Disparities in first evaluation of infants with cystic fibrosis since implementation of newborn screening.

OBJECTIVE: We evaluated whether implementation of cystic fibrosis (CF) newborn screening (NBS) leads to equitable timeliness of initial evaluation. We compared age at first event (AFE, age at sweat test, encounter and/or care episode) between infants categorized as Black/African American, American Indian/ Native Alaskan, Asian, and/or Hispanic and/or other (Group 1) to White and not Hispanic infants (Group 2). METHODS: This retrospective cohort study from the Cystic Fibrosis Foundation Patient Registry (CFFPR) included infants born 2010-2018. Race and ethnicity categories followed US Census definitions. The primary outcome was AFE; the secondary outcome was weight for age (WFA) z-score averaged 12 to < 24 months. We compared distributions by Wilcoxon rank-sum test and proportions by Chi-square or Fisher's exact tests. A nested cohort study used a linear mixed effects model of variables that affect WFA, chosen a priori, to evaluate associations with 1-year WFA z-score. RESULTS: Among 6354 infants, 21% were in Group 1. Group 1 median AFE was 31 days (IQR 19, 49) and Group 2 was 22 days (IQR 14,36) (p< .001). Median WFA z-score at 1-2 years was lower in Group 1. In 3017 infants with complete data on variables of interest, AFE, Black race, CFTR variant class I-III, prematurity and public insurance were associated with lower 1-year WFA z-score. CONCLUSIONS: Differences in AFE for infants with CF from historically marginalized groups may exacerbate long standing health disparities. We speculate that inequitable identification of CFTR gene variants and/or bias may influence timeliness of evaluation after an out-of-range NBS.

Parental Views of Social Worker and Chaplain Involvement in Care and Decision Making for Critically Ill Children with Cancer.

BACKGROUND: Social workers (SWs) and chaplains are trained to support families facing challenges associated with critical illness and potential end-of-life issues. Little is known about how parents view SW/chaplain involvement in care for critically ill children with cancer. METHODS: We studied parent perceptions of SW/chaplain involvement in care for pediatric intensive care unit (PICU) patients with cancer or who had a hematopoietic cell transplant. English- and Spanish-speaking parents completed surveys within 7 days of PICU admission and at discharge. Some parents participated in an optional interview. RESULTS: Twenty-four parents of 18 patients completed both surveys, and six parents were interviewed. Of the survey respondents, 66.7% and 75% interacted with SWs or chaplains, respectively. Most parents described SW/chaplain interactions as helpful (81.3% and 72.2%, respectively), but few reported their help with decision making (18.8% and 12.4%, respectively). Parents described SW/chaplain roles related to emotional, spiritual, instrumental, and holistic support. Few parents expressed awareness about SW/chaplain interactions with other healthcare team members. CONCLUSIONS: Future work is needed to determine SWs'/chaplains' contributions to and impact on parental decision making, improve parent awareness about SW/chaplain roles and engagement with the healthcare team, and understand why some PICU parents do not interact with SWs/chaplains.

The association between pulmonary vascular disease and respiratory improvement in infants with type I severe bronchopulmonary dysplasia.

OBJECTIVE: To describe the association between echocardiographic measures of pulmonary vascular disease and time to respiratory improvement among infants with Type I severe bronchopulmonary dysplasia (sBPD). STUDY DESIGN: We measured the pulmonary artery acceleration time indexed to the right ventricular ejection time (PAAT/RVET) and right ventricular free wall longitudinal strain (RVFWLS) at 34-41 weeks' postmenstrual age. Cox-proportional hazards models were used to estimate the relationship between the PAAT/RVET, RVFWLS, and the outcome: days from 36 weeks' postmenstrual age to room-air or discharge with oxygen (≤0.5 L/min). RESULT: For 102 infants, the mean PAAT/RVET and RVFWLS were 0.27 ± 0.06 and -22.63 ± 4.23%. An abnormal measurement was associated with an increased time to achieve the outcome (PAAT/RVET: 51v24, p < 0.0001; RVFWLS; 62v38, p = 0.0006). A normal PAAT/RVET was independently associated with a shorter time to outcome (aHR = 2.04, 1.11-3.76, p = 0.02). CONCLUSION: The PAAT/RVET may aid in anticipating timing of discharge in patients with type I severe BPD.

