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To assess the stability of the Gross Motor Functional Classification System (GMFCS) in children with cerebral palsy (CP) from time of preliminary diagnosis (~2 years of age) to time of diagnosis (~5 years of age), and to examine factors associated with reclassification.We conducted a longitudinal study using a sample from the Canadian CP Registry. Stability was analysed by using the percentage of agreement between timepoints and a weighted prevalence and bias adjusted kappa statistic. Univariate and multivariate logistic regressions were performed to identify variables associated with reclassification.The study included 1670 children (857 males, 713 females) with a mean age of 11 years 4 months (SD 4 years, range 3 years 5 months-20 years 1 month) at time of data extraction (3rd September 2019), of which 1435 (85.9%) maintained a stable GMFCS, with a weighted kappa of 0.91 (95% confidence interval 0.89-0.92). Univariate logistic regression showed that initial GMFCS level, CP subtype, and the presence of cognitive impairment were associated with the likelihood of change in the GMFCS level (p 0.1). In the multivariate analysis, however, the likelihood was associated with initial GMFCS level only (odds ratio 7.10-8.88, p 0.00).The GMFCS has good stability in early childhood. For the majority of children, it is predictive of their long-term motor function.The Gross Motor Function Classification System (GMFCS) rating in early childhood is stable over time. There is no directionality in the reclassification of the GMFCS. The initial GMFCS level was related to the likelihood of change in follow-up GMFCS level.

Aim To determine whether inequities in health outcomes for Indigenous Canadians are also present in cerebral palsy (CP) by comparing CP profiles between Indigenous and non-Indigenous children. Method Using the Canadian Cerebral Palsy Registry, we conducted a cross-sectional study. CP motor subtype, gross motor severity, comorbidities, perinatal adversity, preterm birth, and parental education were compared between 94 Indigenous (53 males, 41 females) and 1555 non-Indigenous (891 males, 664 females) children (all >5y). Multivariate analysis was done to analyze adverse CP factors, defined as CP gross motor severity and comorbidities. CP etiologies, either prenatal/perinatal or postnatal, were also compared. Results Indigenous children with CP have higher odds of having low parental education (odds ratio [OR] 6.15, 95% confidence interval [CI] 3.36-11.3) and comorbidities (OR 4.46, 95% CI 1.62-12.3), especially cognitive (OR 4.52, 95% CI 2.27-9.05), communication (OR 2.66, 95% CI 1.54-4.61), and feeding (OR 2.25, 95% CI 1.33-3.83) impairment. Indigenous children also have higher CP gross motor severity (p=0.03). Indigenous children are also more likely to have non-accidental head injury (n=4; OR 8.18, 95% CI 1.86-36.0) as the cause of their postnatal CP. Interpretation Indigenous populations have worse health outcomes as a result of intergenerational impacts of colonization. Our study shows that Indigenous children with CP have increased comorbidities and higher CP gross motor severity, reinforcing the need for a multidisciplinary approach to management. Furthermore, targeted prevention programs against preventable causes of CP, such as non-accidental head injury, may be beneficial. What this paper adds Indigenous children with cerebral palsy (CP) have more severe motor impairment and more comorbidities. Non-accidental head injury is a significant cause of postnatal CP.

BACKGROUND AND OBJECTIVES Arteriovenous fistulae (AVF) and grafts (AVG) are preferred permanent vascular access (PVA) for chronic hemodialysis (HD) patients. Our objective was to examine the change in markers of HD efficacy after successful establishment of a PVA among children who started HD with a tunneled cuffed catheter (TCC). MATERIALS AND METHODS Retrospective chart reviews were completed on patients from 20 pediatric dialysis centers. All patients used TCC prior to AVF/AVG, and each patient acted as his/her own control. Data on markers of HD efficacy (single-pool Kt/V, urea reduction ratio (URR), serum albumin and hematocrit (Hct)) were collected at the creation of AVF/AVG and for 2 years thereafter. Statistical methods included hypothesis testing and statistical modeling after adjusting for relevant demographic variables. RESULTS First PVA was created in 98 individual children: 87 (89%) were AVF and 11 (11%) were AVG. The mean TCC vintage prior to AVF/AVG was 10.4 ± 17.3 months. At 1-year follow-up, Kt/V improved by 0.15 ± 0.06 (p = 0.02) and URR improved by 4.54 ± 1.17% (p

Objective:To describe complementary and alternative medicine (CAM) use amongst children with cerebral palsy (CP) in Canada and to identify factors associated with CAM use.Methods:We conducted a cross-sectional study, utilising data from the Canadian CP Registry. We explored the association between CAM use and regional, socioeconomic and CP phenotypic variables, and parental perception of the family-centredness of clinical care using the Measures of Process of Care-56 (MPOC-56). Chi-square analyses were performed, and odds ratios (OR) and 95% confidence intervals (CI) were obtained. Mann–WhitneyUtests were used to compare MPOC-56 scores between CAM users and non-CAM users.Results:The study sample consisted of 313 families of which 27% reported CAM use in the past year. Children with CP using CAM were more likely to reside in Western Canada (OR 3.3, 95% CI 1.6–6.7), live in a two-parent household (OR 3.5, 95% CI 1.5–8.4), have an ataxic/hypotonic or dyskinetic CP subtype (OR 3.0, 95% CI 1.5–6.1) and have a greater motor impairment (OR 2.8, 95% CI 1.7–4.9). MPOC-56 subscale scores were not significantly associated with CAM use.Conclusion:Physicians need to be aware of existing CAM therapies, the level of evidence supporting their efficacy (beneficence), their associated risks of adverse events (non-maleficence) and enable fair access to care that may be of benefit to each child.

RATIONALE & OBJECTIVE: The KDIGO (Kidney Disease: Improving Global Outcomes) guideline on CKD presented an international classification system that ranks patients' risk for CKD progression. Few data on children informed guideline development STUDY DESIGN: Observational cohort study SETTINGS AND PARTICIPANTS: Children aged 1-18 years enrolled in the North American Chronic Kidney Disease in Children (CKiD) cohort study and the European Effect of Strict Blood Pressure Control and ACE Inhibition on the Progression of CRF in Pediatric Patients (ESCAPE) trial. PREDICTOR: Level of estimated glomerular filtration rate (eGFR) and proteinuria (urine protein-creatinine ratio (mg/mg)) at study entry OUTCOME: A composite event of renal replacement therapy, 50% reduction of estimated GFR (eGFR), or eGFR 2.0 at study entry. Six ordered stages with varying combinations of eGFR categories (60-89, 45-59, 30-44 and 15-29 ml/min/1.73m(2)) and UPCR categories (2.0) described the risk continuum. Median times to event ranged from >10 years for eGFR 45-90 and UPCR 2. Children with glomerular disease were estimated to have a 43% shorter time to event than children with nonglomerular disease. Cross-validation demonstrated risk patterns that were consistent across the ten subsample validation models. LIMITATIONS: Observational study, utilized cross validation rather than external validation CONCLUSION: CKD staged by level of eGFR and proteinuria characterizes the timeline of progression and can guide management strategies in children.

