Nita Chahal

Transition to adult health care for adolescents and young adults with congenital heart disease: Perspectives of the patient, parent and health care provider

BACKGROUND Pediatric institutions play a large role in preparing young adults with congenital heart disease to transition to adult care. OBJECTIVE To determine the perspectives of patients, parents and providers on transition preparation. METHODS Patients aged nine to 18 years with congenital heart defects and their respective parent(s) participated in semistructured interviews. Health care providers completed a self-administered survey. RESULTS A total of 23 patients, 22 parents and 45 health care providers were enrolled in the study. Only 36% of patients demonstrated a clear understanding of transition and its implications for their cardiac care. Parents were extensively involved in care activities, with 95% accompanying their child to visits at the clinic, 68% staying with their child for the entire visit and 45% administering their medication. Children more knowledgeable about their diagnosis demonstrated a better understanding about their transition to adult care (100% versus 7%, respectively; P<0.01) and were more likely to communicate directly with their providers than those who were less or not knowledgeable (88% versus 33%, respectively; P=0.03). Nurses were more likely than physicians to view increased parental involvement in care activities as a barrier to transition preparation (37% versus 5%, respectively; P=0.02). CONCLUSIONS A lack of clear role expectations indicates a need for the pediatric health care setting to adapt appropriately to address the shifting needs of adolescent and young adult patients and their families. A formalized approach to transition preparation for adolescents with congenital heart disease needs to emphasize comprehensive education. A delegation of explicit responsibilities and the clear definition of roles for parents, providers and patients are necessary to provide young adults with the resources and support necessary to achieve a successful transition to adult care.

Sleep disturbance and cardiovascular risk in adolescents

Background: Evidence suggests that inadequate or disturbed sleep is associated with increased cardiovascular risk in adults. There are limited data on sleep quality and associated cardiovascular risk in children. Methods: We obtained data on adolescents from the 2009/10 cycle of the Healthy Heart Schools’ Program, a population-based cross-sectional study in the Niagara region of Ontario. Participants underwent measurements of cardiometabolic risk factors, including body mass index (BMI), lipid profile and blood pressure, and they completed questionnaires measuring sleeping habits and nutritional status. We assessed sleep disturbance using the sleep disturbance score derived from the Pittsburgh Sleep Quality Index. We explored associations between sleeping habits and cardiovascular risk factors. Results: Among 4104 adolescents (51% male), the mean hours of sleep per night (± standard deviation) were 7.9 ± 1.1 on weeknights and 9.4 ± 1.6 on weekends. In total, 19% of participants reported their sleep quality as fairly bad or very bad on weeknights and 10% reported it as fairly bad or very bad on weekends. In the multivariable regression models, a higher sleep disturbance score was associated with increased odds of being at high cardiovascular risk (highest v. lowest tertile odds ratio [OR] 1.43 [95% confidence interval (CI) 1.16–1.77], p < 0.001), increased odds of hypertension (highest v. lowest tertile OR 1.44 [95% CI 1.02–2.05], p = 0.05) and increased odds of elevated non-high density lipoprotein cholesterol (highest v. lowest tertile OR 1.28 [95% CI 1.00–1.64], p = 0.05). The mean duration of sleep was not associated with these outcomes. Interpretation: In healthy adolescents, sleep disturbance is associated with cardiovascular risk factor abnormalities. Intervention strategies to optimize sleep hygiene early in life may be important for the prevention of cardiovascular disease.

Kawasaki Disease at the Extremes of the Age Spectrum

OBJECTIVE: We sought to determine outcomes of Kawasaki disease (KD) and to explore factors associated with poor clinical outcomes for patients diagnosed outside the age range of 1 to 4 years. METHODS: A retrospective review of data for all patients seen between January 1990 and April 2007 was performed. Patients were stratified into 5 groups on the basis of age at diagnosis. RESULTS: A total of 1374 patients were identified; 61 (4%) were <6 months of age at diagnosis, 114 (8%) 6 months to <1 year, 854 (62%) 1 to 4 years, 258 (19%) 5 to 9 years, and 87 (6%) >9 years. Patients <1 year of age and those >9 years of age were more likely to have coronary artery abnormalities than were patients diagnosed between 1 and 4 years of age. Patients diagnosed between the ages of 5 and 9 years were at the lowest risk. Patients at both extremes of the age spectrum were more likely to present with <4 of the classic KD features, but only those <6 months or >5 years of age were at increased risk of being diagnosed >12 days after illness onset. Patients <6 months of age had lower albumin levels, and those <1 year of age had higher white blood cell and platelet counts, all of which are known predictors of coronary artery abnormalities. Patients >9 years of age were less likely to receive intravenous immunoglobulin treatment. CONCLUSION: Outcomes for children diagnosed with KD at either extreme of the age spectrum are suboptimal, although the associated factors are different.