The Feasibility of Cross-Linguistic Speech Evaluation in the Care of International Cleft Palate Patients.

ABSTRACT: Many patients with cleft palate in developing countries never receive postoperative speech assessment or therapy. The use of audiovisual recordings could improve access to post-repair speech care. The present study evaluated whether English-speaking speech-language pathologists (SLPs) could assess cleft palate patients speaking an unfamiliar language (Tamil) using recorded media. Recordings obtained from Tamil-speaking participants were rated by 1 Tamil-speaking SLP and 3 English-speaking SLPs. Ratings were analyzed for inter-rater reliability and scored for percent correct. Accuracy of the English SLPs was compared with independent t tests and Analysis of Variance. Sixteen participants (mean age 14.5 years, standard deviation [SD] 7.4 years; mean age of surgery of 2.7 years, SD 3.7 years; time since surgery: 10.8 years, SD 5.7 years) were evaluated. Across the 4 SLPs, 5 speech elements were found to have moderate agreement, and the mean kappa was 0.145 (slight agreement). Amongst the English-speaking SLPs, 10 speech elements were found to have substantial or moderate agreement, and the mean kappa was 0.333 (fair agreement). Speech measures with the highest inter-rater reliability were hypernasality and consonant production errors. The average percent correct of the English SLPs was 60.7% (SD 20.2%). English SLPs were more accurate if the participant was female, under eighteen, bilingual, or had speech therapy. The results demonstrate that English SLPs without training in a specific language (Tamil) have limited potential to assess speech elements accurately. This research could guide training interventions to augment the ability of SLPs to conduct cross-linguistic evaluations and improve international cleft care by global health teams.

Outcomes in Pediatric Thyroidectomy: Results From a Multinational, Multi-institutional Database.

OBJECTIVE: Traditionally, data regarding thyroidectomy were extracted from billing databases, but information may be missed. In this study, a multi-institutional pediatric thyroidectomy database was used to evaluate recurrent laryngeal nerve (RLN) injury and hypoparathyroidism. STUDY DESIGN: Retrospective multi-institutional cohort study. SETTING: Tertiary care pediatric hospital systems throughout North America. METHODS: Data were individually collected for thyroidectomies, then entered into a centralized database and analyzed using univariate and multivariable regression models. RESULTS: In total, 1025 thyroidectomies from 10 institutions were included. Average age was 13.9 years, and 77.8% were female. Average hospital stay was 1.9 nights and 13.5% of patients spent at least 1 night in the pediatric intensive care unit. The most frequent pathology was papillary thyroid carcinoma (42%), followed by Graves' disease (20.1%) and follicular adenoma (18.2%). Overall, 1.1% of patients experienced RLN injury (0.8% permanent), and 7.2% experienced hypoparathyroidism (3.3% permanent). Lower institutional volume (odds ratio [OR], 3.57; 95% CI, 1.72-7.14) and concurrent hypoparathyroidism (OR, 3.51; 95% CI, 1.64-7.53) correlated with RLN injury on multivariable analysis. Graves' disease (OR, 2.27; 95% CI, 1.35-3.80), Hashimoto's thyroiditis (OR, 4.67; 95% CI, 2.39-9.09), central neck dissection (OR, 3.60; 95% CI, 2.36-5.49), and total vs partial thyroidectomy (OR, 7.14; 95% CI, 4.55-11.11) correlated with hypoparathyroidism. CONCLUSION: These data present thyroidectomy information and complications pertinent to surgeons, along with preoperative risk factor assessment. Multivariable analysis showed institutional volume and hypoparathyroidism associated with RLN injury, while hypoparathyroidism associated with surgical indication, central neck dissection, and extent of surgery. Low complication rates support the safety of thyroidectomy in pediatric tertiary care centers.

