Showing papers by "Primary Children's Hospital published in 2008"

Hospital care for children with hydrocephalus in the United States: utilization, charges, comorbidities, and deaths.

Abstract: Object The aims of this study were to measure inpatient health care for pediatric hydrocephalus in the US; describe patient, hospital, and hospitalization characteristics for pediatric hydrocephalus inpatient care; and determine characteristics associated with death. Methods A cross-sectional study was performed using the 1997, 2000, and 2003 Healthcare Cost and Utilization Project Kids' Inpatient Databases (KID), nationally representative weighted data sets of hospital discharges for pediatric patients. A hydrocephalus-related hospitalization was classified as either cerebrospinal fluid (CSF) shunt–related (including initial placements, infections, malfunctions, or other) or non–CSF shunt–related. Patients > 18 years of age were excluded. The KID provided weighted estimates of 6.657, 6.597, and 6.732 million total discharges in the 3 study years. Results Each year there were 38,200–39,900 admissions, 391,000–433,000 hospital days, and total hospital charges of $1.4–2.0 billion for pediatric hydrocephalus...

Forecasting daily patient volumes in the emergency department.

Abstract: Background: Shifts in the supply of and demand for emergency department (ED) resources make the efficient allocation of ED resources increasingly important. Forecasting is a vital activity that guides decision-making in many areas of economic, industrial, and scientific planning, but has gained little traction in the health care industry. There are few studies that explore the use of forecasting methods to predict patient volumes in the ED. Objectives: The goals of this study are to explore and evaluate the use of several statistical forecasting methods to predict daily ED patient volumes at three diverse hospital EDs and to compare the accuracy of these methods to the accuracy of a previously proposed forecasting method. Methods: Daily patient arrivals at three hospital EDs were collected for the period January 1, 2005, through March 31, 2007. The authors evaluated the use of seasonal autoregressive integrated moving average, time series regression, exponential smoothing, and artificial neural network models to forecast daily patient volumes at each facility. Forecasts were made for horizons ranging from 1 to 30 days in advance. The forecast accuracy achieved by the various forecasting methods was compared to the forecast accuracy achieved when using a benchmark forecasting method already available in the emergency medicine literature. Results: All time series methods considered in this analysis provided improved in-sample model goodness of fit. However, postsample analysis revealed that time series regression models that augment linear regression models by accounting for serial autocorrelation offered only small improvements in terms of postsample forecast accuracy, relative to multiple linear regression models, while seasonal autoregressive integrated moving average, exponential smoothing, and artificial neural network forecasting models did not provide consistently accurate forecasts of daily ED volumes. Conclusions: This study confirms the widely held belief that daily demand for ED services is characterized by seasonal and weekly patterns. The authors compared several time series forecasting methods to a benchmark multiple linear regression model. The results suggest that the existing methodology proposed in the literature, multiple linear regression based on calendar variables, is a reasonable approach to forecasting daily patient volumes in the ED. However, the authors conclude that regression-based models that incorporate calendar variables, account for site-specific special-day effects, and allow for residual autocorrelation provide a more appropriate, informative, and consistently accurate approach to forecasting daily ED patient volumes.

Alterations in glucose homeostasis in the pediatric intensive care unit: Hyperglycemia and glucose variability are associated with increased mortality and morbidity.

Abstract: Objectives:Critically ill patients with alterations in glucose equilibrium may experience adverse outcomes. We sought to describe the distribution of blood glucose values in the absence of insulin therapy and to evaluate the association of hyperglycemia, hypoglycemia, and glucose variability with mo

Diagnosis and laparoscopic treatment of surgical diseases during pregnancy: an evidence-based review.

