Health-related quality of life and fatigue in children and adults with pyruvate kinase deficiency.

Pyruvate kinase deficiency (PKD) is the most common cause of congenital nonspherocytic hemolytic anemia. Although recognition of the disease spectrum has recently expanded, data describing its impact on health-related quality of life (HRQoL) are limited. In this prospective international cohort of 254 patients (131 adults and 123 children) with PKD, we used validated measures to assess the impact of disease on HRQoL (EuroQol 5-Dimension Questionnaire, Pediatric Quality of Life Inventory Generic Core Scale version 4.0, and Functional Assessment of Cancer Therapy-Anemia) and fatigue (Patient Reported Outcomes Measurement Information System Fatigue and Pediatric Functional Assessment of Chronic Illness Therapy-Fatigue). Significant variability in HRQoL and fatigue was reported for adults and children, although individual scores were stable over a 2-year interval. Although adults who were regularly transfused reported worse HRQoL and fatigue compared with those who were not (EuroQol-visual analog scale, 58 vs 80; P = .01), this difference was not seen in children. Regularly transfused adults reported lower physical, emotional, and functional well-being and more anemia symptoms. HRQoL and fatigue significantly differed in children by genotype, with the worst scores in those with 2 severe PKLR mutations; this difference was not seen in adults. However, iron chelation was associated with significantly worse HRQoL scores in children and adults. Pulmonary hypertension was also associated with significantly worse HRQoL. Additionally, 59% of adults and 35% of children reported that their jaundice upset them, identifying this as an important symptom for consideration. Although current treatments for PKD are limited to supportive care, new therapies are in clinical trials. Understanding the impact of PKD on HRQoL is important to assess the utility of these treatments. This trial was registered at www.clinicaltrials.gov as #NCT02053480.

Health Care Transition in Type 1 Diabetes: Perspectives of Diabetes Care and Education Specialists Caring for Young Adults.

PURPOSE: The purpose of the study was to describe experiences reported by diabetes care and education specialists caring for young adults with type 1 diabetes and to assess perceived deficiencies in clinical resources and barriers to care delivery. METHODS: A 60-item electronic survey was fielded through email to members of the Association of Diabetes Care and Education Specialists (ADCES). Respondents completed a survey consisting of: (1) clinical practice characteristics and respondents' demographics; (2) health care transition components (eg, referrals) and their perceived importance; (3) framework of current clinical diabetes care delivery and perceived need for additional support; and (4) perceived barriers regarding clinical care delivery. Statistical analyses included descriptive statistics, chi-square tests, and logistic regression. RESULTS: Respondents (N = 531, 96% female, median years in practice = 13; interquartile range = 7-20) represented 49 states plus the District of Columbia. Although 88% of respondents reported reviewing pediatric records as important/very important, only 22% often/always reviewed them. Although 58% of respondents noted easy access to mental health care providers for young adults, 50% stated a need for additional resources. Furthermore, diabetes care and education specialists without easy access to mental health professionals were significantly more likely to report barriers to diabetes management for young adults with depression, substance abuse, eating disorders, and developmental disabilities. CONCLUSION: Study findings highlight modifiable factors that may improve diabetes care coordination for transitioning young adults. Uniform approaches and increased access to trained mental health professionals may help support diabetes care and education specialists in their care of young adults with type 1 diabetes.

Association of Blood Type With Postsurgical Mucosal Bleeding in Pediatric Patients Undergoing Tonsillectomy With or Without Adenoidectomy.