Introduction of an enhanced recovery pathway results in decreased length of stay in patients with adolescent idiopathic scoliosis undergoing posterior spinal fusion: A description of implementation strategies and retrospective before-and-after study of outcomes.

STUDY OBJECTIVE: This study assessed whether implementation of an enhanced recovery-based pathway decreased length of stay without increasing readmissions among patients with adolescent idiopathic scoliosis undergoing posterior spinal fusion. DESIGN: Retrospective observational before-and-after study. SETTING: A tertiary children's hospital. PATIENTS: A total of 117 patients were studied, 78 in the pre-intervention group and 39 in the post-intervention group. All patients underwent posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS) in the same institution with one of two spine surgeons. Age, sex, American Society of Anesthesiologists physical status, and Cobb angle were comparable between the two groups. INTERVENTIONS: Between the pre- and post-intervention groups an enhanced recovery protocol was developed. The pathway included standardized use of nonopioid analgesics, proactive transition to oral analgesics, scheduled antiemetics, plans for diet advancement, and specific physical therapy goals. MEASUREMENTS: Outcome measurements included hospital length of stay, cumulative opioid doses in the first two postoperative days, and time to discontinuation of urinary catheter and patient-controlled analgesia. Postoperative emergency department visits, hospital readmissions and chronic pain management referrals were also measured. Pain scores on postoperative days one through four were recorded. MAIN RESULTS: Hospital length of stay decreased from 4.6 days to 3.8 days. Patient-controlled analgesia (PCA) was discontinued one day earlier on average following pathway implementation. Average cumulative postoperative opioid use, in morphine equivalents, decreased in the first two postoperative days from 2.5 to 2.2 mg/kg. There was no change in hospital readmission rate or postoperative chronic pain referral. CONCLUSIONS: Patients undergoing PSF for AIS experienced shorter hospital stays without increased readmissions following the implementation of an enhanced recovery pathway. Development of this pathway required buy-in from multiple stakeholders and significant coordination among services. The principles used to develop this pathway may be applied in other institutions and to other patient populations using the model outlined here.

Is Gadolinium Contrast Necessary for Pituitary MRI in the Evaluation of Pediatric Short Stature and Growth Hormone Deficiency?

INTRODUCTION: Short stature is a common concern that necessitates pediatric endocrinology evaluation. Growth hormone deficiency (GHD) is a commonly considered etiology. Brain and pituitary magnetic resonance imaging (MRI) with gadolinium-based contrast agents (GBCAs) is the most widely used imaging in assessing patients with GHD. Given the significant strides made in MRI technology, the need for contrast material should be reassessed. METHOD: We performed a retrospective review of healthy patients with short stature and/or GHD who underwent brain and pituitary MRI with and without contrast to assess the added value of contrast administration. RESULTS: 227/318 identified patients underwent growth hormone (GH) stimulation testing; 28 (12.3%) with normal GH response and 62 (27.3%) with severe GHD. We found a low incidence of sellar and suprasellar pathologies. When comparing noncontrast and contrast MRI, we found perfect agreement in detecting abnormal posterior pituitary bright spots (kappa:1.0) and substantial agreement in detecting pars intermedia cysts and posterior superior sellar cysts (kappa: 0.74 and 0.71, respectively). Initially, only moderate agreement was found in detecting infundibular abnormalities (kappa: 0.51), although a revised noncontrast MRI protocol with high-resolution 3D images enabled visualization of the infundibulum. CONCLUSION: The MRI evaluation of healthy patients with short stature and/or isolated GHD may be completed without the use of GBCAs. The slight overestimation of pituitary stalk interruption by noncontrast images can be overcome by adding newer high-resolution sequences.

Impact of an automated multiple emitter whole-room ultraviolet-C disinfection system on hospital acquired infections: A quasi-experimental study.

