Canadian Paediatric Surveillance Program - 2003 Results

Highlights from other national paediatric surveillance units

Australia

The response of paediatricians and child psychiatrists contributing to the surveillance of mental health issues through the Australian Paediatric Surveillance Unit (APSU) has been very positive. Three psychosocial disorders have recently been monitored: Munchausen by proxy syndrome, conversion disorder and early-onset eating disorder (in children 5-13 years of age).

The Munchausen by proxy syndrome study (2000-2003) has enabled documentation of the presenting features and consequences for a sample of 61 children. The study has also yielded some remarkable information on the impact diagnosing this particular form of child abuse had on the clinician.

Conversion disorder has been described in over 150 children during surveillance through the APSU (2002-2003). Of these children, 74% were female and the mean age was 11.8 years (range=3.0-15.9 years). The clinical picture for most children was complex. Children presented with an average of three symptoms/signs, the most common were motor weakness/paralysis (40%), abnormal gait/movements (39%) and pseudoseizure (25%). Many children also had pain (55%) and/or fatigue (30%). The study has demonstrated that health and support needs of affected children can be very high.

The ongoing study of early-onset eating disorder has confirmed 41 children (73% female) with determined food avoidance to date. Clinical features include weight loss in 88% (mean loss of 6.7 kg), fear of weight gain (76%) and excessive exercise (49%). This study is also currently being conducted through the CPSP, presenting an excellent opportunity for international comparison in the future.

Britain

This past year has seen several new studies come onto our orange card. Of particular importance is the study into childhood tuberculosis (TB). TB notification rates are increasing in the UK, particularly in cities such as London where a quadrupling of notification has occurred over the last ten years. Several factors have contributed to this, including immigration from high prevalence countries and HIV infection. This 13-month study will assess current incidence rates, how children are being managed, what services exist for children with TB, and importantly validate the current national TB surveillance scheme.

In-house, 2003 saw the introduction of the Sir Peter Tizard Research Bursary. This bursary aims to encourage junior doctors to undertake research into rare paediatric disorders and is presented in the name of one of the founders of the BPSU. The successful applicant this year will be examining the epidemiology on thyrotoxicosis.

Cyprus/Greece

The Cyprus/Greece Paediatric Surveillance Unit was established in May 2001 with research projects conducted from April 2002 to December 2003. Cases were reported on West syndrome, Kawasaki disease, rickets, pertussis and pancytopenia. The unit is now applying for full membership at the 2004 INoPSU meeting in Portugal.

Germany

Cerebral venous thrombosis in children was studied to assess the association of prothrombotic risk factors and underlying conditions (infections, vascular trauma, immobilization, malignancies, autoimmune diseases, renal diseases, metabolic disorders, obesity, birth asphyxia, cardiac malformations, and use of prothrombotic drugs) with cerebral venous thrombosis (CVT) in children.

Results were published in Circulation 2003;108: 1362-7 (Cerebral venous thrombosis in children -A multifactorial origin). From 1995 to 2002, 149 paediatric patients aged newborn to <18 years (median six years) with CVT were consecutively enrolled. In patients, and in 149 age- and gender-matched children with similar underlying clinical conditions but without CVT, the factor V G1691A mutation, the factor II G20210A variant, lipoprotein(a) [Lp(a)], protein C, protein S, antithrombin, and antiphospholipid antibodies, as well as associated clinical conditions, were investigated. Eighty-four (56.4%) of the patients had at least one prothrombotic risk factor compared with 31 control children (20.8%; P<0.0001). In addition, 105 (70.5%) of 149 patients with CVT presented with an underlying predisposing condition. On univariate analysis, factor V, protein C, protein S, and elevated Lp(a) were found to be significantly associated with CVT. However, in multivariate analysis, only the combination of a prothrombotic risk factor with an underlying condition (OR 3.9, 95% CI 1.8 to 8.6), increased Lp(a) (OR 4.1, 95% CI 2.0 to 8.7), and protein C deficiency (OR 11.1, 95% CI 1.2 to 104.4) had independent associations with CVT in the children investigated.

CVT in children is a multifactorial disease that, in the majority of cases, results from a combination of prothrombotic risk factors and/or underlying clinical condition.

Ireland

The type I diabetes study is going into its second year and will compare current insulin figures to the previous survey in 1997. A large number of reports have been seen; the impression prior to analysis is that there is a continuing increase in new onset type I diabetes in children as in other countries. The last survey put the Republic of Ireland and Northern Ireland into the moderate to high group of countries concerning diabetes mellitus, and the results of the 2003-2004 study are awaited with interest.

The study on alcohol and children concluded that acute, severe alcohol ingestion by children resulted in hospital admissions and, of concern, produced a high number of adolescents, particularly girls, presenting to children's units in an intoxicated state. The results of this study will be presented at the spring meeting of the Irish Paediatric Association and have already generated some media interest.

Knowing that a similar study is ongoing in the British unit, the IPSU put congenital toxoplasmosis on their card, in order to ascertain as completely as possible the frequency of this condition in Ireland.

Latvia

Latvia continues active surveillance in the following areas: hematology/oncology, endocrinology, nephrology and HIV/AIDS.

Netherlands

Of note in 2003 was the rise in the incidence of hemoglobinopathies compared to the surveillance numbers in 1993. A national screening program for sickle cell disease and thalassemia will be set up. Medium-chain acyl-CoA dehydrogenase deficiency surveillance shows that the incidence is as expected and no children are missed by the tandem mass spectrometry screening in the northern part of the Netherlands where a pilot screening is conducted.

