NobleBlocks

Children's Hospital of Western Ontario

Hospital / health systemLondon, Ontario, Canada

Research output, citation impact, and the most-cited recent papers from Children's Hospital of Western Ontario (Canada). Aggregated across the NobleBlocks index of 300M+ scholarly works.

Total works
868
Citations
45.4K
h-index
87
i10-index
805
Also known as
Children's Hospital of Western Ontario

Top-cited papers from Children's Hospital of Western Ontario

The validation of visual analogue scales as ratio scale measures for chronic and experimental pain
Donald D. Price, Patricia McGrath, Amir Rafii, Barbara Buckingham
1983· Pain3.9Kdoi:10.1016/0304-3959(83)90126-4

Visual analogue scales (VAS) of sensory intensity and affective magnitude were validated as ratio scale measures for both chronic and experimental pain. Chronic pain patients and healthy volunteers made VAS sensory and affective responses to 6 noxious thermal stimuli (43, 45, 47, 48, 49 and 51 degrees C) applied for 5 sec to the forearm by a contact thermode. Sensory VAS and affective VAS responses to these temperatures yielded power functions with exponents 2.1 and 3.8, respectively; these functions were similar for pain patients and for volunteers. The power functions were predictive of estimated ratios of sensation or affect produced by pairs of standard temperatures (e.g. 47 and 49 degrees C), thereby providing direct evidence for ratio scaling properties of VAS. Vas sensory intensity responses to experimental pain, VAS sensory intensity responses to different levels of chronic pain, and direct temperature (experimental pain) matches to 3 levels of chronic pain were all internally consistent, thereby demonstrating the valid use of VAS for the measurement of and comparison between chronic pain and experimental heat pain.

Clinical Risk Score for Persistent Postconcussion Symptoms Among Children With Acute Concussion in the ED
Roger Zemek, Nick Barrowman, Stephen B. Freedman, Jocelyn Gravel +4 more
2016· JAMA884doi:10.1001/jama.2016.1203

IMPORTANCE: Approximately one-third of children experiencing acute concussion experience ongoing somatic, cognitive, and psychological or behavioral symptoms, referred to as persistent postconcussion symptoms (PPCS). However, validated and pragmatic tools enabling clinicians to identify patients at risk for PPCS do not exist. OBJECTIVE: To derive and validate a clinical risk score for PPCS among children presenting to the emergency department. DESIGN, SETTING, AND PARTICIPANTS: Prospective, multicenter cohort study (Predicting and Preventing Postconcussive Problems in Pediatrics [5P]) enrolled young patients (aged 5-<18 years) who presented within 48 hours of an acute head injury at 1 of 9 pediatric emergency departments within the Pediatric Emergency Research Canada (PERC) network from August 2013 through September 2014 (derivation cohort) and from October 2014 through June 2015 (validation cohort). Participants completed follow-up 28 days after the injury. EXPOSURES: All eligible patients had concussions consistent with the Zurich consensus diagnostic criteria. MAIN OUTCOMES AND MEASURES: The primary outcome was PPCS risk score at 28 days, which was defined as 3 or more new or worsening symptoms using the patient-reported Postconcussion Symptom Inventory compared with recalled state of being prior to the injury. RESULTS: In total, 3063 patients (median age, 12.0 years [interquartile range, 9.2-14.6 years]; 1205 [39.3%] girls) were enrolled (n = 2006 in the derivation cohort; n = 1057 in the validation cohort) and 2584 of whom (n = 1701 [85%] in the derivation cohort; n = 883 [84%] in the validation cohort) completed follow-up at 28 days after the injury. Persistent postconcussion symptoms were present in 801 patients (31.0%) (n = 510 [30.0%] in the derivation cohort and n = 291 [33.0%] in the validation cohort). The 12-point PPCS risk score model for the derivation cohort included the variables of female sex, age of 13 years or older, physician-diagnosed migraine history, prior concussion with symptoms lasting longer than 1 week, headache, sensitivity to noise, fatigue, answering questions slowly, and 4 or more errors on the Balance Error Scoring System tandem stance. The area under the curve was 0.71 (95% CI, 0.69-0.74) for the derivation cohort and 0.68 (95% CI, 0.65-0.72) for the validation cohort. CONCLUSIONS AND RELEVANCE: A clinical risk score developed among children presenting to the emergency department with concussion and head injury within the previous 48 hours had modest discrimination to stratify PPCS risk at 28 days. Before this score is adopted in clinical practice, further research is needed for external validation, assessment of accuracy in an office setting, and determination of clinical utility.

