OBSOLETE: Acrodysostosis with multiple hormone resistance

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ORPHA:280651
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Overview

Acrodysostosis with multiple hormone resistance is a rare genetic condition that affects bone development and the body's ability to respond to certain hormones. This entry is now considered obsolete in medical databases because the condition has been reclassified and is now recognized as Acrodysostosis type 1 (caused by changes in the PRKAR1A gene). People with this condition typically have shortened bones in the hands and feet (brachydactyly), a flattened nose with a small upturned tip, and short stature. A key feature is that the body does not respond properly to several hormones, most notably parathyroid hormone (PTH) and thyroid-stimulating hormone (TSH). This hormone resistance can lead to low calcium levels, high phosphorus levels, and underactive thyroid function. Many affected individuals also experience some degree of intellectual disability or developmental delay. Facial features may include a broad, flat face and widely spaced eyes. The condition is present from birth, and skeletal abnormalities can often be seen on X-rays early in life. Treatment focuses on managing hormone imbalances with replacement therapy and supporting development through early intervention programs. There is no cure, but with proper medical management, many symptoms can be effectively controlled.

Key symptoms:

Short fingers and toesShort statureFlattened nasal bridge with small upturned noseBroad flat faceResistance to parathyroid hormone causing calcium problemsUnderactive thyroid due to hormone resistanceIntellectual disability or learning difficultiesDevelopmental delayAdvanced bone age on X-raysSmall hands and feetWidely spaced eyesDental problemsHearing difficultiesObesity or weight gain

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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Acrodysostosis with multiple hormone resistance.

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No actively recruiting trials found for OBSOLETE: Acrodysostosis with multiple hormone resistance at this time.

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No specialists are currently listed for OBSOLETE: Acrodysostosis with multiple hormone resistance.

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

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Questions for your doctor

Bring these to your next appointment

  • Q1.Which hormones are affected in my child, and how will they be monitored over time?,What calcium and vitamin D supplements are needed, and how often should blood levels be checked?,Is thyroid hormone replacement needed, and what are the signs it may need adjusting?,What developmental therapies would benefit my child, and when should they start?,Should we consider growth hormone therapy, and what are the expected benefits and risks?,How often should X-rays or bone age assessments be done?,Are there any clinical trials or new research studies we should know about?

Common questions about OBSOLETE: Acrodysostosis with multiple hormone resistance

What is OBSOLETE: Acrodysostosis with multiple hormone resistance?

Acrodysostosis with multiple hormone resistance is a rare genetic condition that affects bone development and the body's ability to respond to certain hormones. This entry is now considered obsolete in medical databases because the condition has been reclassified and is now recognized as Acrodysostosis type 1 (caused by changes in the PRKAR1A gene). People with this condition typically have shortened bones in the hands and feet (brachydactyly), a flattened nose with a small upturned tip, and short stature. A key feature is that the body does not respond properly to several hormones, most notab

How is OBSOLETE: Acrodysostosis with multiple hormone resistance inherited?

OBSOLETE: Acrodysostosis with multiple hormone resistance follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does OBSOLETE: Acrodysostosis with multiple hormone resistance typically begin?

Typical onset of OBSOLETE: Acrodysostosis with multiple hormone resistance is neonatal. Age of onset can vary across affected individuals.