共查询到20条相似文献，搜索用时 31 毫秒
1.
Matthew Wong Janette Reyes Eveline Lapidus-Krol Monping Chiang Tilman Humpl Malikah Al-Faraj Greg Ryan Priscilla P.L. Chiu 《Journal of pediatric surgery》2018,53(5):918-924
Prenatal observed/expected lung–to-head ratio (O/E LHR) by ultrasound correlates with postnatal mortality for congenital diaphragmatic hernia (CDH) patients. The aim of this study is to determine if O/E LHR correlates with pulmonary hypertension (PH) outcomes for CDH patients.
Methods
A single center retrospective chart review was performed for CDH neonates from January 1, 2006, to December 31, 2015, (REB #1000053124) to include prenatal O/E LHR, liver position, first arterial blood gas, repair type, echocardiogram (ECHO), and lung perfusion scan (LPS) results up to 5 years of age.Results
Of 153 newborns, 123 survived (80.4%), 58 (37.9%) had prenatal O/E LHR, and 42 (27.5%) had postnatal ECHO results. High mortality risk neonates (O/E LHR ≤ 45%) correlated with higher right ventricular systolic pressure (RVsp) at birth. Generally PH resolved by age 5 years. LPS results did not change over time (p > 0.05) regardless of initial PH severity, suggesting that PH resolution did not correlate with increased ipsilateral lung perfusion to offload the right ventricle.Conclusion
Prenatal prognostic markers correlated with initial PH severity for CDH newborns, but PH resolved over time despite fixed perfusion bias to the lungs. These results suggest favorable PH outcomes for CDH patients who survive beyond infancy.Type of Study
Retrospective Cohort Study.Level of Evidence
3b 相似文献2.
Loren Berman Jordan Jackson Kristen Miller Rebecca Kowalski Paul Kolm Francois I. Luks 《Journal of pediatric surgery》2018,53(8):1592-1599
Background
Pediatric surgeons frequently offer prenatal consultation for congenital pulmonary airway malformation (CPAM) and congenital diaphragmatic hernia (CDH); however, there is no evidence-based consensus to guide prenatal decision making and counseling for these conditions. Eliciting feedback from experts is integral to defining best practice regarding prenatal counseling and intervention.Methods
A Delphi consensus process was undertaken using a panel of pediatric surgeons identified as experts in fetal therapy to address current limitations. Areas of discrepancy in the literature on CPAM and CDH were identified and used to generate a list of content and intervention questions. Experts were invited to participate in an online Delphi survey. Items that did not reach first-round consensus were broken down into additional questions, and consensus was achieved in the second round.Results
Fifty-four surgeons (69%) responded to at least one of the two survey rounds. During round one, consensus was reached on 54 of 89 survey questions (61%), and 45 new questions were developed. During round two, consensus was reached on 53 of 60 survey questions (88%).Conclusions
We determined expert consensus to establish guidelines regarding perinatal management of CPAM and CDH. Our results can help educate pediatric surgeons participating in perinatal care of these patients.Level of Evidence
V. 相似文献3.
Joseph T. Church Rodrigo Mon Tiffany Wright Megan A. Coughlin Maria Ladino-Torres Christopher Tapley Heather Bowen Niki Matusko George B. Mychaliska 《Journal of pediatric surgery》2018,53(6):1087-1091
Purpose
Survivors of congenital diaphragmatic hernia (CDH) face high morbidity. We studied the neurodevelopmental outcomes of CDH survivors at a single institution.Methods
CDH survivors born July 2006–March 2016 at a free-standing children's hospital were reviewed. Neurodevelopment was assessed using the Peabody Developmental Motor Scales (PDMS-2) broken into gross, fine, and total motor quotients. Data collected included prenatal variables (liver herniation, defect laterality, observed:expected total fetal lung volume (o:eTFLV) on MRI), birth demographics (sex, race, estimated gestational age (EGA), birth weight (BtWt), 5 min APGAR, associated anomalies), and therapies/hospital course (HFOV/HFJV, ECMO, timing of repair, pulmonary hypertension (PHTN) severity, length of stay, ventilator days). Variables were analyzed using mixed linear modeling.Results
Sixty-eight children were included. Most patients had left-sided CDH (55/68, 81%) without liver herniation (42/68, 62%). ECMO utilization was 25/68 (37%). The mean [95% confidence interval] gross motor quotient for the entire cohort was 87 [84–91], fine motor quotient was 92 [88–96], and total motor quotient was 88 [84–93], representing below average, average, and below average functioning, respectively. o:eTFLV predicted fine motor quotient among prenatal variables. Associated anomalies and ECMO use predicted all quotients in the final model.Conclusions
Associated anomalies and ECMO use predict neurodevelopmental delay in CDH survivors.Type of Study
