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1.
J Perinatol ; 40(5): 695-703, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32203174

RESUMO

OBJECTIVE: To compare the prognostic accuracy of six neonatal illness severity scores (CRIB, CRIB II, SNAP, SNAP II, SNAP-PE, and SNAP-PE II), birthweight (BW), and gestational age (GA) for predicting pre-discharge mortality among very low birth weight (VLBW) infants (<1500 g) and very preterm infants (<32 weeks' gestational age). STUDY DESIGN: PubMed, EMBASE, and Scopus were the data sources searched for studies published before January 2019. Data were extracted, pooled, and analyzed using random-effects models and reported as AUC with 95% confidence intervals (CI). RESULTS: Of 1659 screened studies, 24 met inclusion criteria. CRIB was the most discriminate for predicting pre-discharge mortality [AUC 0.88 (0.86-0.90)]. GA was the least discriminate [AUC 0.76 (0.72-0.80)]. CONCLUSIONS: Although the original CRIB score was the most accurate predictor of pre-discharge mortality, significant heterogeneity between studies lowers confidence in this pooled estimate. A more precise illness severity score to predict pre-discharge mortality is still needed.

2.
Pediatr Pulmonol ; 55(3): 788-790, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31986238

RESUMO

Endobronchial inflammatory myofibroblastic tumors (IMTs) rarely occur in children younger than 10 years of age and have intermediate malignant potential. A 7-year-old girl initially presented with pneumonia. After failing outpatient treatment, she re-presented in status asthmaticus. Computed tomography showed a left mainstem endobronchial mass which was resected bronchoscopically. Pathology was consistent with IMT. Surveillance bronchoscopy identified a recurrence. Despite a left upper lobectomy, recurrence led to further treatment with celecoxib and argon plasma coagulation. Follow-up bronchoscopy revealed complete resolution. She remains disease and symptom-free at her six-year follow-up.

3.
J Pediatr Surg ; 55(1): 33-38, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31677822

RESUMO

BACKGROUND: Congenital diaphragmatic hernia (CDH) occurs in 1 out of 2500-3000 live births. Right-sided CDHs (R-CDHs) comprise 25% of all CDH cases, and data are conflicting on outcomes of these patients. The aim of our study was to compare outcomes in patients with right versus left CDH (L-CDH). METHODS: We analyzed a multicenter prospectively enrolled database to compare baseline characteristics and outcomes of neonates enrolled from January 2005 to January 2019 with R-CDH vs. L-CDH. RESULTS: A total of 588, 495 L-CDH, and 93 R-CDH patients with CDH were analyzed. L-CDHs were more frequently diagnosed prenatally (p=0.011). Lung-to-head ratio was similar in both cohorts. R-CDHs had a lower frequency of primary repair (p=0.022) and a higher frequency of need for oxygen at discharge (p=0.013). However, in a multivariate analysis, need for oxygen at discharge was no longer significantly different. There were no differences in long-term neurodevelopmental outcomes assessed at two year follow up. There was no difference in mortality, need for ECMO, pulmonary hypertension, or hernia recurrence. CONCLUSION: In this large series comparing R to L-CDH patients, we found no significant difference in mortality, use of ECMO, or pulmonary complications. Our study supports prior studies that R-CDHs are relatively larger and more often require a patch or muscle flap for repair. TYPE OF STUDY: Prognosis study LEVEL OF EVIDENCE: Level II.

4.
J Pediatr Surg ; 55(1): 29-32, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31672411

RESUMO

BACKGROUND: The practice of "cutting-away" from venoarterial extracorporeal life support (ECLS) and leaving indwelling heparinized cannulas prior to decannulation is controversial. This study aims to determine the safety and efficacy of this strategy in patients with congenital diaphragmatic hernia (CDH) who require ECLS. METHODS: A single-center retrospective review of electronic health records was performed on all patients with CDH who underwent elective ECLS decannulation between January 2014 and September 2018. Descriptive statistics are presented as medians with interquartile range. RESULTS: Seventy-three percent (19/26) of patients who underwent venoarterial ECLS for CDH were electively decannulated. After a median ECLS run of 10.7 days [6.1-19.5], patients were "cut-away" for a median of 26 h [19.8-43] prior to decannulation. One patient required re-initiation at 36 h for a pulmonary hypertensive crisis (5%). There were no major bleeding or embolic events while "cut-away", and four (21%) patients had clots removed from the cannulas without clinical sequelae. One patient was recannulated 16 days following initial decannulation. CONCLUSIONS: Our data suggests that "cutting-away" from ECLS in patients with congenital diaphragmatic hernia is safe and allows a period of observation without significant complications. This strategy may be particularly helpful in patients at risk for recannulation, but better prognostic criteria are needed. LEVEL OF EVIDENCE: Level IV. TYPE OF STUDY: Treatment Study.

