Acute liver failure
Synonyms: Acute hepatic failure · Fulminant hepatic failure
- Type
- Clinical syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly, Infancy
- Status
- active
A rare hepatic disease characterized by acute onset of severe liver dysfunction without evidence of underlying chronic liver disease. Patients present with nonspecific symptoms like jaundice, upper right abdominal pain, nausea, vomiting, pruritus, fatigue, and fever. The condition may rapidly progress to hepatic encephalopathy, coagulopathy, and life-threatening multiorgan failure. Liver biopsy typically shows massive hepatic necrosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acute liver failure | Acute hepatic failure · Fulminant hepatic failure |
| fr | Insuffisance hépatique aiguë | |
| de | Leberversagen, akutes | Leberversagen, fulminantes |
| es | Insuficiencia hepática aguda | Fallo hepático agudo · Fallo hepático fulminante · Insuficiencia hepática fulminante |
| it | Insufficienza epatica acuta | |
| nl | Acuut leverfalen | Acuut hepatisch falen · Fulminant hepatisch falen |
| pt | Insuficiência hepática aguda | |
| pl | Ostra niewydolność wątroby | Piorunująca niewydolność wątroby |
| cs | Akutní selhání jater | Akutní jaterní selhání |
| uk | Гостра печінкова недостатність | Гостра печінкова недостатність · Фульмінантна печінкова недостатність |
| zh | 急性肝功能衰竭 | 暴发性性肝功能衰竭 |
| tr | Akut karaciğer yetmezliği | Akut karaciğer yetmezliği · Fulminan karaciğer yetmezliği |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K72.0 | NTBT | attributed | validated |
| ICD-11 | DB91.1 | NTBT | attributed | validated |
| MONDO | 0019542 | E | validated | |
| MeSH | D017114 | E | validated | |
| UMLS | C0162557 | E | validated | |
| MedDRA | 10000804 | 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:57146Rare hepatic disease
rare hepatic 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 | 20 / 100 000 | Europe | Value and class | Validated | European Medicines Agency[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
50 annotations · validated 2020-03-12| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002910 | Elevated circulating hepatic transaminase concentration | |
| HP:0012115 | Hepatitis | |
| HP:0001404 | Hepatocellular necrosis | |
| HP:0000952 | Jaundice | |
| Frequent (79-30%) | ||
| HP:0002793 | Abnormal pattern of respiration | |
| HP:0002795 | Abnormal respiratory system physiology | |
| HP:0003256 | Abnormality of the coagulation cascade | |
| HP:0000846 | Adrenal insufficiency | |
| HP:0000713 | Agitation | |
| HP:0000978 | Bruising susceptibility | |
| HP:0001289 | Confusion | |
| HP:0002014 | Diarrhea | |
| HP:0002329 | Drowsiness | |
| HP:0000712 | Emotional lability | |
| HP:0002605 | Hepatic necrosis | |
| HP:0002614 | Hepatic periportal necrosis | |
| HP:0001987 | Hyperammonemia | |
| HP:0001943 | Hypoglycemia | |
| HP:0002615 | Hypotension | |
| HP:0030977 | Increased factor VIII activity | |
| HP:0002018 | Nausea | |
| HP:0008151 | Prolonged prothrombin time | |
| HP:0003225 | Reduced coagulation factor V activity | |
| HP:0008169 | Reduced factor VII activity | |
| HP:0008321 | Reduced factor X activity | |
| HP:0001350 | Slurred speech | |
| HP:0001873 | Thrombocytopenia | |
| HP:0002013 | Vomiting | |
| Occasional (29-5%) | ||
| HP:0001892 | Abnormal bleeding | |
| HP:0001941 | Acidosis | |
| HP:0001919 | Acute kidney injury | |
| HP:0001948 | Alkalosis | |
| HP:0001251 | Ataxia | |
| HP:0002181 | Cerebral edema | |
| HP:0001259 | Coma | |
| HP:0002625 | Deep venous thrombosis | |
| HP:0000716 | Depression | |
| HP:0001298 | Encephalopathy | |
| HP:0031844 | Euphoria | |
| HP:0001945 | Fever | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0002883 | Hyperventilation | |
| HP:0012417 | Hypocapnia | |
| HP:0002311 | Incoordination | |
| HP:0002516 | Increased intracranial pressure | |
| HP:0002170 | Intracranial hemorrhage | |
| HP:0007021 | Pain insensitivity | |
| HP:0001250 | Seizure | |
| HP:0031273 | Shock | |
| HP:0000988 | Skin rash | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 30266162[PMID]_30485882[PMID]_27058243[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/90062
- This record as JSON: /data/d/90062.json