Necrotizing enterocolitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Neonatal
- Status
- active
A rare intestinal disease characterized by potentially life-threatening inflammatory bowel necrosis predominantly affecting preterm neonates. Patients may present with feeding intolerance, lethargy, temperature instability, abdominal distention, blood-stained stools, diarrhea, bilious vomiting, apnea, and signs of sepsis. Radiographic features include pneumatosis intestinalis, portal venous gas, presence of fixed, dilated intestinal loops, bowel wall edema, and (in case of bowel perforation) pneumoperitoneum.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Necrotizing enterocolitis | |
| fr | Entérocolite nécrosante | |
| de | Nekrotisierende Enterokolitis | |
| es | Enterocolitis necrotizante | |
| it | Enterocolite necrotizzante | |
| nl | Necrotiserende enterocolitis | |
| pt | Enterocolite necrotizante | |
| pl | Martwicze zapalenie jelit | |
| cs | Nekrotizující enterokolitida | |
| uk | Некротичний ентероколіт | |
| zh | 坏死性小肠结肠炎 | |
| tr | Nekrotizan enterokolit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | P77 | E | specific | validated |
| ICD-11 | KB88 | E | specific | validated |
| MONDO | 0005313 | E | validated | |
| MeSH | D020345 | E | validated | |
| UMLS | C0520459 | E | validated | |
| GARD | 9767 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:117569Rare intestinal disease
rare gastroenterological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 45 / 100 000 | Worldwide | Value and class | Validated | European Medicines Agency 2015[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
34 annotations · validated 2020-03-17| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001622 | Premature birth | |
| Frequent (79-30%) | ||
| HP:0003270 | Abdominal distention | |
| HP:0025085 | Bloody diarrhea | |
| HP:0000961 | Cyanosis | |
| HP:0001875 | Decreased total neutrophil count | |
| HP:0002014 | Diarrhea | |
| HP:0012378 | Fatigue | |
| HP:0012537 | Food intolerance | |
| HP:0030144 | Hypoactive bowel sounds | |
| HP:0002902 | Hyponatremia | |
| HP:0001974 | Increased total leukocyte count | |
| HP:0004396 | Poor appetite | |
| HP:0001518 | Small for gestational age | |
| Occasional (29-5%) | ||
| HP:0032545 | Abdominal rigidity | |
| HP:0011014 | Abnormal glucose homeostasis | |
| HP:0001627 | Abnormal heart morphology | |
| HP:0001941 | Acidosis | |
| HP:0002104 | Apnea | |
| HP:0001541 | Ascites | |
| HP:0001662 | Bradycardia | |
| HP:0005521 | Disseminated intravascular coagulation | |
| HP:0000969 | Edema | |
| HP:0001543 | Gastroschisis | |
| HP:0003074 | Hyperglycemia | |
| HP:0002615 | Hypotension | |
| HP:0002151 | Increased circulating lactate concentration | |
| HP:0001254 | Lethargy | |
| HP:0001942 | Metabolic acidosis | |
| HP:0040187 | Neonatal sepsis | |
| HP:0002586 | Peritonitis | |
| HP:0031273 | Shock | |
| HP:0005968 | Temperature instability | |
| HP:0001873 | Thrombocytopenia | |
| HP:0002013 | Vomiting | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 30793842[PMID]_30691547[PMID]_29196510[PMID]_30020729[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/391673
- This record as JSON: /data/d/391673.json