Abstract: Treatment of surgical disease in the gravid patient requires a unique and careful approach where safety of the mother and fetus are both considered. Approaches to diagnosis and therapy of surgical disease in the gravid patient are increasingly clarified and defined in the literature. Laparoscopy, once described as contraindicated in pregnancy, has been steadily accepted and applied as data supporting its safety and use have accumulated. An extensive review of the literature was performed to define the use of laparoscopy in pregnancy. Diagnoses for independent surgical diseases as well as imaging modalities and techniques during pregnancy are reviewed. Preoperative, intraoperative, and postoperative management of the pregnant patient are described and evaluated with focus on use of laparoscopy. Literature supporting safety and efficacy of laparoscopy in cholecystectomy, appendectomy, solid organ resection, and oophorectomy in the gravid patient is outlined. Based on level of evidence, this review includes recommendations specific to surgical approach, trimester of pregnancy, patient positioning, port placement, insufflation pressure, monitoring, venous thromboembolic prophylaxis, obstetric consultation, and use of tocolytics in the pregnant patient.

Seasonal Invasive Pneumococcal Disease in Children: Role of Preceding Respiratory Viral Infection

Abstract: OBJECTIVE. Our objective was to demonstrate correlations between invasive pneumococcal disease in children and circulating respiratory viruses. METHODS. This retrospective study included 6 winter respiratory viral seasons (2001–2007) in Intermountain Healthcare, an integrated health system in the Intermountain West, including Primary Children9s Medical Center in Salt Lake City, Utah. Children RESULTS. A total of 435 children with invasive pneumococcal disease and 203 with culture-confirmed invasive pneumococcal disease were hospitalized in an Intermountain Healthcare facility or Primary Children9s Medical Center during the study period. During the same period, 6963 children with respiratory syncytial virus, 1860 with influenza virus, 1459 with parainfluenza virus, and 818 with adenoviruses were evaluated at Primary Children9s Medical Center. A total of 253 children with human metapneumovirus were identified during the last 5 months of the study. There were correlations between invasive pneumococcal disease and seasonal respiratory syncytial virus, influenza virus, and human metapneumovirus activity. The correlation with invasive pneumococcal disease was strong up to 4 weeks after respiratory syncytial virus activity. For influenza virus and human metapneumovirus, the correlations were strong at 2 weeks after activity of these viruses. Pneumonia was the most common clinical disease associated with culture-confirmed invasive pneumococcal disease, mostly attributable to serotypes 1, 19A, 3, and 7F. CONCLUSIONS. In the post–pneumococcal conjugate vaccine era, seasonal increases in respiratory syncytial virus, influenza virus, and human metapneumovirus infections in children were associated with increased pediatric admissions with invasive pneumococcal disease, especially pneumonia caused by nonvaccine serotypes.

Pneumococcal Necrotizing Pneumonia in Utah: Does Serotype Matter?

Abstract: Background. Streptococcus pneumoniae is the most common cause of bacterial pneumonia in children. Despite the use of the 7-valent pneumococcal conjugate vaccine, the incidence of pneumococcal necrotizing pneumonia (PNP) has been increasing. Our objectives were to describe temporal trends in PNP and to evaluate pneumococcal serotypes associated with PNP in Utah. Methods. We performed a retrospective review of all children <18 years of age who were cared for at a tertiary care children's hospital and who had blood, lung tissue, broncheoalveolar lavage, or pleural fluid cultures that grew S. pneumoniae, as well as radiographic evidence of pneumonia, from January 1997 through March 2006. All S. pneumoniae isolates were typed. Results. A total of 124 children with pneumococcal pneumonia were identified, and 33 (27%) of these children had radiographic evidence of PNP. During the period 1997-2000, 5 (13%) of 39 cases of culture-confirmed pneumococcal pneumonia were associated with PNP. In contrast, during the period 2001-2006, 28 (33%) of 85 pneumococcal pneumonia cases were complicated by PNP (odds ratio, 3.34; 95% confidence interval, 1.11-12.03). Non-7-valent pneumococcal conjugate vaccine serotypes comprised 49% of the isolates during 1997-2000 and 88% of isolates during 2001-2006 (odds ratio, 7.89; 95% confidence interval, 2.91-21.90). Pneumonia due to serotype 3 was most often associated with PNP. Eleven (79%) of 14 cases of serotype 3-associated pneumonia were associated with PNP. When compared with all other serotypes, serotype 3 was strongly associated with necrosis (odds ratio, 14.67; 95% confidence interval, 3.39-86.25). Conclusions. PNP is a serious and increasingly common complication of S. pneumoniae pneumonia in Utah. Infection with serotype 3 is associated with an increased risk of developing PNP. The increase in the incidence of infection due to nonvaccine serotypes reported worldwide and the changing epidemiology of invasive pneumococcal disease should be considered when developing vaccine strategies.

