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Necrotizing enterocolitis

MedGen UID:
105440
Concept ID:
C0520459
Disease or Syndrome
Synonyms: Enterocolitis, Necrotizing; Necrotizing Enterocolitis
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0033165
Monarch Initiative: MONDO:0005313
Orphanet: ORPHA391673

Definition

Inflammation of the intestine leading to bacterial invasion causing cellular damage and death which causes necrosis of the colon and intestine. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNecrotizing enterocolitis

Conditions with this feature

Fumarase deficiency
MedGen UID:
87458
Concept ID:
C0342770
Disease or Syndrome
Fumarate hydratase (FH) deficiency results in severe neonatal and early infantile encephalopathy that is characterized by poor feeding, failure to thrive, hypotonia, lethargy, and seizures. Dysmorphic facial features include frontal bossing, depressed nasal bridge, and widely spaced eyes. Many affected individuals are microcephalic. A spectrum of brain abnormalities are seen on magnetic resonance imaging, including cerebral atrophy, enlarged ventricles and generous extra-axial cerebral spinal fluid (CSF) spaces, delayed myelination for age, thinning of the corpus callosum, and an abnormally small brain stem. Brain malformations including bilateral polymicrogyria and absence of the corpus callosum can also be observed. Development is severely affected: most affected individuals are nonverbal and nonambulatory, and many die during early childhood. Less severely affected individuals with moderate cognitive impairment and long-term survival have been reported.
Immunodeficiency due to ficolin3 deficiency
MedGen UID:
462576
Concept ID:
C3151226
Disease or Syndrome
Individuals with ficolin-3 deficiency have highly variable manifestations and a variable age of symptom onset. Some patients may show increased susceptibility to infection in the perinatal or neonatal period, whereas others may show autoimmune features as adults. Ficolin-3, also known as H-ficolin, can activate the lectin pathway of the complement system; deficiency may thus lead to defects in the complement system (summary by Munthe-Fog et al., 2009 and Michalski et al., 2015). For a discussion of genetic heterogeneity of lectin complement activation pathway defects, see LCAPD1 (614372).
Very long chain acyl-CoA dehydrogenase deficiency
MedGen UID:
854382
Concept ID:
C3887523
Disease or Syndrome
Deficiency of very long-chain acyl-coenzyme A dehydrogenase (VLCAD), which catalyzes the initial step of mitochondrial beta-oxidation of long-chain fatty acids with a chain length of 14 to 20 carbons, is associated with three phenotypes. The severe early-onset cardiac and multiorgan failure form typically presents in the first months of life with hypertrophic or dilated cardiomyopathy, pericardial effusion, and arrhythmias, as well as hypotonia, hepatomegaly, and intermittent hypoglycemia. The hepatic or hypoketotic hypoglycemic form typically presents during early childhood with hypoketotic hypoglycemia and hepatomegaly, but without cardiomyopathy. The later-onset episodic myopathic form presents with intermittent rhabdomyolysis provoked by exercise, muscle cramps and/or pain, and/or exercise intolerance. Hypoglycemia typically is not present at the time of symptoms.
Hyperphosphatasia with intellectual disability syndrome 6
MedGen UID:
906509
Concept ID:
C4225201
Disease or Syndrome
Hyperphosphatasia with impaired intellectual development syndrome-6 (HPMRS6) is an autosomal recessive multisystem disorder characterized by global developmental delay, dysmorphic features, seizures, and congenital cataracts. Severity is variable, and the disorder may show a range of phenotypic and biochemical abnormalities, including increased serum alkaline phosphatase levels (summary by Ilkovski et al., 2015). The disorder is caused by a defect in glycosylphosphatidylinositol (GPI) biosynthesis. For a discussion of genetic heterogeneity of HPMRS, see HPMRS1 (239300). For a discussion of genetic heterogeneity of GPI biosynthesis defects, see GPIBD1 (610293).
Visceral myopathy 2
MedGen UID:
1783630
Concept ID:
C5543466
Disease or Syndrome
Visceral myopathy-2 (VSCM2) is characterized by gastrointestinal symptoms resulting from intestinal dysmotility and paresis, including abdominal distention, pain, nausea, and vomiting. Some patients exhibit predominantly esophageal symptoms, with hiatal hernia and severe reflux resulting in esophagitis and stricture, whereas others experience chronic intestinal pseudoobstruction. Bladder involvement resulting in megacystis and megaureter has also been observed and may be evident at birth (Dong et al., 2019; Gilbert et al. (2020)).
Immunodeficiency 87 and autoimmunity
MedGen UID:
1794280
Concept ID:
C5562070
Disease or Syndrome
Immunodeficiency-87 and autoimmunity (IMD87) is an autosomal recessive immunologic disorder with wide phenotypic variation and severity. Affected individuals usually present in infancy or early childhood with increased susceptibility to infections, often Epstein-Barr virus (EBV), as well as with lymphadenopathy or autoimmune manifestations, predominantly hemolytic anemia. Laboratory studies may show low or normal lymphocyte numbers, often with skewed T-cell subset ratios. The disorder results primarily from defects in T-cell function, which causes both immunodeficiency and overall immune dysregulation (summary by Serwas et al., 2019 and Fournier et al., 2021).

