Overview
Transient neonatal multiple acyl-CoA dehydrogenase deficiency (also called transient neonatal MADD or transient glutaric aciduria type II) is a rare metabolic condition that affects newborn babies. It belongs to a group of disorders known as fatty acid oxidation defects. In this condition, the baby's body temporarily has trouble breaking down fats and certain amino acids (building blocks of protein) for energy. This happens because enzymes that normally help with these processes do not work properly during the first days to weeks of life. Babies with this condition may show symptoms shortly after birth, including low blood sugar (hypoglycemia), low muscle tone (floppiness), poor feeding, a distinctive sweaty-feet odor, and metabolic acidosis (too much acid in the blood). In some cases, the heart or liver may also be affected. What makes this form unique compared to permanent MADD is that the enzyme problems improve over time, often within weeks to months, and the baby can recover. The exact cause of the transient form is not always fully understood. In some cases, it may be related to the mother's riboflavin (vitamin B2) status or to temporary immaturity of the baby's metabolic pathways. Treatment typically involves riboflavin supplementation, careful management of feeding and blood sugar, and sometimes carnitine supplementation. With early recognition and supportive care, the outlook for most affected babies is generally good, as the metabolic abnormalities tend to resolve. However, prompt treatment during the acute phase is critical to prevent serious complications.
Also known as:
Key symptoms:
Low blood sugar (hypoglycemia)Poor feeding or difficulty feedingLow muscle tone (floppiness)Unusual sweaty-feet body odorMetabolic acidosis (too much acid in the blood)Lethargy or excessive sleepinessVomitingEnlarged liverHeart problems (cardiomyopathy)Breathing difficultiesDehydrationFailure to gain weight
Variable
Can be inherited in different ways depending on the underlying gene
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Transient neonatal multiple acyl-CoA dehydrogenase deficiency.
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Specialists
View all specialists →No specialists are currently listed for Transient neonatal multiple acyl-CoA dehydrogenase deficiency.
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 neonatal multiple acyl-CoA dehydrogenase deficiency.
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Caregiver Resources
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Questions for your doctor
Bring these to your next appointment
- Q1.How can you be sure this is the transient form and not permanent MADD?,How long will my baby need riboflavin and carnitine supplements?,What signs should I watch for that would mean I need to bring my baby to the emergency room?,How often will my baby need follow-up blood tests to check if the condition is resolving?,Are there any long-term effects I should watch for as my child grows?,Should I be tested for riboflavin deficiency or carrier status?,Will future pregnancies be at risk for this same condition?
Common questions about Transient neonatal multiple acyl-CoA dehydrogenase deficiency
What is Transient neonatal multiple acyl-CoA dehydrogenase deficiency?
Transient neonatal multiple acyl-CoA dehydrogenase deficiency (also called transient neonatal MADD or transient glutaric aciduria type II) is a rare metabolic condition that affects newborn babies. It belongs to a group of disorders known as fatty acid oxidation defects. In this condition, the baby's body temporarily has trouble breaking down fats and certain amino acids (building blocks of protein) for energy. This happens because enzymes that normally help with these processes do not work properly during the first days to weeks of life. Babies with this condition may show symptoms shortly a
At what age does Transient neonatal multiple acyl-CoA dehydrogenase deficiency typically begin?
Typical onset of Transient neonatal multiple acyl-CoA dehydrogenase deficiency is neonatal. Age of onset can vary across affected individuals.