A quasi-experimental study performed in a pediatric hematology-oncology unit demonstrated that whole-room ultraviolet-C disinfection was associated with a significant reduction in hospital-onset Clostridioides difficile infection (P< .01, trend and level), but not healthcare-associated viral respiratory infections (P= .06 for trend, P= .36 for level) or central line-associated bloodstream infections (P> 0.75, trend and level).

Tolerance of 7% Hypertonic Saline in Pediatric Cystic Fibrosis Patients.

Background: Clinical trials of 7% hypertonic saline (HTS) in cystic fibrosis (CF) show short- and long-term benefits, including improved pulmonary function and reduced exacerbation risk. Adverse effects of HTS include bronchospasm, and guidelines recommend tolerance be tested in a clinical environment before prescribing. We evaluated the rate of, and risk factors for, intolerance of HTS at a single pediatric CF program. Methods: Single-center retrospective study in patients with CF, aged 6-20 years, who received an HTS test dose between 2006 and 2017. HTS intolerance was defined as either a ≥10% decline in forced expiratory volume in 1 s (FEV1) percent predicted or wheezing/severe cough. Results: Fifty-one patients met inclusion criteria, and 13 (25%) showed intolerance of 7% HTS. There were trends toward higher rates of comorbidities in the patients intolerant versus tolerant of HTS, including allergies and/or rhinitis 85% versus 66% (P = 0.30) and sinus disease 85% versus 58% (P = 0.10). A trend toward more methicillin-sensitive Staphylococcus aureus (58% vs. 31%, P = 0.09) and asthma (42% vs. 24%, P = 0.09) was seen in patients tolerant of HTS. Demographics, pulmonary function, nutritional parameters, laboratory tests, respiratory cultures, chronic therapies, and antibiotics for exacerbations did not differ. Conclusions: In our program, 1:4 children with CF exhibited objective findings of HTS intolerance. There were trends suggesting higher frequency of allergies and rhinosinusitis in intolerant children. Assessing tolerance of HTS before prescribing chronic daily therapy is important. Larger studies are needed to more accurately define the incidence of intolerance and risk factors.

MRI of Pediatric Orbital Masses: Role of Quantitative Diffusion-weighted Imaging in Differentiating Benign from Malignant Lesions.

PURPOSE: To systematically evaluate the utility of different magnetic resonance imaging (MRI) features, including quantitative diffusion-weighted imaging, in differentiating benign from malignant pediatric orbital masses. METHODS: The use of MRI in 40 pediatric patients with orbital masses was retrospectively reviewed. Multiple subjective and objective MRI parameters, including lesion mean apparent diffusion coefficient (ADC) values and lesion-to-thalamus ADC ratio were recorded. Bivariate analysis was done to identify parameters that were significantly different between benign and malignant subgroups. Receiver operating curves were used to establish optimal cut-off values for lesion mean ADC and lesion-to-thalamus ADC ratio for predicting benign versus malignant lesions. RESULTS: Lesion mean ADC, lesion-to-thalamus ADC ratio and extent of contrast enhancement showed statistically significant differences between the two subgroups. For distinguishing benign from malignant lesions, a lesion mean ADC cut-off value of 1.14â€¯× 10-3 mm2/s provided a sensitivity of 84% and specificity of 100%, while an ADC ratio of 1.4 provided a sensitivity of 81% and specificity of 89%. CONCLUSION: Quantitative diffusion-weighted imaging can be a useful adjunct in characterizing pediatric orbital masses by MRI, and thus help in clinical decision making.

Variables associated with pediatric emergency department visits for uncontrolled pain in postoperative adenotonsillectomy patients.