Courthouse facility dogs are increasingly used to support child witnesses and complainants during testimony in courtrooms across the globe. Although already commonplace in the United States, this practice has been largely unexplored in the Australian context. This paper puts forward the case for courthouse facility dogs in Australia and offers some insight into striking the delicate balance between protecting defendants’ rights to a fair trial, and improving the efficiency and quality of the legal system for vulnerable witnesses and victims.

Permanent vascular access (PVA) is preferred for long-term hemodialysis. Arteriovenous fistulae (AVF) have the best patency and the lowest complication rates compared to arteriovenous grafts (AVG) and tunneled cuffed catheters (TCC). However, AVF need time to mature. This study aimed to investigate predictors of time to first cannulation for AVF in pediatric hemodialysis patients. Data on first AVF and AVG of patients at 20 pediatric dialysis centers were collected retrospectively, including demographics, clinical information, dialysis markers, and surgical data. Statistical modeling was used to investigate predictors of outcome. First PVA was created in 117 children: 103 (88%) AVF and 14 (12%) AVG. Mean age at AVF creation was 15.0 ± 3.3 years. AVF successfully matured in 89 children (86.4%), and mean time to first cannulation was 3.6 ± 2.5 months. In a multivariable regression model, study center, age, duration of non-permanent vascular access (NPVA), and Kt/V at AVF creation predicted time to first cannulation, with study center as the strongest predictor (p < 0.01). Time to first cannulation decreased with increasing age (p = 0.03) and with increasing Kt/V (p = 0.01), and increased with duration of NPVA (p = 0.03). Secondary failure occurred in 10 AVF (11.8%). Time to first cannulation did not predict secondary failure (p = 0.29), but longer time to first cannulation tended towards longer secondary patency (p = 0.06). Study center is the strongest predictor of time to first cannulation for AVF and deserves further investigation. Time to first cannulation is significantly shorter in older children, with more efficient dialysis treatments, and increases with longer NPVA duration.

Introduction/Background Lyme disease, a tick-borne zoonosis caused by the bacterium Borrelia burgdorferi, has emerged in Nova Scotia (NS) as a common illness. Since 2002 when Lyme disease was first diagnosed in NS, >1000 cases have been reported. Seventh cranial nerve palsy (CNP-7) is said to be the most common presentation of early disseminated Lyme disease in children in endemic areas. Objectives We aimed to determine the frequency of CNP-7 in NS and if physicians are considering Lyme disease as an etiology. Design/Methods A retrospective review of health records of children seen at the IWK Health Centre from 2000-2018 who were ≤18 years of age with an ICD-9 or 10 diagnosis of Bell’s palsy (CNP-7) was conducted. CNP-7 due to local infection, trauma, malignancy, or systemic neurologic disease was excluded. Results Of 237 ICD “Bell’s palsy” diagnoses, 66 cases were eligible, of which 60.6 % (n=40) were female. The median age was 10 years (range 0-16). Five cases of Lyme disease-associated CNP-7 were recognized (7.6%), all since 2013. No bilateral CNP-7 occurred; 59.1% of cases were on the left. Most children presented within 3 days of symptom onset (84.8%) to the emergency department (95.4%), and 56.1% subsequently saw a pediatric neurologist. The most common associated symptom with CNP-7 was headache (22.7%). Lyme disease was considered in the differential diagnosis in 34.8 % (n=23) of cases, and only since 2012. Systemic steroids were prescribed to 51.5 % (34/66) of children in the emergency department, for durations varying from 1 to 10 days. The most common steroid course length was 5 days. Antimicrobials were prescribed for 18 (27.3%) children including acyclovir, beta lactams and tetracyclines. Resolution of the facial palsy findings was documented in 45 children, of whom 36 (54.5%) had complete resolution and 9 (13.6%) had partial resolution. Four children with Lyme disease associated CNP-7 had complete resolution, and one had partial resolution. Conclusion There does not appear to be a standard approach to diagnosis and management of CNP-7 in this pediatric health centre. Lyme disease is not regularly considered in the differential diagnosis, which is surprising given the high incidence of Lyme disease in NS. These findings will be shared with health care providers most likely to see CNP-7 in order to develop a standard algorithm to the initial presentation of 7th cranial nerve palsy in children.

ObjectiveTo specifically report on ataxic-hypotonic cerebral palsy (CP) using registry data and to directly compare its features with other CP subtypes.MethodsData on prenatal, perinatal, and neonatal characteristics and gross motor function (Gross Motor Function Classification System [GMFCS]) and comorbidities in 35 children with ataxic-hypotonic CP were extracted from the Canadian Cerebral Palsy Registry and compared with 1,804 patients with other subtypes of CP.ResultsPerinatal adversity was detected significantly more frequently in other subtypes of CP (odds ratio [OR] 4.3, 95% confidence interval [CI] 1.5–11.7). The gestational age at birth was higher in ataxic-hypotonic CP (median 39.0 weeks vs 37.0 weeks,p= 0.027). Children with ataxic-hypotonic CP displayed more intrauterine growth restriction (OR 2.6, 95% CI 1.0–6.8) and congenital malformation (OR 2.4, 95% CI 1.2–4.8). MRI was more likely to be either normal (OR 3.8, 95% CI 1.4–10.5) or to show a cerebral malformation (OR 4.2, 95% CI 1.5–11.9) in ataxic-hypotonic CP. There was no significant difference in terms of GMFCS or the presence of comorbidities, except for more frequent communication impairment in ataxic-hypotonic CP (OR 4.2, 95% CI 1.5–11.6).ConclusionsOur results suggest a predominantly genetic or prenatal etiology for ataxic-hypotonic CP and imply that a diagnosis of ataxic-hypotonic CP does not impart a worse prognosis with respect to comorbidities or functional impairment. This study contributes toward a better understanding of ataxic-hypotonic CP as a distinct nosologic entity within the spectrum of CP with its own pathogenesis, risk factors, clinical profile, and prognosis compared with other CP subtypes.

Aim To identify characteristics of young children with cerebral palsy (CP), and intrinsic and extrinsic factors, that may be associated with parental perceptions regarding family-centred health care services. Method We conducted a cross-sectional study, drawing our sample from the Canadian Cerebral Palsy Registry (CCPR). Parents rated the extent of family-centred care provided by their child's health care teams using the 56-item Measures of Process of Care (MPOC) questionnaire. Environmental and CP phenotypic variables were extracted from the CCPR for group comparisons. Low and high MPOC-56 raters were also compared. Results Valid responses were obtained from 282 families (90%). All MPOC-56 subscales were highly rated (median ≥6.0), indicating satisfaction with health care services, with the exception of the Providing General Information subscale (median 4.8, interquartile range 3.2-6.0). Parents from Nova Scotia rated all subscales significantly higher than parents from other regions. CP subtype and severity were not significantly associated with MPOC-56 subscale scores. Higher socio-economic status was associated with lower MPOC-56 subscale scores. Higher paternal educational attainment and household income were significantly associated with lower scores on the Providing General Information and Providing Specific Information about the Child subscales respectively. Interpretation Participants affirmed the provision of family-centred services from Canadian pediatric rehabilitation centres. Sociodemographic factors were associated with parental perceptions of family-centred services. What this paper adds Sociodemographic factors were associated with parental perceptions of family-centred care. Factors intrinsic to the child's cerebral palsy were not associated with parental perceptions.

