Alberta Children's Hospital Research Articles

Beta-blockers for the treatment of problematic hemangiomas

To examine treatment indications, efficacy and side effects of oral beta-blockers for the treatment of problematic hemangiomas. A retrospective review of patients with hemangiomas presenting to the Alberta Children's Hospital Vascular Birthmark Clinic (Calgary, Alberta) between 2009 and 2011 was conducted. The subset of patients treated with oral beta-blockers was further characterized, investigating indication for treatment, response to treatment, time to resolution of indication, duration of treatment, occurrence of rebound growth and side effects of therapy. Between 2009 and 2011, 311 new patients with hemangiomas were seen, of whom 105 were treated with oral beta-blockers. Forty-five patients completed beta-blocker treatment while the remainder continue to receive therapy. Indications for treatment were either functional concerns (68.6%) or disfigurement (31.4%). Functional concerns included ulceration (29.5%), periocular location with potential for visual interference (28.6%), airway interference (4.8%), PHACES syndrome (3.8%), auditory interference (0.95%) and visceral location with congestive heart failure (0.95%). The median age at beta-blocker initiation was 3.3 months; median duration of therapy was 10.6 months; and median maximal treatment dose was 1.5 mg/kg/day for propranolol and 1.6 mg/kg/day for atenolol. Ninety-nine patients (94.3%) responded to therapy with size reduction, colour changes, softened texture and/or healing of ulceration. Rebound growth requiring an additional course of therapy was observed in 23 patients. Side effects from beta-blockers included cool extremities (26.7%), irritability (17.1%), lower gastrointestinal upset (14.3%), emesis (11.4%), hypotension (10.5%), poor feeding (7.6%), lethargy (4.8%), bronchospasm (0.95%) and rash (0.95%). Side effects did not result in complete discontinuation of beta-blocker treatment in any case; however, they prompted a switch to a different beta-blocker preparation in some cases. Resolution of the primary indication, requiring a median time of three months, occurred in 87 individuals (82.9%). Treatment of infantile hemangiomas with oral beta-blocker therapy is highly effective and well tolerated, with more than 94% of patients demonstrating a response to treatment and 90% showing resolution of the primary functional indication for treatment.

Hemimegalencephaly: A Foetal Tauopathy

Researchers at University of Calgary and Alberta Children's Hospital, Canada; and University of Pennsylvania, Philadelphia studied brain resections from 3 male infants with hemimegalencephaly (HME) and refractory epilepsy.

Posterior to the ridge laser treatment for severe stage 3 retinopathy of prematurity

Current methods of treating the avascular retina with laser photocoagulation for severe retinopathy of prematurity (ROP) are not completely effective in the reduction of visual morbidity. We report a case series in which additional laser treatment, called 'posterior laser', was delivered posterior to the neovascular ridge, for eyes with severe stage 3 ROP in zone II with avascular retina posterior to the ridge. Retrospective chart review. Infants who underwent laser treatment, posterior to the neovascular ridge for severe ROP at the Alberta Children's Hospital, between January 2005 and October 2008. Charts were reviewed for 18 eyes of 11 patients and collected information included demographic data, clinical examination results, and digital retinal images. Structural and functional outcomes of treatment. Four (22%) of 18 eyes received 'posterior laser' as primary treatment and the remainder of eyes (78%) received 'posterior laser' following previous laser photocoagulation anterior to the neovascular ridge. Mean birthweight was 688 g (552-930) and mean gestational age was 24 weeks (23-28). There were no complications because of the posterior laser treatment. In all, 16 of 18 eyes experienced rapid regression of the ridge and subsequent decrease in vascular dilation and tortuosity within 1 week. Two eyes required vitrectomy for 4A retinal detachment; however, no eyes developed stage 4B ROP. Posterior to the ridge laser in the setting of the morphological criteria described had no increased safety concerns and resulted in rapid regression of ROP with good outcomes.