Sleep disordered breathing and obstructive sleep apnea in the cleft population.

Abstract: Objectives/Hypothesis: Children with cleft deformities have the tendency for multilevel airway obstruction. The incidence of sleep disordered breathing (SDB) in this population has not been well studied. This study attempts to describe the high incidence and the results of intervention. Study Design: A three-year retrospective chart review by a tertiary cleft and craniofacial team. Methods: The symptoms of sleep disordered breathing and polysomnographic data were reviewed and analyzed using descriptive statistics and multivariate analysis. Results: Of the 539 children seen during the period, 120 (22%) had symptoms suggestive of SDB. Twenty-four of them had a tonsillectomy with or without partial adenoidectomy without polysomnogram (PSG). Sixty-nine (57%) had a PSG, and 28 (40%) had a follow-up PSG. Syndromic children had significantly more symptoms of SDB (P < .001) and were more likely to undergo PSG (P < .05). Of those children who underwent a PSG, only six had a normal obstructive apnea–hypopnea index (OAHI) with a mean (±standard deviation) respiratory disturbance index (RDI) of 15.5 (±17.5) and OAHI of 12.05 (±15.57). Post intervention PSG showed significant improvement in RDI (P = .048) and OAHI (P = .012) using a single-tailed Wilcoxan analysis. Unfortunately, most of these children still had significant sleep apnea. There was also a high percentage of children with periodic leg motion syndrome (24.7%), some of whom also had OSA. Conclusion: There is a high incidence of SDB and definable OSA in the cleft population. Though there is a statistically significant improvement after intervention, some were not cured. Sleep disturbance and OSA is likely under-reported and treated in the cleft population. PSG should be done more frequently, and post intervention PSG should be strongly considered.

Familial occurrence of schwannomas and malignant rhabdoid tumour associated with a duplication in SMARCB1

Abstract: Background: The role of germline and somatic SMARCB1 gene mutations in malignant rhabdoid tumour (MRT) predisposition is well known. Germline SMARCB1 mutations have also recently been identified in a subset of individuals with schwannomatosis. Surprisingly, MRT predisposition and schwannomatosis have never been reported to co-occur in a family. The correlation between genotype and phenotype for mutations in SMARCB1 has not been determined. Results: We have identified a germline 2631 bp duplication that includes exon 6 of SMARCB1 in a unique family with a four generation history of MRT predisposition and schwannomatosis. This duplication segregates with disease in individuals affected with both conditions, linking MRT predisposition and schwannomatosis as components of the same syndrome in this family. Conclusion: The unique combination of tumours that result from the duplication described in this report may provide important clues about the mechanisms that influence the phenotype associated with a given SMARCB1 mutation.