Professional guidelines

PubMed

Bering J, DiBaise JK
Nutr Clin Pract 2023 May;38 Suppl 1:S46-S58. doi: 10.1002/ncp.10978. PMID: 37115034
Szajewska H, Berni Canani R, Domellöf M, Guarino A, Hojsak I, Indrio F, Lo Vecchio A, Mihatsch WA, Mosca A, Orel R, Salvatore S, Shamir R, van den Akker CHP, van Goudoever JB, Vandenplas Y, Weizman Z; ESPGHAN Special Interest Group on Gut Microbiota and Modifications
J Pediatr Gastroenterol Nutr 2023 Feb 1;76(2):232-247. Epub 2022 Oct 11 doi: 10.1097/MPG.0000000000003633. PMID: 36219218
Burris AD, Burris J, Järvinen KM
Neoreviews 2020 Dec;21(12):e795-e808. doi: 10.1542/neo.21-12-e795. PMID: 33262206

Recent clinical studies

Etiology

Duess JW, Sampah ME, Lopez CM, Tsuboi K, Scheese DJ, Sodhi CP, Hackam DJ
Gut Microbes 2023 Jan-Dec;15(1):2221470. doi: 10.1080/19490976.2023.2221470. PMID: 37312412Free PMC Article
Kaplina A, Kononova S, Zaikova E, Pervunina T, Petrova N, Sitkin S
Int J Mol Sci 2023 Jan 27;24(3) doi: 10.3390/ijms24032471. PMID: 36768793Free PMC Article
Duchon J, Barbian ME, Denning PW
Clin Perinatol 2021 Jun;48(2):229-250. doi: 10.1016/j.clp.2021.03.002. PMID: 34030811
Meister AL, Doheny KK, Travagli RA
Exp Biol Med (Maywood) 2020 Jan;245(2):85-95. Epub 2019 Dec 6 doi: 10.1177/1535370219891971. PMID: 31810384Free PMC Article
Alganabi M, Lee C, Bindi E, Li B, Pierro A
F1000Res 2019;8 Epub 2019 Jan 25 doi: 10.12688/f1000research.17228.1. PMID: 30740215Free PMC Article

Diagnosis

Neu J
Neonatology 2020;117(2):240-244. Epub 2020 Mar 10 doi: 10.1159/000506866. PMID: 32155645
Meister AL, Doheny KK, Travagli RA
Exp Biol Med (Maywood) 2020 Jan;245(2):85-95. Epub 2019 Dec 6 doi: 10.1177/1535370219891971. PMID: 31810384Free PMC Article
Bellodas Sanchez J, Kadrofske M
Neurogastroenterol Motil 2019 Mar;31(3):e13569. doi: 10.1111/nmo.13569. PMID: 30793842
Rich BS, Dolgin SE
Pediatr Rev 2017 Dec;38(12):552-559. doi: 10.1542/pir.2017-0002. PMID: 29196510
Neu J, Walker WA
N Engl J Med 2011 Jan 20;364(3):255-64. doi: 10.1056/NEJMra1005408. PMID: 21247316Free PMC Article

Therapy

Rausch LA, Hanna DN, Patel A, Blakely ML
Clin Perinatol 2022 Dec;49(4):955-964. Epub 2022 Oct 9 doi: 10.1016/j.clp.2022.07.005. PMID: 36328610
Sowden M, van Weissenbruch MM, Bulabula ANH, van Wyk L, Twisk J, van Niekerk E
Nutrients 2022 Aug 12;14(16) doi: 10.3390/nu14163305. PMID: 36014810Free PMC Article
Mitra S, Florez ID, Tamayo ME, Mbuagbaw L, Vanniyasingam T, Veroniki AA, Zea AM, Zhang Y, Sadeghirad B, Thabane L
JAMA 2018 Mar 27;319(12):1221-1238. doi: 10.1001/jama.2018.1896. PMID: 29584842Free PMC Article
Robinson JR, Rellinger EJ, Hatch LD, Weitkamp JH, Speck KE, Danko M, Blakely ML
Semin Perinatol 2017 Feb;41(1):70-79. Epub 2016 Nov 8 doi: 10.1053/j.semperi.2016.09.020. PMID: 27836422Free PMC Article
Ritchie ML, Romanuk TN
PLoS One 2012;7(4):e34938. Epub 2012 Apr 18 doi: 10.1371/journal.pone.0034938. PMID: 22529959Free PMC Article

