Overview
Transient infantile hypertriglyceridemia and hepatosteatosis (sometimes abbreviated as TIHHS) is a rare metabolic condition that affects babies during infancy. In this condition, infants develop very high levels of triglycerides (a type of fat) in their blood, along with fatty liver (hepatosteatosis), meaning fat builds up in the liver. The word 'transient' is key — this condition is temporary and typically resolves on its own as the child grows, usually by the time they are a toddler or in early childhood. The condition is caused by genetic changes that temporarily affect how the body processes fats during infancy. Babies with this condition may have an enlarged liver, elevated liver enzymes found on blood tests, and very high triglyceride levels that can sometimes be detected because the blood appears milky or creamy (lipemic). Some infants may also show poor feeding, failure to thrive, or abdominal swelling. Because the condition resolves with time, treatment is mainly supportive. Doctors may recommend dietary modifications, such as using medium-chain triglyceride (MCT)-enriched formulas, to help manage fat levels while the infant's metabolism matures. Close monitoring of blood fat levels and liver function is important. The long-term outlook is generally very good, as most children outgrow the condition completely without lasting liver damage or metabolic problems.
Also known as:
Key symptoms:
Very high triglyceride (fat) levels in the bloodFatty liver (fat buildup in the liver)Enlarged liverElevated liver enzymes on blood testsMilky or creamy-looking bloodAbdominal swelling or distensionPoor feeding or difficulty feedingFailure to thrive or poor weight gainIrritabilityPale or fatty stools
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
FDA & Trial Timeline
2 eventsZirgan: FDA approved
Treatment of acute herpetic keratitis (dendritic ulcers)
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableZirgan
Treatment of acute herpetic keratitis (dendritic ulcers)
Clinical Trials
View all trials with filters →No actively recruiting trials found for Transient infantile hypertriglyceridemia and hepatosteatosis at this time.
New trials open frequently. Follow this disease to get notified.
Specialists
View all specialists →No specialists are currently listed for Transient infantile hypertriglyceridemia and hepatosteatosis.
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 Transient infantile hypertriglyceridemia and hepatosteatosis.
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Caregiver Resources
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Family & Caregiver Grants
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Social Security Disability
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Questions for your doctor
Bring these to your next appointment
- Q1.How high are my baby's triglyceride levels, and how often will they be monitored?,What type of formula or diet should my baby be on, and for how long?,How will we know when the condition is resolving?,Are there any signs I should watch for at home that would need urgent medical attention?,Does my baby need a liver ultrasound, and how often should it be repeated?,Should my other children or future children be tested for this condition?,Are there any long-term effects I should be aware of even after the condition resolves?
Common questions about Transient infantile hypertriglyceridemia and hepatosteatosis
What is Transient infantile hypertriglyceridemia and hepatosteatosis?
Transient infantile hypertriglyceridemia and hepatosteatosis (sometimes abbreviated as TIHHS) is a rare metabolic condition that affects babies during infancy. In this condition, infants develop very high levels of triglycerides (a type of fat) in their blood, along with fatty liver (hepatosteatosis), meaning fat builds up in the liver. The word 'transient' is key — this condition is temporary and typically resolves on its own as the child grows, usually by the time they are a toddler or in early childhood. The condition is caused by genetic changes that temporarily affect how the body proces
How is Transient infantile hypertriglyceridemia and hepatosteatosis inherited?
Transient infantile hypertriglyceridemia and hepatosteatosis follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Transient infantile hypertriglyceridemia and hepatosteatosis typically begin?
Typical onset of Transient infantile hypertriglyceridemia and hepatosteatosis is infantile. Age of onset can vary across affected individuals.