Importance: Blood type (BT) O has been identified as a risk factor for bleeding complications, while non-O BTs may increase risk for thromboembolic events. Limited data are available in children undergoing tonsillectomy with or without adenoidectomy. Objective: To determine whether BT O is associated with hemorrhage after tonsillectomy with or without adenoidectomy. Design, Setting, and Participants: Retrospective cohort study of patients younger than 22 years who underwent tonsillectomy with or without adenoidectomy at a single institution between January 1, 2008, and August 7, 2017. Statistical analysis was performed from November 2017 to January 2019. Main Outcomes and Measures: Prevalence of hemorrhage following surgery was defined as any bleeding requiring cauterization up to 1 month after the procedure. Data on sex, age, von Willebrand disease (VWD) status, BT, white blood cell counts, and platelet counts closest to date of surgery were collected from an electronic medical record system, and the association of these factors with hemorrhage following surgery was investigated. Results: A total of 14 951 pediatric patients (median [range] age, 5.6 [0.8-21.9] years; 6956 [46.5%] female) underwent tonsillectomy with or without adenoidectomy. Prevalence of hemorrhage following the procedure was 3.9% (578 patients) for the full cohort and 2.8% (362 of 13 065) for patients with no BT identified or preprocedure VWD panel results at baseline. Children who had a BT identified and/or a VWD panel before surgery had higher bleeding rates (BT only, 14.9% [172 of 1156]; preprocedure VWD panel only, 4.6% [28 of 607]; and BT and preprocedure VWD panel, 13.0% [16 of 123]), all of which were significantly different from the baseline bleeding rate (P < .001). While the bleeding rates in children with BT O were not statistically different from those with non-O BT (14.8% and 14.6%, respectively; P > .99), mean von Willebrand factor values were statistically different (mean [SD] von Willebrand factor antigen level in O group, 86.9 [42.4] IU/dL in the O group vs 118.0 [53.8] IU/dL in the non-O group; P = .002; and mean [SD] von Willebrand factor ristocetin-cofactor in the O group, 72.2 [44.3] IU/dL vs 112.6 [68.0] IU/dL in the non-O group; P = .001). In addition, children older than 12 years had increased bleeding rates in the full cohort (8.3% vs 3.2%), in the testing-naive cohort (6.5% vs 2.3%), and in those with a preprocedure VWD panel only (13.5% vs 3.1%) compared with children aged 12 years or younger. Conclusions and Relevance: Type O blood was not a risk factor associated with hemorrhage after tonsillectomy with or without adenoidectomy despite lower baseline von Willebrand factor antigen and von Willebrand factor ristocetin-cofactor values in children with BT O vs those with non-O BT in our study cohort. No association was found between VWD status and bleeding, and there was no difference in VWD panel values in those who experienced hemorrhage vs those who did not within BT groups. Further studies elucidating the utility of von Willebrand factor values for children undergoing tonsillectomy with or without adenoidectomy are needed.

Macronutrient Analysis of Modified-Fat Breast Milk Produced by 3 Methods of Fat Removal.

BACKGROUND: Infants with chylothorax after congenital heart disease surgery are commonly treated using modified-fat breast milk. The effect of fat removal on breast milk macronutrients remains unclear. We compared macronutrient content of breast milk with breast milk skimmed using 3 methods, including a novel device, a cream separator. METHODS: Thawed frozen breast milk samples from 30 women were defatted using refrigerated centrifuge, cream separator, and manual separation after refrigeration. We used standard assays to measure energy, protein, and fat content of breast milk samples. RESULTS: All fat removal methods yielded skimmed breast milk with substantially lower fat and energy content. Mean energy content in breast milk skimmed by centrifuge (36.7 [SD 3.6] kcal/100 mL) was similar to that from cream separator (38.8 [3.5] kcal/100 mL). Both centrifuge and cream separator methods removed almost all fat and substantially more fat than the manual fat removal method. For unprocessed milk, energy and fat content estimated by creamatocrit was similar to reference method measurements; in skimmed milk, the creamatocrit significantly overestimated fat content. Mean protein content of skimmed breast milk was similar to unprocessed breast milk (mean 1.25 [0.31] g/100 mL). CONCLUSION: Breast milk fat removal did not significantly alter protein levels. In skimmed breast milk, the overestimation of fat content using creamatocrit method suggests a need for more accurate bedside methods to assess macronutrient content. The similar macronutrient composition of breast milk skimmed by cream separator and centrifuge suggests the potential for cream separator use as a new, portable defatting method for hospitals and families.