Prognosis

Horbar JD, Greenberg LT, Buzas JS, Ehret DEY, Soll RF, Edwards EM
Pediatrics 2024 Jan 1;153(1) doi: 10.1542/peds.2023-064153. PMID: 38053449
Sowden M, van Weissenbruch MM, Bulabula ANH, van Wyk L, Twisk J, van Niekerk E
Nutrients 2022 Aug 12;14(16) doi: 10.3390/nu14163305. PMID: 36014810Free PMC Article
Altobelli E, Angeletti PM, Verrotti A, Petrocelli R
Nutrients 2020 May 6;12(5) doi: 10.3390/nu12051322. PMID: 32384652Free PMC Article
Askie LM, Darlow BA, Finer N, Schmidt B, Stenson B, Tarnow-Mordi W, Davis PG, Carlo WA, Brocklehurst P, Davies LC, Das A, Rich W, Gantz MG, Roberts RS, Whyte RK, Costantini L, Poets C, Asztalos E, Battin M, Halliday HL, Marlow N, Tin W, King A, Juszczak E, Morley CJ, Doyle LW, Gebski V, Hunter KE, Simes RJ; Neonatal Oxygenation Prospective Meta-analysis (NeOProM) Collaboration
JAMA 2018 Jun 5;319(21):2190-2201. doi: 10.1001/jama.2018.5725. PMID: 29872859Free PMC Article
Srinivasan PS, Brandler MD, D'Souza A
Clin Perinatol 2008 Mar;35(1):251-72, x. doi: 10.1016/j.clp.2007.11.009. PMID: 18280885

Clinical prediction guides

Neu J
Neonatology 2020;117(2):240-244. Epub 2020 Mar 10 doi: 10.1159/000506866. PMID: 32155645
Meister AL, Doheny KK, Travagli RA
Exp Biol Med (Maywood) 2020 Jan;245(2):85-95. Epub 2019 Dec 6 doi: 10.1177/1535370219891971. PMID: 31810384Free PMC Article
Rose AT, Patel RM
Semin Fetal Neonatal Med 2018 Dec;23(6):374-379. Epub 2018 Aug 1 doi: 10.1016/j.siny.2018.07.005. PMID: 30115546Free PMC Article
Samuels N, van de Graaf RA, de Jonge RCJ, Reiss IKM, Vermeulen MJ
BMC Pediatr 2017 Apr 14;17(1):105. doi: 10.1186/s12887-017-0847-3. PMID: 28410573Free PMC Article
Robinson JR, Rellinger EJ, Hatch LD, Weitkamp JH, Speck KE, Danko M, Blakely ML
Semin Perinatol 2017 Feb;41(1):70-79. Epub 2016 Nov 8 doi: 10.1053/j.semperi.2016.09.020. PMID: 27836422Free PMC Article

Recent systematic reviews

Ferreira RG, Mendonça CR, Gonçalves Ramos LL, de Abreu Tacon FS, Naves do Amaral W, Ruano R
J Matern Fetal Neonatal Med 2022 Dec;35(25):6199-6212. Epub 2021 Apr 25 doi: 10.1080/14767058.2021.1909563. PMID: 33899664
Mitra S, Florez ID, Tamayo ME, Mbuagbaw L, Vanniyasingam T, Veroniki AA, Zea AM, Zhang Y, Sadeghirad B, Thabane L
JAMA 2018 Mar 27;319(12):1221-1238. doi: 10.1001/jama.2018.1896. PMID: 29584842Free PMC Article
Samuels N, van de Graaf RA, de Jonge RCJ, Reiss IKM, Vermeulen MJ
BMC Pediatr 2017 Apr 14;17(1):105. doi: 10.1186/s12887-017-0847-3. PMID: 28410573Free PMC Article
Downes KL, Grantz KL, Shenassa ED
Am J Perinatol 2017 Aug;34(10):935-957. Epub 2017 Mar 22 doi: 10.1055/s-0037-1599149. PMID: 28329897Free PMC Article
Pammi M, Cope J, Tarr PI, Warner BB, Morrow AL, Mai V, Gregory KE, Kroll JS, McMurtry V, Ferris MJ, Engstrand L, Lilja HE, Hollister EB, Versalovic J, Neu J
Microbiome 2017 Mar 9;5(1):31. doi: 10.1186/s40168-017-0248-8. PMID: 28274256Free PMC Article

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