Retrospective observational study; Prognostic.Level of Evidence
II. 相似文献4.
Lasantha Gunasekara Mustafa Al-Saiedy Francis Green Ryan Pratt Candice Bjornson Ailian Yang W. Michael Schoel Ian Mitchell Mary Brindle Mark Montgomery Elizabeth Keys John Dennis Grishma Shrestha Matthias Amrein 《Journal of cystic fibrosis》2017,16(5):565-572
Background
Airway surfactant is impaired in cystic fibrosis (CF) and associated with declines in pulmonary function. We hypothesized that surfactant dysfunction in CF is due to an excess of cholesterol with an interaction with oxidation.Methods
Surfactant was extracted from bronchial lavage fluid from children with CF and surface tension, and lipid content, inflammatory cells and microbial flora were determined. Dysfunctional surfactant samples were re-tested with a lipid-sequestering agent, methyl-β-cyclodextrin (MβCD).Results
CF surfactant samples were unable to sustain a normal low surface tension. MβCD restored surfactant function in a majority of samples.Mechanistic studies showed that the dysfunction was due to a combination of elevated cholesterol and an interaction with oxidized phospholipids and their pro-inflammatory hydrolysis products.Conclusion
We confirm that CF patients have impaired airway surfactant function which could be restored with MβCD. These findings have implications for improving lung function and mitigating inflammation in patients with CF. 相似文献5.
Stephanie M. Cruz Patricio E. Lau Craig G. Rusin Candace C. Style Darrell L. Cass Caraciolo J. Fernandes Timothy C. Lee Christopher J. Rhee Sundeep Keswani Rodrigo Ruano Stephen E. Welty Oluyinka O. Olutoye 《Journal of pediatric surgery》2018,53(1):152-158
Background/purpose
The purpose of this study was to develop a computational algorithm that would predict the need for ECMO in neonates with congenital diaphragmatic hernia (CDH).Methods
CDH patients from August 2010 to 2016 were enrolled in a study to continuously measure cerebral tissue oxygen saturation (cStO2) of left and right cerebral hemispheres. NIRS devices utilized were FORE-SIGHT, CASMED and INVOS 5100, Somanetics. Using MATLAB©, a data randomization function was used to deidentify and blindly group patient's data files as follows: 12 for the computational model development phase (6 ECMO and 6 non-ECMO) and the remaining patients for the validation phase.Results
Of the 56 CDH patients enrolled, 22 (39%) required ECMO. During development of the algorithm, a difference between right and left hemispheric cerebral oxygenation via NIRS (ΔHCO) was noted in CDH patients that required ECMO. Using ROC analysis, a ΔHCO cutoff > 10% was predictive of needing ECMO (AUC: 0.92; sensitivity: 85%; and specificity: 100%). The algorithm predicted need for ECMO within the first 12 h of life and at least 6 h prior to the clinical decision for ECMO with 88% sensitivity and 100% specificity.Conclusion
This computational algorithm of cerebral NIRS predicts the need for ECMO in neonates with CDH.Level of evidence
II 相似文献6.
