Joseph M. Geskey

Transmission of surfactant protein variants and haplotypes in children hospitalized with respiratory syncytial virus.

Severity of lung injury with respiratory syncytial virus (RSV) infection is variable and may be related to genetic variations. This preliminary report describes a prospective, family-based association study of children hospitalized secondary to RSV, aimed to determine whether intragenic and other haplotypes of surfactant proteins (SP)-A and SP-D are transmitted disproportionately from parents to offspring with RSV disease. Genomic DNA was genotyped for several SP-A and SP-D single nucleotide polymorphisms (SNPs). Transmission disequilibrium test analysis was used to determine transmission of variants and haplotypes from parents to affected offspring. Three hundred seventy-five individuals were studied, including 148 children with active RSV disease and one or both parents. The SP-A2 intragenic haplotype 1A2 was found to be protective (p = 0.013). The SP-D SNP DA160_A may possibly be an “at-risk” marker (p = 0.0058). Additional two- and three-marker haplotypes were associated with severe RSV disease, with two being protective (DA11_T/DA160_G and DA160_G/SP-A2 1A0/SP-A1 6A2). We conclude that there may be associations between SP-A and SP-D and RSV disease. Further study is required to determine whether these variants can be used to target a high-risk patient population in clinical trials aimed at reducing either the symptoms of acute infection or long-term pulmonary sequelae.

Palivizumab prophylaxis to prevent respiratory syncytial virus mortality after pediatric bone marrow transplantation: a decision analysis model.

Objective Palivizumab, a monoclonal antibody against respiratory syncytial virus (RSV), has been demonstrated to be safe and effective in young children, but evidence is lacking as to whether palivizumab is effective in preventing RSV-induced morbidity and mortality in children who are immunosuppressed after bone marrow transplantation (BMT). As a randomized, double-blind, placebo-controlled trial is lacking, we chose to examine this issue with the use of decision analysis methodology. Methods A decision tree was designed to determine mortality from RSV-related lung disease in children who received palivizumab after BMT. Probabilities were derived by meta-analysis methodology on the basis of the available literature. Sensitivity analyses were performed across a broad range of biologically plausible probabilities to judge the robustness of the results of the model. Results The model revealed that there is a 10% increase in survival in BMT patients who receive palivizumab. The absolute survival rate increased from 83% to 92%. A practitioner would need to treat 12 children to save 1 post-BMT child from dying from RSV-related lung disease. Conclusions Decision analysis modeling demonstrates a decrease in mortality in pediatric BMT patients with the addition of palivizumab to protect against RSV-related lung disease. A well-designed, randomized controlled trial is necessary.

Evaluating Communication Between Pediatric Primary Care Physicians and Hospitalists

To determine the preferences for and satisfaction with communication between pediatric primary care physicians (PCPs) and hospitalists, 2 surveys (PCP and hospitalist versions with matching questions) were developed. Overall, PCPs were less satisfied than hospitalists with communication (P < .01). The 2 provider types had differing opinions on responsibility for care after hospital discharge, with hospitalists more likely than PCPs to assign responsibility to the PCP for pending labs (65% vs 49%; P < .01), adverse events (85% vs 67%; P < .01), or status changes (85% vs 69%; P < .01). Whereas satisfaction with and preferences for patient-related communication differed between hospitalists and PCPs, the incongruent views on the responsibility for care after patient discharge have major implications for safety particularly if poor communication occurs at discharge. Successful transitions from the hospital to primary care require communication between hospitalists and PCPs to be consistent, timely, and informative with responsibility for care discussed at discharge.

Palivizumab in congenital heart disease: should international guidelines be revised?

Palivizumab has reduced the incidence of respiratory syncytial virus hospitalization in infants and children with congenital heart disease by 45%. Although the mortality rate of children with congenital heart disease hospitalized with respiratory syncytial virus infection has declined from 37% to ∼ 3% over the past 3 decades, palivizumab has not been shown to improve mortality. There has been considerable controversy over the cost-effectiveness of administering palivizumab according to international guidelines, including children with congenital heart disease. In particular, the number of children that need to be treated with palivizumab to prevent one respiratory syncytial virus hospitalization increases dramatically in children > 12 months of age. As a result, the authors recommend that countries re-examine their recommendations for providing palivizumab up to age 24 months in children with congenital heart disease.