OBJECTIVE: Returns to the emergency department (ED) for pain or dehydration after adenotonsillectomy (T&A) are frequent. Attempts to associate the specific pain regimens with these visits have been unrevealing, suggesting a need to assess for other potential factors associated with readmission. METHODS: A review of a 2:1 cohort matched by age, gender and payer status compared post-T&A patients who did not return ED for pain or dehydration within 21 days to those who returned. Factors investigated included patient demographics, comorbidities, medication regimen and the presence of postoperative telephone encounters. Patients returning to the ED were further assessed for rates of medication adherence. RESULTS: 7493 patients underwent T&A during the period. Of these, 144 (1.9%) returned for pain/dehydration. Comparison to 285 matched patients revealed an association between ED returns and Hispanic ethnicity (p < 0.001), Spanish language (p = 0.0002), and comorbid Down syndrome and ADHD (p = 0.011 in both). The incidence of parent telephone calls to the office was associated with ED returns (58.7 in the ED cohort, 28.4% in non-ED cohort, p < 0.0001). On multivariable analysis, Hispanic ethnicity and phone calls were associated with ED returns (p < 0.0001 and p < 0.0001, respectively). Only 64.0% of patients returning to the ED were adherent with postoperative pain regimens. CONCLUSIONS: While demographic factors may be associated with rate of ED returns for pain and dehydration, post-operative phone calls were most highly associated with returns. The majority of patients returning to the ED were non-adherent with recommended pain regimens, suggesting an opportunity to investigate medication adherence in all post-tonsillectomy patients.

Risk factors for ocular complications in periocular infantile hemangiomas.

BACKGROUND/OBJECTIVES: Infantile hemangiomas are the most common benign tumors of childhood. Although some children with periocular infantile hemangiomas do not require treatment, these lesions may result in amblyopia and visual impairment if not properly treated. We have attempted to characterize clinical features of periocular infantile hemangiomas that predict negative ocular outcomes and thus require prompt referral to an ophthalmologist and initiation of therapy. METHODS: This study included children with periocular infantile hemangiomas consecutively seen at Ann & Robert H. Lurie Children's Hospital of Chicago from January 1994 through December 2014. Only patients evaluated by both a dermatologist and an ophthalmologist were included. Medical records of patients who met inclusion criteria were reviewed. Ocular findings were reviewed for the presence of ptosis, refractive errors, strabismus, proptosis, and amblyopia. RESULTS: Ninety-six patients (74% female, median age of onset 0.48 months) were included. Periocular infantile hemangiomas larger than 1 cm in diameter, with a deep component, and with involvement of the upper eyelid were significantly associated with astigmatism (P = .002, P = .02, and P = .003, respectively) and amblyopia (P = .002, P = .02, and P = .04, respectively). Using logistic regression, diameter greater than 1 cm (odds ratio = 14.13, P = .01) and amblyopia (odds ratio = 21.00, P = .04) were the strongest predictors of astigmatism. Lower lid and medial and lateral canthal involvement were not predictive of ocular complications. CONCLUSION: Predictive factors for ocular complications in patients with periocular infantile hemangiomas are diameter greater than 1 cm, a deep component, and upper eyelid involvement, with size being the most consistent predictor. These patients should be promptly referred to an ophthalmologist, and treatment should be strongly considered.

Analysis of factors predicting surgical intervention and associated costs in pediatric breast masses: a single center study.

PURPOSE: Finding a breast mass in a child provokes apprehension in parents, especially in those with a family history of breast cancer. Clinicians must decide between serial imaging or biopsy of the mass. Herein, we identify management differences in those with and without a positive family history, as well as identify cost differences. METHODS: An institutional retrospective review was performed of patients (2-18 years of age) with a diagnosis of breast mass. Patient demographics, presentation, medical and surgical history, physical exam, imaging, and pathologic diagnosis were collected. Cost data were acquired from the pediatric health information system (PHIS). Costs were compared between patients managed by biopsy versus serial ultrasounds. Bivariate analyses including Pearson's Chi-square, student's t tests, and logistic regression were performed. RESULTS: The probability of biopsy increases with age (p = 0.0001) and female gender (p = 0.006). Biopsy rate is higher for larger masses (p < 0.0001), growing size (p < 0.0001), and in patients with a positive family history of breast cancer (p < 0.0001). The average cost of care for management with initial excisional biopsy was $4491 versus those with serial ultrasounds ($986) (p < 0.0001). CONCLUSIONS: In patients with small lesions, even with a family history of breast cancer, non-operative monitoring is a safe and cost-effective alternative to invasive biopsy.