Hemodialysis (HD) guidelines recommend permanent vascular access (PVA) in children unlikely to receive kidney transplant within 1 year of starting HD. We aimed to determine predictors of primary and secondary patency of PVA in pediatric HD patients. Retrospective chart reviews were performed for first PVAs in 20 participating centers. Variables collected included patient demographics, complications, interventions, and final outcome. There were 103 arterio-venous fistulae (AVF) and 14 AV grafts (AVG). AVF demonstrated superior primary (p = 0.0391) and secondary patency (p = 0.0227) compared to AVG. Primary failure occurred in 16 PVA (13.6%) and secondary failure in 14 PVA (12.2%). AVF were more likely to have primary failure (odds ratio (OR) = 2.10) and AVG had more secondary failure (OR = 3.33). No demographic, clinical, or laboratory variable predicted primary failure of PVA. Anatomical location of PVA was predictive of secondary failure, with radial having the lowest risk compared to brachial (OR = 12.425) or femoral PVA (OR = 118.618). Intervention-free survival was predictive of secondary patency for all PVA (p = 0.0252) and directly correlated with overall survival of AVF (p = 0.0197) but not AVG. Study center demonstrated statistically significant effect only on intervention-free AVF survival (p = 0.0082), but not number of complications or interventions, or outcomes. In this multi-center pediatric HD cohort, AVF demonstrated primary and secondary patency advantages over AVG. Radial PVA was least likely to develop secondary failure. Intervention-free survival was the only predictor of secondary patency for AVF and directly correlated with overall access survival. The study center effect on intervention-free survival of AVF deserves further investigation.

To study the effects of single versus serial casting post-Botulinum toxin A (BoNT-A) injections on hypoextensibility of triceps surae in children, 2-7 years old, with cerebral palsy and equinus gait.A randomized, stratified, parallel, two-group trial was conducted at a pediatric health center with assessments at baseline, precast, postcast and, 1-, 2-, and 6-month follow-ups. One week following BoNT-A injections into triceps surae muscle, a single below-knee cast (n = 10) or 3 serial casts (n = 10) were applied for 3 weeks. Primary outcome measure was the Modified Tardieu Scale (MTS), secondary outcome measures were Modified Ashworth Scale (MAS), GAITRite™, Gross Motor Function Measure-66 (GMFM-66), and Pediatric Evaluation of Disability Inventory (PEDI).Significant effects of time, but not group-by-time, were found for MTS R1 (P0.001), MTS R2 (P0.001), MAS (P = 0.001), GMFM-66 (P = 0.002), and PEDI (P0.001-0.009). One participant who received a single cast did not complete the 6-month assessment.Magnitudes of improvements were similar using single or serial casting. If these findings are corroborated in a larger scale study, the recommendation of a single cast may be appropriate due to its greater convenience for families and clinicians.

BACKGROUND: Few prospective studies have systematically evaluated the extent of recovery from incident acquired demyelinating syndromes (ADS) of the central nervous system in children. METHODS: In a national cohort study of pediatric ADS, severity of the incident attack and extent of recovery by 12 months were evaluated. Annual evaluations were used to determine current diagnoses (monophasic ADS or multiple sclerosis [MS]) and new deficits. RESULTS: Of 283 children, 244 (86%) required hospitalization for a median (interquartile range [IQR]) of 6 (3–10) days, and 184 had moderate or severe deficits; 41 children were profoundly encephalopathic, 129 were unable to ambulate independently, and 59 with optic neuritis (ON) had moderately or severely impaired vision. Those with transverse myelitis (TM) and patients with monophasic disease were more likely to have moderate or severe deficits at onset. Twenty-seven children (10%) did not experience full neurologic recovery from their incident attack; 12 have severe residual deficits. Monophasic illness, TM, and moderate or severe deficits at onset were associated with poor recovery. After a median (IQR) follow-up of 5.06 (3.41–6.97) years, 59 children (21%) were diagnosed with MS; all recovered fully from their incident ADS attacks, although 6 subsequently acquired irreversible deficits after a median (IQR) observation period of 5.93 (4.01–7.02) years. CONCLUSIONS: ADS is a serious illness, with 86% of affected Canadian children requiring hospitalization. More than 90% of children recovered physically from their ADS event, including those children experiencing onset of MS. However, permanent visual or spinal cord impairment occurred in some children with ON or TM.

Summary Background MRI and laboratory features have been incorporated into international diagnostic criteria for multiple sclerosis. We assessed the pattern of MRI lesions and contributions of cerebrospinal fluid (CSF) and serum antibody findings that best identifies children with multiple sclerosis, and the applicability of international diagnostic criteria in the paediatric context. Methods In this prospective cohort study, detailed clinical assessments, serum and CSF studies, and MRI scans were done in youth (aged 0·46–17·87 years) with incidental acquired demyelinating syndrome. Participants were examined prospectively to identify relapsing disease. All MRI scans were assessed using a validated scoring method. A random forest classifier identified imaging and laboratory features that best predicted a multiple sclerosis or monophasic outcome. Performance of the 2001, 2010, and 2017 international McDonald criteria for the diagnosis of multiple sclerosis, the 2016 MRI in multiple sclerosis (MAGNIMS) criteria, and our 2011 proposed (Verhey) criteria were determined; performance was adjudicated with generalised linear models. Findings Between Sept 1, 2004, and June 30, 2017, we included 324 participants with median follow-up of 72 months (range 6–150), 71 (22%) participants with multiple sclerosis, 237 (73%) with monophasic acquired demyelinating syndrome, 14 (4%) with relapsing non-multiple sclerosis, and two (1%) with alternative diagnoses. We scored 2391 brain, 444 spinal, and 67 dedicated orbital MRI scans. One or more T1 hypointense lesions plus one or more periventricular lesions (Verhey criteria) best predicted multiple sclerosis outcome. Performance of the 2017 McDonald criteria was comparable to the 2010 McDonald criteria and was easier to adjudicate. The ability of CSF oligoclonal bands to substitute for the requirement for both enhancing and non-enhancing lesions in the 2017 McDonald criteria improved its performance compared with the 2010 criteria. Myelin oligodendrocyte testing at baseline did not improve performance of the 2017 McDonald criteria. Interpretation The 2017 McDonald criteria for the diagnosis of multiple sclerosis, as applied at the time of incident attack, perform well in identifying children and youth with multiple sclerosis, indicating that the same diagnostic criteria for multiple sclerosis apply across the age span. The presence of so-called black holes on MRI and periventricular lesions at baseline (Verhey criteria) also effectively distinguish children with multiple sclerosis from children with monophasic demyelination. The presence of CSF oligoclonal bands improve diagnostic accuracy. Myelin oligodendrocyte glycoprotein antibodies identify children with acute disseminated encephalomyelitis, and those with relapsing non-multiple sclerosis, most of whom do not meet 2017 McDonald criteria at onset. Funding The Multiple Sclerosis Scientific Research Foundation and The Children's Hospital of Philadelphia.