5.
J Pediatr Surg ; 2019 Nov 26.
Artigo em Inglês | MEDLINE | ID: mdl-31813580

RESUMO

PURPOSE: Prenatal risk assessment of congenital diaphragmatic hernia (CDH) relies on prenatal ultrasound (U/S) and fetal magnetic resonance imaging (MRI). When the modalities differ in prognosis, it is unclear which is more reliable. METHODS: Retrospective chart review identified cases of prenatally diagnosed CDH from 4/2010-6/2018 meeting inclusion criteria. Demographic, radiologic, and postnatal outcomes data were collected. Ultrasound- versus MRI-based prognosis (mild, moderate, and severe) was compared with clinical outcomes. Kappa measures compared congruency in disease severity scaling between imaging modalities, while logistic regression and receiver operating characteristics curves compared the ability of each modality to predict outcomes. RESULTS: Forty-two patients met criteria. Both U/S- and MRI-based prognosis categories differentiated for survival. MRI categories differentiated for ECMO use, surgical repair, and defect type. O/e TFLV better discriminated for survivors and defect type than o/e LHR. Seventeen (40.5%) had discordant prenatal prognostic categories. In 13/17 (76.5%), o/e TFLV predicted higher severity when compared to o/e LHR, but sample size was insufficient to compare accuracy in cases of discordance. CONCLUSIONS: Clinical outcomes suggest fetal MRI may more accurately predict severe pulmonary hypoplasia compared to prenatal ultrasound. Our analysis suggests fetal MRI is a valuable adjunct in the prenatal evaluation of CDH. LEVEL OF EVIDENCE: Level III. TYPE OF STUDY: Retrospective Review.

6.
Fetal Diagn Ther ; : 1-9, 2019 Dec 20.
Artigo em Inglês | MEDLINE | ID: mdl-31865353

RESUMO

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a potentially lethal birth defect, and identifying prenatal predictors of outcome is important. Observed-to-expected total fetal lung volume (o/e TFLV) has been shown to be a predictor of severity and useful in risk stratification but is variable due to different TFLV formulas. OBJECTIVES: To calculate o/e TFLV for CDH patients part of a twin gestation using the unaffected sibling as an internal control and comparing these values to those calculated using published formulas for TFLV. METHODS: Seven twin gestations with one fetus affected by CDH were identified between 2006 and 2017. The lung volume for each twin was calculated using magnetic resonance imaging (MRI), and o/e TFLV was calculated using the unaffected twin's TFLV. This percentage was then compared to the o/e TFLV calculated using published formulas. RESULTS: Lung volumes in the unaffected twins were within normal ranges at the lower end of the spectrum. No single TFLV formula was found to correlate perfectly. Intraclass correlation coefficient estimate was most consistent for o/e TFLV calculated with the Meyers formula and supported by Bland-Altman plots. CONCLUSIONS: O/e TFLV measured in CDH/non-CDH twin gestations using the unaffected sibling demonstrated agreement with o/e TFLV calculated using the Meyers formula. We urge the fetal community to standardize the method, use, and interpretation of fetal MRI in the prenatal evaluation of CDH.