Classification criteria for autoinflammatory recurrent fevers
Marco Gattorno, Michaël Hofer, Silvia Federici, Federica Vanoni +4 more
2019· Annals of the Rheumatic Diseases555doi:10.1136/annrheumdis-2019-215048

BACKGROUND: Different diagnostic and classification criteria are available for hereditary recurrent fevers (HRF)-familial Mediterranean fever (FMF), tumour necrosis factor receptor-associated periodic fever syndrome (TRAPS), mevalonate kinase deficiency (MKD) and cryopyrin-associated periodic syndromes (CAPS)-and for the non-hereditary, periodic fever, aphthosis, pharyngitis and adenitis (PFAPA). We aimed to develop and validate new evidence-based classification criteria for HRF/PFAPA. METHODS: Step 1: selection of clinical, laboratory and genetic candidate variables; step 2: classification of 360 random patients from the Eurofever Registry by a panel of 25 clinicians and 8 geneticists blinded to patients' diagnosis (consensus ≥80%); step 3: statistical analysis for the selection of the best candidate classification criteria; step 4: nominal group technique consensus conference with 33 panellists for the discussion and selection of the final classification criteria; step 5: cross-sectional validation of the novel criteria. RESULTS: The panellists achieved consensus to classify 281 of 360 (78%) patients (32 CAPS, 36 FMF, 56 MKD, 37 PFAPA, 39 TRAPS, 81 undefined recurrent fever). Consensus was reached for two sets of criteria for each HRF, one including genetic and clinical variables, the other with clinical variables only, plus new criteria for PFAPA. The four HRF criteria demonstrated sensitivity of 0.94-1 and specificity of 0.95-1; for PFAPA, criteria sensitivity and specificity were 0.97 and 0.93, respectively. Validation of these criteria in an independent data set of 1018 patients shows a high accuracy (from 0.81 to 0.98). CONCLUSION: Eurofever proposes a novel set of validated classification criteria for HRF and PFAPA with high sensitivity and specificity.

A Longitudinal Study of Siblings of Children with Chronic Disabilities
Sandra Fisman, Lucille C. Wolf, Deborah Ellison, Tom Freeman
2000· The Canadian Journal of Psychiatry235doi:10.1177/070674370004500406

OBJECTIVE: To examine the unaffected siblings of 2 different groups with chronic disabilities, pervasive developmental disorder (PDD) and Down syndrome (DS), over 3 years, comparing their adjustment with each other and with the siblings of a nondisabled group. METHOD: This study examines 137 siblings of children with PDD, children with DS, and developmentally normal children (control group) initially and 127 siblings at follow-up 3 years later. Their adjustment is measured by the Survey Diagnostic Instrument (SDI), completed by caregivers and teachers. Predictor variables include sibling self-perception, social support, and relationship with sibling, as indicated by siblings; caregiver psychosocial factors such as parental stress, caregiver depression, and marital relationship; family systems characteristics as viewed by both caregiver and sibling; and difficulty that disabled child causes as perceived by the primary caregiver. RESULTS: Significantly more adjustment problems are found in the siblings of PDD children at both times when compared with siblings of DS and control children. Caregivers of PDD children report the highest levels of distress and depression, and this persists over time. Parent distress was found, at both times, to be related to sibling adjustment problems, regardless of study group. CONCLUSION: These results have implications for preventive intervention for the unaffected siblings of PDD children.