Serologic Protection to Routine Vaccinations in Children with Inflammatory Bowel Disease

Background: Treatment for inflammatory bowel disease [IBD] frequently involves the aggressive use of immunosuppressants that increases susceptibility to and severity of other infections. Consequently, many individuals postpone or refuse immunizations due to unstable disease activity and of fear of disease exacerbation. The aim was to evaluate serologic protection to and completeness of routine vaccinations in children with IBD. Methods: In this single-center cross-sectional study, children with IBD followed at the Alberta Children's Hospital were recruited from September 15, 2011 to August 15, 2012. Demographic data, IBD medication use, infection risk factors, and vaccination records were collected. Serum was also collected for rubella, hepatitis A virus [HAV], and hepatitis B virus [HBV] serology and analyzed by the Provincial Laboratory of Public Health. From review of vaccination records, the proportion with complete series for each vaccine according to the Alberta schedule (at age of vaccination) was evaluated. Results: 155 children with IBD (93 Crohn’s disease, 46 ulcerative colitis, 16 IBD-unclassified) underwent serum collection; complete vaccine records were available for 152 of these children. At enrolment, 93 subjects (60%) were currently using immunosuppressive medications (systemic corticosteroids n=20; immunomodulators n=70; and biologic n=48); an additional 30 subjects had a past history of immunosuppressive medication use. Of 155 participants, 69.7% had up to date MMR, DTap-IPV-Hib, and HBV immunizations. Of 152 participants, 81.9% mounted serologic protection to rubella; 20.6% to hepatitis A, and 65.8% to hepatitis B. Of those who had completed the specific vaccinations, serologic immunity was mounted by 113 of the 142 participants (79.6%) to rubella, 79 of the 114 participants (69.3%) to HBV, and 100% of the participants to HAV. Conclusions: Children with IBD are at risk for vaccine-preventable illnesses due to lack of receiving complete vaccine series and mounting an inadequate serologic response to vaccinations. Therefore, clinicians caring for patients with IBD should be conscientious about adherence to recommended vaccination schedules, measurement of immune response to vaccines, and administering booster vaccinations where appropriate.

Detecting epilepsy‐related cognitive problems in clinically referred children with epilepsy: Is the WISC‐IV a useful tool?

The Wechsler Intelligence Scale for Children - Fourth Edition is the most widely used intelligence quotient (IQ) test in use today. However, despite numerous studies on IQ in childhood epilepsy, data exist almost exclusively from prior editions of the test, and no studies to date provide information on the sensitivity of specific WISC-IV scores (full-scale IQ [FSIQ], index, and subtest scores) to epilepsy-related cognitive impairments. The goal of this study was to determine the relative sensitivity of WISC-IV index and subscale scores in detecting cognitive problems in a group of clinically referred children with epilepsy compared to matched controls, and to define the relationship among WISC-IV scales, demographic factors, and epilepsy-related variables. WISC-IV data for children with epilepsy and high seizure burden were obtained from the Alberta Children's Hospital (ACH) and the New York University Comprehensive Epilepsy Center (NYU), two tertiary care medical centers for pediatric epilepsy. All children were clinically referred and received a standard assessment including WISC-IV. Matched controls were obtained from the WISC-IV Canadian and American standardization samples. WISC-IV scores from 212 children were included: 106 children with epilepsy (46 girls, 60 boys; mean age 11.0 years, standard deviation [SD] 3.1; parental education 14.5 years, SD 2.8), and 106 controls matched for age, gender, ethnicity, and parental education. Of the children with epilepsy, 44 had a clearly lateralized focus on electroencephalography (EEG) involving either the right or left hemisphere (26 left, 18 right). FSIQ for the epilepsy group was significantly lower than for controls, and 36.8% of children had IQs compatible with intellectual disability (FSIQ < 70), versus <1% of controls. In children with epilepsy, Working Memory and Processing Speed Index scores were lower than those for Verbal Comprehension and Perceptual Reasoning (p < 0.01). At the subtest level, scores for children with epilepsy were highest on visual and verbal subtests measuring reasoning skills such as Matrix Reasoning, and lowest on Coding (mean 5.93, SD 3.6). In terms of percentage of children on each subtest with low scores (i.e., scores below 2 SDs from the expected normative mean of 10), the Coding subtest identified the most children (28.3%) with low scores, and the Similarities subtest identified the fewest (16%). Later age at onset and shorter epilepsy duration were both correlated with higher WISC-IV FSIQ and index scores (r correlation coefficient values ranging from 0.36 to 0.44, p < 0.0001), and number of current and previous antiepileptic drug trials were both inversely correlated with FSIQ and index scores (r -0.27 to -0.47, all p-values < 0.01). Neither the FSIQ nor the index scores were significantly related to seizure frequency. A similar pattern was found for subtest scores. No differences in FSIQ, index scores, or subtest scores were found between children with left- and right-hemisphere seizure foci, or between those with positive or negative magnetic resonance imaging (MRI) findings. The WISC-IV is sensitive to epilepsy-related cognitive problems in clinically referred children with high seizure burden, particularly problems relating to expressive verbal, working memory, and processing speed difficulties. Compared to healthy children, these children have a very high rate of cognitive difficulties as assessed by the WISC-IV. The usefulness of the WISC-IV in detecting cognitive deficits in children with milder forms of epilepsy remains to be determined.