7.
ASAIO J ; 2019 Sep 30.
Artigo em Inglês | MEDLINE | ID: mdl-31577624

RESUMO

The modalities of vascular access for the extracorporeal artificial placenta (AP) have undergone many iterations over the past decade. We hypothesized that single lumen jugular cannulation (SLC) using tidal flow extracorporeal life (ECLS) support is a feasible alternative to venovenous (VV) umbilical-jugular cannulation and double lumen cannulation (DLC) and can maintain fetal circulation, stable hemodynamics, and adequate gas exchange for 24 hours. After in vitro evaluation of the tidal flow system, six preterm lambs at estimated gestational age 118-124 days (term 145 days) were delivered and underwent VV-ECLS. Three were supported using DLC and three with SLC utilizing tidal flow AP support. Hemodynamics, circuit flow, and gas exchange were monitored. Target fetal parameters were as follows: mean arterial pressure 40-60 mmHg, heart rate 140-240 beats per minute (bpm), SatO2% 60-80%, PaO2 25-50 mmHg, PaCO2 30-55 mmHg, oxygen delivery >5 ml O2/dl/kg/min, and circuit flow 100 ± 25 ml/kg/min. All animals survived 24 hours and maintained fetal circulation with stable hemodynamics and adequate gas exchange. Parameters of the tidal flow group were comparable with those of DLC. Single lumen jugular cannulation using tidal flow is a promising vascular access strategy for AP support. Successful miniaturization holds great potential for clinical translation to support extremely premature infants.

8.
J Surg Res ; 244: 291-295, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31302327

RESUMO

BACKGROUND: Advances in prenatal imaging is increasing detection of abnormally dilated bowel. There is no literature to date defining the criteria for a dilated rectum or its association with postnatal pathology. The aim of this study is to investigate the clinical significance of a prenatally identified dilated rectum. METHODS: A retrospective review was performed of all cases of "dilated bowel" on prenatal ultrasound between January 2000 and December 2017 at a single institution. We excluded ventral wall defects from review and sought to include only cases of a prominent or dilated rectum. Collected data included prenatal bowel measurements, postnatal diagnoses, need for surgical intervention, and outcomes. Descriptive statistics were applied. RESULTS: One hundred and ninety-three cases of prenatal "dilated bowel" were identified in which 12 (6.2%) had specifically visualized a prominent or dilated rectum. Nine of these (75.0%) had no rectal or intestinal abnormality on postnatal evaluation and were discharged feeding and defecating normally. The remaining three cases exhibited clinical pathology necessitating additional management: (1) meconium plug, (2) jejunal atresia with cecal perforation, and (3) rectal perforation with retroperitoneal abscess. All three had rectal biopsies with identification of ganglionated submucosa. CONCLUSIONS: Although a prenatal dilated rectum is a normal variant in the vast majority of cases, it may be associated with a gastrointestinal abnormality requiring surgical intervention. Interestingly, there were no cases of Hirschsprung's disease or anorectal malformations in this cohort. These results, in conjunction with continued efforts to identify and define rectal dilation, are useful for prenatal counseling and postnatal evaluation.


Assuntos
Reto/diagnóstico por imagem , Reto/patologia , Ultrassonografia Pré-Natal , Dilatação Patológica , Feminino , Humanos , Recém-Nascido , Gravidez , Estudos Retrospectivos
9.
J Pediatr Surg ; 54(6): 1147-1152, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30902457

RESUMO

INTRODUCTION: The purpose of this study is to evaluate splenic effects during artificial placenta (AP) support. METHODS: AP lambs (118-121 d, n = 14) were delivered and placed on the AP support for a goal of 10-14 days. Cannulation used right jugular drainage and umbilical vein reinfusion. Early (ETC; 115-120 d; n = 7) and late (LTC; 125-131 d; n = 7) tissue controls were delivered and immediately sacrificed. Spleens were formalin fixed, H&E stained, and graded for injury, response to inflammation, and extramedullary hematopoiesis (EMH). CD68 and CD163 stains were used to assess for macrophage activation and density. Clinical variables were correlated with splenic scores. Groups were compared using Fisher's Exact Test and descriptive statistics. p < 0.05 indicated significance. RESULTS: Mean survival for AP lambs was 12 ±â€¯5 d. There was no necrosis found in any of the groups. Vascular congestion and sinusoidal histiocytosis did not significantly differ between AP and control groups (p = 0.72; p = 0.311). There were significantly more pigmented macrophages (p = 0.008), CD163 (p = <0.001), and CD68 (p = <0.001) stained cells in the AP group. ETC and LTC demonstrated more EMH than AP spleens (p = <0.001). CONCLUSIONS: During AP support, spleens appear to develop normally and exhibit an appropriate inflammatory response. After initiation of AP support, EMH transitions away from the spleen. STUDY TYPE: Research Paper/Therapeutic Potential. LEVEL OF EVIDENCE: N/A.