Transcriptional Profiling of Antigen-Dependent Murine B Cell Differentiation and Memory Formation
Deepta Bhattacharya, Ming T. Cheah, Christopher B. Franco, Naoki Hosen +3 more
2007· The Journal of Immunology169doi:10.4049/jimmunol.179.10.6808

Humoral immunity is characterized by the generation of Ab-secreting plasma cells and memory B cells that can more rapidly generate specific Abs upon Ag exposure than their naive counterparts. To determine the intrinsic differences that distinguish naive and memory B cells and to identify pathways that allow germinal center B cells to differentiate into memory B cells, we compared the transcriptional profiles of highly purified populations of these three cell types along with plasma cells isolated from mice immunized with a T-dependent Ag. The transcriptional profile of memory B cells is similar to that of naive B cells, yet displays several important differences, including increased expression of activation-induced deaminase and several antiapoptotic genes, chemotactic receptors, and costimulatory molecules. Retroviral expression of either Klf2 or Ski, two transcriptional regulators specifically enriched in memory B cells relative to their germinal center precursors, imparted a competitive advantage to Ag receptor and CD40-engaged B cells in vitro. These data suggest that humoral recall responses are more rapid than primary responses due to the expression of a unique transcriptional program by memory B cells that allows them to both be maintained at high frequencies and to detect and rapidly respond to antigenic re-exposure.

Neuropeptide Y is produced in visceral adipose tissue and promotes proliferation of adipocyte precursor cells <i>via</i> the Y1 receptor
Kaiping Yang, Haiyan Guan, Edith Arany, David J. Hill +1 more
2008· The FASEB Journal157doi:10.1096/fj.07-100735

Neuropeptide Y (NPY) is synthesized in neural tissue of the central and peripheral nervous systems and has a number of important functions besides regulating appetite and energy homeostasis. Here we identify a novel site of NPY biosynthesis and a role for NPY in promoting proliferation of adipocyte precursor cells. We show that NPY mRNA is not only expressed in visceral adipose tissue (VAT) but that its levels are up-regulated 6-fold in our early-life programmed rat model of increased visceral adiposity. This is accompanied by a parallel rise in NPY protein, demonstrating that VAT is a novel peripheral site of NPY biosynthesis. Furthermore, NPY mRNA expression is also elevated >2-fold in VAT of obese Zucker rats. Importantly, NPY stimulates proliferation of primary rat preadipocytes as well as 3T3-L1 preadipocytes in vitro. This mitogenic effect appears to be mediated by the Y1 receptor and involves the activation of extracellular related kinase 1/2. In addition, insulin and glucocorticoid up-regulate VAT NPY expression in lean but not obese Zucker rats. Taken together, these results suggest that an enhanced local expression of NPY within VAT may be a common feature of and contribute to the molecular mechanisms underlying increased visceral adiposity.

Vasopressin in Pediatric Vasodilatory Shock: A Multicenter Randomized Controlled Trial
Karen Choong, Desmond Bohn, Douglas D. Fraser, Isabelle Gaboury +4 more
2009· American Journal of Respiratory and Critical Care Medicine144doi:10.1164/rccm.200902-0221oc

RATIONALE: Vasopressin has been proposed as a potent vasoactive agent in the treatment of vasodilatory shock in adults and children. The objective of this trial was to evaluate the efficacy and safety of vasopressin as an adjunctive agent in pediatric vasodilatory shock. METHODS: In this multicenter, double-blind trial, children with vasodilatory shock were randomized to receive low-dose vasopressin (0.0005-0.002 U/kg/min) or placebo in addition to open-label vasoactive agents. Vasoactive infusions were titrated to clinical endpoints of adequate perfusion. The primary outcome was time to vasoactive-free hemodynamic stability. Secondary outcomes included mortality, organ-failure-free days, length of critical care unit stay, and adverse events. MEASUREMENTS AND MAIN RESULTS: Sixty-five of 69 children (94%) who were randomized received the study drug (33 vasopressin, 32 placebo) and were included in the analysis. There was no significant difference in the primary outcome between the vasopressin and placebo groups (49.7 vs. 47.1 hours; P = 0.85). There were 10 deaths (30%) in the vasopressin group and five (15.6%) in the placebo group (relative risk, 1.94; 95% confidence interval, 0.75-5.05; P = 0.24). There were no significant differences with respect to organ failure-free days (22 vs. 25.5 days; P = 0.11), ventilator-free days (16.5 23 days; P = 0.15), length of stay (8 vs. 8.5 days; P = 0.93), or adverse event rate ratios (12.0%; 95% confidence interval, -2.6 to 26.7; P = 0.15). CONCLUSIONS: Low-dose vasopressin did not demonstrate any beneficial effects in this pediatric trial. Although not statistically significant, there was a concerning trend toward increased mortality. Clinical trial registered with www.controlled-trials.com (ISRCTN11597444).

