Zollinger-Ellison syndrome
Synonyms: Gastrinoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare neuroendocrine neoplasm (NEN) characterized by severe peptic disease (ulcers/esophageal disease) caused by hypergastrinemia secondary to a gastrin-secreting NEN (gastrinoma) resulting in increased gastric acid secretion.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Zollinger-Ellison syndrome | Gastrinoma |
| fr | Syndrome de Zollinger-Ellison | Gastrinome |
| de | Zollinger-Ellison-Syndrom | Gastrinom |
| es | Síndrome de Zollinger-Ellison | = en |
| it | Sindrome di Zollinger-Ellison | = en |
| nl | Syndroom van Zollinger-Ellison | Gastrinoom |
| pt | Síndrome Zollinger-Ellison | = en |
| pl | Zespół Zollingera i Ellisona | = en |
| cs | Zollingerův-Ellisonův syndrom | Gastrinom · Zollinger-Ellisonův syndrom |
| uk | Синдром Золлінгера-Еллісона | Гастринома |
| zh | Zollinger-Ellison 综合征 | 胃泌素瘤 |
| tr | Zollinger-Ellison sendromu | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E16.4 | NTBT | inclusion | validated |
| ICD-11 | 5A43.1 | E | specific | validated |
| MONDO | 0019610 | E | validated | |
| MeSH | D015043 | E | validated | |
| UMLS | C0043515 | E | validated | |
| GARD | 7918 | E | validated | |
| MedDRA | 10017852 | 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 (2):
- ORPHA:100076Duodenal neuroendocrine tumor
rare gastroenterological diseases; rare endocrine diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:506060Functioning neuroendocrine tumor of pancreas
rare gastroenterological diseases; rare endocrine diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
6 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.15 / 100 000 | Europe | Value and class | Validated | [EXPERT] |
| Point prevalence | 1-9 / 100 000 | — | Europe | Class only | Validated | [EXPERT] |
| Annual incidence | 1-9 / 1 000 000 | 0.15 / 100 000 | Denmark | Value and class | Validated | ISBN:0849359937[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.3 / 100 000 | Switzerland | Value and class | Not yet validated | ISBN:0849359937[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.05 / 100 000 | Ireland | Value and class | Validated | 2575263[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.125 / 100 000 | Worldwide | Value and class | Validated | 22261919[PMID] |
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 2017-01-25| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0100634 | Neuroendocrine neoplasm | |
| HP:0002044 | Zollinger-Ellison syndrome | |
| Very frequent (99-80%) | ||
| HP:0002014 | Diarrhea | |
| HP:0002588 | Duodenal ulcer | |
| HP:0002574 | Episodic abdominal pain | |
| HP:0100633 | Esophagitis | |
| HP:0002018 | Nausea | |
| HP:0004398 | Peptic ulcer | |
| Frequent (79-30%) | ||
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0008256 | Adrenocortical adenoma | |
| HP:0000845 | Elevated circulating growth hormone concentration | |
| HP:0003165 | Elevated circulating parathyroid hormone level | |
| HP:0010783 | Erythema | |
| HP:0012334 | Extrahepatic cholestasis | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0030404 | Glucagonoma | |
| HP:0002573 | Hematochezia | |
| HP:0003072 | Hypercalcemia | |
| HP:0000843 | Hyperparathyroidism | |
| HP:0003118 | Increased circulating cortisol level | |
| HP:0030688 | Increased glucagon level | |
| HP:0012030 | Increased urinary cortisol level | |
| HP:0005214 | Intestinal obstruction | |
| HP:0000952 | Jaundice | |
| HP:0012032 | Lipoma | |
| HP:0001012 | Multiple lipomas | |
| HP:0008208 | Parathyroid hyperplasia | |
| HP:0002893 | Pituitary adenoma | |
| HP:0008291 | Pituitary corticotropic cell adenoma | |
| HP:0011760 | Pituitary growth hormone cell adenoma | |
| HP:0011761 | Pituitary null cell adenoma | |
| HP:0006767 | Pituitary prolactin cell adenoma | |
| HP:0000854 | Thyroid adenoma | |
| Very rare (<4-1%) | ||
| HP:0006744 | Adrenocortical carcinoma | |
Run the differential on the 8 obligate / very frequent terms
Annotation source: 23582916[PMID]_22052063[PMID]_23645327[PMID]_21443889[PMID]_21315717[PMID]_19854317[PMID]_19836486[PMID]_18270260[PMID]_19059523[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/913
- This record as JSON: /data/d/913.json