Second-line treatments in children with immune thrombocytopenia: Effect on platelet count and patient-centered outcomes.

Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder with isolated thrombocytopenia and hemorrhagic risk. While many children with ITP can be safely observed, treatments are often needed for various reasons, including to decrease bleeding, or to improve health related quality of life (HRQoL). There are a number of available second-line treatments, including rituximab, thrombopoietin-receptor agonists, oral immunosuppressive agents, and splenectomy, but data comparing treatment outcomes are lacking. ICON1 is a prospective, multi-center, observational study of 120 children starting second-line treatments for ITP designed to compare treatment outcomes including platelet count, bleeding, and HRQoL utilizing the Kids ITP Tool (KIT). While all treatments resulted in increased platelet counts, romiplostim had the most pronounced effect at 6 months (P = .04). Only patients on romiplostim and rituximab had a significant reduction in both skin-related (84% to 48%, P = .01 and 81% to 43%, P = .004) and non-skin-related bleeding symptoms (58% to 14%, P = .0001 and 54% to 17%, P = .0006) after 1 month of treatment. HRQoL significantly improved on all treatments. However, only patients treated with eltrombopag had a median improvement in KIT scores at 1 month that met the minimal important difference (MID). Bleeding, platelet count, and HRQoL improved in each treatment group, but the extent and timing of the effect varied among treatments. These results are hypothesis generating and help to improve our understanding of the effect of each treatment on specific patient outcomes. Combined with future randomized trials, these findings will help clinicians select the optimal second-line treatment for an individual child with ITP.

Disparities in the delivery of pediatric oncology nursing care by country income classification: International survey results.

BACKGROUND: In 2014, a task force of the International Society of Paediatric Oncology (SIOP) Paediatric Oncology in Developing Countries Nursing Workgroup published six baseline standards to provide a framework for pediatric oncology nursing care in low- and lower-middle income countries (L/LMIC). We conducted an international survey in 2016-2017 to examine the association between country income level and nurses' resporting of conformity to the standards at their respective institutions. PROCEDURE: Data from a cross-sectional web-based survey completed by nurses representing 54 countries were analyzed (N = 101). Responses were clustered by relevance to each standard and compared according to the 2017 World Bank-defined country income classification (CIC) of hospitals. RESULTS: CIC and nurse-to-patient ratios in inpatient wards were strongly associated (P < 0.0001). Nurses in L/LMIC prepared chemotherapy more often (P < 0.0001) yet were less likely to have access to personal protective equipment such as nitrile gloves (P = 0.0007) and fluid-resistant gowns (P = 0.011) than nurses in high-resource settings. Nurses in L/LMIC were excluded more often from physician/caregiver meetings to discuss treatment options (P = 0.04) and at the time of diagnosis (P = 0.002). Key educational topics were missing from nursing orientation programs across all CICs. An association between CIC and the availability of written policies (P = 0.009) was found. CONCLUSIONS: CIC and the ability to conform to pediatric oncology baseline nursing standards were significantly associated in numerous elements of the baseline standards, a likely contributor to suboptimal patient outcomes in L/LMIC. To achieve the goal of high-quality cancer care for children worldwide, nursing disparities must be addressed.

Validation of a pediatric early warning system for hospitalized pediatric oncology patients in a resource-limited setting.