Repeated systematic surveillance of Kawasaki disease in Ontario from 1995 to 2006

Background:  Rising incidences of Kawasaki disease (KD) have been reported worldwide. Reported herein are the results of 4 triennial KD surveillances conducted in Ontario.

Spectrum and Management of Hypertriglyceridemia Among Children in Clinical Practice

OBJECTIVES. The prevalence and identification of hypertriglyceridemia in youths will likely will increase in the future as a consequence of childhood obesity and increased screening for dyslipidemias. We sought to review our clinical experience with hypertriglyceridemia, evaluate factors associated with increased triglyceride levels, and review treatment options to provide guidance for management. METHODS. Clinical review of data for all patients who had ≥1 elevated triglyceride level (>4 mmol/L [>350 mg/dL]) while being monitored in a specialized lipid disorders clinic was performed. RESULTS. The study population consisted of 76 patients with 761 clinic visits. Hypertriglyceridemia was secondary to lifestyle factors for 13 patients. The rest had primary hypertriglyceridemia, with 32 patients having familial combined hypertriglyceridemia and hypercholesterolemia (type II), 25 patients having primary hypertriglyceridemia (type IV), 4 patients having familial lipase deficiency (type I), and 2 patients having hyperlipoproteinemia E2/E2 phenotype (type III). Triglyceride levels were highest in type I and III hypertriglyceridemia (>10 mmol/L [>900 mg/dL]), followed by type IV and adiposity-related hypertriglyceridemia (>4 mmol/L [>350 mg/dL]) and finally type II familial combined hypertriglyceridemia and hypercholesterolemia (>2 mmol/L [>180 mg/dL]). A total of 34 patients received 37 trials of drug therapy as part of triglyceride level management (bile acid–binding resins, n = 12; fibrates, n = 19; statins, n = 6). Triglyceride levels were found to decrease over time with the use of fibrates, to increase with the use of bile acid–binding resins, and not to change with the use of statins. CONCLUSIONS. Lifestyle modifications remain the primary therapeutic avenue for the management of pediatric hypertriglyceridemia. We propose an algorithm for the management of this heterogeneous population to guide clinicians in their treatment decisions.

Comparison of Factors Associated With Coronary Artery Dilation Only Versus Coronary Artery Aneurysms in Patients With Kawasaki Disease

We sought to determine whether differences exist in the factors associated with transient coronary artery (CA) dilation only compared to CA aneurysms after Kawasaki disease (KD). We reviewed all patients with KD assessed and treated at a single tertiary institution from January 1990 to April 2007. Of 1,374 patients (63% men) with KD, 1,108 (81%) had no CA abnormalities; 180 (13%) had CA dilation only, and 86 (6%) had CA aneurysms. The factors associated with any CA abnormality included male gender (odds ratio [OR] 1.8, p <0.001), lower albumin (OR per 1 g/dl, 1.07; p < 0.001), lower hemoglobin (OR per 10 g/dl; 1.02, p = 0.008), diagnosed outside of the 1 to 9-year-age range (OR for <1 year old, 1.5; p = 0.04; OR for >9 years old, 1.9, p = 0.03), longer duration of fever before treatment (OR per day, 1.05, p = 0.002), greater platelet count (OR per 10 x 10(9)/L, 1.14, p = 0.008), and nonresponse to initial intravenous immunoglobulin treatment (OR 2.4, p <0.001). Only age at diagnosis, duration of fever before treatment, greater platelet count, and nonresponse to initial intravenous immunoglobulin discriminated between CA dilation only versus CA aneurysms (c-statistic, 0.80, p <0.001). In conclusion, CA dilation only and CA aneurysms differ only in the total duration of inflammation, as measured by the number of days from fever onset to defervescence.