BACKGROUND: Pediatric arterial ischemic stroke remains incompletely understood. Population-based epidemiological data inform clinical trial design but are scant in this condition. We aimed to determine age-specific epidemiological characteristics of arterial ischemic stroke in neonates (birth to 28 days) and older children (29 days to 18 years). METHODS: We conducted a 16-year, prospective, national population-based study, the Canadian Pediatric Ischemic Stroke Registry, across all 16 Canadian acute care children's hospitals. We prospectively enrolled children with arterial ischemic stroke from January 1992 to December 2001 and documented disease incidence, presentations, risk factors, and treatments. Study outcomes were assessed throughout 2008, including abnormal clinical outcomes (stroke-related death or neurological deficit) and recurrent arterial ischemic stroke or transient ischemic attack. RESULTS: Among 1129 children enrolled with arterial ischemic stroke, stroke incidence was 1.72/100,000/year, (neonates 10.2/100,000 live births). Detailed clinical and radiological information were available for 933 children (232 neonates and 701 older children, 55% male). The predominant clinical presentations were seizures in neonates (88%), focal deficits in older children (77%), and diffuse neurological signs (54%) in both. Among neonates, 44% had no discernible risk factors. In older children, arteriopathy (49% of patients with vascular imaging), cardiac disorders (28%), and prothrombotic disorders (35% of patients tested) predominated. Antithrombotic treatment increased during the study period (P < 0.001). Stroke-specific mortality was 5%. Outcomes included neurological deficits in 60% of neonates and 70% of older children. Among neonates, deficits emerged during follow-up in 39%. Overall, an initially decreased level of consciousness, a nonspecific systemic presentation, and the presence of stroke risk factors predicted abnormal outcomes. For neonates, predictors were decreased level of consciousness, nonspecific systemic presentation, and basal ganglia infarcts. For older children, predictors were initial seizures, nonspecific systemic presentation, risk factors, and lack of antithrombotic treatment. Recurrent arterial ischemic stroke or transient ischemic attack developed in 12% of older children and was predicted by arteriopathy, presentation without seizures, and lack of antithrombotic treatment. Emerging deficit was predicted by neonatal age at stroke and by cardiac disease. CONCLUSIONS: This national data set provides a population-based disease incidence rate and demonstrates the protective effect of antithrombotic treatment in older children, and frequent long-term emerging deficits in neonates and in children with cardiac disorders. Further clinical trials are required to develop effective age-appropriate treatments for children with acute arterial ischemic stroke.

The original version of this article unfortunately contained a mistake. The name of Vimal Chadha was presented incorrectly. The corrected author list is given above.

Understanding what patients and their parents want is essential to plan appropriate patient-centered care. Questionnaires were distributed to 500 consecutive children and parents seen for their first pediatric neurology consultation. Both patients and their families answered questions about their expectations of the consultation, their level of worry, and the Penn State Worry Questionnaire. The 5 most important issues for the parents were to get information, to work with the doctor to manage the problem, to have questions answered, to find out what was wrong, and to discuss the impact on the child’s life. The children had very similar priorities. The 5 least important concerns for parents were to get a prescription, blood tests, to talk to others with similar problems, to get a radiograph/computed tomography/magnetic resonance imaging (MRI) and to be told nothing is wrong. The pediatric neurologists did well in anticipating these priorities but had more difficulty appreciating parent and patient level of worry.

Pediatric intracranial calcification may be caused by inherited or acquired factors. We describe the identification of a novel rearrangement in which a downstream pseudogene translocates into exon 9 of OCLN, resulting in band-like brain calcification and advanced chronic kidney disease in early childhood. SNP genotyping and read-depth variation from whole exome sequencing initially pointed to a mutation in the OCLN gene. The high degree of identity between OCLN and two pseudogenes required a combination of multiplex ligation-dependent probe amplification, PCR, and Sanger sequencing to identify the genomic rearrangement that was the underlying genetic cause of the disease. Mutations in exon 3, or at the 5-6 intron splice site, of OCLN have been reported to cause brain calcification and polymicrogyria with no evidence of extra-cranial phenotypes. Of the OCLN splice variants described, all make use of exon 9, while OCLN variants that use exons 3, 5, and 6 are tissue specific. The genetic rearrangement we identified in exon 9 provides a plausible explanation for the expanded clinical phenotype observed in our individuals. Furthermore, the lack of polymicrogyria associated with the rearrangement of OCLN in our patients extends the range of cranial defects that can be observed due to OCLN mutations.

Background Children and youth with epilepsy have long been subjected to excessive restrictions on extracurricular activities due to concerns over risk of injury. Over time physicians and medical regulatory associations have liberalized the advice given for people with epilepsy to promote independence, self-esteem and general health benefits of physical activity. Current evidence suggests that few restrictions are needed for children with epilepsy beyond water-related precautions and avoidance of very high-risk activities. However, more stringent restrictions on daily activities may be imposed by caregivers. This study was aimed at exploring current perceptions of parents regarding restrictions on activity for children with epilepsy and the child’s perspective on restrictions related to the diagnosis. Methods A self-administered questionnaire was offered to a sample of parent-child dyads of children/youth with epilepsy attending summer camp for children with epilepsy age 8–18 years. A 10-item validated HARCES Parent Scale of Childhood Epilepsy was completed by the parent/guardian and a modified-HARCES completed by the child. The primary objective was to assess the degree of restrictions placed on children with epilepsy from the perspective of child and parent assessed independently. Agreement of perceived restrictions between parent-child dyads was also determined. Results 21 parent/guardian-child pairs were recruited with mean age of children/youth 12.7 years (range 9–16 years). Total HARCES scores for parents and guardians ranged from 11–26 (x = 16.5; SD 4.9) while total scores for children with epilepsy similarly ranged from 10–25 (x = 15.2; SD 4.9). There were no differences in total parent scores when analyzed by child's age ( 13 years), gender, age of seizure onset, seizure frequency or seizure type. Total HARCES scores showed no agreement between parent and child pairs with correlation of 0.2798 (95% CI − 0.173–0.635). Conclusions Children and youth with epilepsy often face activity restrictions based on fear of perceived risk of injury. This small sample shows evidence that even more permissive parents and his/her children still feel limited by such restrictions. Parents and children do not perceive these restrictions in the same way despite similar education by physicians highlighting an important secondary role of epilepsy camps in targeting misperceptions and educating families on appropriate precautions.