BACKGROUND: Pediatric oncology patients are at high risk of clinical deterioration, particularly in hospitals with resource limitations. The performance of pediatric early warning systems (PEWS) to identify deterioration has not been assessed in these settings. This study evaluates the validity of PEWS to predict the need for unplanned transfer to the pediatric intensive care unit (PICU) among pediatric oncology patients in a resource-limited hospital. METHODS: A retrospective case-control study comparing the highest documented and corrected PEWS score before unplanned PICU transfer in pediatric oncology patients (129 cases) with matched controls (those not requiring PICU care) was performed. RESULTS: Documented and corrected PEWS scores were found to be highly correlated with the need for PICU transfer (area under the receiver operating characteristic, 0.940 and 0.930, respectively). PEWS scores increased 24 hours prior to unplanned transfer (P = .0006). In cases, organ dysfunction at the time of PICU admission correlated with maximum PEWS score (correlation coefficient, 0.26; P = .003), patients with PEWS results ≥4 had a higher Pediatric Index of Mortality 2 (PIM2) (P = .028), and PEWS results were higher in patients with septic shock (P = .01). The PICU mortality rate was 17.1%; nonsurvivors had higher mean PEWS scores before PICU transfer (P = .0009). A single-point increase in the PEWS score increased the odds of mechanical ventilation or vasopressors within the first 24 hours and during PICU admission (odds ratio 1.3-1.4). CONCLUSIONS: PEWS accurately predicted the need for unplanned PICU transfer in pediatric oncology patients in this resource-limited setting, with abnormal results beginning 24 hours before PICU admission and higher scores predicting the severity of illness at the time of PICU admission, need for PICU interventions, and mortality. These results demonstrate that PEWS aid in the identification of clinical deterioration in this high-risk population, regardless of a hospital's resource-level. Cancer 2017;123:4903-13. © 2017 American Cancer Society.

Improved outcomes after successful implementation of a pediatric early warning system (PEWS) in a resource-limited pediatric oncology hospital.

BACKGROUND: Hospitalized pediatric oncology patients are at high risk of clinical decline and mortality, particularly in resource-limited settings. Pediatric early warning systems (PEWS) aid in the early identification of clinical deterioration; however, there are limited data regarding their feasibility or impact in low-resource settings. This study describes the successful implementation of PEWS at the Unidad Nacional de Oncología Pediátrica (UNOP), a pediatric oncology hospital in Guatemala, resulting in improved inpatient outcomes. METHODS: A modified PEWS was implemented at UNOP with systems to track errors, transfers to a higher level of care, and high scores. A retrospective cohort study was used to evaluate clinical deterioration events in the year before and after PEWS implementation. RESULTS: After PEWS implementation at UNOP, there was 100% compliance with PEWS documentation and an error rate of <10%. Implementation resulted in 5 high PEWS per week, with 30% of patients transferring to a higher level of care. Among patients requiring transfer to the pediatric intensive care unit (PICU), 93% had an abnormal PEWS before transfer. The rate of clinical deterioration events decreased after PEWS implementation (9.3 vs 6.5 per 1000-hospitalpatient-days, p = .003). Despite an 18% increase in total hospital patient-days, PICU utilization for inpatient transfers decreased from 1376 to 1088 PICU patient-days per year (21% decrease; P<.001). CONCLUSIONS: This study describes the successful implementation of PEWS in a pediatric oncology hospital in Guatemala, resulting in decreased inpatient clinical deterioration events and PICU utilization. This work demonstrates that PEWS is a feasible and effective quality improvement measure to improve hospital care for children with cancer in hospitals with limited resources. Cancer 2017;123:2965-74. © 2017 American Cancer Society.

Creation of a novel simulator for minimally invasive neurosurgery: fusion of 3D printing and special effects.

