raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:284

Alveolar echinococcosis

Synonyms: Echinococcus multilocularis infection

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Adult, Elderly
Status
active

A rare parasitic disorder that occurs after ingestion of eggs of <i>Echinococcus multilocularis</i> and characterized by an initial asymptomatic incubation period of many years followed by a chronic course where the clinical manifestations include epigastric pain and jaundice.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAlveolar echinococcosisEchinococcus multilocularis infection
frEchinococcose alvéolaireInfection à l'echinococcus multilocularis
deEchinokokkose, alveoläreEchinococcus multilocularis-Infektion
esEquinococosis alveolarInfección por Echinococcus multilocularis
itEchinococcosi alveolare
nlAlveolaire echinokokkoseInfectie door Echinococcus multilocularis
ptEquinococose alveolar
plEchinokokoza pęcherzykowaZakażenie tasiemcem wielojamowym
csAlveolární echinokokóza= en
ukАльвеолярний ехінококозІнфекція Echinococcus multilocularis
zh泡状棘球蚴病多房棘球绦虫感染
trAlveoler ekinokokkozEchinococcus multilocularis enfeksiyonu

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10B67.5BTNTspecificvalidated
ICD-10B67.6BTNTspecificvalidated
ICD-10B67.7BTNTspecificvalidated
ICD-111F73.YNTBTindexvalidated
MONDO0017282Evalidated
MeSHC536591Evalidated
UMLSC0152069Evalidated
GARD207Evalidated
MedDRA10053042Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

6 records
TypeClassValueAreaQualificationValidationSource
Annual incidence1-9 / 1 000 0000.16 / 100 000EuropeValue and classValidated23301116[PMID]_ORPHANET
Annual incidence1-9 / 1 000 0000.26 / 100 000SwitzerlandValue and classValidated17553227[PMID]
Annual incidence<1 / 1 000 0000.014 / 100 000PolandValue and classValidated23301116[PMID]
Annual incidence1-9 / 100 0002.8 / 100 000AustriaValue and classValidated23628138[PMID]
Annual incidence<1 / 1 000 0000.026 / 100 000FranceValue and classValidatedFrancEchino[OTHER]_OTHERitut de Veille Sanitaire 2010[OTHER]
Point prevalenceUnknown—EuropeClass onlyNot yet validatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

50 annotations · validated 2020-11-24
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0003565Elevated erythrocyte sedimentation rate
HP:0001407Hepatic cysts
HP:0010702Increased circulating immunoglobulin concentration
HP:0001880Increased total eosinophil count
Frequent (79-30%)
HP:0002027Abdominal pain
HP:0001903Anemia
HP:0025406Asthenia
HP:0001410Decreased liver function
HP:0410019Epigastric pain
HP:0012378Fatigue
HP:0001945Fever
HP:0000952Jaundice
HP:0003419Low back pain
HP:0010741Pedal edema
Occasional (29-5%)
HP:0025408Abnormal spleen morphology
HP:0000766Abnormal sternum morphology
HP:0011732Abnormality of adrenal morphology
HP:0100749Chest pain
HP:0030151Cholangitis
HP:0012735Cough
HP:0031292Cutaneous abscess
HP:0002094Dyspnea
HP:0010766Ectopic calcification
HP:0002315Headache
HP:0002105Hemoptysis
HP:0032445Pulmonary cyst
HP:0000107Renal cyst
HP:0001824Weight loss
Very rare (<4-1%)
HP:0040163Abnormal pelvis bone morphology
HP:0001697Abnormal pericardium morphology
HP:0011805Abnormal skeletal muscle morphology
HP:0003468Abnormal vertebral morphology
HP:0025487Abnormality of bladder morphology
HP:0100016Abnormality of mesentery morphology
HP:0000775Abnormality of the diaphragm
HP:0002381Aphasia
HP:0001251Ataxia
HP:0002613Biliary cirrhosis
HP:0012062Bone cyst
HP:0002639Budd-Chiari syndrome
HP:0001260Dysarthria
HP:0001269Hemiparesis
HP:0002516Increased intracranial pressure
HP:0010576Intracranial cystic lesion
HP:0100523Liver abscess
HP:0001737Pancreatic cysts
HP:0001409Portal hypertension
HP:0001250Seizure
HP:0002321Vertigo
HP:0002013Vomiting

Run the differential on the 4 obligate / very frequent terms

Annotation source: 27082120[PMID]_29564733[PMID]_20683265[PMID]

7

Sources for this card