Assuntos
Órgãos Artificiais , Placenta/fisiologia , Nascimento Prematuro , Carneiro Doméstico/crescimento & desenvolvimento , Baço , Animais , Feminino , Gravidez , Nascimento Prematuro/mortalidade , Nascimento Prematuro/veterinária , Ovinos , Baço/crescimento & desenvolvimento , Baço/imunologia , Baço/fisiologia
11.
Arch Dis Child Fetal Neonatal Ed ; 104(4): F372-F377, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30049725

RESUMO

BACKGROUND: Although fetal ultrasound, fetal MRI and postnatal CT are now widely used in the evaluation of congenital lung malformations (CLM), their diagnostic accuracy remains undefined. OBJECTIVE: To correlate prenatal and postnatal imaging studies with pathological data after CLM resection. DESIGN: Retrospective, descriptive case series study. SETTING: A North American tertiary care centre. PATIENTS: One hundred and three consecutive lung resections for a suspected CLM between 1 January 2005 and 31 December 2015. MAIN OUTCOME MEASURES: Diagnostic accuracy of imaging diagnosis compared with pathological evaluation. RESULTS: Pathological diagnoses included congenital pulmonary airway malformation ((CPAM) n=45, 44%), bronchopulmonary sequestration (BPS; n=25, 24%), CPAM/BPS hybrid lesions (n=22, 21%) and pleuropulmonary blastoma (n=2, 2%). Fetal ultrasound detected 85 (82.5%) lesions and correctly diagnosed whether or not a lesion was a CPAM in 75% of cases (sensitivity 93%, specificity 32%). Fetal MRI had a similar concordance rate (73%) but was superior in correctly determining whether a systemic feeding vessel was present in 80% of cases (sensitivity 71%, specificity 88%) compared with an ultrasound accuracy rate of 72% (sensitivity 49%, specificity 93%). By comparison, postnatal CT correctly diagnosed whether a CPAM was present in 84% of cases (sensitivity 86%, specificity 77%) and whether a systemic feeding vessel was present in 90% of cases (sensitivity 92%, specificity 88%). CONCLUSIONS: Fetal ultrasound remains an important tool in the detection and evaluation of congenital lung malformations. However, it does not correctly predict histology in approximately 25% of prenatally detected CLMs and remains limited by relatively poor sensitivity for systemic feeding vessels pathognomic for a bronchopulmonary sequestration. These data suggest the importance of obtaining additional cross-sectional imaging, preferably a postnatal CT scan, in all patients to help counsel families and to guide in the optimal management of these lesions.


Assuntos
Pneumopatias/congênito , Pulmão/anormalidades , Pulmão/diagnóstico por imagem , Diagnóstico Pré-Natal/métodos , Anormalidades do Sistema Respiratório/diagnóstico por imagem , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Feminino , Humanos , Pulmão/embriologia , Imagem por Ressonância Magnética/métodos , Gravidez
12.
Am J Perinatol ; 36(7): 742-750, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30372770

RESUMO

PURPOSE: We hypothesized that surgical energy could be used to create hysterotomies in open fetal surgery. STUDY DESIGN: Initial studies compared the LigaSure Impact and Harmonic ACE + 7 Shears in the efficiency of hysterotomy and thermal damage. Pregnant ewes at an estimated gestational age (EGA) of 116 to 120 days (term = 145; n = 7) underwent hysterotomy using either device. Hysterotomy edges were resected, and thermal injury extent was determined by histopathological assessment. Upon determining a superior device, subsequent studies compared this to the AutoSuture Premium Poly CS*-57 Stapler in uterine healing. Pregnant ewes (n = 6) at an EGA of 87 to 93 days underwent 6-cm hysterotomy in each gravid horn with either the stapler (n = 5) or Harmonic (n = 5) followed by closure and animal recovery. After 37 to 42 days, uterine healing was assessed by evaluating tensile strength and histopathology. RESULTS: Thermal damage was more extensive with the LigaSure (n = 11 hysterotomies) than with the Harmonic (n = 11; 5.6 ± 1 vs. 3.1 ± 0.6 mm; p < 0.0001);therefore, the Harmonic was selected for healing studies. Gross scar appearance and tensile strength were the same between the Harmonic and stapler. The stapler caused more fibrosis (4/7 samples with "moderate" fibrosis vs. 0/8 with the Harmonic; p = 0.02). CONCLUSION: The Harmonic ACE + 7 caused less thermal injury than the LigaSure Impact and performed similar to the CS*-57 Stapler in uterine healing with continued gestation.