Pilomyxoid Astrocytoma: Expanding the Imaging Spectrum

Abstract: BACKGROUND AND PURPOSE: Pilomyxoid astrocytoma (PMA) is a recently described variant of pilocytic astrocytoma (PA) with unique clinical and histopathologic characteristics. Because the histopathology of PMA is distinct from that of PA, we hypothesized that PMAs would display distinctive imaging characteristics. We retrospectively reviewed the imaging findings in a large number of patients with PMA to identify these characteristics. MATERIALS AND METHODS: CT and MR images, pathology reports, and clinical information from 21 patients with pathology-confirmed PMA from 7 institutions were retrospectively reviewed. CT and MR imaging findings, including location, size, signal intensity, hemorrhage, and enhancement pattern, were tabulated. RESULTS: Patients ranged in age from 9 months to 46 years at initial diagnosis. Sex ratio was 12:9 (M/F). Twelve of 21 (57%) tumors were located in the hypothalamic/chiasmatic/third ventricular region. Nine (43%) occurred in other locations, including the parietal lobe (2/21), temporal lobe (2/21), cerebellum (2/21), basal ganglia (2/21), and fourth ventricle (1/21). Ten (48%) tumors showed heterogeneous rim enhancement, 9 (43%) showed uniform enhancement, and 2 (9%) showed no enhancement. Five (24%) masses demonstrated intratumoral hemorrhage. CONCLUSION: This series expands the clinical and imaging spectrum of PMA and identifies characteristics that should suggest consideration of this uncommon diagnosis. One third of patients were older children and adults. Almost half of all tumors were located outside the typical hypothalamic/chiasmatic region. Intratumoral hemorrhage occurred in one quarter of patients. PMA remains a histologic diagnosis without definitive imaging findings that distinguish it from PA.

Jugular venous valved conduit (Contegra) matches allograft performance in infant truncus arteriosus repair.

Abstract: Objective:Limitedavailabilityanddurabilityofallograftconduitsrequirethatalternativesbeconsidered.Wecomparedbovinejugularvenous valved (JVV) and allograft conduit performance in 107 infants who survived truncus arteriosus repair. Methods: Children were prospectively recruited between 2003 and 2007 from 17 institutions. The median z-score for JVV (n = 27, all 12 mm) was +2.1 (range +1.2 to +3.2) and allograft (n = 80, 9—15 mm) was +1.7 (range 0.4 to +3.6). Propensity-adjusted comparison of conduit survival was undertaken using parametric riskhazard analysis and competing risks techniques. All available echocardiograms (n = 745) were used to model deterioration of conduit function in regression equations adjusted for repeated measures. Results: Overall conduit survival was 64 9% at 3 years. Conduit replacement was for conduit stenosis (n = 16) and/or pulmonary artery stenosis (n = 18) or regurgitation (n = 1). The propensity-adjusted 3-year freedom from replacement for in-conduit stenosis was 96 4% for JVVand 69 8% for allograft (p = 0.05). The risk of intervention or replacement for branch pulmonary artery stenosis was similar for JVV and allograft. Smaller conduit z-score predicted poor conduit performance (p < 0.01) with best outcome between +1 and +3. Although JVV conduits were a uniform diameter, their z-score more consistently matched this ideal. JVVexhibited a non-significant trend towards slower progression of conduit regurgitation and peak right ventricular outflow tract (RVOT) gradient. In addition, catheter intervention was more successful at slowing subsequent gradient progression in children with JVV versus those with allograft (p < 0.01). Conclusions: JVV does match allograft performance and may be advantageous. It is an appropriate first choice for repair of truncus arteriosus, and perhaps other small infants requiring RVOT reconstruction. # 2008 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Pediatric palliative care: an assessment of physicians' confidence in skills, desire for training, and willingness to refer for end-of-life care.

