and mutations in the remainder.

4. In Canada, hemolytic uremic syndrome is the principal cause of acute renal failure in children, and in most cases it is secondary to infection with Escherichia coli O157:H7.

5. Prenatal screening for Smith-Lemli-Opitz syndrome is beneficial because early treatment with dietary cholesterol supplementation has clearly improved the quality of life for families by reducing frequency of infections, accelerating growth and ameliorating behaviour. Beneficial results may improve the final developmental outcome of patients with Smith-Lemli-Opitz syndrome.

6. The CPSP condition is acute flaccid paralysis and the disease under eradication is poliomyelitis. The elimination of indigenous wild poliovirus transmission in Canada, and the rest of the American region, was certified in September 1994. However, until global polio eradication is attained (estimated to be in the year 2005), there remains an ongoing risk of wild poliovirus importation from polio-endemic regions to Canada. Consequently, active surveillance of acute flaccid paralysis is used to monitor potential cases of paralytic poliomyelitis.

7. Management of the CHARGE association/syndrome child should include surgery (general, cardiac, ear, nose and throat, ophthalmology, orthopedic, plastic, urology), medical (gastroenterology, endocrinology, psychiatry/psychology) and paramedical (occupational therapy, physiotherapy, speech therapy, social and educational services) specialists.

8. Through an international comparison, the CPSP showed that congenital rubella syndrome is rare but persists in the following groups: non-immunized women, including immigrant populations; women in early pregnancy travelling to countries with incomplete or absent rubella immunization programs; and women experiencing primary vaccine failure.

9. The secondary infection is invasive group A beta-hemolytic streptococcal disease. Healthy children affected by varicella have a 40- to 60-fold increased risk of developing this complication, including necrotizing fasciitis.

10. Vitamin D deficiency rickets is mostly confined to darker-skinned infants and children who have been breastfed without vitamin D supplementation. Dark-skinned children are more at risk for rickets because melanin acts as a natural sunscreen and competes with provitamin D3 for solar ultraviolet-B photons.

11. Glucose 6-phosphatase dehydrogenase deficiency is an X-linked disorder affecting 3.4% of the world population. The incidence is higher in the Mediterranean and Middle East and reaches 15% to 26% in Africa and Southeast Asia.