A Systematic Review on the Diagnosis of Pediatric Bacterial Pneumonia: When Gold Is Bronze
Tim Lynch, Liza Bialy, James D. Kellner, Martin H. Osmond +4 more
2010· PLoS ONE143doi:10.1371/journal.pone.0011989

BACKGROUND: In developing countries, pneumonia is one of the leading causes of death in children under five years of age and hence timely and accurate diagnosis is critical. In North America, pneumonia is also a common source of childhood morbidity and occasionally mortality. Clinicians traditionally have used the chest radiograph as the gold standard in the diagnosis of pneumonia, but they are becoming increasingly aware that it is not ideal. Numerous studies have shown that chest radiography findings lack precision in defining the etiology of childhood pneumonia. There is no single test that reliably distinguishes bacterial from non-bacterial causes. These factors have resulted in clinicians historically using a combination of physical signs and chest radiographs as a 'gold standard', though this combination of tests has been shown to be imperfect for diagnosis and assigning treatment. The objectives of this systematic review are to: 1) identify and categorize studies that have used single or multiple tests as a gold standard for assessing accuracy of other tests, and 2) given the 'gold standard' used, determine the accuracy of these other tests for diagnosing childhood bacterial pneumonia. METHODS AND FINDINGS: Search strategies were developed using a combination of subject headings and keywords adapted for 18 electronic bibliographic databases from inception to May 2008. Published studies were included if they: 1) included children one month to 18 years of age, 2) provided sufficient data regarding diagnostic accuracy to construct a 2x2 table, and 3) assessed the accuracy of one or more index tests as compared with other test(s) used as a 'gold standard'. The literature search revealed 5,989 references of which 256 were screened for inclusion, resulting in 25 studies that satisfied all inclusion criteria. The studies examined a range of bacterium types and assessed the accuracy of several combinations of diagnostic tests. Eleven different gold standards were studied in the 25 included studies. Criterion validity was calculated for fourteen different index tests using eleven different gold standards. The most common gold standard utilized was blood culture tests used in six studies. Fourteen different tests were measured as index tests. PCT was the most common measured in five studies each with a different gold standard. CONCLUSIONS: We have found that studies assessing the diagnostic accuracy of clinical, radiological, and laboratory tests for bacterial childhood pneumonia have used a heterogeneous group of gold standards, and found, at least in part because of this, that index tests have widely different accuracies. These findings highlight the need for identifying a widely accepted gold standard for diagnosis of bacterial pneumonia in children.

Biallelic mutations in DNAJC21 cause Shwachman-Diamond syndrome
Santhosh Dhanraj, Anna Matveev, Hongbing Li, Supanun Lauhasurayotin +4 more
2017· Blood142doi:10.1182/blood-2016-08-735431

Abstract There is an Inside Blood Commentary on this article in this issue.

The Relationship Between Elbow Position and Grip Strength
Elizabeth Ann Kuzala, Michael Charles Vargo
1992· American Journal of Occupational Therapy137doi:10.5014/ajot.46.6.509

Forty-six graduate students participated in a study to investigate the effect of elbow position on grip-strength measurements. Sixteen males and 30 females, aged 21 to 46 years, participated in the study. Data collection procedures followed standardized grip-strength testing guidelines established by the American Society of Hand Therapists, with the exception of elbow position. Grip-strength measurements were taken with the elbow positioned at 0 degree, 45 degrees, 90 degrees, and 135 degrees of flexion. The results showed a significantly stronger grip-strength measurement at 0 degree of flexion and the weakest grip-strength measurement at 135 degrees of flexion. This study supports the use of a standardized method for hand-strength testing. The knowledge that grip-strength measurement is highest when the elbow is fully extended could affect treatment planning.