Pediatric arterial catheters: Complications and associated risk factors.

BACKGROUND/PURPOSE: Arterial catheter complications are a common problem in a pediatric critical care setting, but reported complication rates and risk factors associated with peripheral arterial catheter complications vary. We conducted a retrospective cohort study to identify risk factors in a pediatric patient population. METHODS: We performed a detailed abstraction of provider notes in the electronic medical records of inpatients ≤18years of age who underwent arterial line placement between January 1, 2008 and January 1, 2013 at a university-affiliated standalone pediatric hospital. Inpatient records were assessed for complications associated with arterial catheterization and risk factors inherent to arterial catheter insertion. RESULTS: Two hundred twenty-eight children were identified, of whom 75 (33%) had a total of 106 arterial catheter complications. Complications included line malfunctions (59%, n=63), bleeding (16%, n=17), multiple complications (11%, n=12), infiltration (8%, n=9), and hematoma (4%, n=4). Line malfunction was reported in all patients with multiple complications. Independent predictors of complications associated with arterial catheterization were the presence of more than one provider during the insertion (p=0.007) and insertion attempts at multiple sites (p=0.036). CONCLUSIONS: Our analysis suggests the need for a prospective study to comprehensively assess provider-related risk factors associated with arterial catheter complications in children. LEVEL OF EVIDENCE: IV.

Preoperative Use of CT Angiography in Infants With Coarctation of the Aorta.

BACKGROUND: Computed tomographic angiography (CTA) and echocardiography (echo) are used preoperatively in coarctation of the aorta to define arch hypoplasia and great vessel branching. We sought to determine differences in quantitative measurements, as well as surgical utility, between modalities. METHODS: Infants (less than six months) with both CTA and echo prior to coarctation repair from 2004 to 2013 were included. Measurements were compared and correlated with surgical approach. Three surgeons reviewed de-identified images to predict approach and characterize utility. Computed tomographic angiography radiation dose was calculated. RESULTS: Thirty-three patients were included. No differences existed in arch measurements between echo and CTA ( z-score: -2.59 vs -2.43; P = .47). No differences between modalities were seen for thoracotomy ( z-score: -2.48 [echo] vs -2.31 [CTA]; P = .48) or sternotomy ( z-score: -3.13 [echo] vs -3.08 [CTA]; P = .84). Computed tomographic angiography delineated great vessel branching pattern in two patients with equivocal echo findings ( P = .60). Surgeons rated CTA as far more useful than echo in understanding arch hypoplasia and great vessel branching in cases where CTA was done to resolve anatomical questions that remain after echo evaluation. Two of three surgeons were more likely to choose the surgical approach taken based on CTA (surgeon A, P = .02; surgeon B, P = .01). Radiation dose averaged 2.5 (1.6) mSv and trended down from 2.9 mSv (1.8 mSv; n = 20) to 1.6 mSv (0.5 mSv; n = 7) ( P = .06) with new technology. CONCLUSION: Although CTA and echo measurements of the aorta do not differ, CTA better delineates branching and surgeons strongly prefer it for three-dimensional arch anatomy. We recommend CTA for patients with anomalous arch branching patterns, diffuse or complex hypoplasia, or unusual arch morphology not fully elucidated by echo.

Health-related quality of life and adherence to hydroxyurea in adolescents and young adults with sickle cell disease.