The epidemiological profile of the Vascular Birthmark Clinic at the Alberta Children's Hospital

The Vascular Birthmark (VBM) Clinic at the Alberta Children's Hospital (Calgary, Alberta) is a multidisciplinary clinic dedicated to the evaluation of children with vascular anomalies. To review the characteristics of patients seen at the VBM Clinic. A retrospective data analysis of all pediatric patients presenting to the VBM Clinic between 1998 and 2009 was performed. Data including demographic, referring, diagnostic and treatment information were obtained from the clinic's database. Of 932 patients, 621 with hemangiomas and 311 patients with vascular malformations were found in the database. Hemangiomas were more commonly found in girls (68.5%), and most commonly located on the head and neck (54%), with most patients (72.6%) having only one lesion. Of the patients with hemangiomas, 14.7% underwent diagnostic imaging investigation and 23.7% received treatment including medications, surgery, pulsed-dye laser or dressings. The sex distribution among the 311 patients with vascular malformations was almost equal. Venous malformations accounted for 38.9% of patients, isolated capillary malformations for 31.5%, lymphatic malformations for 11.6%, mixed low-flow malformations for 14.8% and arteriovenous malformations for 2.9%. Overall, 37.9% of patients underwent diagnostic imaging investigation and 42.4% received treatment - either pulsed-dye laser, surgical excision, sclerotherapy or other treatment. Hemangiomas and vascular malformations can occur at any anatomical site. There is a large variation in clinical presentation necessitating expertise in a variety of diagnostic approaches and treatment modalities. Vascular anomalies are best managed in a multidisciplinary setting.

A shared founder mutation underlies restrictive dermopathy in Old Colony (Dutch‐German) Mennonite and Hutterite patients in North America

A Shared Founder Mutation Underlies Restrictive Dermopathy in Old Colony (Dutch-German) Mennonite and Hutterite Patients in North America Catrina Loucks, Jillian S. Parboosingh, Jessica X. Chong, Carole Ober, Victoria M. Siu, Robert A. Hegele, C. Anthony Rupar, D. Ross McLeod, Alfredo Pinto, Albert E. Chudley, and A. Micheil Innes* Department of Medical Genetics, Alberta Children’s Hospital and Alberta Children’s Hospital Research Institute for Child and Maternal Health, University of Calgary, Calgary, Alberta, Canada Department of Human Genetics, The University of Chicago, Chicago, Illinois Department of Obstetrics and Gynecology, The University of Chicago, Chicago, Illinois Department of Paediatrics and Biochemistry, University of Western Ontario, London, Ontario, Canada Departments of Biochemistry and Medicine, Robarts Research Institute and Schulich School of Medicine and Dentistry, University of Western Ontario, London, Ontario, Canada Department of Pathology and Lab Medicine, University of Calgary, Calgary, Alberta, Canada Program in Genetic and Metabolism, Children’s Hospital, Department of Pediatrics and Child Health and Biochemistry and Medical Genetics, University of Manitoba, Winnipeg, Manitoba, Canada