Background Advanced maternal age is associated with higher frequencies of antenatal and perinatal conditions, as well as a higher risk of cerebral palsy in offspring. We explore the association between maternal age and specific cerebral palsy risk factors. Methods Data were extracted from the Canadian Cerebral Palsy Registry. Maternal age was categorized as ≥35 years of age and less than 20 years of age at the time of birth. Chi-square and multivariate logistic regressions were performed to calculate odds ratios and their 95% confidence intervals. Results The final sample consisted of 1391 children with cerebral palsy, with 19% of children having mothers aged 35 or older and 4% of children having mothers below the age of 20. Univariate analyses showed that mothers aged 35 or older were more likely to have gestational diabetes (odds ratio 1.9, 95% confidence interval 1.3 to 2.8), to have a history of miscarriage (odds ratio 1.8, 95% confidence interval 1.3 to 2.4), to have undergone fertility treatments (odds ratio 2.4, 95% confidence interval 1.5 to 3.9), and to have delivered by Caesarean section (odds ratio 1.6, 95% confidence interval 1.2 to 2.2). These findings were supported by multivariate analyses. Children with mothers below the age of 20 were more likely to have a congenital malformation (odds ratio 2.4, 95% confidence interval 1.4 to 4.2), which is also supported by multivariate analysis. Conclusions The risk factor profiles of children with cerebral palsy vary by maternal age. Future studies are warranted to further our understanding of the compound causal pathways leading to cerebral palsy and the observed greater prevalence of cerebral palsy with increasing maternal age.

Summary Background HLA-DRB1*15 genotype, previous infection with Epstein-Barr virus, and vitamin D insufficiency are susceptibility factors for multiple sclerosis, but whether they act synergistically to increase risk is unknown. We aimed to assess the contributions of these risk factors and the effect of established precursors of multiple sclerosis, such as brain lesions on MRI and oligoclonal bands in CSF at the time of incident demyelination, on development of multiple sclerosis in children. Methods In our prospective national cohort study, we assessed children who presented with incident CNS demyelination to any of the 16 paediatric health-care facilities or seven regional health-care facilities in Canada. We did univariate and multivariable analyses to assess contributions of HLA-DRB1*15 , Epstein-Barr virus, vitamin D status, MRI evidence of brain lesions, and CSF oligoclonal bands as determinants of multiple sclerosis. We used classification and regression tree analyses to generate a risk stratification algorithm for clinical use. Findings Between Sept 1, 2004, and June 30, 2010, we screened 332 children of whom 302 (91%) were eligible and followed-up for a median of 3·14 years (IQR 1·61–4·51). 63 (21%) children were diagnosed with multiple sclerosis after a median of 127 days (99–222). Although the risk of multiple sclerosis was increased with presence of one or more HLA-DRB1*15 alleles (hazard ratio [HR] 2·32, 95% CI 1·25–4·30), reduced serum 25-hydroxyvitamin D concentration (HR per 10 nmol/L decrease 1·11, 1·00–1·25), and previous Epstein-Barr-virus infection (HR 2·04, 0·99–4·20), no interactions between these variables were detected on multivariate analysis. Multiple sclerosis was strongly associated with baseline MRI evidence of one or more brain lesion (HR 37·9, 5·26–273·85) or CSF oligoclonal bands (6·33, 3·35–11·96), suggesting established disease. One patient diagnosed with multiple sclerosis had a normal MRI scan, and therefore sensitivity of an abnormal MRI scan for multiple sclerosis diagnosis was 98·4%. Interpretation Risk of multiple sclerosis in children can be stratified by presence of HLA-DRB1*15 alleles, remote Epstein-Barr virus infection, and low serum 25-hydroxyvitamin D concentrations. Similar to previous studies in adults, brain lesions detected on MRI and CSF oligoclonal bands in children are probable precursors to the clinical onset of multiple sclerosis. Children with a normal MRI are very likely to have a monophasic illness. Funding Canadian Multiple Sclerosis Scientific Research Foundation.

We explored whether parents of our pediatric patients valued the diagnostic terms "concussion," "minor traumatic brain injury," and "mild traumatic brain injury" as equivalent or nonequivalent. 1734 of 2304 parents attending a regional pediatric emergency department completed a brief questionnaire assessing the equivalence or nonequivalence of the diagnostic terms "concussion," "minor traumatic brain injury," and "mild traumatic brain injury" in a pairwise fashion. Many parents viewed these diagnostic terms as equivalent, when assessed side by side. For those who considered these diagnostic terms nonequivalent, concussion was regarded as considerably "better" (or less "worse") than minor traumatic brain injury (P0.001, χ(2) test) or mild traumatic brain injury (P0.001, χ(2) test). A moderate degree of variability was evident in parent/guardian responses. As a group, parents reported that concussion or mild/minor traumatic brain injuries are valued equivalently. However, many parents considered them different, with concussion reflecting a "better" (or less "worse") outcome.

Many children with chronic kidney disease have urinary incontinence due to urological disorders and/or a urine concentrating defect. We determined the prevalence and impact of incontinence on health related quality of life in children with chronic kidney disease.The Chronic Kidney Disease in Children study is a prospective, observational cohort of children recruited from 47 sites in the United States and Canada. Eligibility requirements are age 1 to 16 years and an estimated glomerular filtration rate of 30 to 90 ml per minute per 1.73 m(2). Demographics, continence status, glomerular filtration rate and physical examination were assessed at study entry. Health related quality of life was measured using the parent and child versions of PedsQL. PedsQL scores in participants 5 years old or older were compared among children who were toilet trained and not bed-wetting, bed-wetting or not toilet trained using multivariate linear regression.Overall median age of the 329 participants was 12.5 years, 61.4% were male, 70% were white and 55.5% had a urological disorder. Of participants 71.4% were toilet trained at study enrollment, 23.1% had bed-wetting and 5.5% were not toilet trained. Children who were not yet toilet trained had an average total score that was 13.5 points lower (95% CI -25.2, -1.8) on the PedsQL child report than in those who were toilet trained (p = 0.02). Physical functioning (-15.0, 95% CI -28.2, -1.9) and school functioning (-15.3, 95% CI -29.8, -0.8) were also lower in this group (p = 0.03 and 0.04, respectively). On the PedsQL parent proxy report physical functioning (-14.2, 95% CI -26.7, -1.6) was similarly affected by child incontinence (p = 0.03).Urinary incontinence is common in pediatric patients with chronic kidney disease and associated with lower health related quality of life on the PedsQL child and parent proxy reports. Early recognition of and treatment for urinary incontinence may improve health related quality of life in this population.

PURPOSE The purpose of this study was to investigate feasibility of an assessment protocol for a trial of post-Botox casting to treat equinus gait in cerebral palsy. METHODS Ten children (ages, 26-75 months) were recruited. Nine were assessed 1 week before botulinum toxin-A injections and reassessed 1 week after removal of the final cast. The assessment protocol included Modified Ashworth Scale (MAS), Modified Tardieu Scale (MTS), Gross Motor Function Measure-66 (GMFM-66), Pediatric Evaluation of Disability Inventory (PEDI), and GAITRite. Feasibility was based on acceptability of the protocol, inter-rater reliability, and responsiveness of outcome measures. RESULTS The assessment protocol was acceptable and practical. Inter-rater reliability for MAS, MTS, and GMFM ranged from moderate to excellent. Improvements were found in MTS and MAS scores for dorsiflexion and hamstring (p < 0.01), GMFM-66 (p = 0.01), and Pediatric Evaluation of Disability Inventory mobility (p = 0.01), self-care (p = 0.01), and social function (p = 0.00). GAITRite revealed reductions in speed (p = 0.00) and cadence (p = 0.01). CONCLUSIONS Feasibility was confirmed. Recommendations include raising minimum age and delaying gait analysis.