13.
ASAIO J ; 65(7): 690-697, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30585874

RESUMO

An artificial placenta (AP) utilizing extracorporeal life support (ECLS) could protect premature lungs from injury and promote continued development. Preterm lambs at estimated gestational age (EGA) 114-128 days (term = 145) were delivered by Caesarian section and managed in one of three groups: AP, mechanical ventilation (MV), or tissue control (TC). Artificial placenta lambs (114 days EGA, n = 3; 121 days, n = 5) underwent venovenous (VV)-ECLS with jugular drainage and umbilical vein reinfusion for 7 days, with a fluid-filled, occluded airway. Mechanical ventilation lambs (121 days, n = 5; 128 days, n = 5) underwent conventional MV until failure or maximum 48 hours. Tissue control lambs (114 days, n = 3; 121 days, n = 5; 128 days, n = 5) were sacrificed at delivery. At the conclusion of each experiment, lungs were procured and sectioned. Hematoxylin and eosin (H&E) slides were scored 0-4 in seven injury categories, which were summed for a total injury score. Slides were also immunostained for platelet-derived growth factor receptor (PDGFR)-α and α-actin; lung development was quantified by the area fraction of double-positive tips of secondary alveolar septa. Support duration of AP lambs was 163 ± 9 (mean ± SD) hours, 4 ± 3 for early MV lambs, and 40 ± 6 for late MV lambs. Total injury scores at 121 days were 1.7 ± 2.1 for AP vs. 5.5 ± 1.6 for MV (p = 0.02). Using immunofluorescence, double-positive tip area fraction at 121 days was 0.017 ± 0.011 in AP lungs compared with 0.003 ± 0.003 in MV lungs (p < 0.001) and 0.009 ± 0.005 in TC lungs. At 128 days, double-positive tip area fraction was 0.012 ± 0.007 in AP lungs compared with 0.004 ± 0.004 in MV lungs (p < 0.001) and 0.016 ± 0.009 in TC lungs. The AP is protective against lung injury and promotes lung development compared with mechanical ventilation in premature lambs.

14.
PLoS Genet ; 14(12): e1007822, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30532227

RESUMO

Congenital diaphragmatic hernia (CDH) is a severe birth defect that is often accompanied by other congenital anomalies. Previous exome sequencing studies for CDH have supported a role of de novo damaging variants but did not identify any recurrently mutated genes. To investigate further the genetics of CDH, we analyzed de novo coding variants in 362 proband-parent trios including 271 new trios reported in this study. We identified four unrelated individuals with damaging de novo variants in MYRF (P = 5.3x10(-8)), including one likely gene-disrupting (LGD) and three deleterious missense (D-mis) variants. Eight additional individuals with de novo LGD or missense variants were identified from our other genetic studies or from the literature. Common phenotypes of MYRF de novo variant carriers include CDH, congenital heart disease and genitourinary abnormalities, suggesting that it represents a novel syndrome. MYRF is a membrane associated transcriptional factor highly expressed in developing diaphragm and is depleted of LGD variants in the general population. All de novo missense variants aggregated in two functional protein domains. Analyzing the transcriptome of patient-derived diaphragm fibroblast cells suggest that disease associated variants abolish the transcription factor activity. Furthermore, we showed that the remaining genes with damaging variants in CDH significantly overlap with genes implicated in other developmental disorders. Gene expression patterns and patient phenotypes support pleiotropic effects of damaging variants in these genes on CDH and other developmental disorders. Finally, functional enrichment analysis implicates the disruption of regulation of gene expression, kinase activities, intra-cellular signaling, and cytoskeleton organization as pathogenic mechanisms in CDH.


