Low oxygen affinity gamma chain hemoglobin disease

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ORPHA:280615OMIM:613977D58.2
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Overview

Low oxygen affinity gamma chain hemoglobin disease is a rare blood condition caused by changes (mutations) in the gamma-globin genes, which produce a type of hemoglobin found mainly in newborns called fetal hemoglobin (hemoglobin F). In this condition, the fetal hemoglobin releases oxygen to the body's tissues more easily than normal — meaning it has a lower than usual attraction (affinity) for oxygen. Fetal hemoglobin is the main oxygen-carrying protein during pregnancy and in the first months of life, and it is gradually replaced by adult hemoglobin during infancy. Because the abnormal gamma chain hemoglobin lets go of oxygen too readily, the body may appear to have lower oxygen levels on certain measurements, even though tissues are actually receiving adequate oxygen. Newborns with this condition may appear bluish (cyanotic), which can be alarming. However, because fetal hemoglobin is naturally replaced by adult hemoglobin within the first several months of life, symptoms typically resolve on their own as the baby grows. This condition is generally considered benign and self-limiting. Most affected infants do not require specific treatment. The main challenge is distinguishing this condition from more serious causes of cyanosis in newborns, such as heart defects or lung problems. Accurate diagnosis through hemoglobin analysis and genetic testing can prevent unnecessary invasive procedures and provide reassurance to families.

Key symptoms:

Bluish skin color (cyanosis) in newbornsLow oxygen saturation readings on pulse oximetryGrayish or dusky skin toneApparent low blood oxygen levels that do not match the baby's overall healthSymptoms that gradually improve over the first few months of life

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Low oxygen affinity gamma chain hemoglobin disease.

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No actively recruiting trials found for Low oxygen affinity gamma chain hemoglobin disease at this time.

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No specialists are currently listed for Low oxygen affinity gamma chain hemoglobin disease.

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

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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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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.How can you be sure my baby's cyanosis is from this hemoglobin condition and not from a heart or lung problem?,How long will it take for my baby's skin color to return to normal?,What follow-up tests will be needed to track my baby's hemoglobin levels?,Is there any risk of long-term health problems from this condition?,Could my future children also have this condition?,Should other family members be tested for this hemoglobin variant?,Are there any warning signs I should watch for at home that would require emergency care?

Common questions about Low oxygen affinity gamma chain hemoglobin disease

What is Low oxygen affinity gamma chain hemoglobin disease?

Low oxygen affinity gamma chain hemoglobin disease is a rare blood condition caused by changes (mutations) in the gamma-globin genes, which produce a type of hemoglobin found mainly in newborns called fetal hemoglobin (hemoglobin F). In this condition, the fetal hemoglobin releases oxygen to the body's tissues more easily than normal — meaning it has a lower than usual attraction (affinity) for oxygen. Fetal hemoglobin is the main oxygen-carrying protein during pregnancy and in the first months of life, and it is gradually replaced by adult hemoglobin during infancy. Because the abnormal gamm

How is Low oxygen affinity gamma chain hemoglobin disease inherited?

Low oxygen affinity gamma chain hemoglobin disease follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Low oxygen affinity gamma chain hemoglobin disease typically begin?

Typical onset of Low oxygen affinity gamma chain hemoglobin disease is neonatal. Age of onset can vary across affected individuals.