In adults, caffeine has been shown to enhance the effectiveness of most analgesics, including ibuprofen. This double-blind cross-over pilot study evaluated the effect of ibuprofen and caffeine compared with ibuprofen and placebo in 12 children with headaches. Patients completed diaries for both headaches. Outcome measures included a five-faces severity scale, a measure of clinical disability, and a scale of pain severity. Comparison of the cumulative response scores revealed a trend toward a greater response to ibuprofen-caffeine treatment of headaches (P = 0.14, P = 0.09, and P = 0.07 for the three measures, respectively). Further larger studies are needed to confirm this effect and to identify potential responders.

To determine the stability of Gross Motor Function Classification System (GMFCS) levels between approximately 12 years of age and adulthood (i.e. > 16y) using a matched chart review. Adult health records from the Ottawa Rehabilitation Centre were matched with childhood health records from the Ottawa Children's Treatment Centre (OCTC). Health records were available for 103 adults (52 males, 51 females) with cerebral palsy (CP; age range 17-38y; mean age 22y [SD 4y]) who had also been seen at the OCTC at a mean age of 12 years (SD 1y). GMFCS levels as adults were: Level I, n= 10; Level II, n= 24; Level III, n= 21; Level IV, n= 30; and Level V, n= 18. Adult participants were classified using the GMFCS at the time they were last seen by a rehabilitation specialist, sometime between June 2002 and June 2005. Corresponding paediatric charts were reviewed and classified by two independent raters blinded to the adult GMFCS levels. GMFCS levels around age 12 were: Level I, n= 20; Level II, n= 13; Level III, n= 22; Level IV, n= 35; and Level V, n= 13. Interrater reliability for childhood health records was determined with a quadratic weighted kappa and was 0.978. Stability of GMFCS levels was also assessed using the quadratic weighted kappa and was 0.895. The positive predictive value of the GMFCS at 12 years of age to predict walking without mobility aids by adulthood is 0.88. If the child is a wheelchair user at around age 12 years, the positive predictive value is 0.96 that the individual will still be a wheelchair user as an adult. This study supports previous findings that interrater reliability when using the GMFCS is very high. It also shows that the GMFCS level observed around the age of 12 years is highly predictive of adult motor function. This provides important information for individuals with CP, their families, and care providers as they plan for future care needs and rehabilitation intervention.

This study explored the relationships between the Gross Motor Function Classification System (GMFCS), limb distribution, and type of motor impairment. Data used were collected in the Ontario Motor Growth study, a longitudinal cohort study with a population-based sample of children with cerebral palsy (CP) in Canada (n=657; age 1 to 13 years at study onset). The majority (87.8%) of children with hemiplegia were classified as level I. Children with a bilateral syndrome were represented in all GMFCS levels, with most in levels III, IV, and V. Classifications by GMFCS and 'limb distribution' or by GMFCS and 'type of motor impairment' were statistically significantly associated (Pearson's chi2 p

Aim We sought to investigate how brain injury and severity, and neurological subtype of cerebral palsy (CP) differed in term-born children with CP after neonatal encephalopathy, between those with suspected birth asphyxia and those without. Method Using the Canadian CP Registry, which included 1001 children, those with CP born at ≥36wks after moderate or severe neonatal encephalopathy, were dichotomized according to the presence or absence of suspected birth asphyxia. Gross Motor Function Classification System (GMFCS) scores, neurological subtypes, comorbidities, and magnetic resonance imaging findings were compared. Results Of the 147 term-born children with CP (82 males, 65 females; median age 37 months, interquartile range [IQR] 26–52.5) who after moderate or severe neonatal encephalopathy had the required outcome data, 61 (41%) met criteria for suspected birth asphyxia. They had a higher frequency of non-ambulatory GMFCS status (odds ratio [OR] 3.4, 95% confidence interval [CI] 1.72–6.8), spastic quadriplegia (OR 2.8, 95% CI 1.4–5.6), non-verbal communication skills impairment (OR 4.2, 95% CI 2.0–8.6), isolated deep grey matter injury (OR 4.1, 95% CI 1.8–9.5), a lower frequency of spastic hemiplegia (OR 0.17, 95% CI 0.07–0.42), focal injury (OR 0.20; 95% CI 0.04–0.93), and more comorbidities (p=0.017) than those who did not meet criteria. Interpretation Term-born children who develop CP after neonatal encephalopathy with suspected birth asphyxia have a greater burden of disability than those without suspected birth asphyxia.

OBJECTIVES. It is unclear whether declines in neonatal and infant mortality have led to changes in the occurrence of cerebral palsy. We conducted a study to examine and investigate recent temporal changes in the prevalence of cerebral palsy in a population-based cohort of very preterm infants who were 24 to 30 weeks of gestational age.METHODS. A population-based cohort of very preterm infants who were born between January 1, 1993, and December 31, 2002, was evaluated by the Perinatal Follow-up Program of Nova Scotia. Follow-up extended to age 2 years to ascertain the presence or absence of cerebral palsy and for overall survival. Infant survival and cerebral palsy rates were compared by year and also in two 5-year periods, 1993–1997 and 1998–2002. Logistic regression analyses were used to identify factors that potentially were responsible for temporal changes in cerebral palsy rates.RESULTS. A total of 672 liveborn very preterm infants were born to mothers who resided in Nova Scotia between 1993 and 2002. Infant mortality among very preterm infants decreased from 256 per 1000 live births in 1993 to 114 per 1000 live births in 2002, whereas the cerebral palsy rates increased from 44.4 per 1000 live births in 1993 to 100.0 per 1000 live births in 2002. Low gestational age, postnatal dexamethasone use, patent ductus arteriosus, severe hyaline membrane disease, resuscitation in the delivery room, and intraventricular hemorrhage were associated with higher rates of cerebral palsy, whereas antenatal corticosteroid use was associated with a lower rate.CONCLUSION. Cerebral palsy has increased substantially among very preterm infants in association with and possibly as a consequence of large declines in infant mortality.

OBJECTIVE. We examined the effect of pregnancy and neonatal factors on the subsequent development of childhood epilepsy in a population-based cohort study. PATIENTS AND METHODS. Children born between January 1986 and December 2000 in Nova Scotia, Canada were followed up to December 2001. Data on pregnancy and neonatal events and on diagnoses of childhood epilepsy were obtained through record linkage of 2 population-based databases: the Nova Scotia Atlee Perinatal Database and the Canadian Epilepsy Database and Registry. Factors analyzed included events during the prenatal, labor and delivery, and neonatal time periods. Cox proportional hazards regression models were used to estimate relative risks and 95% confidence intervals. RESULTS. There were 648 new cases of epilepsy diagnosed among 124207 live births, for an overall rate of 63 per 100000 person-years. Incidence rates were highest among children CONCLUSIONS. Our study supports the concept that prenatal factors contribute to the occurrence of subsequent childhood epilepsy.