Abstract: This study determines the confidence levels of physicians in providing components of pediatric palliative care and identifies their willingness to obtain training and to make palliative care referrals. Surveys were mailed to all physicians at Primary Children's Medical Center. The survey instrument includes 3 demographic items, 9 items designed to assess physician confidence in core palliative care skills, and 4 items designed to assess what steps physicians would be likely to take to assure that patients receive palliative care. Physicians were asked to rate their confidence levels to provide palliative care components on a 4-point scale for each of the items. Five hundred ninety-seven surveys were mailed, with 323 usable surveys returned. The proportion of physicians who rate their ability to provide palliative care as "confident" or "very confident" ranges from 74% for giving difficult news to families to 23% for managing end-of-life symptoms. Thirty-six percent of the physicians say they would be "likely" or "very likely" to attend training to improve their ability to provide palliative care to children. Eighty-six percent would be "likely" or "very likely" to refer for a palliative care consult and 91% to a home health agency or hospice. There is wide variation in the confidence levels of physicians to provide the core components of palliative care. Few are interested in obtaining additional training, but most are willing to obtain consultation or to refer to a palliative care service. These results argue in favor of hospital-based palliative care teams and for specialty training and certification in pediatric palliative care.

Value of overnight monitoring of intracranial pressure in hydrocephalic children.

Abstract: Objective: Exaggerated nocturnal intracranial pressure (ICP) dynamics are commonly observed in hydrocephalic children with a compromise of CSF compensatory reserve capacity. Success

Adult GM1 gangliosidosis: clinical and biochemical studies on two patients and comparison to other patients called variant or adult GM1 gangliosidosis.

Abstract: Two young adult siblings were diagnosed as having a deficiency of acid beta-galactosidase activity in leukocytes and fibroblasts. The parents had enzyme levels approximately half of the normal level, consistent with this being the primary enzymatic lesion. Sialidose activities measured with natural and synthetic substrates in the patient's skin fibroblast cultures were normal. Hybridization of one of these patient's cells with cells from a patient with GM1 gangliosidosis, Type 1 did not show complementation of beta-galactosidase activity. However, when the cells from the patient were hybridized with cells from a patient with combined sialidase and beta-galactosidase deficiency, complementation was observed. These two siblings have ataxia, mild intellectual deterioration, slurred speech, mild vertebral changes and little, if any, visceromegaly. They do not have myoclonus, seizures or cherry-red spots, which are found in most patients with combined sialidase and beta-galactosidase deficiency. These patients are discussed with regard to other patients in the literature called variant or adult GM1 gangliosidosis.

Factors influencing nurses' attitudes toward simulation-based education.

Abstract: Objective:To identify barriers to nurses’ participation in simulation, and to determine whether prior simulation exposure, professional experience, and practice location influence their tendency to perceive specific issues as barriers. We also sought to identify nurses’ educational priorities, and t

Pulmonary Regurgitation: Determining Severity by Echocardiography and Magnetic Resonance Imaging

Abstract: Objective Pulmonary regurgitation (PR) is common after repair of congenital heart disease involving the right ventricular outflow tract. Because PR results in chronic right ventricular volume overload and associated morbidity and mortality, accurate assessment of its severity is important. The aim of this study was to compare echocardiography with the gold standard of PR quantitation by magnetic resonance imaging (MRI) in a young population with repaired congenital heart disease. Design/methods Patients with congenital heart disease who had undergone right ventricular outflow tract reconstruction and/or pulmonary valve replacement and had an MRI within 3 months of an echocardiogram formed the study group. Echocardiographic indices were compared with MRI-determined pulmonary regurgitant fraction (PRF) to determine the most accurate measurements to quantitate PR. Results Of the 69 MRI/echocardiography pairs in 64 patients, 53 data sets were complete and used in the analysis. For the prediction of MRI PRF > or =20%, PR jet width/annulus ratio > or =0.5 demonstrated excellent sensitivity (94%), specificity (100%), positive predictive value (PPV 100%), and negative predictive value (NPV 82%). For the prediction of MRI PRF > or =40%, jet width/annulus ratio > or =0.7 and diastolic flow reversal in the branch pulmonary arteries showed useful sensitivity (92%), specificity (68%), PPV (76%), and NPV (88%). Conclusion Pulmonary regurgitation jet width/annulus ratio combined with diastolic flow reversal is the most valuable echocardiographic measure for assessing PR severity after right ventricular outflow tract reconstruction or pulmonary valve replacement; however, this surrogate measure does not replace the importance of MRI evaluation.