Peripheral artery tonometry demonstrates altered endothelial function in children with type 1 diabetes
Michael J. Haller, Jennifer Stein, Jon Shuster, Douglas W. Theriaque +4 more
2007· Pediatric Diabetes135doi:10.1111/j.1399-5448.2007.00246.x

OBJECTIVES: To assess the ability of reactive hyperemia-peripheral artery tonometry (RH-PAT) to serve as a surrogate marker of endothelial dysfunction in children with type 1 diabetes (T1D). RESEARCH DESIGN AND METHODS: Forty-four children with T1D [age 14.6 +/- 2.7 yr; duration of diabetes 6.01 +/- 4 yr; range of diabetes duration 1-16 yr; and hemoglobin A1c (HbA1c) 8.34 +/- 1.2%] and 20 children without diabetes (age 14.1 +/- 1.5 yr) underwent RH-PAT endothelial function testing after an overnight fast. Height, weight, body mass index (BMI), blood pressure (BP), fasting lipid profile, and glucose level were determined in each child. Children with T1D underwent a second RH-PAT study 4 wk after their initial study to determine the intrapatient variability of the technique. RESULTS: Children with T1D had endothelial dysfunction as evidenced by lower mean RH-PAT scores (1.63 +/- 0.5) when compared with children without diabetes (mean RH-PAT score 1.95 +/- 0.3) (p = 0.01). Repeat RH-PAT scores were predicted by initial RH-PAT scores (p = 0.0025). Mean intrapatient standard deviation of RH-PAT score was 0.261 and mean coefficient of variation was 14.8. Variations in RH-PAT score were not explained by differences in glucose, HbA1c, BMI, systolic BP, diastolic BP, or lipids. CONCLUSIONS: Although larger validation studies are required, RH-PAT is a promising non-invasive technique to assess endothelial function in children with T1D. Non-invasive measures of endothelial dysfunction may provide the additional risk stratification data needed to justify more aggressive primary prevention of cardiovascular disease in children with T1D.

Guideline for the prevention of oral and oropharyngeal mucositis in children receiving treatment for cancer or undergoing haematopoietic stem cell transplantation
Lillian Sung, Paula D. Robinson, Nathaniel S. Treister, Tina Baggott +4 more
2015· BMJ Supportive & Palliative Care134doi:10.1136/bmjspcare-2014-000804

PURPOSE: To develop an evidence-based clinical practice guideline for the prevention of oral mucositis in children (0-18 years) receiving treatment for cancer or undergoing haematopoietic stem cell transplantation (HSCT). METHODS: The Mucositis Prevention Guideline Development Group was interdisciplinary and included internationally recognised experts in paediatric mucositis. For the evidence review, we included randomised controlled trials (RCTs) conducted in either children or adults evaluating the following interventions selected according to prespecified criteria: cryotherapy, low level light therapy (LLLT) and keratinocyte growth factor (KGF). We also examined RCTs of any intervention conducted in children. For all systematic reviews, we synthesised the occurrence of severe oral mucositis. The Grades of Recommendation, Assessment, Development and Evaluation approach was used to describe quality of evidence and strength of recommendations. RESULTS: We suggest cryotherapy or LLLT may be offered to cooperative children receiving chemotherapy or HSCT conditioning with regimens associated with a high rate of mucositis. We also suggest KGF may be offered to children receiving HSCT conditioning with regimens associated with a high rate of severe mucositis. However, KGF use merits caution as there is a lack of efficacy and toxicity data in children, and a lack of long-term follow-up data in paediatric cancers. No other interventions were recommended for oral mucositis prevention in children. CONCLUSIONS: All three specific interventions evaluated in this clinical practice guideline were associated with a weak recommendation for use. There may be important organisational and cost barriers to the adoption of LLLT and KGF. Considerations for implementation and key research gaps are highlighted.