Assuntos
Variação Genética , Hérnias Diafragmáticas Congênitas/genética , Proteínas de Membrana/genética , Mutação , Fatores de Transcrição/genética , Pré-Escolar , Variações do Número de Cópias de DNA , Deficiências do Desenvolvimento/genética , Feminino , Cardiopatias Congênitas/genética , Hérnias Diafragmáticas Congênitas/metabolismo , Humanos , Recém-Nascido , Estudos Longitudinais , Masculino , Proteínas de Membrana/metabolismo , Mutação de Sentido Incorreto , Fenótipo , Análise de Sequência de RNA , Síndrome , Fatores de Transcrição/metabolismo , Sequenciamento Completo do Exoma , Sequenciamento Completo do Genoma
15.
J Pediatr Surg ; 53(10): 1896-1903, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29960740

RESUMO

PURPOSE: An artificial placenta (AP) utilizing extracorporeal life support (ECLS) could avoid the harm of mechanical ventilation (MV) while allowing the lungs to develop. METHODS: AP lambs (n = 5) were delivered at 118 days gestational age (GA; term = 145 days) and placed on venovenous ECLS (VV-ECLS) with jugular drainage and umbilical vein reinfusion. Lungs remained fluid-filled. After 10 days, lambs were ventilated. MV control lambs were delivered at 118 ("early MV"; n = 5) or 128 days ("late MV"; n = 5), and ventilated. Compliance and oxygenation index (OI) were calculated. After sacrifice, lungs were procured and H&E-stained slides scored for lung injury. Slides were also immunostained for PDGFR-α and α-actin; alveolar development was quantified by the area fraction of alveolar septal tips staining double-positive for both markers. RESULTS: Compliance of AP lambs was 2.79 ±â€¯0.81 Cdyn compared to 0.83 ±â€¯0.19 and 3.04 ±â€¯0.99 for early and late MV, respectively. OI in AP lambs was lower than early MV lambs (6.20 ±â€¯2.10 vs. 36.8 ±â€¯16.8) and lung injury lower as well (1.8 ±â€¯1.6 vs. 6.0 ±â€¯1.2). Double-positive area fractions were higher in AP lambs (0.012 ±â€¯0.003) than early (0.003 ±â€¯0.0005) and late (0.004 ±â€¯0.002) MV controls. CONCLUSIONS: Lung development continues and lungs are protected from injury during AP support relative to mechanical ventilation. LEVEL OF EVIDENCE: n/a (basic/translational science).


Assuntos
Órgãos Artificiais , Oxigenação por Membrana Extracorpórea , Pulmão/crescimento & desenvolvimento , Nascimento Prematuro/terapia , Animais , Animais Recém-Nascidos , Modelos Animais de Doenças , Feminino , Idade Gestacional , Pulmão/fisiologia , Placenta/fisiologia , Gravidez , Ovinos
16.
J Pediatr Surg ; 53(6): 1240-1245, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29605266

RESUMO

BACKGROUND: An Artificial Placenta (AP) utilizing extracorporeal life support (ECLS) could revolutionize care of extremely premature newborns, but its effects on gastrointestinal morphology and injury need investigation. METHODS: Lambs (116-121days GA, term=145; n=5) were delivered by C-section, cannulated for ECLS, had total parenteral nutrition (TPN) provided, and were supported for 7days before euthanasia. Early and Late Tissue Controls (ETC, n=5 and LTC, n=5) delivered at 115-121days and 125-131days, respectively, were immediately sacrificed. Standardized jejunal samples were formalin-fixed for histology. Crypt depth (CD), villus height (VH), and VH:CD ratios were measured. Measurements also included enterocyte proliferation (Ki-67), Paneth cell count (Lysozyme), and injury scores (H&E). ANOVA and Chi Square were used with p<0.05 considered significant. RESULTS: CD, VH, and VH:CD were similar between groups (p>0.05). AP demonstrated more enterocyte proliferation (95.7±21.8) than ETC (49.4±23.4; p=0.003) and LTC (66.1+11.8; p=0.04), and more Paneth cells (81.7±17.5) than ETC (41.6±7.0; p=0.0005) and LTC (40.7±8.2, p=0.0004). Presence of epithelial injury and congestion in the bowel of all groups were not statistically different. No villus atrophy or inflammation was present in any group. CONCLUSIONS: This suggests preserved small bowel mucosal architecture, high cellular turnover, and minimal evidence of injury. STUDY TYPE: Research paper/therapeutic potential. LEVEL OF EVIDENCE: N/A.