OBJECTIVE Recent advances in optics and miniaturization have enabled the development of a growing number of minimally invasive procedures, yet innovative training methods for the use of these techniques remain lacking. Conventional teaching models, including cadavers and physical trainers as well as virtual reality platforms, are often expensive and ineffective. Newly developed 3D printing technologies can recreate patient-specific anatomy, but the stiffness of the materials limits fidelity to real-life surgical situations. Hollywood special effects techniques can create ultrarealistic features, including lifelike tactile properties, to enhance accuracy and effectiveness of the surgical models. The authors created a highly realistic model of a pediatric patient with hydrocephalus via a unique combination of 3D printing and special effects techniques and validated the use of this model in training neurosurgery fellows and residents to perform endoscopic third ventriculostomy (ETV), an effective minimally invasive method increasingly used in treating hydrocephalus. METHODS A full-scale reproduction of the head of a 14-year-old adolescent patient with hydrocephalus, including external physical details and internal neuroanatomy, was developed via a unique collaboration of neurosurgeons, simulation engineers, and a group of special effects experts. The model contains "plug-and-play" replaceable components for repetitive practice. The appearance of the training model (face validity) and the reproducibility of the ETV training procedure (content validity) were assessed by neurosurgery fellows and residents of different experience levels based on a 14-item Likert-like questionnaire. The usefulness of the training model for evaluating the performance of the trainees at different levels of experience (construct validity) was measured by blinded observers using the Objective Structured Assessment of Technical Skills (OSATS) scale for the performance of ETV. RESULTS A combination of 3D printing technology and casting processes led to the creation of realistic surgical models that include high-fidelity reproductions of the anatomical features of hydrocephalus and allow for the performance of ETV for training purposes. The models reproduced the pulsations of the basilar artery, ventricles, and cerebrospinal fluid (CSF), thus simulating the experience of performing ETV on an actual patient. The results of the 14-item questionnaire showed limited variability among participants' scores, and the neurosurgery fellows and residents gave the models consistently high ratings for face and content validity. The mean score for the content validity questions (4.88) was higher than the mean score for face validity (4.69) (p = 0.03). On construct validity scores, the blinded observers rated performance of fellows significantly higher than that of residents, indicating that the model provided a means to distinguish between novice and expert surgical skills. CONCLUSIONS A plug-and-play lifelike ETV training model was developed through a combination of 3D printing and special effects techniques, providing both anatomical and haptic accuracy. Such simulators offer opportunities to accelerate the development of expertise with respect to new and novel procedures as well as iterate new surgical approaches and innovations, thus allowing novice neurosurgeons to gain valuable experience in surgical techniques without exposing patients to risk of harm.

Thrombopoietin Receptor Agonist Use in Children: Data From the Pediatric ITP Consortium of North America ICON2 Study.

BACKGROUND: Data on second-line treatment options for pediatric patients with immune thrombocytopenia (ITP) are limited. Thrombopoietin receptor agonists (TPO-RA) provide a nonimmunosuppressive option for children who require an increased platelet count. PROCEDURE: We performed a multicenter retrospective study of pediatric ITP patients followed at ITP Consortium of North America (ICON) sites to characterize TPO-RA use. RESULTS: Seventy-nine children had a total of 87 treatments (28 eltrombopag, 43 romiplostim, and eight trialed on both). The majority had primary ITP (82%) and most (60.8%) had chronic ITP. However, 22% had persistent ITP and 18% had newly diagnosed ITP. During the first 3 months of treatment, 89% achieved a platelet count ≥ 50 × 10(9) /l (86% romiplostim, 81% eltrombopag, P = 0.26) at least once in the absence of rescue therapy. The average time to a response was 6.4 weeks for romiplostim and 7.0 weeks for eltrombopag (P = 0.83). Only 40% of patients demonstrated a stable response with consistent dosing over time. An intermittent response with constant dose titration was seen in 15%, and an initial response that waned to no response was seen in 13%. Significant adverse events were minimal with the exception of two patients with thrombotic events and one who developed a neutralizing antibody. CONCLUSIONS: Our results demonstrate that TPO-RA agents are being used in children with ITP of varying duration and severity. The response was similar to clinical trials, but the sustainability of response varied. Future studies need to focus on the ideal timing and rationale for these medications in pediatric patients.

Validation of a Pediatric Early Warning Score in Hospitalized Pediatric Oncology and Hematopoietic Stem Cell Transplant Patients.