Congenital heart defect case ascertainment by the Alberta Congenital Anomalies Surveillance System

Congenital heart defects (CHDs) are the most common type of congenital anomaly, with a wide range of reported birth prevalence estimates. This quality assurance study describes CHD case ascertainment by the Alberta Congenital Anomalies Surveillance System (ACASS). ACASS data for CHD cases were compared with additional sources including the two Pediatric Cardiology clinics in Alberta, the Alberta Children's Hospital Department of Pathology, and hospital records. Cases included live births, stillbirths, and fetal deaths at less than 20 weeks' gestation born in Alberta, Canada, between 1995 and 2002. The birth prevalence of cases and chi-square linear trend analyses were calculated for specific types of heart defects for the total study period. The ascertainment of CHD cases by ACASS was 45%. The total prevalence of CHD cases was 5.59 per 1000 total births (TBs; 95% confidence interval [CI], 5.32-5.86) when ACASS was the only data source and increased to 12.42 per 1000 TBs (95% CI, 12.03-12.83) when all data sources were used. Although the total prevalence of CHD cases remained stable during 1995 to 2002, the prevalence of atrial septal defect (ASD) and cases with an ASD and ventricular septal defect (VSD) significantly increased. The prevalence of left ventricular outflow tract obstruction cases significantly decreased during the study period. Pediatric cardiology clinics are worth including as additional ascertainment sources to contribute to more accurate prevalence estimates. The significant increases of ASD and cases with both an ASD and VSD may reflect differences in diagnostic and ascertainment practices.

Assessing the representativeness of Canadian Hospitals Injury Reporting and Prevention Programme (CHIRPP) sport and recreational injury data in Calgary, Canada

The objective of this study was to assess the representativeness of sport and recreational injury data from Canadian Hospital Injury Reporting and Prevention Programme (CHIRPP) in Calgary. Internal representativeness was assessed by comparing CHIRPP and regional health administrative data (ambulatory care classification system-ACCS) at Alberta Children's Hospital (ACH). External representativeness was assessed by comparing CHIRPP with ACCS at all hospitals. Comparisons were performed using descriptive statistics for top injury-producing sports and sports that produced severe injuries. Stratified distributions of injury-producing sports by gender, age group and severity of injury in CHIRPP and ACCS were compared. The proportion of all injuries in Calgary captured by CHIRPP was 64.8% (99%CI: 64.02–65.54%) (16,977/26,206). CHIRPP captured more cases of top injury-producing sports than ACCS at ACH. Rankings of top injury-producing sports in CHIRPP and ACCS at ACH were remarkably consistent (ρ = 0.92, p < 0.0001). Rankings of top injury-producing sports in CHIRPP and ACCS at all hospitals were almost identical (ρ = 0.98, p < 0.0001). Stratified distributions of top injury-producing sports by gender, age group and the severity of injury showed strong consistency between CHIRPP and ACCS. It is concluded that CHIRPP in Calgary provides a representative profile of injuries compared to regional health administrative data. This project supports the use of CHIRPP for establishing injury prevention priorities.

Mitochondrial Hypermetabolic Neurons in Epilepsy

Researchers at the University of Calgary and Alberta Children's Hospital, Canada studied histochemically frozen sections of 10 brain resections from 7 epileptic children (ages 2 months to 17 years).