Carmen Mesas Burgos Björn Frenckner Matias Luco Matthew T. Harting Pamela A. Lally Kevin P. Lally 《Journal of pediatric surgery》2018,53(1):113-117
Background
Right-sided congenital diaphragmatic hernias (CDH) and bigger defect sizes have been associated with poorer outcomes.Aim
The aim of this study was to evaluate right- and left-sided CDH in terms of size, survival, associated anomalies, and morbidity.Material and methods
We used information from a multicenter, multinational database including patients with CDH born between 2007 and 2015. All infants with data on defect side were included for this analysis. We compared differences in outcomes between right- and left-sided CDH. Further analysis on the association between side, size of the defect, and outcome was performed.Results
A total of 3754 cases of CDH were entered in the registry between January 2007 and September 2015, with an overall survival of 71%. Of those, 598 (16%) were right-sided and 3156 left-sided, with a survival rate of 67% and 72%, respectively. Right-sided CDH had a larger proportion of C and D defects (p < 0.001 and 0.04, respectively). Survival rates for the same size defect were similar, independent of the side of the defect. Multivariable logistic regression analysis with survival as dependent variable identified a significant correlation with defect size, but not side.Conclusions
The higher proportion of large defects (C & D) in right-sided CDH, not the side itself, accounts for the reported poorer survival in right-sided CDH.Level of evidence
Level I for a prognosis study – This is a high-quality, prospective cohort study with 99% of patients followed to the study end point (death or discharge). 相似文献7.
Sarah K. Lucas Robert Yang Jordan M. Dunitz Holly C. Boyer Ryan C. Hunter 《Journal of cystic fibrosis》2018,17(2):204-212
Background
Metastasis of upper airway microbiota may have significant implications in the development of chronic lung disease. Here, we compare bacterial communities of matched sinus and lung mucus samples from cystic fibrosis (CF) subjects undergoing endoscopic surgery for treatment of chronic sinusitis.Methods
Mucus from one maxillary sinus and expectorated sputum were collected from twelve patients. 16S rRNA gene sequencing was then performed on sample pairs to compare the structure and function of CF airway microbiota.Results
Bacterial diversity was comparable between airway sites, though sinuses harbored a higher prevalence of dominant microorganisms. Ordination analyses revealed that samples clustered more consistently by airway niche rather than by individual. Finally, predicted metagenomes suggested that anaerobiosis was enriched in the lung.Conclusions
Our findings indicate that while the lung may be seeded by individual sinus pathogens, airway microenvironments harbor distinct bacterial communities that should be considered in selecting antimicrobial therapies. 相似文献8.
Heather B. Howell Christiana Farkouh-Karoleski Marilyn Weindler Rakesh Sahni 《Journal of pediatric surgery》2018,53(11):2100-2104
Background
Infants with congenital diaphragmatic hernia (CDH) are at risk for growth failure because of inadequate caloric intake and high catabolic stress. There is limited data on resting energy expenditure (REE) in infants with CDH.Aims
To assess REE via indirect calorimetry (IC) in term infants with CDH who are no longer on respiratory support and nearing hospital discharge with advancing post-conceptional age and to assess measured-to-predicted REE using predictive equations.Methods
A prospective cohort study of term infants with CDH who were no longer on respiratory support and nearing hospital discharge was conducted to assess REE via IC and caloric intake. Baseline characteristics and hospital course data were collected. Three day average caloric intake around time of IC testing was calculated. Change in REE with advancing post-conceptional age and advancing post-natal age was assessed. The average measured-to-predicted REE was calculated for the cohort using predictive equations [22].Results
Eighteen infants with CDH underwent IC. REE in infants with CDH increased with advancing postconceptional age (r2?=?0.3, p?<?0.02). The mean REE for the entire group was 53.2 +/? 10.9?kcal/kg/day while the mean caloric intake was 101.2 +/? 17.4?kcal/kg/day. The mean measured-to-predicted ratio for the cohort was in the normal metabolic range (1.10 +/? 0.17) with 50% of infants considered hypermetabolic and 11% of infants considered hypo-metabolic.Conclusions
Infant survivors of CDH repair who are without respiratory support at time of neonatal hospital discharge have REE, as measured by indirect calorimetry, that increases with advancing post-conceptional age and that is within the normal metabolic range when compared to predictive equations.Level of Evidence
III 相似文献9.