Objective. Most children enjoy healthy childhoods with little need for specialized health care services. However, some children experience difficulties in early childhood and require access to and utilization of considerable health care resources over time. Although impaired motor function is the hallmark of the cerebral palsy (CP) syndromes, many children with this development disorder also experience sensory, communicative, and intellectual impairments and may have complex limitations in self-care functions. Although caregiving is a normal part of being the parent of a young child, this role takes on an entirely different significance when a child experiences functional limitations and possible long-term dependence. One of the main challenges for parents is to manage their child's chronic health problems effectively and juggle this role with the requirements of everyday living. Consequently, the task of caring for a child with complex disabilities at home might be somewhat daunting for caregivers. The provision of such care may prove detrimental to both the physical health and the psychological well-being of parents of children with chronic disabilities. It is not fully understood why some caregivers cope well and others do not. The approach of estimating the “independent” or “direct” effects of the care recipient's disability on the caregiver's health is of limited value because (1) single-factor changes are rare outside the context of constrained experimental situations; (2) assumptions of additive relationships and perfect measurements rarely hold; and (3) such approaches do not provide a complete perspective, because they fail to examine indirect pathways that occur between predictor variables and health outcomes. A more detailed analytical approach is needed to understand both direct and indirect effects simultaneously. The primary objective of the current study was to examine, within a single theory-based multidimensional model, the determinants of physical and psychological health of adult caregivers of children with CP.Methods. We developed a stress process model and applied structural equation modeling with data from a large cohort of caregivers of children with CP. This design allowed the examination of the direct and indirect relationships between a child's health, behavior and functional status, caregiver characteristics, social supports, and family functioning and the outcomes of caregivers' physical and psychological health. Families (n = 468) of children with CP were recruited from 19 regional children's rehabilitation centers that provide outpatient disability management and supports in Ontario, Canada. The current study drew on a population available to the investigators from a previous study, the Ontario Motor Growth study, which explored patterns of gross motor development in children with CP. Data on demographic variables and caregivers' physical and psychological health were assessed using standardized, self-completed parent questionnaires as well as a face-to-face home interview. Structural equation modeling was used to test specific hypotheses outlined in our conceptual model. This analytic approach involved a 2-step process. In the first step, observed variables that were hypothesized to measure the underlying constructs were tested using confirmatory factor analysis; this step led to the so-called measurement model. The second step tested hypotheses about relationships among the variables in the structural model. All of the hypothesized paths in the conceptual model were tested and included in the structural model. However, only paths that were significant were shown in the final results. The direct, indirect, and total effects of theoretical constructs on physical and psychological health were calculated using the structural model.Results. The most important predictors of caregivers' well-being were child behavior, caregiving demands, and family function. A higher level of behavior problems was associated with lower levels of both psychological (β = −.22) and physical health (β = −.18) of the caregivers, whereas fewer child behavior problems were associated with higher self-perception (β = −.37) and a greater ability to manage stress (β = −.18). Less caregiving demands were associated with better physical (β = .23) and psychological (β = .12) well-being of caregivers, respectively. Similarly, higher reported family functioning was associated with better psychological health (β = .33) and physical health (β = .33). Self-perception and stress management were significant direct predictors of caregivers' psychological health but did not directly influence their physical well-being. Caregivers' higher self-esteem and sense of mastery over the caregiving situation predicted better psychological health (β = .23). The use of more stress management strategies was also associated with better psychological health of caregivers (β = .11). Gross income (β = .08) and social support (β = .06) had indirect overall effects only on psychological health outcome, whereas self-perception (β = .22), stress management (β = .09), gross income (β = .07), and social support (β = .06) had indirect total effects only on physical health outcomes.Conclusions. The psychological and physical health of caregivers, who in this study were primarily mothers, was strongly influenced by child behavior and caregiving demands. Child behavior problems were an important predictor of caregiver psychological well-being, both directly and indirectly, through their effect on self-perception and family function. Caregiving demands contributed directly to both the psychological and the physical health of the caregivers. The practical day-to-day needs of the child created challenges for parents. The influence of social support provided by extended family, friends, and neighbors on health outcomes was secondary to that of the immediate family working closely together. Family function affected health directly and also mediated the effects of self-perception, social support, and stress management. In families of children with CP, strategies for optimizing caregiver physical and psychological health include supports for behavioral management and daily functional activities as well as stress management and self-efficacy techniques. These data support clinical pathways that require biopsychosocial frameworks that are family centered, not simply technical and short-term rehabilitation interventions that are focused primarily on the child. In terms of prevention, providing parents with cognitive and behavioral strategies to manage their child's behaviors may have the potential to change caregiver health outcomes. This model also needs to be examined with caregivers of children with other disabilities.

Objective.—To validate our previous estimates of the prevalence of frequent headache and associated factors in a new sample of 12- to 13-year-old adolescent Canadians, and to explore if estimates of the prevalence of frequent headache and associated factors remain stable within the original cohort after a two-year interval. Methods.—We analyzed the self-administered questionnaire microdata files of the National Longitudinal Survey of Children and Youth (NLSCY: 1998–1999). 1694 respondents representing 724 400 youth aged 12 to 13 years were used to validate the prevalence in our initial estimates. From the initial cohort, 1764 respondents representing 819 600 youth, now aged 14 to 15 years, were used to examine whether frequent headache prevalence or associated factors had changed in the intervening two years. Results.—Among the analyzed individuals, 26.3% of 12- to 13-year olds and 31.2% of 14- to 15-year olds reported frequent headaches of “about once a week” or more often (P= .001). Our previous study of the NLSCY: 1996–1997 found a prevalence of 26.6% in 12- to 13-year olds. We previously identified 22 risk factors, of which 15 were replicated and one proxy was available within this iteration of the survey. For the 12- to 13-year olds, 14/16 were associated (P < .01, chi-square) with frequent headaches. For the initial cohort, now 14- to 15-year olds, 15/16 risk factors remained associated (P < .01, chi-square) with an increased risk of headaches. Conclusions.—Our estimates of frequent headache prevalence are reliable and consistent in two large nationally representative cohorts of 12- to 13-year olds. In the first of these groups there was an increase in prevalence over 2 years as adolescence progresses. Our identified risk factors for frequent headaches have been validated within another cohort and are maintained as the cohort ages.

For most consultations the diagnosis is established by a thorough clinical history. We prospectively assessed the impact of each component of the pediatric neurologic consultation in 500 consecutive referrals to a tertiary care pediatric neurology clinic. Diagnosis and management decisions were declared for each patient by the consultant after each stage of the consultation process (1: receipt of consultation letter, 2: history, 3: physical examination and 4: receipt of investigation results). We found that the physical examination and investigations influenced management decisions for less than 6%. Specifically the examination was never influential for children with headaches, Tourette syndrome, developmental delay or attention-deficit-hyperactivity disorder (ADHD). Investigations never influenced management for those with Tourette syndrome, ADHD, or cerebral palsy. Therefore for the majority of children the critical component of the pediatric neurology consultation is a detailed clinical history.