THE USE OF OLDER DONOR LIVERS FOR HEPATIC TRANSPLANTATION
William Wall, Richard Mimeault, David Grant, MICHAEL S. BLOCH
1990· Transplantation128doi:10.1097/00007890-199002000-00030

The function and outcome of liver grafts from "older" donors (more than 50 years old) were compared with grafts from younger donors (less than 50 years old). Of 184 consecutive liver transplants, 23 grafts were from older donors (50.2-65.3 years, mean 54.3 years). The liver preservation period was short, averaging less than 4 hr with the maximum under 8 hr for the older grafts. The majority of livers were preserved with Collins' solution. All transplants were performed using consistent methods that had proved to be successful over time. The medical status of the patients who received the older and younger grafts was similar but a higher percentage of older grafts were transplanted into ABO blood group--incompatible recipients. Graft function--as determined by peak aminotransferase levels, duration of prolonged prothrombin time, retransplantation rate within 30 days and incidence of primary nonfunction--was not significantly different in older versus younger grafts. Actual 30-day graft survival was 86.9% in the older grafts and 85.1% in the younger grafts. Actuarial 1-year graft and patient survival rates were 65.0% and 71.4%, respectively, in recipients of older grafts and 68.8% and 75.6%, respectively, in recipients of younger grafts. It is concluded that donor livers older than 50 years can be transplanted with the same success as younger livers provided that other generally accepted donor criteria are satisfied and the preservation period is short. The upper age limit for liver donation is not yet known.

Can We Predict Which Children With Clinically Suspected Pneumonia Will Have the Presence of Focal Infiltrates on Chest Radiographs?
Tim Lynch, Robert W. Platt, Serge Gouin, Charles P. Larson +1 more
2004· PEDIATRICS127doi:10.1542/peds.113.3.e186

Objective. To determine predictive factors for the presence of focal infiltrates in children with clinically suspected pneumonia in a pediatric emergency department. Methods. Children (1–16 years) with clinically suspected pneumonia were studied prospectively. The presenting features were compared between the children with and without focal infiltrates using χ2 analysis, t test, and odds ratio with 95% confidence intervals. A multivariate prediction rule was developed using logistic regression. Results. A total of 570 were studied. Risk factors (odds ratio; 95% confidence interval) for the presence of focal infiltrates included history of fever (3.1; 1.7–5.3), decreased breath sounds (1.4; 1.0–2.0), crackles (2.0; 1.4–2.9), retractions (2.8; 1.0–7.6), grunting (7.3; 1.1–48.1), fever (1.5; 1.2–1.9), tachypnea (1.8; 1.3–2.5), and tachycardia (1.3; 1.0–1.6). We then used logistic regression to develop a candidate prediction rule for the variables of fever, decreased breath sounds, crackles, and tachypnea, which had an area under the receiver operating curve of 0.668. This rule had excellent sensitivity (93.1%–98%) yet poor specificity (5.7%–19.4%). Conclusions. Multiple predictive factors for children with suspected pneumonia have been identified. Patients with focal infiltrates were more likely in our study to have a history of fever, tachypnea, increased heart rate, retractions, grunting, crackles, or decreased breath sounds. A multivariate prediction rule shows promise for the accurate prediction of pneumonia in children. However, the prospective evaluation of this multivariate prediction rule in a clinical setting is still required.

NFIA Haploinsufficiency Is Associated with a CNS Malformation Syndrome and Urinary Tract Defects
Lu W, Fabiola Quintero‐Rivera, Yanli Fan, Fowzan S. Alkuraya +4 more
2007· PLoS Genetics126doi:10.1371/journal.pgen.0030080

Complex central nervous system (CNS) malformations frequently coexist with other developmental abnormalities, but whether the associated defects share a common genetic basis is often unclear. We describe five individuals who share phenotypically related CNS malformations and in some cases urinary tract defects, and also haploinsufficiency for the NFIA transcription factor gene due to chromosomal translocation or deletion. Two individuals have balanced translocations that disrupt NFIA. A third individual and two half-siblings in an unrelated family have interstitial microdeletions that include NFIA. All five individuals exhibit similar CNS malformations consisting of a thin, hypoplastic, or absent corpus callosum, and hydrocephalus or ventriculomegaly. The majority of these individuals also exhibit Chiari type I malformation, tethered spinal cord, and urinary tract defects that include vesicoureteral reflux. Other genes are also broken or deleted in all five individuals, and may contribute to the phenotype. However, the only common genetic defect is NFIA haploinsufficiency. In addition, previous analyses of Nfia(-/-) knockout mice indicate that Nfia deficiency also results in hydrocephalus and agenesis of the corpus callosum. Further investigation of the mouse Nfia(+/-) and Nfia(-/-) phenotypes now reveals that, at reduced penetrance, Nfia is also required in a dosage-sensitive manner for ureteral and renal development. Nfia is expressed in the developing ureter and metanephric mesenchyme, and Nfia(+/-) and Nfia(-/-) mice exhibit abnormalities of the ureteropelvic and ureterovesical junctions, as well as bifid and megaureter. Collectively, the mouse Nfia mutant phenotype and the common features among these five human cases indicate that NFIA haploinsufficiency contributes to a novel human CNS malformation syndrome that can also include ureteral and renal defects.