Introduction
Congenital pulmonary airway malformation (CPAM) is the most common lung pathology diagnosed antenatally. Thoracoscopic lobectomy has shown increasing popularity, but the long-term result is still lacking. In this study we compared long -term pulmonary function after thoracoscopic and open lobectomy.Methods
All CPAM patients with lobectomy between 2000 and 2008 were recruited into the study. Pulmonary function test (PFT) was performed at least 7?years after operation. Demographic data and PFT results were analyzed. Comparison was made between the thoracoscopic and open group.Results
Twelve patients were included in each group. PFT was performed at a mean age of 9.8 (thoracoscopic) and 12.2?years (open), respectively (p?=?0.17). The thoracoscopic group showed better performance in forced vital capacity (FVC) (98.9 vs 84.3% predicted, p?=?0.03), forced expiratory volume in 1?s (FEV1) (88.5 vs 76.1% predicted, p?=?0.04), and alveolar volume adjusted diffusion capacity of carbon monoxide (106.4 vs 91.4% predicted, p?=?0.03). FEV1 to FVC ratio, total lung capacity, and residual volume showed no statistical difference.Conclusion
The long term PFT result following thoracoscopic lobectomy is better than open lobectomy. This may be due to impaired respiratory musculature after thoracotomy. Further study with larger sample size is necessary to determine this hypothesis.Level-of-evidence
III 相似文献10.
Emily A. Partridge Marcus G. Davey Matthew Hornick Kevin C. Dysart Aliza Olive Robert Caskey James T. Connelly Holly L. Hedrick William H. Peranteau Alan W. Flake 《Journal of pediatric surgery》2018,53(8):1453-1460
Background
Extracorporeal membrane oxygenation (ECMO) is commonly required in neonates with congenital diaphragmatic hernia (CDH) complicated by pulmonary hypertension (PH). ECMO carries significant risk, and is contraindicated in the setting of extreme prematurity or intracranial hemorrhage. Pumpless arteriovenous ECMO (P-ECMO) may represent an alternative for respiratory support. The present study summarizes our initial experience with P-ECMO in a lamb model of CDH.Study design
Surgical creation of CDH was performed at 65–75 days' gestation. At term (135–145 days), lambs were delivered into the P-ECMO circuit. Three animals were maintained on a low-heparin infusion protocol (target ACT 160–180) and three animals were maintained with no systemic heparinization.Results
Animals were supported by the circuit for 380.7 +/? 145.6 h (range, 102–504 h). Circuit flow rates ranged from 97 to 208 ml/kg/min, with adequacy of organ perfusion demonstrated by stable serum lactate levels (3.0 +/? 1.7) and pH (7.4 +/? 0.3). Necropsy demonstrated no evidence of thrombogenic complications.Conclusion
Pumpless extracorporeal membrane oxygenation achieved support of CDH model lambs for up to three weeks. This therapy has the potential to bridge neonates with decompensated respiratory failure to CDH repair with no requirement for systemic anticoagulation, and may be applicable to patients currently precluded from conventional ECMO support. 相似文献11.
12.
Jeffrey S. Wagener Michael J. Williams Stefanie J. Millar Wayne J. Morgan David J. Pasta Michael W. Konstan 《Journal of cystic fibrosis》2018,17(4):496-502
Background
Patients with cystic fibrosis (CF) who experience acute declines in percent predicted FEV1 (ppFEV1 decreased ≥10% relative to baseline) are often not treated with antibiotics for pulmonary exacerbations (PEx), whereas other patients are treated even when they have not experienced a decline in lung function.Methods
We analyzed 2 patient cohorts using 3?years of Epidemiologic Study of CF data. Cohort 1 (12,837 patients) experienced a ≥10% acute decline in ppFEV1 (n?=?22,898) and Cohort 2 (10,416 patients) had a clinician-diagnosed PEx (n?=?20,731).Results
70.7% of ≥10% decline events were treated with antibiotics; with intravenous antibiotics used 67.1% of the time. 32.0% of clinician-diagnosed PEx declined <10%; with intravenous antibiotics used 36.9% of the time.Conclusions
A clinician's decision to diagnose a PEx and treat with antibiotics often is not defined by measured lung function: a ≥10% FEV1 decline is not considered an absolute indication of a PEx and the lack of a decline does not contraindicate a PEx. Clinicians appear to use the history of prior PEx plus other variables as factors for diagnosing PEx. 相似文献13.