OBJECTIVES: To evaluate the correlation of a Pediatric Early Warning Score with unplanned transfer to the PICU in hospitalized oncology and hematopoietic stem cell transplant patients. DESIGN: We performed a retrospective matched case-control study, comparing the highest documented Pediatric Early Warning Score within 24 hours prior to unplanned PICU transfers in hospitalized pediatric oncology and hematopoietic stem cell transplant patients between September 2011 and December 2013. Controls were patients who remained on the inpatient unit and were matched 2:1 using age, condition (oncology vs hematopoietic stem cell transplant), and length of hospital stay. Pediatric Early Warning Scores were documented by nursing staff at least every 4 hours as part of routine care. Need for transfer was determined by a PICU physician called to evaluate the patient. SETTING: A large tertiary/quaternary free-standing academic children's hospital. PATIENTS: One hundred ten hospitalized pediatric oncology patients (42 oncology, 68 hematopoietic stem cell transplant) requiring unplanned PICU transfer and 220 matched controls. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Using the highest score in the 24 hours prior to transfer for cases and a matched time period for controls, the Pediatric Early Warning Score was highly correlated with the need for PICU transfer overall (area under the receiver operating characteristic = 0.96), and in the oncology and hematopoietic stem cell transplant groups individually (area under the receiver operating characteristic = 0.95 and 0.96, respectively). The difference in Pediatric Early Warning Score results between the cases and controls was noted as early as 24 hours prior to PICU admission. Seventeen patients died (15.4%). Patients with higher Pediatric Early Warning Scores prior to transfer had increased PICU mortality (p = 0.028) and length of stay (p = 0.004). CONCLUSIONS: We demonstrate that our institution's Pediatric Early Warning Score is highly correlated with the need for unplanned PICU transfer in hospitalized oncology and hematopoietic stem cell transplant patients. Furthermore, we found an association between higher scores and PICU mortality. This is the first validation of a Pediatric Early Warning Score specific to the pediatric oncology and hematopoietic stem cell transplant populations, and supports the use of Pediatric Early Warning Scores as a method of early identification of clinical deterioration in this high-risk population.

Quality of life and parental adjustment in pediatric pulmonary hypertension.

BACKGROUND: This study examines the impact of pulmonary hypertension (PH) on the quality of life (QoL) of affected youth, as well as the relationships among PH disease severity, parental adjustment variables, and family relational functioning. METHODS: Subjects were 47 eligible parents of children with PH aged 2 to 18 years who were evaluated at Boston Children's Hospital. Measures of patient QoL and of parental stress, coping, and adjustment were administered to the caregivers. Clinicians rated illness severity and family relational functioning. RESULTS: Youth with PH scored significantly lower than healthy norms on a measure of parent-reported QoL (total, psychosocial, and physical QoL, each P < .0001). The parents reported encountering stressful events more frequently than published norms of parents of children with cancer (P < .0001). Thirty-four percent of parents of children with PH met criteria for presumed psychiatric diagnosis, and they reported using psychologic coping strategies significantly more often than a normative sample. A summary parental stress measure correlated inversely with child QoL, an effect that held true even after controlling for disease severity (P = .03). CONCLUSIONS: PH takes a major toll on children and their families. Decreased QoL among youth with PH was significantly associated with high levels of parental stress, over and above the effect of illness severity. These findings suggest that interventions to better support the caretakers of affected children and adolescents should accompany medical treatment advances so as to improve QoL for patients facing pediatric PH.

Neuropsychological outcomes of a randomized trial of prednisone versus dexamethasone in acute lymphoblastic leukemia: findings from Dana-Farber Cancer Institute All Consortium Protocol 00-01.

BACKGROUND: Dexamethasone is more efficacious than prednisone in the treatment of acute lymphoblastic leukemia (ALL), but has also been associated with greater toxicity. We compared neuropsychological outcomes for patients treated on DFCI ALL Consortium Protocol 00-01, which included a randomized comparison of the two steroid preparations during post-induction therapy in children and adolescents with ALL. PROCEDURE: Between 2000 and 2005, 408 children with standard-risk or high-risk ALL treated on Dana-Farber Cancer Institute Consortium Protocol 00-01 were randomly assigned to prednisone or dexamethasone administered as 5-day pulses every 3 weeks for 2 years, beginning at week 7 of treatment. Blinded neuropsychological testing was completed for 170 randomized patients (prednisone, N = 76; dexamethasone, N = 94), all of whom were in continuous complete remission after completion of therapy. RESULTS: Outcomes were comparable for most variables, although patients on the dexamethasone arm performed more poorly on a measure of fluid reasoning (P = 0.02). They also tended to be more likely to be enrolled in special education (dexamethasone, 33% vs. prednisone, 20%, P = 0.09). CONCLUSIONS: Dexamethasone has well documented benefit in treatment of ALL. Although formal testing provided little indication of increased risk for neurotoxicity relative to prednisone, the somewhat greater utilization of special education services by patients treated with dexamethasone merits further investigation.