The epidemiological profile of the Vascular Birthmark Clinic at the Alberta Children's Hospital

The epidemiological profile of the Vascular Birthmark Clinic at the Alberta Children's Hospital

The Opportunities and Obstacles in Developing a Vascular Birthmark Database for Clinical and Research Use

INTRODUCTION Databases are useful tools in clinical settings. The authors review the benefits and challenges associated with the development and implementation of an efficient electronic database for the multidisciplinary Vascular Birthmark Clinic at the Alberta Children's Hospital, Calgary, Alberta. METHODS The content and structure of the database were designed using the technical expertise of a data analyst from the Calgary Health Region. Relevant clinical and demographic data fields were included with the goal of documenting ongoing care of individual patients, and facilitating future epidemiological studies of this patient population. After completion of this database, 10 challenges encountered during development were retrospectively identified. Practical solutions for these challenges are presented. RESULTS The challenges identified during the database development process included: identification of relevant data fields; balancing simplicity and user-friendliness with complexity and comprehensive data storage; database expertise versus clinical expertise; software platform selection; linkage of data from the previous spreadsheet to a new data management system; ethics approval for the development of the database and its utilization for research studies; ensuring privacy and limited access to the database; integration of digital photographs into the database; adoption of the database by support staff in the clinic; and maintaining up-to-date entries in the database. CONCLUSIONS There are several challenges involved in the development of a useful and efficient clinical database. Awareness of these potential obstacles, in advance, may simplify the development of clinical databases by others in various surgical settings.

Temporal trends in radiologic tests performed at diagnosis in a cohort of children with inflammatory bowel disease (1983-2011)

Children with inflammatory bowel disease (IBD) undergo radiologic tests at diagnosis to determine disease extent, behaviour, and complications. Over the past three decades, novel radiologic modalities have been introduced including computed tomography (CT), magnetic resonance imaging (MRI), and ultrasound (US). The objective of this study was to characterize the trends in radiologic investigations used within six months of diagnosis in children with inflammatory bowel disease. We retrospectively identified all children ≤ 17 years old diagnosed with IBD between 1983 and 2011 and followed at the Alberta Children's Hospital, the sole tertiary care pediatric center in southern Alberta. We recorded radiologic investigations performed within six months of diagnosis (barium enema, small bowel follow through [SBFT], CT, MRI, US). Temporal trends were evaluated using joinpoint regression analysis. Subjects were categorized into 5-year intervals by year of diagnosis. Between January 1983 and June 2011, 565 children were diagnosed with IBD (300 Crohn's disease, 219 ulcerative colitis, and 46 IBD-unclassified or indeterminate colitis). During 1983-1987, 33 (89%) and 26 (70%) subjects received a barium enema and SBFT, respectively. During 1988-1992, 25 (63%) and 33 (83%) subjects received a barium enema and SBFT, respectively. During 1993-1997, 29 (31%) and 79 (84%) subjects received a barium enema and SBFT, respectively. During 1998-2002, 12 (11%) and 91 (83%) subjects received a barium enema and SBFT, respectively. During 2003-2007, 9 (5%), 124 (74%), and 1 (1%) subjects received a barium enema, SBFT, and CT/MRI, respectively. During 2008-2011, 1 (1%), 78 (67%), 20 (17%), 19 (16%), and 70 (60%) subjects received a barium enema, SBFT, CT, MRI, and US, respectively. At diagnosis, no subjects received an US before 2008 and no subjects received a CT or MRI before 2003. During 1983-2011, the use of barium enema and SBFT within six months of diagnosis decreased significantly (annual percent change -11% and -0.4%, respectively). During 2008-2011, the use of CT decreased significantly (annual percent change - 14%) and the use of MRI increased significantly (annual percent change +32%). During 2008-2011, subjects with Crohn's disease were more likely to receive CT, MRI, or US at diagnosis than subjects without Crohn's disease (odds ratio 4.5, 95% CI 1.8-12.2). As the types of radiologic modalities used in IBD have increased over the past three decades with the introduction of MRI, US, and CT, the use of modalities involving radiation (barium enema, SBFT, and CT) has decreased, affirming the goal to minimize radiation exposure in children. Enhanced understanding of the unique roles of each modality will allow further reduction of radiation exposure and improved abilities to determine disease extent, behaviour, and complications at diagnosis.