Population Trends Toward Increasing Cardiovascular Risk Factors in Canadian Adolescents

OBJECTIVE To determine prevalence and cross-sectional trends over time for cardiovascular risk factors in Canadian adolescents. STUDY DESIGN Cross-sectional trends in cardiovascular risk and lifestyle factors were gathered annually in 14- to 15-year-old students in the Niagara region, Ontario, Canada. RESULTS A total of 20 719 adolescents were screened between 2002 and 2008. The proportion of obese adolescents (>95th percentile for body mass index [BMI]) increased significantly, by +0.34%/year (P = .002). The proportions of adolescents with borderline high cholesterol (4.4-5.1 mmol/L) (+0.57%/year; P <.001) and with high cholesterol (≥5.2 mmol/L) (+0.43%/year; P <.001) both increased significantly over time. The proportion of adolescents with prehypertension decreased by -0.23%/year (P = .02), whereas the proportion of those with stage I hypertension (5%-6%) or stage II hypertension (2%-4%) remained constant. The proportion of adolescents classified as being at high cardiovascular risk increased by +0.67%/year (P <.001). Family history, low levels of physical activity, sedentary behaviors, poor nutrition, and lower socioeconomic status were all independently and negatively associated with all aspects of cardiovascular risk. CONCLUSIONS A significant proportion of 14- to 15-year-old Canadian adolescents have at least one cardiovascular risk factor, and the cross-sectional trends worsened during the period 2002-2008.

Macrophage activation syndrome in the acute phase of Kawasaki disease.

Rare cases of macrophage activation syndrome (MAS) occurring during the acute phase of Kawasaki disease (KD) have been reported. We sought to characterize, review treatment, and outcomes of KD patients with clinical features of MAS. Medical histories of patients treated for KD and MAS between January 2001 and March 2008 were reviewed. Of 638 KD patients seen, 12 (1.9%) had additional clinical findings usually associated with MAS; 7 of them were males older than 5 years (6.1%; odds ratio: 6.8, P=0.002). Clinically, 9 patients had at least 4 of 5 KD clinical signs, and all patients had prolonged fever beyond initial intravenous immunoglobulin treatment. Hepatosplenomegaly, cytopenia in two or more cell lines, hypertriglyceridemia and/or hypofibrinogenemia, and increased D-dimers were seen in 11 patients. Hyperferritinemia and elevated hepatic enzymes were seen in all patients. Four patients had biopsy-proven evidence of hemophagocytosis. All but 2 patients met at least 5 of 8 criteria necessary for MAS diagnosis. Treatment beyond the standard KD protocol (aspirin + intravenous immunoglobulin) was necessary in all but 1 patient. All patients eventually recovered with no long-term sequelae. A high index of suspicion for clinical features associated with MAS is warranted for KD patients to provide appropriate and timely treatment.

Association Between Parental Anxiety and Compliance With Preoperative Requirements for Pediatric Outpatient Surgery

PURPOSE The purpose of this study was to determine if parental anxiety interferes with the ability to follow preoperative requirements. METHOD In a single center observational study of parents of children admitted to a same-day surgical unit at a tertiary pediatric hospital, parental preoperative anxiety was measured by the State-Trait Anxiety Inventory (STAI) questionnaire. Anxiety was correlated to a four points assessment of adherence with the following preoperative requirements: dietary restrictions, timely arrival at the hospital, arrival at the assigned room, and completion of required medical forms, because those requirements are the greatest external contributors to surgical cancellation according to hospital statistics. RESULTS A total of 203 families completed the study. The average STAI score on the day of surgery was 38+/-12 (population average, 36+/-11). Only 130 families (66%) complied with all four preoperative requirements. A higher level of anxiety was significantly associated with lower probability of compliance (odds ratio, 0.88; 95% confidence interval, 0.78-1.00, P=05). In univariate models, factors associated with higher STAI scores included younger parent age, younger children, only child, childs first surgery, and no medical consultation between surgical assessment and surgery. DISCUSSION Parental anxiety could be associated with a lower likelihood of parents following preoperative requirements and could contribute to increased likelihood of surgical cancellation.

Intravenous immunoglobulin preparation type: association with outcomes for patients with acute Kawasaki disease.

Manlhiot C, Yeung RSM, Chahal N, McCrindle BW. Intravenous immunoglobulin preparation type: Association with outcomes for patients with acute Kawasaki disease.
Pediatr Allergy Immunol 2010: 21: 515–521.
© 2010 John Wiley & Sons A/S