Michael B. Mundorff

Impact of fundoplication versus gastrojejunal feeding tubes on mortality and in preventing aspiration pneumonia in young children with neurologic impairment who have gastroesophageal reflux disease.

OBJECTIVE. Aspiration pneumonia is the most common cause of death in children with neurologic impairment who have gastroesophageal reflux disease. Fundoplications and gastrojejunal feeding tubes are frequently employed to prevent aspiration pneumonia in this population. Which of these approaches is more effective in preventing aspiration pneumonia and/or improving survival is unknown. The objective of this study was to compare outcomes for children with neurologic impairment and gastroesophageal reflux disease after either a first fundoplication or a first gastrojejunal feeding tube. PATIENTS AND METHODS. This was a retrospective, observational cohort study of children with neurologic impairment who had either a fundoplication or gastrojejunal feeding tube between January 1997 and December 2005 at a tertiary care childrens hospital. Main outcome measures were postprocedure aspiration pneumonia–free survival and mortality. Propensity analyses were used to control for bias in treatment assignment and prognostic imbalances. RESULTS. Of the 366 children with neurologic impairment and gastroesophageal reflux disease, 43 had a first gastrojejunal feeding tube and 323 underwent a first fundoplication. Median length of follow-up was 3.4 years. Children who received a first fundoplication had similar rates of aspiration pneumonia and mortality after the procedure compared with those who had a first gastrojejunal feeding tube, when adjusting for the treatment assignment using propensity scores. CONCLUSIONS. Aspiration pneumonia and mortality are not uncommon events after either a first fundoplication or a first gastrojejunal feeding tube for the management of gastroesophageal reflux disease in children with neurologic impairment. Neither treatment option is clearly superior in preventing the subsequent aspiration pneumonia or improving overall survival for these children. This complex clinical scenario needs to be studied in a prospective, multicenter, randomized control trial to evaluate definitively whether 1 of these 2 management options is more beneficial.

The burden of inherited leukodystrophies in children.

Objectives: Leukodystrophies are diseases of the white matter for which data concerning clinical characteristics, incidence, disease burden, and description of outcomes are sparse. The purpose of our study was to determine the incidence and most common types of inherited leukodystrophies in a population, the mortality and time course of deaths, common neurologic features in patients, and health care costs associated with leukodystrophies. Methods: We conducted a retrospective, hospital- and clinic-based surveillance of inherited leukodystrophies among children younger than 18 years presenting to a regional childrens hospital. We enrolled children evaluated from January 1, 1999, through December 31, 2007; clinical information was obtained from medical records. We calculated incidence based on state birth rates. Results: A total of 122 children with an inherited leukodystrophy were identified; 542 patients were excluded. A total of 49% had epilepsy, 43% required a gastrostomy tube, and 32% had a history of developmental regression. Mortality was 34%; average age at death was 8.2 years. No final diagnosis was reported in 51% of patients. The most common diagnoses were metachromatic leukodystrophy (8.2%), Pelizaeus-Merzbacher disease (7.4%), mitochondrial diseases (4.9%), and adrenoleukodystrophy (4.1%). Endocrine abnormalities and hypoplastic cerebellum were noted in significant portions of patients (15% and 14%). Average yearly per-patient medical costs were

Hospital admission medication reconciliation in medically complex children: an observational study

22,579. Population incidence was 1 in 7,663 live births. Conclusions: Inherited leukodystrophies are associated with substantial morbidity and mortality in children. Overall population incidence is higher than generally appreciated (1 in 7,663 live births). Most leukodystrophies remain undiagnosed, but a logical algorithm based on prevalence could aid testing.

Spinal muscular atrophy type 1: are proactive respiratory interventions associated with longer survival?

Objective To evaluate admission medication reconciliation in children with medically complex conditions (MCC) by determining the availability and accuracy of five information sources and characterising admitting order errors. Design Prospective quality improvement cohort study. Setting Tertiary care free-standing childrens hospital in the Intermountain west, USA. Participants 23 children with MCC identified from 219 admissions between 16 December 2004 and 7 January 2005. Intervention Medication reconciliation at hospital admission using information from five sources. Main outcomes The accuracy of information sources was determined by sensitivity and specificity compared with verified outpatient medication lists. Errors were determined by comparing admitting orders with reconciled inpatient medication lists and categorised by frequency, type and clinical risk. Results Children with MCC averaged 5.3 chronic medications. The reconciliation process took an average of 90 min. Availability/sensitivity/specificity respectively were parents 52%/0.75/0.96, pharmacy 61%/0.64/0.74, primary provider 43%/0.25/0.86, last admission electronic health record 87%/0.74/0.33 and admitting history 65%/0.31/0.94. Thirty-nine errors were identified in 182 admission medications (21%) including 17 omissions, affecting 13 patients (57%). The estimated clinical risk, if an adverse drug event had occurred, was serious or life-threatening in five instances. Conclusions In children with MCC admitted at our institution during the study period, no medication information source was optimally available, sensitive and specific. Admitting order medication errors affected more than half of patients, the most common being omissions. Efforts to improve medication reconciliation at hospital admission in this population must account for availability and accuracy of information sources and medication omissions at the time of hospital admission.