046 Safety of Endomyocardial Biopsy in Two Pediatric Non-Surgical Centers Using the New Atc Bioptome with Variable Flexibility

BackgroundRight ventricular (RV) endomyocardial biopsy (EMB) is the “gold standard” for surveillance and acute detection of rejection following cardiac transplantation.Several bioptomes are in use. This study examined the safety of EMB in two pediatric non-surgical cardiac centers using the new Sparrowhawk bioptome with variable flexibility (ATC Technologies, Woburn, MA, USA).MethodsHealth records of all the EMB performed in two non-surgical Pediatric Cardiology Centers were reviewed: Variety Children's Heart Center, Winnipeg MB, and The Alberta Children's Hospital, Calgary AB. From October 2005 to January 2011, 79 RV EMB were performed in 33 children. The main indication for biopsy was routine surveillance for rejection following heart transplantation (n = 30) and the rest as part of cardiomyopathy work-up (n = 3). All procedures were performed under general anesthesia using custom pre-shaped guiding sheaths. The median age of the patients was 8 years (0.25-18 years) and median weight 22 kg (4.4-72.3Kg). Ten procedures were performed on children under 1 year of age. Femoral vein (FV) approach was used in most of our cases and internal jugular vein in bilateral FV occlusion. Four to five EMB specimens were obtained at each session, suitable for pathological examination.ResultsThere was no death, vascular damage or anesthetic complication. Only a small pericardial effusion in a 12 months old baby occured after the last biopsy. This complication did not require further intervention and it occurred during our early experience. There was no mechanical malfunction of the bioptome.ConclusionWe conclude that the EMB by using the new Sparrowhawk bioptome is safe in the infants & children and can be performed in cardiac non-surgical centers. BackgroundRight ventricular (RV) endomyocardial biopsy (EMB) is the “gold standard” for surveillance and acute detection of rejection following cardiac transplantation.Several bioptomes are in use. This study examined the safety of EMB in two pediatric non-surgical cardiac centers using the new Sparrowhawk bioptome with variable flexibility (ATC Technologies, Woburn, MA, USA). Right ventricular (RV) endomyocardial biopsy (EMB) is the “gold standard” for surveillance and acute detection of rejection following cardiac transplantation. Several bioptomes are in use. This study examined the safety of EMB in two pediatric non-surgical cardiac centers using the new Sparrowhawk bioptome with variable flexibility (ATC Technologies, Woburn, MA, USA). MethodsHealth records of all the EMB performed in two non-surgical Pediatric Cardiology Centers were reviewed: Variety Children's Heart Center, Winnipeg MB, and The Alberta Children's Hospital, Calgary AB. From October 2005 to January 2011, 79 RV EMB were performed in 33 children. The main indication for biopsy was routine surveillance for rejection following heart transplantation (n = 30) and the rest as part of cardiomyopathy work-up (n = 3). All procedures were performed under general anesthesia using custom pre-shaped guiding sheaths. The median age of the patients was 8 years (0.25-18 years) and median weight 22 kg (4.4-72.3Kg). Ten procedures were performed on children under 1 year of age. Femoral vein (FV) approach was used in most of our cases and internal jugular vein in bilateral FV occlusion. Four to five EMB specimens were obtained at each session, suitable for pathological examination. Health records of all the EMB performed in two non-surgical Pediatric Cardiology Centers were reviewed: Variety Children's Heart Center, Winnipeg MB, and The Alberta Children's Hospital, Calgary AB. From October 2005 to January 2011, 79 RV EMB were performed in 33 children. The main indication for biopsy was routine surveillance for rejection following heart transplantation (n = 30) and the rest as part of cardiomyopathy work-up (n = 3). All procedures were performed under general anesthesia using custom pre-shaped guiding sheaths. The median age of the patients was 8 years (0.25-18 years) and median weight 22 kg (4.4-72.3Kg). Ten procedures were performed on children under 1 year of age. Femoral vein (FV) approach was used in most of our cases and internal jugular vein in bilateral FV occlusion. Four to five EMB specimens were obtained at each session, suitable for pathological examination. ResultsThere was no death, vascular damage or anesthetic complication. Only a small pericardial effusion in a 12 months old baby occured after the last biopsy. This complication did not require further intervention and it occurred during our early experience. There was no mechanical malfunction of the bioptome. There was no death, vascular damage or anesthetic complication. Only a small pericardial effusion in a 12 months old baby occured after the last biopsy. This complication did not require further intervention and it occurred during our early experience. There was no mechanical malfunction of the bioptome. ConclusionWe conclude that the EMB by using the new Sparrowhawk bioptome is safe in the infants & children and can be performed in cardiac non-surgical centers. We conclude that the EMB by using the new Sparrowhawk bioptome is safe in the infants & children and can be performed in cardiac non-surgical centers.