Ashley S. Bruns Patricio E. Lau Gurpreet S. Dhillon Joseph Hagan Joshua A. Kailin George B. Mallory Pablo Lohmann Oluyinka O. Olutoye Rodrigo Ruano Caraciolo J. Fernandes 《Journal of pediatric surgery》2018,53(9):1675-1680
Background & objectives
Congenital Diaphragmatic Hernia (CDH) is associated with significant morbidity and mortality. This study compares the efficacy of the highest oxygenation index in the first 48 h (HiOI) versus current prenatal indices to predict survival and morbidity.Methods
Medical records of 50 prenatally diagnosed, isolated, left-sided CDH patients treated from January 2011 to April 2016 were reviewed. Data abstracted included HiOI, lung to head ratio (LHR), observed to expected total fetal lung volume (O/E TFLV), percent liver herniation (%LH), 6 month survival, respiratory support at discharge, ventilator days and length of stay. Data were analyzed using parametric and nonparametric tests and regression analyses as appropriate.Results
HiOI was associated with significantly increased LOS (p < 0.001), respiratory support at discharge (p < 0.001), greater ventilator days (p = 0.001) and higher odds of death (p = 0.004) with risk of death increasing by 5% for every one-unit increase in OI. HiOI was statistically a better predictor of LOS than O/E TFLV (p = 0.007) and %LH (p = 0.02).Conclusions
In isolated, left-sided CDH patients, HiOI is associated with higher mortality, greater length of stay, more ventilator days and increased respiratory support at discharge. HiOI is a better predictor of length of stay than O/E TFLV and %LH.Type of study
Retrospective StudyLevel of evidence
II 相似文献14.
Lily Paemka Brian N. McCullagh Mahmoud H. Abou Alaiwa David A. Stoltz Qian Dong Christoph O. Randak Robert D. Gray Paul B. McCray 《Journal of cystic fibrosis》2017,16(4):471-474
Background
We sought to address whether CF macrophages have a primary functional defect as a consequence of CFTR loss and thus contribute to the onset of infection and inflammation observed in CF lung disease.Methods
Monocyte derived macrophages (MDMs) were prepared from newborn CF and non-CF pigs. CFTR mRNA expression was quantified by rtPCR and anion channel function was determined using whole cell patch clamp analysis. IL8 and TNFα release from MDMs in response to lipopolysaccharide stimulation was measured by ELISA.Results
CFTR was expressed in MDMs by Q-rtPCR at a lower level than in epithelial cells. MDMs exhibited functional CFTR current at the cell membrane and this current was absent in CF MDMs. CF MDMs demonstrated an exaggerated response to lipopolysaccharide stimulation.Conclusions
In the absence of CFTR function, macrophages from newborn CF pigs exhibit an increased inflammatory response to a lipopolysaccharide challenge. This may contribute to the onset and progression of CF lung disease. 相似文献15.
Kathryn A. Ramsey Caroline McGirr Stephen M. Stick Graham L. Hall Shannon J. Simpson 《Journal of cystic fibrosis》2017,16(6):713-718
Background
We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease.Methods
Multiple breath washout (MBW) was performed in seated and supine postures in 25 healthy children and 21 children with CF. Children with CF also underwent a chest CT scan. Functional residual capacity (FRC), lung clearance index (LCI) and moment ratios were calculated from the MBW test. CT scans were evaluated for CF-related structural lung disease.Results
FRC was lower in the supine than in the seated posture, whereas LCI was higher in the supine than in the seated posture. In children with CF, associations between LCI and the extent of structural lung disease were stronger when performed in the supine posture.Conclusions
Body posture influences lung volumes and ventilation distribution in both healthy children and children with CF. MBW testing in the supine posture strengthened associations with structural lung damage. 相似文献16.