Neurologically intact children with an isolated skull fracture may be safely discharged after brief observation

Context: Spinal muscular atrophy type 1, an autosomal recessive motor neuron disease, is a leading genetic cause of death in infancy and early childhood. Objective: To determine whether the early initiation of noninvasive respiratory interventions is associated with longer survival. Design: Single-institution retrospective cohort study identified children with spinal muscular atrophy type 1 from January 1, 2002 to May 1, 2009 who were followed for 2.3 mean yrs. Setting: Tertiary care children’s hospital and outpatient clinics in a vertically integrated healthcare system. Patients or Other Participants: Forty-nine children with spinal muscular atrophy type 1 were grouped according to the level of respiratory support their caregivers chose within the first 3 months after diagnosis: proactive respiratory care (n = 26) and supportive care (n = 23). Interventions: Proactive respiratory care included bilevel noninvasive ventilation during sleep and twice a day cough assist while supportive respiratory care included suctioning, with or without supplemental oxygen. Measurements and Main Results: Kaplan–Meier survival curves were assessed based on intention to treat. Children treated with early proactive respiratory support had statistically longer survival compared to supportive care (log rank 0.047); however, the adjusted hazard ratio for survival was not statistically different (2.44 [95% confidence interval 0.84-7.1]). Children in the proactive group were more likely to be hospitalized for respiratory insufficiency (83% vs. 46%) and had shortened time after diagnosis until first hospital admission for respiratory insufficiency (median 118 vs. 979 days). Conclusion: Longer survival time with spinal muscular atrophy type 1 is associated with early, noninvasive respiratory care interventions after diagnosis.

Macrophage activation syndrome in children with systemic lupus erythematosus and children with juvenile idiopathic arthritis

PURPOSE The management of children presenting with an isolated skull fracture (ISF) posttrauma is highly variable. We sought to estimate the risk of neurologic deterioration in children with a Glasgow coma score (GCS) 15 and ISF to reduce unnecessary hospital admissions. METHODS A retrospective review at a level I pediatric trauma referral center was conducted for patients with ISF on head computed tomography from 2003 to 2008. Patients were excluded for injury greater than 24 hours prior, GCS less than 15, intracranial pathology, significant fracture depression, or complex fractures involving facial bones or skull base. RESULTS A total of 235 patients were identified with an ISF. The median age was 11 months, with falls accounting for 87% of the injuries. One hundred seventy-seven patients were admitted, and 58 patients were discharged from the emergency department after a period of observation (median, 3.3 hours). Median length of stay for those admitted to the hospital was 18.2 hours. One patient developed vomiting following overnight observation and a repeat computed tomography scan demonstrated a small extra-axial hematoma that required no intervention. The mean total costs for patients discharged from the emergency department were

Improving transitions of care at hospital discharge--implications for pediatric hospitalists and primary care providers.

291 vs

Determinants of health care use in a population-based leukodystrophy cohort.

1447 for those admitted for observation (P < .001). CONCLUSION Patients with a presenting GCS of 15 and an ISF can be safely discharged from the emergency department after a short period of observation if they are asymptomatic and have a reliable social environment. This could result in significant savings by eliminating inpatient costs.

Effect of gastrojejunal feedings on visits and costs in children with neurologic impairment

OBJECTIVE To describe patient demographics, interventions, and outcomes in hospitalized children with macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE) or juvenile idiopathic arthritis (JIA). METHODS We performed a retrospective cohort study using data recorded in the Pediatric Health Information System (PHIS) database from October 1, 2006 to September 30, 2010. Participants had International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes for MAS and either SLE or JIA. The primary outcome was hospital mortality (for the index admission). Secondary outcomes included intensive care unit (ICU) admission, critical care interventions, and medication use. RESULTS A total of 121 children at 28 childrens hospitals met the inclusion criteria, including 19 children with SLE and 102 children with JIA. The index admission mortality rate was 7% (8 of 121 patients). ICU admission (33%), mechanical ventilation (26%), and inotrope/vasopressor therapy (26%) were common. Compared to children with JIA, those with SLE had a similar mortality rate (6% versus 11%, respectively; exact P = 0.6). More patients with SLE than those with JIA received ICU care (63% versus 27%; P = 0.002), received mechanical ventilation (53% versus 21%; P = 0.003), and had cardiovascular dysfunction (47% versus 23% received inotrope/vasopressor therapy; P = 0.02). Children with SLE and those with JIA received cyclosporine at similar rates, but more children with SLE received cyclophosphamide and mycophenolate mofetil, and more children with JIA received interleukin-1 antagonists. CONCLUSION Organ system dysfunction is common in children with rheumatic diseases complicated by MAS, and more organ system support is required in children with underlying SLE than in children with JIA. Current treatment of pediatric MAS varies based on the underlying rheumatic disease.

Outcomes After Skin and Soft Tissue Infection in Infants 90 Days Old or Younger

&NA; Delays, omissions, and inaccuracy of discharge information are common at hospital discharge and put patients at risk for adverse outcomes. We assembled an interdisciplinary team of stakeholders to evaluate our current discharge process between hospitalists and primary care providers (PCPs). We used a fishbone diagram to identify potential causes of suboptimal discharge communication to PCPs. Opportunities for improvement (leverage points) to achieve optimal transfer of discharge information were identified using tally sheets and Pareto charts. Quality improvement strategies consisted of training and implementation of a new discharge process including: (1) enhanced PCP identification at discharge, (2) use of an electronic discharge order and instruction system, and (3) autofaxing discharge information to PCPs. The new discharge processs impact was evaluated on 2,530 hospitalist patient discharges over a 34‐week period by measuring: (1) successful transfer of discharge information (proportion of discharge information sheets successfully faxed to PCPs), (2) timeliness (proportion of sheets faxed within 2 days of discharge), and (3) content (presence of key clinical elements in discharge sheets). Postintervention, success, and timeliness of discharge information transfer between pediatric hospitalists and PCPs significantly improved while content remained high.