ORPHA:243367
Acute fatty liver of pregnancy
Synonyms: AFLP
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial
- Age of onset
- Adult
- Status
- active
A rare, severe complication occurring in the third trimester of pregnancy or in early postpartum period bearing a risk for perinatal and maternal mortality and characterized by jaundice, rise of hepatic injuries and evolving to acute liver failure and encephalopathy.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acute fatty liver of pregnancy | AFLP |
| fr | Stéatose hépatique aiguë gravidique | AFLP · SHAG · Stéatose hépatique aiguë de la grossesse |
| de | Akute Schwangerschaft-Fettleber | |
| es | Hígado graso agudo del embarazo | = en |
| it | Fegato grasso acuto della gravidanza | = en |
| nl | Acute leververvetting tijdens zwangerschap | = en |
| pt | Fígado gordo agudo da gravidez | |
| pl | Ostre stłuszczenie wątroby w ciąży | = en |
| cs | Akutní těhotenská steatóza | = en |
| uk | Гостра жирова дистрофія печінки при вагітності | = en |
| zh | 妊娠期急性脂肪肝 | = en |
| tr | Hamilelikte akut yağlı karaciğer | = en |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | O26.6 | NTBT | attributed | validated |
| ICD-11 | JA65.0 | NTBT | index | validated |
| MONDO | 0016573 | E | validated | |
| MeSH | C537957 | E | validated | |
| UMLS | C1455728 | E | validated | |
| GARD | 9578 | E | validated | |
| MedDRA | 10000746 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:163637Rare disorder related with pregnancy, childbirth and puerperium
rare gynecological and obstetric diseases - ORPHA:101939Rare parenchymal liver disease
rare hepatic diseases
No children.
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Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| HADHA | Disease-causing germline mutation(s) in | Assessed | HGNC:4801 | ENSG00000084754 | 600890 | 2p23.3 | hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha | 28515471[PMID] |
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Prevalence and incidence
0 recordsNo prevalence record in this release.
6
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/243367
- This record as JSON: /data/d/243367.json