Background
Outcome data for UK cystic fibrosis centres are publicly available in an annual report, which ranks centres by median FEV1% predicted. We wished to assess whether there are differences in lung function outcomes between adult centres that might imply differing standards of care.Methods
UK Registry data from 4761 subjects at 34 anonymised adult centres were used to calculate mean FEV1% and rate of change of lung function for 2007–13. These measures were used to rank centres and compare outcomes.Results
There are minor differences between centres for mean FEV1% for some years of the study and for rate of change of lung function over the study period. However, rankings are critically dependent on the outcome measure chosen and centre variation becomes negligible once patient population characteristics are taken into account.Conclusions
We have demonstrated that the ranking of centres is biased and any apparent difference in respiratory outcomes is unlikely to be related to differing standards of care between centres. 相似文献17.
18.
Caroline Q. Stephens Stephanie Dukhovny Kathryn J. Rowland Nicholas A. Hamilton 《Journal of pediatric surgery》2018,53(11):2145-2149
Background
Duodenal obstruction (DO) is associated with congenital cardiac anomalies that may complicate the delivery of anesthesia during surgical repair. As most infants undergo fetal ultrasounds that identify cardiac anomalies, our aim was to determine the utility of obtaining preoperative neonatal echocardiograms in all DO patients.Methods
We conducted a retrospective cohort study of all DO patients treated at two tertiary care children's hospitals between January 2005 and February 2016. Prenatal ultrasounds were compared to neonatal echocardiograms to determine concordance. Binomial exact analyses were used to estimate the negative predictive value (NPV) of prenatal imaging.Results
We identified 65 infants with DO. The majority of patients (93.8%) had prenatal ultrasounds, including twenty patients that underwent fetal echocardiogram. Fourteen (21.5%) were diagnosed with cardiac lesions in utero, and neonatal echocardiograms confirmed 12 lesions, without identifying any new lesions. No changes to anesthetic management were made because of cardiac lesions. The NPV of prenatal imaging was 100% (95% Confidence Interval: 91.0–100.0).Conclusions
Neonatal echocardiogram is unlikely to identify new cardiac lesions in DO patients with negative fetal imaging and delays in surgical care are unwarranted.Levels of evidence
Study of Diagnostic Test-Level II. 相似文献19.
Austin E. Gillen Rui Yang Calvin U. Cotton Aura Perez Scott H. Randell Shih-Hsing Leir Ann Harris 《Journal of cystic fibrosis》2018,17(4):444-453
Background
Robust methods to culture primary airway epithelial cells were developed several decades ago and these cells provide the model of choice to investigate many diseases of the human lung. However, the molecular signature of cells from different regions of the airway epithelium has not been well characterized.Methods
We utilize DNase-seq and RNA-seq to examine the molecular signatures of primary cells derived from human tracheal and bronchial tissues, as well as healthy and diseased (cystic fibrosis (CF)) donor lung tissue.Results
Our data reveal an airway cell signature that is divergent from other epithelial cell types and from common airway epithelial cell lines. The differences between tracheal and bronchial cells are clearly evident as are common regulatory features. Only minor variation is seen between bronchial cells from healthy or CF donors.Conclusions
These data are a valuable resource for functional genomics analysis of airway epithelial tissues in human disease. 相似文献20.
Harriet Corvol Nathalie Rousselet Kristin E. Thompson Laura Berdah Guillaume Cottin Tobias Foussigniere Elisabeth Longchampt Laurence Fiette Edouard Sage Céline Prunier Mitchell Drumm Craig A. Hodges Pierre-Yves Boëlle Loic Guillot 《Journal of cystic fibrosis》2018,17(2):190-203