Childhood traumatic brain injury: neuropsychological status at the time of hospital discharge

Abstract: The present study examined the status at the time of hospital discharge of 22 children who had sustained moderate to severe closed head injuries. Despite an average length of stay of 46 days, on average the children performed significantly below normal on cognitive, memory, and motor tests. The children who did the worst were those who sustained their injuries riding in a vehicle, as opposed to being hit by a vehicle while walking or riding a bicycle. No reliable associations were found between neuroimaging data and cognitive measures; however, children whose scans showed more diffuse severe injury (e.g. white matter hemorrhages) performed more poorly than those who did not. Of further importance were detailed morphometric analyses of tissue quantity and ventricular volume that showed significantly increased ventricle to brain ratios (VBR) over a 3 to 4 week period following injury.

Prognostic value of B-type natriuretic peptide in surgical palliation of children with single-ventricle congenital heart disease.

Abstract: The objective of this prospective study was to assess the prognostic role of perioperative B-type natriuretic peptide (BNP) levels in infants and children with single-ventricle congenital heart disease undergoing Norwood, bidirectional cavopulmonary anastomosis (BCPA), or Fontan operation. BNP levels were measured at baseline, after cardiopulmonary bypass, 6 to 12 hours after surgery, and then daily until indwelling vascular catheters were removed. Outcome measures included length of mechanical ventilation, inotropic support, and hospital stay. Twenty subjects underwent 23 surgical procedures (13 Norwood, 5 BCPA, and 5 Fontan). BNP levels were significantly higher in patients undergoing a Norwood procedure compared with a BCPA or Fontan procedure (p < 0.01). BNP levels measured 6 to 12 hours after surgery were predictive of duration of hospitalization (p = 0.005) and inotropic support (p = 0.01). An increase in BNP level within 48 hours of extubation was observed in 92% of patients undergoing a Norwood procedure. Early postoperative BNP levels correlate significantly with the ensuing duration of inotropic support and length of hospitalization. An increase in BNP after extubation may be reflective of the degree of underlying cardiopulmonary instability. Further investigation is necessary to define this important relation.

Expert Survey for the Management of Adolescent Depression in Primary Care

Abstract: OBJECTIVE: Primary care clinics have become the "de facto" mental health clinics for teens with mental health problems such as depression; however, there is little guidance for primary care professionals who are faced with treating this population. This study surveyed experts on key management issues regarding adolescent depression in primary care where empirical literature was scant or absent. METHODS: Participants included experts from family medicine, pediatrics, nursing, psychology, and child psychiatry, identified through nonprobability sampling. The expert survey was developed on the basis of information from focus groups with patients, families, and professionals and from the research literature and included sections on early identification, assessment and diagnosis, initial management, treatment, and ongoing management. Means, standard deviations, and confidence intervals were calculated for each survey item. RESULTS: Seventy-eight of 81 experts agreed to participate (return rate of 96%). Fifty-three percent of the experts (n = 40) were primary care professionals. Experts endorsed routine surveillance for youth at high risk for depression, as well as the use of standardized measures as diagnostic aids. For treatment, "active monitoring" was deemed appropriate in mild depression with recent onset. Medication and psychotherapy were considered acceptable options for treatment of moderate depression without complicating factors such as comorbid illness. Fluoxetine was rated as the most appropriate antidepressant for use in this population. Finally, experts agreed that patients who are started on antidepressants should be followed within 2 weeks after initiation. CONCLUSIONS: Survey results support the identification and management of adolescent depression in the primary care setting and, in specific situations, referral and co-management with specialty mental health professionals. Even with the recent controversies around treatment, experts across primary care and specialty mental health alike agreed that active monitoring, pharmacotherapy with selective serotonin reuptake inhibitors, and psychotherapy can be appropriate under certain clinical circumstances when initiated within primary care settings. Language: en

Reducing irinotecan-associated diarrhea in children.