Supplemental feedings are commonly recommended for young children on dialysis but their effect on growth parameters and mortality has not been well documented. We report the results of a North American Pediatric Renal Transplant Cooperative Study (NAPRTCS) survey on the impact of supplemental feedings on growth and mortality in children

The factors associated with a greater mortality risk in infants and young children undergoing dialysis have not been clearly determined. We report the results of a North American Pediatric Renal Transplant Cooperative Study designed to assess risk factors in patients aged younger than 6 years at initiation of dialysis therapy. Sixty-four nonsurvivors were matched with 110 survivors for age at dialysis initiation, primary renal disease, and year of entry onto the database. Questionnaires on 137 patients (51 nonsurvivors, 86 survivors) were completed by participating centers. Seventy-five percent (103 of 137 patients) of the patients were aged younger than 2 years at dialysis initiation; 42% (58 of 137 patients) had renal aplasia, dysplasia, and/or hypoplasia or obstructive uropathy; 62% were boys; and 62% were white. One-year patient survival rates were 83% in infants beginning dialysis at younger than 3 months of age, 89% in 3- to 23-month-olds, and 95% in 2- to 5-year-olds (P = 0.001). Comorbid nonrenal disease occurred in 37 of 51 nonsurvivors (74%) versus 46 of 84 survivors (55%; P = 0.027). Nonsurvivors had pulmonary disease and/or hypoplasia more often (14 of 37 nonsurvivors; 37.8% versus 8 of 46 survivors; 17.4%; P = 0.04). Oliguria or anuria was present in 23 of 33 nonsurvivors (70%) aged younger than 2 years versus 26 of 64 survivors (41%; P = 0.007). Infection accounted for 15 of 51 deaths (29.4%). In summary, these results suggest that age at dialysis initiation; presence of nonrenal disease, particularly pulmonary disease and/or hypoplasia; and oliguria or anuria in children aged younger than 2 years are identifiable as risk factors for mortality in these young patients.

Background and Purpose. Development of gross motor function in children with cerebral palsy (CP) has not been documented. The purposes of this study were to examine a model of gross motor function in children with CP and to apply the model to construct gross motor function curves for each of the 5 levels of the Gross Motor Function Classification System (GMFCS). Subjects. A stratified sample of 586 children with CP, 1 to 12 years of age, who reside in Ontario, Canada, and are known to rehabilitation centers participated. Methods. Subjects were classified using the GMFCS, and gross motor function was measured with the Gross Motor Function Measure (GMFM). Four models were examined to construct curves that described the nonlinear relationship between age and gross motor function. Results. The model in which both the limit parameter (maximum GMFM score) and the rate parameter (rate at which the maximum GMFM score is approached) vary for each GMFCS level explained 83% of the variation in GMFM scores. The predicted maximum GMFM scores differed among the 5 curves (level I=96.8, level II=89.3, level III=61.3, level IV=36.1, and level V=12.9). The rate at which children at level II approached their maximum GMFM score was slower than the rates for levels I and III. The correlation between GMFCS levels and GMFM scores was −.91. Logistic regression, used to estimate the probability that children with CP are able to achieve gross motor milestones based on their GMFM total scores, suggests that distinctions between GMFCS levels are clinically meaningful. Conclusion and Discussion. Classification of children with CP based on functional abilities and limitations is predictive of gross motor function, whereas age alone is a poor predictor. Evaluation of gross motor function of children with CP by comparison with children of the same age and GMFCS level has implications for decision making and interpretation of intervention outcomes.

We report on 4 of 9 sibs with a syndrome of stenosis of the renal arteries and chronic hypertension, variable stenosis or occlusion of cerebral, abdominal and probably coronary arteries due to suspected fibromuscular dysplasia, congenital cardiac abnormalities, brachydactyly and syndactyly of the hands and feet, and increased bone fragility consistent with a mild form of osteogenesis imperfecta. Three affected individuals have had mild to moderate learning disabilities. The parents and the remaining 5 sibs have normal hands and feet and no history of excessive fractures. Individual components of this syndrome may appear as isolated conditions, including fibromuscular dysplasia, brachydactyly, syndactyly, and osteogenesis imperfecta, and are autosomal dominant traits in many cases. Explanations for this familial occurrence include autosomal recessive inheritance, autosomal dominant inheritance with decreased penetrance, or parental gonadal mosaicism for a mutation involving a single gene or several contiguous genes.

Somatometric parameters, renal size, and systolic blood pressure (SBP) were studied in 406 patients referred to pediatric nephrology and urology clinics. These patients included 269 females (66%), 67 African Americans (17%), and 87 patients with essential hypertension (21%). Z scores for the study population were comparable to published standards for height, kidney length, and SBP. Weight and body mass index scores were significantly greater than predicted from the standards, especially in the subset of patients with essential hypertension. Age, height, weight, body mass index, kidney length, and SBP all correlated with one another; however, on multiple regression analysis of SBP with the other five independent variables, only weight proved to have a significant correlation. Furthermore, the relationship of kidney length with SBP was positive and hypertensive patients had greater kidney size than published standards. These data do not support reduced kidney size in the population with essential hypertension, nor is there support for a convincing correlation between kidney length and SBP in the general pediatric population. Body weight correlates best with blood pressure. These findings warrant further study in a less-select population. Prevention and treatment of obesity may thus be of prime importance in addressing hypertension in children.

To define the earliest renal morphological changes in patients with type I diabetes, we studied renal function and morphometric analysis of renal biopsies in 59 patients with diabetes for 5 – 12 years and normal blood pressure, normal creatinine clearance (C Cr), and negative dipstick urinary protein. Arteriolar hyalinization and intimal fibrous thickening were noted in 43%. Glomerular basement membrane thickness and fractional mesangial volume were increased in 51% and 56%, respectively. The pre-pubertal and post-pubertal years of diabetes were associated with similar degrees of renal structural changes, but during the pre-pubertal years normal urinary albumin excretion (UAE) was seen. Principal factor analysis of morphometric structural parameters yielded four clusters of variables: “glomerular size” correlated with patient age, C Cr, and UAE; “peripheral capillary decrease” correlated with glycosylated hemoglobin, diastolic blood pressure, glomerular filtration rate, and UAE; “mesangial increase” correlated with UAE; and “interstitial scarring” correlated with diastolic blood pressure. This study provides unique documentation of renal structural abnormalities which precede clinically evident renal functional abnormalities and documents that these early structural abnormalities are present in the pre-pubertal years of diabetes as well as postpuberty, and are associated with each other in constellations that correspond to postulated mechanisms in diabetic nephropathy.

We studied kidney biopsy specimens from three children with sickle cell anemia and microangiopathic glomerulopathy. One child also had cyanotic congenital heart disease. Laboratory evaluation revealed proteinuria and normal serum creatinine in all and normal serum complement in two of the three children at the time of biopsy. In all biopsies, glomeruli were enlarged with diffuse hypercellularity and focal segmental mesangial interposition; capillary loop lumens were congested with sickled erythrocytes. Immune labeling identified segmental immunoglobulin G, C3, and properdin over the glomerular capillary loop walls in each case. Ultrastructurally, the subendothelial zone of the glomerular basement membrane was widened with new lamina densa formation with focal mesangial interposition. The glomerular lesion we describe in these children may be due to endothelial injury related to the altered erythrocytes, glomerular hemodynamics, and the hypercoagulable state characteristic of sickle cell disease.