BACKGROUND: Complications related to sickle cell disease (SCD) result in significant declines in health-related quality of life (HRQOL). While hydroxyurea reduces SCD complications, adherence remains suboptimal. The study's objectives were to assess the feasibility of Internet-based electronic assessment of HRQOL in SCD clinic and to examine the relationship between HRQOL and hydroxyurea adherence in adolescents and young adults (AYAs) with SCD. PROCEDURE: A cross-sectional survey was administered on tablets to 34 AYAs (12-22 years old) in a SCD clinic from January through December 2015. Study measures included Patient Reported Outcomes Measurement Information System (PROMIS® ) computerized adaptive testing and ©Modified Morisky Adherence Scale 8-items (©MMAS-8). RESULTS: Participants (59% male, 91% Black) had median age of 13.5 (range 12-18) years. Ninety-one percent completed PROMIS® measures electronically in the clinic, meeting our feasibility criterion of ≥85% completion rate. ©MMAS-8 scores positively correlated with fetal hemoglobin (HbF) (rs = 0.34, P = 0.04) and mean corpuscular volume (MCV) (rs = 0.42, P = 0.01) and inversely correlated with fatigue (rs = -0.45, P = 0.01), depression (rs = -0.3, P = 0.08), and social isolation (rs = -0.78, P = 0.02). Low ©MMAS-8 scores, indicating poor adherence, were associated with worse fatigue (P = 0.001) and trended toward significance for pain (P = 0.07) and depression (P = 0.06). Homozygous hemoglobin S disease patients with low HbF (<16%) had worse social isolation (P = 0.04) and those with low MCV (<102 fl) reported worse fatigue (P = 0.001), pain (P = 0.01), mobility (P = 0.01), and social isolation (P = 0.04). CONCLUSIONS: HRQOL assessment in the SCD clinic is feasible. SCD patients with low hydroxyurea adherence and/or low HbF or MCV levels had worse HRQOL scores, particularly fatigue. Future prospective studies examining the relationship between HRQOL and hydroxyurea adherence are warranted.

Reducing Fluoroscopic Radiation Exposure During Endomyocardial Biopsy in Pediatric Transplant Recipients.

Endomyocardial biopsy (EMB) with fluoroscopy is used for rejection surveillance in pediatric heart transplantation. Lowering frame rate may reduce radiation, but decreases temporal resolution and image quality. We undertook a quality initiative reducing frame rate from 10 frames per second (FPS) to 5 FPS. To assess whether lowering frame rate can reduce radiation exposure without compromising safety, data on EMBs from 9/2009 to 4/2013 without angiography or intervention were reviewed. Effective dose was calculated from dose area product (DAP) and fluoroscopy time. Complications were reviewed. Independent t test compared pre- and post-data and a general linear model were used to control for confounders. Paired t test of most proximate data was used for pts with EMB before and after our change. Eighty-six patients had 543 EMB. After adjusting for weight, attending, and presence of a fellow, the lower FPS group had a 60.3% reduction in DAP (p < 0.0001) and 53.8% drop in effective dose (p < 0.0001). Fluoroscopy time did not differ. Twenty-eight pts had EMBs both before and after the FPS change. Pair-wise analysis of this group demonstrated a 33% reduction in DAP (p < 0.05) and 37% drop in effective dose (p < 0.01), without difference in fluoroscopy time. No patient had an increase in TR > 1 grade by ECHO. There were no deaths or perforations. Lowering the frame rate reduces radiation exposure by >50% without compromising safety. Efforts to further minimize radiation exposure of this vulnerable population should be considered.

Current Practice of Pharmacological Thromboprophylaxis for Prevention of Venous Thromboembolism in Hospitalized Children: A Survey of Pediatric Hemostasis and Thrombosis Experts in North America.

Pharmacological thromboprophylaxis (pTP) is the most effective intervention to prevent venous thromboembolism (VTE) in hospitalized adults. High-quality studies investigating the role of pTP in children are lacking. The aim of this study is to understand pediatric hematologists' current practices of pTP prescription and to explore their opinion about universal adoption of pTP for high-risk hospitalized children. An electronic survey was sent to members of Hemostasis and Thrombosis Research Society of North America. The response rate was 47.3% (53/112). VTE was perceived as a major hospital acquired complication by all and 96% (51/53) prescribed pTP in select cases. Majority would consider prescribing pTP for personal history of thrombosis, inheritance of severe thrombophilic conditions, and teen age. The majority of respondents (55%, 29/53) were either not in support of or uncertain about the universal adoption of pTP policy for high-risk hospitalized children. In total, 62% of respondents (33/53) did not support the use of pTP for central venous lines. Respondents reported on the presence of pharmacological (32%, 17/53) and mechanical (45%, 24/53) thromboprophylaxis policies at their institutions. Pediatric hematologists considered pTP a useful intervention to prevent VTE and prescribed pTP in select cases. Universal adoption of pTP was not supported. Wide variability in clinical practice was observed.