Inconclusive Diagnosis of Cystic Fibrosis After Newborn Screening
Chee Y. Ooi, Carlo Castellani, Katherine Keenan, Julie Avolio +4 more
2015· PEDIATRICS126doi:10.1542/peds.2014-2081

OBJECTIVES: To prospectively study infants with an inconclusive diagnosis of cystic fibrosis (CF) identified by newborn screening (NBS; "CF screen positive, inconclusive diagnosis" [CFSPID]) for disease manifestations. METHODS: Infants with CFSPID and CF based on NBS from 8 CF centers were prospectively evaluated and monitored. Genotype, phenotype, repeat sweat test, serum trypsinogen, and microbiology data were compared between subjects with CF and CFSPID and between subjects with CFSPID who did (CFSPID→CF) and did not (CFSPID→CFSPID) fulfill the criteria for CF during the first 3 years of life. RESULTS: Eighty-two subjects with CFSPID and 80 subjects with CF were enrolled. The ratio of CFSPID to CF ranged from 1:1.4 to 1:2.9 in different centers. CFTR mutation rates did not differ between groups; 96% of subjects with CFSPID and 93% of subjects with CF had 2 mutations. Subjects with CFSPID had significantly lower NBS immunoreactive trypsinogen (median [interquartile range]:77 [61-106] vs 144 [105-199] μg/L; P < .0001) than did subjects with CF. Pseudomonas aeruginosa and Stenotrophomonas maltophilia were isolated in 12% and 5%, respectively, of subjects with CFSPID. CF was diagnosed in 9 of 82 (11%) subjects with CFSPID (genotype and abnormal sweat chloride = 3; genotype alone = 4; abnormal sweat chloride only = 2). Sweat chloride was abnormal in CFSPID→CF patients at a mean (SD) age of 21.3 (13.8) months. CFSPID→CF patients had significantly higher serial sweat chloride (P < .0001) and serum trypsinogen (P = .009) levels than did CFSPID→CFSPID patients. CONCLUSIONS: A proportion of infants with CFSPID will be diagnosed with CF within the first 3 years. These findings underscore the need for clinical monitoring, repeat sweat testing at age 2 to 3 years, and extensive genotyping.

Clinical outcomes for permanent incisor luxations in a pediatric population. II. Extrusions
Raymond Lee, Edward J. Barrett, David J. Kenny
2003· Dental Traumatology125doi:10.1034/j.1600-9657.2003.00208.x

A longitudinal outcome study was undertaken to identify variables that significantly influenced outcomes for extruded permanent maxillary incisors of children and adolescents. Clinical and radiographic data was available for 35 patients (18 males, 17 females) representing 55 incisors. Mean age at the time of injury was 10.6 years (range: 7.1-17.8 years). Mean time elapsed to follow up was 1320 days (range: 423-2887 days). Survival analysis was used to identify variables significantly related to the prognosis of these teeth. The loss of an incisor following extrusion was uncommon as only one tooth (1/55) required extraction. There was no statistically significant difference in survival between severely extruded teeth and avulsions that had been stored in physiological media (P > 0.10). Pulp necrosis (PN) was the most common complication following injury (43%) and it most often occurred during the first year. Although not statistically significant, a trend towards increased PN was found with more severely extruded teeth (P = 0.20, relative risk = 2.08). Pulp canal obliteration (PCO) was the second most common outcome (35%). The degree of extrusion was proven to be significantly associated with the development of PCO (P = 0.03, relative risk = 0.33). Root resorption was an uncommon outcome (3/55). This study represents the first outcome data on extrusions to permanent maxillary incisors in an exclusively pediatric population.