Impact of trauma system development on pediatric injury care.

PURPOSE: Trauma systems improve survival by directing severely injured patients to trauma centers. This study analyzes the impact of trauma systems on pediatric triage and injury mortality rates. METHODS: Population-based data were collected on injured children less than 15 years who were admitted to any hospital in New England from 1996 to 2006. Data from three trauma system states were compared to three non-trauma system states. The percentages of injured children, severely injured children, and brain-injured children admitted to trauma centers were determined as well as injury hospitalization and death rates. Time trend analysis examined the pace of change between the groups. RESULTS: A total of 58,583 injured children were hospitalized during the study period. Injury hospitalization rates were initially similar between the two groups (with and without trauma systems) and decreased over time in both. Rates decreased more rapidly in trauma system states compared to those without, (P = 0.003). Injury death rates decreased over time in both groups with no difference between the groups, (P = 0.20). A higher percentage of injured children were admitted to trauma centers in non-trauma system states throughout the study period, and this percentage increased in both groups of states. A higher percentage of severely injured children and brain-injured children were admitted to trauma centers in non-trauma system states and both percentages increased over time. The increase was more rapid in trauma system states for children with severe injuries (P < 0.001) and children with brain injuries (P < 0.001). DISCUSSION: Trauma systems decreased childhood injury hospitalization rates and increased the percentage of severely injured children and brain-injured children admitted to trauma centers. Mortality and overall triage rates were unaffected.

Anterior versus posterior approach to iliac crest for alveolar cleft bone grafting.

PURPOSE: The goal of this study was to compare perioperative outcomes for the anterior versus posterior approach to the iliac crest for alveolar bone grafting in patients with cleft lip and palate (CLP). MATERIALS AND METHODS: A retrospective cohort study of patients with CLP who had an anterior or posterior iliac crest bone graft during a 10-year period was performed. Available medical records were reviewed and the outcome variables documented included the operation site for bone graft procurement (anterior or posterior), estimated blood loss, duration of operation, concurrent procedures, and length of hospital stay. The 2 groups were compared by t test, Fisher exact test, and multivariate regression to show differences. RESULTS: There were 239 patients with CLP who had an iliac crest bone graft to the alveolar cleft; 133 had an anterior and 106 a posterior approach for bone graft procurement. The mean estimated blood loss was significantly less with the posterior approach (85 vs 177 mL; P < .0001). The mean operation duration with the posterior approach was longer (4.6 vs 3.5 hours; P < .0001). Most patients in this group (67%), however, had 2 or more additional procedures during the same anesthetic, whereas only 16% of patients in the anterior group had 2 or more additional procedures. The mean length of stay was significantly shorter for the posterior approach (1.7 vs 2.2 days; P < .0001). CONCLUSIONS: These results suggest that patients having a bone graft from the posterior iliac crest have a smaller estimated blood loss and a shorter length of stay. In addition, this approach permits repair of the alveolar cleft and additional procedures, including augmentation of the median tubercle and correction of nasolabial distortions, which are often present in patients with CLP.

A trigger tool fails to identify serious errors and adverse events in pediatric otolaryngology.