Abstract: Irinotecan is increasingly being used in pediatric oncology. Amelioration of diarrhea associated with protracted irinotecan administration may reduce morbidity and improve dose intensity. In this review, we discuss what is known about the pathogenesis of this toxicity as well as potential predisposing genetic factors. We comprehensively summarize the literature regarding available prevention and treatment strategies, and report data on the use of cephalosporin prophylaxis in 51 patients treated on various pediatric trials. This approach is feasible in children and allows for tolerance of higher doses of protracted irinotecan.

Progressive cerebral vascular degeneration with mitochondrial encephalopathy.

Abstract: MELAS (mitochondrial encephalopathy with lactic acidosis and stroke-like episodes) is a maternally inherited disorder characterized by recurrent cerebral infarctions that do not conform to discreet vascular territories. Here we report on a patient who presented at 7 years of age with loss of consciousness and severe metabolic acidosis following vomiting and dehydration. She developed progressive sensorineural hearing loss, myopathy, ptosis, short stature, and mild developmental delays after normal early development. Biochemical testing identified metabolites characteristic of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency (hexanoylglycine and suberylglycine), but also severe lactic acidemia (10-25 mM) and, in urine, excess of lactic acid, intermediates of the citric cycle, and marked ketonuria, suggesting mitochondrial dysfunction. She progressed rapidly to develop temporary cortical blindness. Brain imaging indicated generalized atrophy, more marked on the left side, in addition to white matter alterations consistent with a mitochondrial disorder. Magnetic resonance angiography indicated occlusion of the left cerebral artery with development of collateral circulation (Moyamoya syndrome). This process worsened over time to involve the other side of the brain. A muscle biopsy indicated the presence of numerous ragged red fibers. Molecular testing confirmed compound heterozygosity for the common mutation in the MCAD gene (985A>G) and a second pathogenic mutation (233T>C). MtDNA testing indicated that the muscle was almost homoplasmic for the 3243A>T mutation in tRNALeu, with a lower mutant load (about 50% heteroplasmy) in blood and skin fibroblasts. These results indicate that mitochondrial disorders may be associated with severe vascular disease resulting in Moyamoya syndrome. The contribution of the concomitant MCAD deficiency to the development of the phenotype in this case is unclear.

Validation of a nutrition risk screening tool for children and adolescents with cystic fibrosis ages 2-20 years.

Abstract: Objectives: According to the 2002 Cystic Fibrosis (CF) Foundation nutrition consensus report, children with CF should grow normally. Cross-sectional data from the foundation’s patient registry concluded that a body mass index at or greater than the 50th percentile is associated with better lung function. A consistent, evidence-based screening process can identify those individuals with CF having nutrition risk factors associated with a decrease in pulmonary function, target early intervention, and prevent further decline. A tool for screening nutrition risk is described to identify those children with CF who would benefit from more extensive nutrition intervention. Methods: The proposed screening tool is a risk-based classification system with 3 categories: weight gain, height velocity, and body mass index. The CF Foundation recommendations regarding these parameters are incorporated, with risk points assigned when minimum body mass index, weight gain, and/or height gain standards are unmet. Results: An interrater measure of agreement determined a satisfactory level of reliability (k ¼ 0.85). Patient records (n ¼ 85) were reviewed to determine nutrition status category (no risk or at risk) of this tool compared with the CF Foundation 2002 Nutrition Consensus, yielding sensitivity and specificity at 84% and 75%, respectively. A second comparison was made with combined, independent nutrition risk factors not included in the screening tool. The sensitivity and specificity of the screening tool compared with the combined risk factors were 86% and 78%, respectively. Conclusions: This tool for screening nutrition risk for CF is reliable and valid, with consistent, reproducible results, free from subject or observer bias. JPGN 46:438–446, 2008.