Assuntos
Órgãos Artificiais , Oxigenação por Membrana Extracorpórea/métodos , Mucosa Gástrica/crescimento & desenvolvimento , Mucosa Intestinal/crescimento & desenvolvimento , Placenta , Animais , Contagem de Células , Proliferação de Células , Enterócitos/citologia , Feminino , Mucosa Gástrica/patologia , Humanos , Mucosa Intestinal/patologia , Jejuno/crescimento & desenvolvimento , Jejuno/patologia , Celulas de Paneth/citologia , Nutrição Parenteral Total , Gravidez , Nascimento Prematuro , Ovinos
17.
J Pediatr Surg ; 53(6): 1234-1239, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29605267

RESUMO

PURPOSE: We evaluated whether brain development continues and brain injury is prevented during Artificial Placenta (AP) support utilizing extracorporeal life support (ECLS). METHODS: Lambs at EGA 118days (term=145; n=4) were placed on AP support (venovenous ECLS with jugular drainage and umbilical vein reinfusion) for 7days and sacrificed. Early (EGA 118; n=4) and late (EGA 127; n=4) mechanical ventilation (MV) lambs underwent conventional MV for up to 48h and were sacrificed, and early (n=5) and late (n=5) tissue control (TC) lambs were sacrificed at delivery. Brains were harvested, formalin-fixed, rehydrated, and studied by magnetic resonance imaging (MRI). The gyrification index (GI), a measure of cerebral folding complexity, was calculated for each brain. Diffusion-weighted imaging was used to determine fractional anisotropy (FA) and apparent diffusion coefficient (ADC) in multiple structures to assess white matter (WM) integrity. RESULTS: No intracranial hemorrhage was observed. GI was similar between AP and TC groups. ADC and FA did not differ between AP and late TC groups in any structure. Compared to late MV brains, AP brains demonstrated significantly higher ADC (0.45±0.08 vs. 0.27±0.11, p=0.02) and FA (0.61±0.04 vs. 0.44±0.05; p=0.006) in the cerebral peduncles. CONCLUSIONS: After 7days of AP support, WM integrity is preserved relative to mechanical ventilation. TYPE OF STUDY: Research study.


Assuntos
Órgãos Artificiais , Lesões Encefálicas/prevenção & controle , Encéfalo/crescimento & desenvolvimento , Oxigenação por Membrana Extracorpórea/métodos , Recém-Nascido Prematuro/fisiologia , Placenta , Animais , Anisotropia , Encéfalo/diagnóstico por imagem , Lesões Encefálicas/diagnóstico por imagem , Modelos Animais de Doenças , Feminino , Humanos , Imagem por Ressonância Magnética , Gravidez , Respiração Artificial , Ovinos
18.
J Pediatr Surg ; 53(6): 1087-1091, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29580786

RESUMO

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.


Assuntos
Hérnias Diafragmáticas Congênitas/complicações , Transtornos do Neurodesenvolvimento/etiologia , Criança , Pré-Escolar , Terapia Combinada , Oxigenação por Membrana Extracorpórea , Feminino , Seguimentos , Hérnias Diafragmáticas Congênitas/diagnóstico , Hérnias Diafragmáticas Congênitas/terapia , Herniorrafia , Humanos , Lactente , Recém-Nascido , Masculino , Transtornos do Neurodesenvolvimento/diagnóstico , Transtornos do Neurodesenvolvimento/epidemiologia , Prognóstico , Estudos Retrospectivos
19.
J Pediatr Surg ; 53(7): 1301-1304, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29459043

