Necrotizing enterocolitis totalis: High mortality in the absence of an aggressive surgical approach

Dukleska, Katerina; Devin, Courtney L.; Martin, Abigail E.; Miller, Jonathan M.; Sullivan, Kevin M.; Levy, Carly; Prestowitz, Sky; Flathers, Kristina; Vinocur, Charles D.; Berman, Loren

doi:10.1016/j.surg.2019.03.005

Cited by 28 publications

(15 citation statements)

References 53 publications

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“…Definitions of NEC-Totalis vary in the literature, but generally refer to patients with massive, neartotal bowel ischemia and necrosis. 4 The vast majority of patients diagnosed with NEC-totalis will succumb to the disease, with a reported 93.3% mortality rate. 4 The pathogenesis of NEC is multifactorial and is an area of ongoing research with most existing literature identifying risk factors for developing NEC without regard for disease severity.…”

Section: Introductionmentioning

confidence: 99%

“…4 The vast majority of patients diagnosed with NEC-totalis will succumb to the disease, with a reported 93.3% mortality rate. 4 The pathogenesis of NEC is multifactorial and is an area of ongoing research with most existing literature identifying risk factors for developing NEC without regard for disease severity. Major risk factors include preterm birth, low birthweight, congenital cardiac anomalies, and formula-feeding.…”

Section: Introductionmentioning

confidence: 99%

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Effect of Feeding Strategies on the Development of Fulminant Necrotizing Enterocolitis

et al. 2020

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Introduction Necrotizing enterocolitis (NEC) causes significant neonatal morbidity. A subset of infants experience precipitous decline and death from fulminant-NEC (F-NEC). We sought to determine the effect of feeding practices on the development of this more virulent form of NEC. Materials and Methods Premature neonates developing Bell's stage II or III NEC between May 2011 and June 2017 were reviewed. Infants were stratified as having NEC or F-NEC, defined as NEC-totalis or NEC causing rapid decline and death within 72 hours. Risk factors extracted included demographics, gestational age, and weight at NEC diagnosis. Feeding data extracted included age at first feed, caloric density, type of feed (breast milk or formula), and whether full volume feeds were reached. Univariate analysis and multivariate analysis were performed. Results A total of 98 patients were identified, of which 80 were included. In total, 57 patients had NEC and 23 had F-NEC. Reaching full volume feeds was associated with F-NEC on both univariate and multivariate analysis (37.9 vs. 4.5%; odds ratio: 67, 95% confidence interval: 6.606–2041, p = 0.003). Infants developing F-NEC achieved full feeds earlier (22.5 vs. 19.8 days, p = 0.025) on univariate but not multivariate analysis. There was no difference in the rates of NEC and F-NEC among infants receiving breast milk (standard or fortified) or formula (standard or increased caloric density; p = 0.235). Conclusion Among premature neonates with NEC, reaching full volume feedings was associated with a nearly 70-fold increased risk of F-NEC. Assuming it was possible to predict an infant's development of NEC, alternative feeding regimens might reduce the risk of F-NEC in this population.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Effect of Feeding Strategies on the Development of Fulminant Necrotizing Enterocolitis

et al. 2020

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“…Ten percent of neonatal intensive care deaths are attributed to necrotizing enterocolitis (NEC) [ 1 ]. Necrotizing enterocolitis totalis ( t NEC), a rare form of NEC characterized by near-total bowel necrosis, is associated with almost 100% fatality [ 2 ]. Of infants who require surgery for NEC, 11% have t NEC [ 3 ], and, amongst those who die from NEC, 33% have total bowel necrosis[ 1 ].…”

Section: Introductionmentioning

confidence: 99%

“…Of infants who require surgery for NEC, 11% have t NEC [ 3 ], and, amongst those who die from NEC, 33% have total bowel necrosis[ 1 ]. However, the true incidence of this disease is not known, as t NEC cannot currently be preoperatively identified and is subjectively defined in the literature [ 2 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

Characteristics and outcomes of a multi-institution cohort of infants with necrotizing enterocolitis totalis

et al. 2021

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Objective To characterize the presentation, management and outcomes of infants with necrotizing enterocolitis totalis ( t NEC) vs surgical non-totalis NEC ( s NEC). Study design This retrospective study identified infants undergoing surgery for NEC through The Children’s Hospitals Neonatal Database. Demographic, surgical and mortality characteristics were compared. Results Of 1059 infants, 161 (15.2%) had t NEC. Perinatal characteristics did not differ. t NEC infants were older and were less likely to have pneumoperitoneum at referral (5.6% vs 13.1%, p < 0.001) or intestinal perforation at surgery (38.5% vs 66.7%, p < 0.001). Infants with t NEC were more acidotic preoperatively (7.1, [IQR 7, 7.3] vs 7.3, [IQR 7.2, 7.4], p < 0.001). Mortality was 96.9% for t NEC and 26.5% for s NEC ( p < 0.001). t NEC cases varied by center, accounting for 0–43% of all surgical NEC cases. Conclusions Mortality is high for t NEC infants, who present at older age, with greater illness severity but are less likely to have intestinal perforation than s NEC infants.

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“…necrotizing enterocolitis (nec) is an acute life-threatening gastrointestinal disease that predominantly affects premature neonates (1,2). The morbidity of nec is rising rapidly with the continuing increased rate of survival of preterm infants (3). Patients that survive severe nec may develop serious sequelae, including gastrointestinal complications and abnormal neurodevelopment (4).…”

Section: Introductionmentioning

confidence: 99%

Integrated analysis of a lncRNA‑mRNA network reveals a potential mechanism underlying necrotizing enterocolitis

et al. 2020

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Previous studies have shown that long non-coding rnas (lncrnas) serve important roles in necrotizing enterocolitis (nec). However, the underlying mechanisms remain largely unknown. in order to examine the potential role of lncrnas in nec, the present study investigated lncrna and mRNA expression profiles in NEC lesions and adjacent intestinal tissues using next Generation Sequencing. a total of 4,202 differentially expressed lncrnas (fold-change >2; P<0.05) and 7,860 differentially expressed mrnas (fold-change >2; P<0.05) were identified. Moreover, 5 dysregulated lncrnas and 5 mrnas were randomly selected, and further assessed by reverse transcription-quantitative Pcr in vitro. Gene ontology and Kyoto encyclopedia of Genes and Genomes analyses demonstrated that the differentially expressed lncrnas were closely associated with nec, and were enriched in 'inflammatory response', 'Toll-like receptor binding', 'PPar signaling pathway', 'Pi3K-akt signaling pathway', 'transforming growth factor-β signaling pathway' and 'hypoxia-inducible factor 1 signaling pathway'. in addition, co-expression analysis demonstrated that these lncrnas, including lncrna enST00000623580, lncrna nonHSaT180418.1, lncrna nonHSaT125636.2 and nonHSaT087855.2, may mediate the pathogenesis and development of nec via lncrna-mrna network interactions. Therefore, the present study provided a novel insight into the role of lncrnas in nec.

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Necrotizing enterocolitis totalis: High mortality in the absence of an aggressive surgical approach

Cited by 28 publications

References 53 publications

Effect of Feeding Strategies on the Development of Fulminant Necrotizing Enterocolitis

Effect of Feeding Strategies on the Development of Fulminant Necrotizing Enterocolitis

Characteristics and outcomes of a multi-institution cohort of infants with necrotizing enterocolitis totalis

Integrated analysis of a lncRNA‑mRNA network reveals a potential mechanism underlying necrotizing enterocolitis

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