Combined malonic and methylmalonic acidemia

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ORPHA:289504OMIM:614265E71.1
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Overview

Combined malonic and methylmalonic acidemia (CMAMMA) is a rare inherited metabolic disorder in which the body cannot properly break down certain fats and proteins. This leads to a buildup of two specific acids — malonic acid and methylmalonic acid — in the blood and urine. The condition is caused by mutations in the ACSF3 gene, which provides instructions for making an enzyme needed in an important energy-producing pathway inside cells called the mitochondria. Symptoms of CMAMMA can vary widely, even among people in the same family. Some individuals are diagnosed in infancy or early childhood with feeding difficulties, failure to thrive, low blood sugar, seizures, and developmental delays. Others may not show symptoms until later in childhood or even adulthood, when they may experience fatigue, memory and concentration problems, psychiatric symptoms like depression or anxiety, and neurological issues such as movement difficulties. During times of illness or fasting, symptoms can worsen significantly, sometimes leading to a metabolic crisis. CMAmMA has historically been underdiagnosed because standard newborn screening for methylmalonic acidemia may not always detect it. Treatment is mainly supportive and focuses on preventing metabolic crises through dietary management, avoiding prolonged fasting, and sometimes supplementing with specific vitamins or nutrients. There is currently no cure, but early diagnosis and careful management can help improve quality of life and reduce the risk of serious complications.

Also known as:

Key symptoms:

Fatigue and low energyFailure to thrive in infancyFeeding difficultiesLow blood sugar (hypoglycemia)SeizuresDevelopmental delaysMemory and concentration problemsDepression or anxietyMovement difficulties or clumsinessMuscle weaknessEpisodes of metabolic crisis during illnessLearning difficultiesSpeech delaysNausea and vomitingIrritability

Clinical phenotype terms (26)— hover any for plain English
Methylmalonic acidemiaHP:0002912Dicarboxylic aciduriaHP:0003215Methylmalonic aciduriaHP:0012120Dicarboxylic acidemiaHP:0040145AcidosisHP:0001941KetoacidosisHP:0001993Intermittent diarrheaHP:0002254
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Combined malonic and methylmalonic acidemia.

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No actively recruiting trials found for Combined malonic and methylmalonic acidemia at this time.

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No specialists are currently listed for Combined malonic and methylmalonic acidemia.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Combined malonic and methylmalonic acidemia.

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Community

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Caregiver Resources

NORD Caregiver Resources

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Mental Health Support

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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.How severe is my (or my child's) form of CMAMMA, and what symptoms should I watch for?,What dietary plan should we follow, and do we need to see a metabolic dietitian?,What should we do during illness to prevent a metabolic crisis?,Should we carry an emergency letter, and what should it say?,Are there any supplements like carnitine that might help?,How often should we have follow-up appointments and lab tests?,Are there any clinical trials or new treatments being studied for CMAMMA?

Common questions about Combined malonic and methylmalonic acidemia

What is Combined malonic and methylmalonic acidemia?

Combined malonic and methylmalonic acidemia (CMAMMA) is a rare inherited metabolic disorder in which the body cannot properly break down certain fats and proteins. This leads to a buildup of two specific acids — malonic acid and methylmalonic acid — in the blood and urine. The condition is caused by mutations in the ACSF3 gene, which provides instructions for making an enzyme needed in an important energy-producing pathway inside cells called the mitochondria. Symptoms of CMAMMA can vary widely, even among people in the same family. Some individuals are diagnosed in infancy or early childhood

How is Combined malonic and methylmalonic acidemia inherited?

Combined malonic and methylmalonic acidemia follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.