OBJECTIVE: To identify and quantify errors and adverse events on an inpatient academic tertiary-care pediatric otolaryngology service, a trigger tool was developed and validated as part of a quality improvement initiative. STUDY DESIGN: Retrospective record review. SETTING: Children's Hospital Boston quality improvement initiative. SUBJECTS AND METHODS: Fifty inpatient admissions were reviewed. The gold standard for errors and adverse events identification was a detailed chart review by two board-certified otolaryngologists blinded to trigger tool findings. RESULTS: Trigger tool interrater reliability ranged from poor to high for admission triggers (kappa = 0.35, 95% confidence interval [95% CI] -0.07 to 0.76), discharge triggers (kappa = 0.63, 95% CI 0.27-0.99), medical records triggers (kappa = 0.61, 95% CI 0.11-1.00), and medication triggers (kappa = 0.90, 95% CI 0.71-1.00). Errors and adverse events were found in all admissions: three percent were potentially harmful, and 93 percent were documentation-related. CONCLUSION: The trigger tool was successful in identifying clerical and administrative errors and adverse events but failed to identify complex errors and adverse events. A hybrid approach for chart review may be cost-effective in pediatric otolaryngology.

Final adult height of children with inflammatory bowel disease is predicted by parental height and patient minimum height Z-score.

BACKGROUND: This study was designed to elucidate the contribution of parental height to the stature of children with inflammatory bowel disease (IBD), who often exhibit growth impairment. Accordingly, we compared patients' final adult heights and target heights based on measured parental heights and examined predictors of final adult height in pediatric IBD patients. METHODS: We prospectively analyzed the growth of 295 patients diagnosed between ages 1 and 18 (211 Crohn's disease [CD], 84 ulcerative colitis [UC]) and their family members (283 mothers, 231 fathers, 55 siblings). RESULTS: Twenty-two percent had growth impairment (height for age Z-score <-1.64, equivalent to <5th percentile on growth curve) in more than 1 measurement since diagnosis; most growth-impaired patients had CD (88% CD versus 12% UC). Parents of the growth-impaired group had lower mean height Z-scores compared to parents of nongrowth-impaired patients (-0.67 versus 0.02 for mothers [P < 0.001]; -0.31 versus 0.22 for fathers [P = 0.002]). For 108 patients who reached adult heights and had available parental heights, the growth-impaired group continued to demonstrate lower adult height Z-scores (-1.38 versus 0.07; P < 0.001). Adult heights were within 1 SD of target heights even for the growth-impaired group. Only 11.3% remained persistently growth-impaired in adulthood. Multivariate regression analysis demonstrated lower parental height and minimum patient height Z-score as significant predictors of lower final adult height in IBD. CONCLUSIONS: Parental height is a powerful determinant of linear growth even in the presence of chronic inflammation, and should be an integral part of the evaluation of growth in IBD children.

Hospital volumes for common pediatric specialty operations.

OBJECTIVES: To describe hospital volumes for common pediatric specialty operations, to evaluate hospital and patient characteristics associated with operations performed at a low-volume hospital, and to evaluate outcomes with hospital volume. DESIGN: Retrospective cohort using the Kids' Inpatient Database 2003. SETTING: Discharges from 3438 hospitals in 36 states from 2003. PARTICIPANTS: Children aged 0 to 18 years undergoing ventriculoseptal defect surgery (n = 2301), tracheotomy (n = 2674), ventriculoperitoneal shunt placement (n = 3378), and posterior spinal fusion (n = 4002). MAIN EXPOSURE: Hospital volume. MAIN OUTCOME MEASURES: In-hospital mortality and postoperative complications. RESULTS: For tracheotomy and posterior spinal fusion, at least one fourth of the hospitals performed only 1 operation for children aged 0 to 18 years in 2003. For these same operations, at least half of hospitals treated 4 or fewer cases per year. For all operations, discharges from low-volume hospitals were less likely to be from children's or teaching hospitals compared with discharges from higher-volume hospitals. For tracheotomy, children were less likely to experience postoperative complications in high-volume hospitals compared with low-volume hospitals (odds ratio, 0.48; 95% confidence interval, 0.21-1.09). CONCLUSIONS: Many children undergoing common pediatric specialty operations had these procedures performed in low-volume hospitals. Low-volume hospitals were less likely to be children's or teaching hospitals. Children undergoing tracheotomy experienced higher rates of complications in low-volume hospitals. Further research is needed to identify the reasons why so many children have these operations performed in low-volume hospitals.