Overview
Fever-associated acute infantile liver failure syndrome (also known as FIALF or infantile liver failure syndrome type 2) is a very rare and serious genetic condition that affects the liver in young children, typically triggered by a common fever or viral illness. In this condition, the liver suddenly stops working properly when a baby or young child develops a fever, even from a mild infection. The liver is a vital organ that helps the body process nutrients, remove toxins, and make important proteins for blood clotting. When the liver fails, toxins can build up in the body, bleeding problems can occur, and other organs may be affected. The disease is caused by mutations in the NBAS gene, which plays a role in transporting proteins within cells. Children with this condition may experience repeated episodes of acute liver failure during febrile illnesses, especially in the first few years of life. Symptoms during an episode include yellowing of the skin and eyes (jaundice), vomiting, lethargy, poor feeding, and abnormal bleeding. Between episodes, liver function may return to normal or near-normal in many cases. Treatment is mainly supportive and focuses on managing liver failure episodes aggressively, including early fever control, intravenous fluids, glucose supplementation, and sometimes intensive care. In severe cases, liver transplantation may be considered. Early recognition and prompt treatment of febrile episodes are critical to improving outcomes. Some children also have additional features such as short stature, skeletal abnormalities, and immune system differences.
Key symptoms:
Sudden liver failure triggered by fever or infectionYellowing of the skin and eyes (jaundice)VomitingExtreme tiredness or lethargyPoor feeding or refusal to eatAbnormal bleeding or easy bruisingLow blood sugarSwelling of the liverShort stature or growth problemsSkeletal abnormalitiesRecurrent episodes of liver crisisElevated liver enzymes in blood testsReduced alertness or confusion during episodesImmune system problems or frequent infections
Autosomal recessive
Passed on when both parents carry the same gene change; often skips generations
Infantile
Begins in infancy, roughly 1 month to 2 years old
Treatments
No FDA-approved treatments are currently listed for Fever-associated acute infantile liver failure syndrome.
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Specialists
View all specialists →No specialists are currently listed for Fever-associated acute infantile liver failure syndrome.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Fever-associated acute infantile liver failure syndrome.
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Caregiver Resources
NORD Caregiver Resources
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Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What should I do at the very first sign of a fever in my child?,What are the specific warning signs that mean I should go to the emergency room?,How often should my child have liver function tests and other monitoring?,Are there any medications or supplements my child should avoid?,Should my child receive any special vaccinations or avoid certain vaccines?,At what point would liver transplantation be considered?,Are there any clinical trials or new treatments being studied for this condition?
Common questions about Fever-associated acute infantile liver failure syndrome
What is Fever-associated acute infantile liver failure syndrome?
Fever-associated acute infantile liver failure syndrome (also known as FIALF or infantile liver failure syndrome type 2) is a very rare and serious genetic condition that affects the liver in young children, typically triggered by a common fever or viral illness. In this condition, the liver suddenly stops working properly when a baby or young child develops a fever, even from a mild infection. The liver is a vital organ that helps the body process nutrients, remove toxins, and make important proteins for blood clotting. When the liver fails, toxins can build up in the body, bleeding problems
How is Fever-associated acute infantile liver failure syndrome inherited?
Fever-associated acute infantile liver failure syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Fever-associated acute infantile liver failure syndrome typically begin?
Typical onset of Fever-associated acute infantile liver failure syndrome is infantile. Age of onset can vary across affected individuals.