A Technical Note on Submuscular Plate Removal for Symptomatic Children and Youth

Clinical Assistant Professor Department of Surgery University of Calgary Alberta Children's Hospital No financial gain is expected as a product of this commentary study. The author declares no conflicts of interest.

Endocrine Complications Following Pediatric Bone Marrow Transplantation

Pediatric bone marrow transplantation (BMT) for various diseases can lead to endocrine system dysfunction owing to preparative regimens involving chemotherapy and radiation therapy. We assessed the prevalence of post-BMT endocrine complications in children treated at the Alberta Children's Hospital (ACH) from 1991 to 2001. Time of onset of endocrine dysfunction, underlying disease processes, chemotherapy, radiation therapy and age at BMT were characterized. Subjects of <18 years of age at the time of allogeneic or autologous BMT for whom 1-year follow-up through the ACH and a chart were available for review were included in the study. Subjects with a pre-existing endocrine condition were excluded. Of the 194 pediatric BMT procedures performed at the ACH between January 1, 1991 and December 31, 2001, 150 complete charts were available for review. Sixty five subjects received follow-up care at other centers and were excluded. Therefore, a total of 85 subjects were included in the review. The prevalence of endocrine complications identified was: primary hypothyroidism 1.2%, compensated hypothyroidism 7.0%, hyperthyroidism 2.4%, hypergonadotrophic hypogonadism 22.4%, abnormal bone density 2.4%, and secondary diabetes mellitus 1.2%. These findings emphasize the need to screen for endocrine system dysfunction, particularly hypergonadotrophic hypogonadism, in children who have undergone BMT. Children need long-term follow-up so that endocrine complications can be diagnosed and treated promptly.

Vitamin D concentrations among healthy children in Calgary, Alberta

To examine the relationship between serum vitamin D concentrations, dietary intake and body mass index among healthy children living in Calgary, Alberta. The present cross-sectional study included healthy children two to 13 years of age who presented to the Alberta Children's Hospital for elective surgery during a 12-month period. Data including the child's weight, height, age, sex, ethnicity, dietary intake, use of vitamin supplements, physical activity and time spent outdoors were collected. Serum concentrations of 25-hydroxyvitamin D (25[OH]D) were measured using commercial immunoradiometric assay kits. Serum 25(OH)D concentrations were available for 1442 of 1862 participants, of whom 862 (59.8%) were boys. The mean (± SD) serum 25(OH)D concentration was 86.1±35.1 nmol/L (range 10 nmol/L to 323 nmol/L). Five hundred thirty-nine (37.4%) participants had insufficient vitamin D status (25[OH]D between 25 nmol/L and lower than 75 nmol/L), and vitamin D deficiency (25[OH]D 25 nmol/L or lower) was present in 29 subjects (2.0%). Children in the older age group (nine to 13 years) were more likely to have suboptimal vitamin D (P<0.001). Other risk factors significantly associated with suboptimal vitamin D status included overweight or obesity, nonwhite ethnicity, winter months, dietary vitamin D intake of less than 200 IU/day and less time spent outdoors. A high rate of suboptimal vitamin D concentrations was observed among the participants. Beyond promoting a vitamin D-enriched diet, physicians should also consider the body mass index and other risk factors to determine the optimal vitamin D intake for children living in the area studied.