Managing the Morbidity Associated with Respiratory Viral Infections in Children with Congenital Heart Disease

Children with congenital heart disease (CHD) are at risk for increased morbidity from viral lower respiratory tract infections because of anatomical cardiac lesions than can worsen an already compromised respiratory status. Respiratory syncytial virus (RSV) remains an important pathogen in contributing toward the morbidity in this population. Although the acute treatment of RSV largely remains supportive, the development of monoclonal antibodies, such as palivuzumab, has reduced the RSV-related hospitalization rate in children with CHD. This review highlights the specific cardiac complications of RSV infection, the acute treatment of bronchiolitis in patients with CHD, and the search for new therapies against RSV, including an effective vaccine, because of the high cost associated with immunoprophylaxis and its lack of reducing RSV-related mortality.

Superior mesenteric artery syndrome in intellectually disabled children.

Objectives Superior mesenteric artery syndrome (SMAS) is a rare cause of small intestinal obstruction in pediatric patients. Children with intellectual disabilities are a challenging patient population in which to make this diagnosis. The goal of this study was to determine the frequency, presenting symptoms, and outcomes of SMAS in intellectually disabled and non–intellectually disabled children. Methods Retrospective chart review of pediatric patients with SMAS admitted to Penn State Hershey Children’s Hospital between January 1999 and July 2010. Results Sixteen children with SMAS were identified. The majority were male (n = 9) and more than two thirds had an intellectual disability (n = 11). Presenting symptoms were similar between groups, but 78% (7/9) of intellectually disabled patients who had a gastrostomy tube presented with feeding intolerance. Although intellectually disabled patients had a higher number of comorbidities and the number of health care visits before diagnosis was higher, this did not reach statistical significance. There were also no significant differences in length of symptoms before diagnosis and amount of weight loss. However, the weight-for-age percentiles in intellectual disabled children were significantly lower in those without an intellectual disability (9.09 [20.31] vs 48 [20.19], respectively, P ⩽ 0.001). Seventy-five percent of patients responded favorably to conservative treatment, but 36% (4/11) of intellectually disabled patients required multiple treatments before there was an improvement in their condition. Conclusions Superior mesenteric artery syndrome was found more often in children with an intellectual disability. These data highlight the need to consider SMAS if there is difficulty tolerating gastrostomy tube feedings in patients with weight-for-age percentiles less than 5%.

Improved Physician Consult Response Times in an Academic Emergency Department After Implementation of an Institutional Guideline

BACKGROUND Physician consultation in the Emergency Department (ED) can account for a significant portion of ED length of stay, which can lead to poor clinical outcomes. OBJECTIVE The purpose of this study was to determine whether an institutional guideline could lead to a reduction in time between consult request and admission decision. This guideline codified a 90-min expected time interval to arrive and complete an admission disposition where the consulting and admitting service were the same in an academic ED with weekly audits and reports to departmental chairs and hospital administrators. METHODS This was a study of consultation times of patients who presented to an academic ED 6 months before the adoption of an institutional guideline and 6 months after the adoption of the guideline. Data measurement in both periods included the length of time from ED consult order to admission disposition, time of ED discharge, number of ED consultations (single and multiple), ED admissions, and the hospital discharge time of admitted patients. RESULTS Physician consult response time decreased from 121 min to 100 min (p < 0.0001), and patients left the ED 18 min earlier (p = 0.0221) after implementation of the consultation guideline despite more ED visits, consultations, and admissions in the post-implementation time period. Patients were discharged from the inpatient setting 50 min later (p < 0.0001) after implementation of the guideline. CONCLUSION An institutional guideline codifying timely ED consultations led to a significant reduction in the time from ED consultation to admission disposition while also allowing patients to leave the ED earlier in a high-occupancy academic medical center. However, the discharge time of admitted hospital patients was later after implementation of the guideline.

Pulmonary Arteriovenous Malformation: An Unusual Case of Hypoxemia in an Infant:

Hypoxemia in infants during the winter months can often be attributed to respirator y syncytial virus (RSV) bronchiolitis.1 However, other causes of hypoxemia must be kept in the differential diagnosis, especially when patients do not follow the typical disease course. One rare cause of hypoxemia is pulmonary arteriovenous malformations (PAVMs).2 The majority of PAVMs occur in patients with hereditar y hemorrhagic telangiectasias (HHT, Rendu-Osler-Weber syndrome), but they can also be seen in children with congenital heart disease or rarely as an isolated occurrence.3 HHT is an autosomal dominant disorder characterized by epistaxis, multiple mucocutaneous telangiectasias, visceral lesions such as pulmonary or cerebral AVMs, and a f irst-degree relative with HHT.4 The present report describes a 1-year-old female who presented to our institution with profound hypoxemia and was diagnosed with a pulmonary AVM without evidence of HHT. It also reviews the diagnostic evaluation and treatment for this condition.