New Zealand

The continuation of a very high response rate, consistently 94 to 96%, demonstrates the interest expressed by paediatricians in using the scheme for their research. It has also helped to secure a further three years of funding from the New Zealand Ministry of Health.

Portugal

The Portuguese Paediatric Surveillance Unit (PPSU) is a scientific branch of the Portuguese Paediatric Society, with no state institutional links. Based on the British Paediatric Surveillance Unit, the PPSU was created in June 2000 and began active surveillance in April 2001. The population under national surveillance is estimated to be 1,673,600 children and adolescents less than 15 years of age. The PPSU includes on its mailing list all registered paediatricians, paediatric surgeons, neurologists and cardiologists, as well as the residents of these specialties for a total of 1,506 participants.

Notification is individual, not institutional. Both postal and electronic notification is available for the return of the postal card and the case inquiry.

During the year 2003, the return rate of notifying cards oscillated between 20 and 25%, amounting to circa 300 cards returned monthly. Of the 3,340 cards returned in 2003, 145 (4.34%) participants reported cases. More than one hundred cards are returned electronically each month. The confirmation of notified cases is not yet closed, as circa 50% of the case inquiries have not yet been returned.

So far, the PPSU has been able to confirm the national dimension of the surveillance system. Data confirm the utility of including non-hospital-based physicians on the mailing list, particularly for some conditions under surveillance such as diabetes. The large mailing list includes every paediatrician (or related specialist) in the country, both active and retired, as well as residents. The PPSU knows that this decision has had a negative effect on the return rates, but feels that it has elements to suggest that reporting has not been negatively affected. This fact supports the decision of keeping every member of the Portuguese Paediatric Society on the mailing list, despite low return rates, at least for the time being.

Switzerland

In July 2002, shaken baby syndrome was included in the SPSU program (under the scientific direction of Dr Ulrich Lips, paediatric university hospital, Zurich). The goal of the study was to ascertain the incidence of children presenting with typical symptoms or "forme-fruste" of shaken baby syndrome and to sensitize the medical profession to this special type of infantile abuse. Unfortunately, in Switzerland, incidence data relating to this syndrome is not available. The public ministry of the Zurich district is the only one (approximately 17% of the Swiss population) that inventoried about ten cases in the years 1999-2001. During this same time frame, the paediatric hospital of Zurich had three children with severe shaken baby syndrome. The number of clinical findings should be higher as the syndrome is not well known. The case definition bases itself on the following points: 1) clinical symptoms (bulging fontanel, convulsions, disturbances, impaired conscience); 2) eye examination signs (unilateral or bilateral retinal and/or vitreous hemorrhages); 3) medical imagery signs tomodensitometry/sub-dural or sub-arachnoidal hematomas parenchymatous lesions (differentiation of gray/white/MRI matter, contusions, shearing injuries, etc.); 4) clinical history (child shaken in the sagittal direction). Depending on the situation, a child deceased as a result of a shaking trauma is not transferred to a clinic but rather directly to a medical legal institution where data collection is carried out simultaneously with these institutions. From July to December 2002, seven shaken baby syndrome cases were reported in children six months old or less, the average age being 5.3 months. Two children succumbed very quickly, another did not present any clinical or radiological sequelae at the time of hospital discharge, and four had neurological sequelae. In 2003, only five cases were declared. Long-term follow-up is undertaken through a second questionnaire that is sent nine months after the initial trauma.

Wales

The incidence of juvenile idiopathic arthritis based on reporting to the WPSU in the first six months suggests an incidence of five per 100,000 population under the age of 16 years. This is lower than expected and approximately half the incidence rate seen in the previous UK study of hospital referred juvenile arthritis. It is likely that there is under-reporting, but it is also possible that these six months may be unrepresentative and that the incidence may be different when looked at over the intended two years of the survey period. The number of children referred for physiotherapy, occupational therapy and orthotics is lower than expected (ideally all patients with a diagnosis of juvenile arthritis should receive specialist advice from a paediatric physiotherapist experienced in dealing with juvenile arthritis). The numbers of patients receiving disease-modifying drugs and anti-TNF (tumor necrosis factor) treatment is appropriate for a newly diagnosed population.

Call for New Studies

Research opportunities

Wanted

• Investigators to initiate new CPSP studies on childhood disorders that are high in disability, morbidity, mortality and economic costs to society, despite their low frequency.
  
• The paediatric community to take up the challenge of proposing a wide range of research studies.
  
• Interested individuals prepared to assume a leadership role in developing protocols and analyzing study data.
  

The tool

The CPSP is:

• A well-established, timely, cost-effective surveillance infrastructure.
  
• A multi-faceted surveillance tool capable of collecting reliable data in a variety of different fields.
  
• An effective means of monitoring low-frequency, high-impact diseases and conditions.
  

Track record

• An 83% overall initial response from more than 2,300 paediatricians.
  
• An impressive 96% data completion rate for the 583 cases reported in 2003.
  
• High duplicate reporting rate (21.3%) assuring case ascertainment and participant commitment.
  

International flavour

• Be part of INoPSU (International Network of Paediatric Surveillance Units), a growing network of national paediatric surveillance units that exists in 13 countries around the world.
  
• Take advantage of international collaboration, as INoPSU studies provide a remarkable opportunity to compare similar data and learn more about rare diseases worldwide.
  

Looking for ideas?

Here are a few examples of studies suggested through the program evaluation survey:

The potential for new studies in different paediatric subspecialties is endless. If you have a research project in mind, please contact the CPSP Senior Coordinator at 613-526-9397, ext. 239, for more information.

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