Endocrine complications following pediatric bone marrow transplantation

Pediatric bone marrow transplantation (BMT) for various diseases can lead to endocrine system dysfunction owing to preparative regimens involving chemotherapy and radiation therapy. We assessed the prevalence of post-BMT endocrine complications in children treated at the Alberta Children's Hospital (ACH) from 1991 to 2001. Time of onset of endocrine dysfunction, underlying disease processes, chemotherapy, radiation therapy and age at BMT were characterized. Subjects of <18 years of age at the time of allogeneic or autologous BMT for whom 1-year follow-up through the ACH and a chart were available for review were included in the study. Subjects with a pre-existing endocrine condition were excluded. Of the 194 pediatric BMT procedures performed at the ACH between January 1, 1991 and December 31, 2001, 150 complete charts were available for review. Sixty five subjects received follow-up care at other centers and were excluded. Therefore, a total of 85 subjects were included in the review. The prevalence of endocrine complications identified was: primary hypothyroidism 1.2%, compensated hypothyroidism 7.0%, hyperthyroidism 2.4%, hypergonadotrophic hypogonadism 22.4%, abnormal bone density 2.4%, and secondary diabetes mellitus 1.2%. These findings emphasize the need to screen for endocrine system dysfunction, particularly hypergonadotrophic hypogonadism, in children who have undergone BMT. Children need long-term follow-up so that endocrine complications can be diagnosed and treated promptly.

The opportunities and obstacles in developing a vascular birthmark database for clinical and research use

Databases are useful tools in clinical settings. The authors review the benefits and challenges associated with the development and implementation of an efficient electronic database for the multidisciplinary Vascular Birthmark Clinic at the Alberta Children's Hospital, Calgary, Alberta. The content and structure of the database were designed using the technical expertise of a data analyst from the Calgary Health Region. Relevant clinical and demographic data fields were included with the goal of documenting ongoing care of individual patients, and facilitating future epidemiological studies of this patient population. After completion of this database, 10 challenges encountered during development were retrospectively identified. Practical solutions for these challenges are presented. THE CHALLENGES IDENTIFIED DURING THE DATABASE DEVELOPMENT PROCESS INCLUDED: identification of relevant data fields; balancing simplicity and user-friendliness with complexity and comprehensive data storage; database expertise versus clinical expertise; software platform selection; linkage of data from the previous spreadsheet to a new data management system; ethics approval for the development of the database and its utilization for research studies; ensuring privacy and limited access to the database; integration of digital photographs into the database; adoption of the database by support staff in the clinic; and maintaining up-to-date entries in the database. There are several challenges involved in the development of a useful and efficient clinical database. Awareness of these potential obstacles, in advance, may simplify the development of clinical databases by others in various surgical settings.

The Use of a Clinical Resource Nurse for Newly Graduated Nurses in a Pediatric Oncology Setting

The pediatric oncology nursing unit at the Alberta Children's Hospital experienced a large influx of new staff nurses between May 2008 and November 2008. There were 16 in total, and only a few had previous experience, whereas the majority was newly graduated nurses. As a solution to the high numbers of new staff nurses, the role of a Resource Nurse was developed as a temporary position to assist new staff nurses with their patient assignment, prioritize their day, and deal with complex patient procedures/treatments. Also, the Resource Nurse assisted all staff on the unit in dealing with increased patient acuity, chemotherapy administration, acuity issues, family teaching, and complicated family situations. A total of 55 prebooked shifts were scheduled from November 2008 to January 2009. A questionnaire was handed out to the staff nurses as a means to determine the effectiveness of having a Resource Nurse work on the unit. Twenty-three nurses responded by filling out the confidential questionnaire. Overall, respondents reported that the Resource Nurse was beneficial to their practice on the unit.