Implementation of a Multicenter Rapid Response System in Pediatric Academic Hospitals Is Effective
Afrothite Kotsakis, Anna-Theresa Lobos, Christopher S. Parshuram, Jonathan Gilleland +4 more
2011· PEDIATRICS123doi:10.1542/peds.2010-0756

OBJECTIVES: This is the first large multicenter study to examine the effectiveness of a pediatric rapid response system (PRRS). The primary objective was to determine the effect of a PRRS using a physician-led team on the rate of actual cardiopulmonary arrests, defined as an event requiring chest compressions, epinephrine, or positive pressure ventilation. The secondary objectives were to determine the effect of PRRSs on the rate of PICU readmission within 48 hours of discharge and PICU mortality after readmission and urgent PICU admission. METHODS: A PRRS was developed, implemented, and evaluated in a standardized manner across 4 pediatric academic centers in Ontario, Canada. The team responded to activations for inpatients and followed patients discharged from the PICU for 48 hours. A 2-year, prospective, observational study was conducted after implementation, and outcomes were compared with data collected 2 years before implementation. RESULTS: After PRRS implementation, there were 55 963 hospital admissions and a team activation rate of 44 per 1000 hospital admissions. There were 7302 patients followed after PICU discharge. Implementation of the PRRS was not associated with a reduction in the rate of actual cardiopulmonary arrests (1.9 vs 1.8 per 1000 hospital admissions; P=.68) or PICU mortality after urgent admission (1.3 vs 1.1 per 1000 hospital admissions; P=.25). There was a reduction in the PICU mortality rate after readmission (0.3 vs 0.1 death per 1000 hospital admissions; P=.05). CONCLUSION: The standardized implementation of a multicenter PRRS was associated with a decrease in the rate of PICU mortality after readmission but not actual cardiopulmonary arrests.

Intramedullary Nailing of Pediatric Femoral Fractures
Robert D. Galpin, R. Baxter Willis, Nabil Sabano
1994· Journal of Pediatric Orthopaedics117doi:10.1097/01241398-199403000-00010

This retrospective study reviews our results with intramedullary nail fixation of 37 fractures of the femur in 35 skeletally immature patients. Five of these fractures were open. Twenty-two patients (average age 12 + 9 years) were treated with reamed intramedullary nails. Fifteen patients (average age 9 + 6 years) were treated with nonreamed nails. All fractures united in 6-12 weeks. There were no infections, delayed or nonunions, nor were there any incidences of avascular necrosis. There were very few significant complications. One patient required excision of heterotopic bone to restore hip motion. When surgical treatment of pediatric femur fractures is indicated, we prefer intramedullary nail fixation (either reamed or nonreamed) depending on age, fracture pattern (level, degree of comminution), and size of femoral canal. Experience and careful surgical judgment are required to appropriately individualize treatment for these patients.

Pediatric trauma: differences in pathophysiology, injury patterns and treatment compared with adult trauma.
Niranjan Kissoon, Jon Dreyer, Mandeep Walia
1990· PubMed116

Although multiple trauma remains the leading cause of death among children, fewer resources and less attention have been directed to treatment of the injured child than to treatment of the injured adult. Insufficient training of medical personnel and hence lack of expertise in the management of injured children are factors contributing to disability and death in such children. Although the principles of resuscitation of injured children are similar to those for adults, appreciation of the differences in cardiorespiratory variables, airway anatomy, response to blood loss, thermoregulation and equipment required is essential for successful initial resuscitation. Cerebral, abdominal and thoracic injuries account for most of the disability and death among injured children. Cerebral damage may be due to secondary injuries to the brain and is potentially preventable. The need to preserve the spleen in children complicates the management of abdominal trauma. Although children usually have large cardiorespiratory reserves, they are likely to need airway control and ventilation with thoracic injuries. The psychologic effect of trauma may pose long-term problems and needs close follow-up.