RESUMO

BACKGROUND: Neurologic complications are common, and amongst the most devastating complications in pediatric patients undergoing extracorporeal life support (ECLS). Carotid artery cannulation (CAN) has been associated with an increase in these complications, thereby shaping practices to avoid this approach in most pediatric patients in which other cannulation approaches are viable. METHODS: A retrospective review of children (0-18years) in the ELSO database was undertaken from 1989 through 2013. Multivariate logistic regression analysis of rates of stroke and other neurologic complications based on cannulation technique was undertaken, adjusting for patient factors including age, underlying disease process, and severity of illness. RESULTS: A total of 30,282 ECLS runs were found in the database. CAN was associated with higher rates of stroke (5.15% vs 3.74%) and overall neurologic complications. However, when correcting for patient factors, including age, underlying disease process, and support type, CAN was not associated with an increased rate of neurologic complications or stroke (p>0.05 for both). CONCLUSION: When correcting for patient related factors CAN is not associated with an increase in stroke or neurologic compilcations. CAN should be re-examined as a cannulation technique for older pediatric patients. LEVEL OF EVIDENCE: III.


Assuntos
Cateterismo/efeitos adversos , Oxigenação por Membrana Extracorpórea/métodos , Acidente Vascular Cerebral/etiologia , Adolescente , Fatores Etários , Cateterismo/métodos , Criança , Pré-Escolar , Bases de Dados Factuais , Oxigenação por Membrana Extracorpórea/efeitos adversos , Feminino , Humanos , Lactente , Recém-Nascido , Modelos Logísticos , Masculino , Doenças do Sistema Nervoso/epidemiologia , Doenças do Sistema Nervoso/etiologia , Estudos Retrospectivos , Fatores de Risco , Acidente Vascular Cerebral/epidemiologia
20.
Neonatology ; 113(4): 313-321, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29478055

RESUMO

BACKGROUND: Extremely premature neonates suffer high morbidity and mortality. An artificial placenta (AP) using extracorporeal life support (ECLS) is a promising therapy. OBJECTIVES: We hypothesized that intratracheal perfluorocarbon (PFC) instillation during AP support would reduce lung injury and promote lung development relative to intratracheal amniotic fluid or crystalloid. METHODS: Lambs at an estimated gestational age (EGA) 116-121 days (term 145 days) were placed on venovenous ECLS with jugular drainage and umbilical vein reinfusion and intubated. Airways were managed by the instillation of amniotic fluid and tracheal occlusion (TO; n = 4), or lactated Ringer's (LR; n = 4) or perfluorodecalin (a PFC) without occlusion (n = 4). After 7 days, the animals were sacrificed. Early (EGA 116-121 days) and late (EGA 125-131 days) tissue control lambs were delivered and sacrificed. Lungs were formalin-inflated to 30 cm H2O and sectioned for histology. Injury was scored by an unbiased pathologist. Slides were immunostained for PDGFR-α and α-actin; development was quantified by the area fraction of double-positive tips. Surfactant protein-C (SP-C) concentration in bronchoalveolar lavage fluid was quantified using ELISA. RESULTS: Total injury scores were lower in PFC lungs (1.8 ± 1.7) than in TO (6.5 ± 2.1; p = 0.01) and LR lungs (5.5 ± 2.4; p = 0.01). The area fraction of double-positive alveolar tips appeared higher in PFC lungs than in TO lungs (0.18 ± 0.007 vs. 0.008 ± 0.004; p = 0.07). SP-C concentration was higher in PFC lungs than in TO lungs (37.9 ± 7.6 vs. 20.0 ± 5.4 pg/mL; p = 0.005), and both early (12.4 ± 1.7 g/mL; p = 0.007) and late tissue control lungs (15.1 ± 5.0 pg/mL; p = 0.0008). CONCLUSION: During AP support, intratracheal PFC prevents lung injury and promotes normal lung development better than crystalloid or amniotic fluid with TO.


Assuntos
Animais Recém-Nascidos , Órgãos Artificiais , Oxigenação por Membrana Extracorpórea , Fluorcarbonetos/administração & dosagem , Lesão Pulmonar/prevenção & controle , Animais , Feminino , Pulmão/crescimento & desenvolvimento , Placenta/fisiologia , Gravidez , Ovinos
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