Growth and Obesity Among Older Single Ventricle Patients Presenting for Fontan Conversion.

INTRODUCTION: Long-term growth outcomes and the prevalence of obesity among older single ventricle (SV) patients have not been well characterized. We investigated these parameters, as well as the impact of obesity on survival, in an older cohort of SV patients presenting for Fontan conversion. METHODS: We analyzed preoperative height, weight, and body mass index (BMI) of patients who underwent Fontan conversion. Overweight and obese were defined as BMI ≥85 percentile and ≥95 percentile for patients <20 years and BMI 25 to 30 kg/m(2) and ≥30 kg/m(2) for patients ≥20 years, respectively. Postoperative transplant-free survival was assessed among obese, overweight, and normal weight patients. RESULTS: We evaluated 139 patients presenting for Fontan conversion at a median age of 23.2 years. Patients had shorter stature compared to the normal population (mean Z score -0.6, P < .001). Younger patients had lower BMI compared to the normal population (<20 years: mean Z score -0.5, P = .02), while older patients had elevated BMI (≥20 years: mean Z score +0.4, P < .001). The mean BMI among older patients approached overweight at 24.6 kg/m(2). The prevalence of obesity increased with advancing age, with 36% overweight and 14% obese at >30 years. At a median of 8.2 years following Fontan conversion, obesity and overweight status were not associated with transplant-free survival. CONCLUSION: Older SV patients presenting for Fontan conversion had shorter stature compared to the normal population as well as a high prevalence of overweight and obesity. Although there was no relationship between weight status and early postoperative survival, further investigation of long-term outcomes is warranted.

Prevalence of BK polyomavirus infection and association with renal dysfunction in pediatric heart transplant recipients.

BACKGROUND: BK polyomavirus (BKV) infection and nephropathy complicate renal allografts; however, their effect in the native kidneys of pediatric heart transplant (HTx) recipients is unknown. We assessed the prevalence of BKV infection and its association with kidney dysfunction in survivors of pediatric HTx. METHODS: A single-center retrospective study compared pediatric (aged <18 years ) HTx recipients, with and without BKV (controls), who received an allograft from May 1989 to July 2013. Screening of urine for BKV was performed in patients with chronic kidney disease (CKD) stage ≥2 since 2006, and since April 2012 in all HTx recipients at least at an annual evaluation. Serum for BKV DNA was assayed if BK viruria was present. Data collected included recipient and donor demographics, the immunosuppressive regimen, and history of Epstein-Bar virus (EBV) and cytomegalovirus infection. Statistics included Fisher's exact test, chi-square test, Student's t-test, and multivariate logistic regression. RESULTS: Of 98 eligible recipients, 83 (85%) were screened: 28 (34%) had BK viruria, and 7 had BK viremia. One viremic patient had biopsy-proven BKV nephropathy that progressed to end-stage renal disease. Risk factors for BK viruria were (1) longer duration since HTx (6.02 vs 2.95 years; p = 0.01), (2) worsening estimated glomerular filtration rate (71.3 vs 86.3 ml/min/1.73 m(2), p = 0.03), (3) history of EBV infection (p = 0.0002), and (4) use of sirolimus (p = 0.0003). After multivariate logistic-regression, only history of EBV infection remained associated with BKV infection (p = 0.015). CONCLUSIONS: BKV may lead to BK viremia and BK nephropathy in pediatric HTx patients. Routine screening for BK viruria should be considered.