Albright hereditary osteodystrophy

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3Active trials21Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Albright hereditary osteodystrophy (AHO) is a rare genetic disorder characterized by a distinctive constellation of physical features including short stature, obesity, round face, subcutaneous ossifications (calcium deposits under the skin), brachydactyly (shortening of the fingers and toes, particularly the fourth and fifth metacarpals), and often mild intellectual disability. The condition is caused by heterozygous loss-of-function mutations in the GNAS gene, which encodes the alpha subunit of the stimulatory G protein (Gsα), a critical signaling molecule involved in the response of many tissues to hormones. AHO can present in two distinct clinical forms depending on the parental origin of the mutation. When the GNAS mutation is inherited from the mother, patients develop pseudohypoparathyroidism type 1A (PHP1A), which includes the AHO phenotype along with multi-hormone resistance — most notably resistance to parathyroid hormone (PTH), leading to hypocalcemia and hyperphosphatemia, as well as resistance to thyroid-stimulating hormone (TSH) and gonadotropins. When the same mutation is inherited from the father, patients develop pseudopseudohypoparathyroidism (PPHP), in which the AHO physical features are present but without hormone resistance. This parent-of-origin effect is due to tissue-specific genomic imprinting of the GNAS locus. Treatment of AHO is primarily supportive and directed at managing specific manifestations. For patients with PHP1A, calcium and active vitamin D supplementation are essential to correct hypocalcemia and suppress elevated PTH levels. Thyroid hormone replacement is used for TSH resistance. Growth hormone therapy may be considered for short stature. Regular monitoring of calcium, phosphorus, PTH, and thyroid function is important. Genetic counseling is recommended for affected families given the autosomal dominant inheritance pattern and the significant impact of parental imprinting on clinical expression.

Inheritance

Autosomal dominant

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

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗NORD ↗

FDA & Trial Timeline

3 events
Jul 2020Theophylline Treatment for Pseudohypoparathyroidism - Children 2-12 Years Old

Vanderbilt University Medical Center — PHASE2

TrialACTIVE NOT RECRUITING
Sep 2018Theophylline Treatment for Pseudohypoparathyroidism

Vanderbilt University Medical Center — PHASE2

TrialACTIVE NOT RECRUITING
Jan 2003Albright Hereditary Osteodystrophy: Natural History, Growth, and Cognitive/Behavioral Assessments

Connecticut Children's Medical Center — NA

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Albright hereditary osteodystrophy.

3 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

3 recruitingView all trials with filters →
Phase 22 trials
Theophylline Treatment for Pseudohypoparathyroidism - Children 2-12 Years Old
Phase 2
Active
PI: Ashley Shoemaker, MD (Vanderbilt University Medical Center) · Sites: Nashville, Tennessee · Age: 212 yrs
Theophylline Treatment for Pseudohypoparathyroidism
Phase 2
Active
PI: Ashley Shoemaker, MD (Vanderbilt University Medical Center) · Sites: Nashville, Tennessee · Age: 1399 yrs
N/A1 trial
Albright Hereditary Osteodystrophy: Natural History, Growth, and Cognitive/Behavioral Assessments
N/A
Actively Recruiting
PI: Emily L Germain-Lee, MD (Connecticut Children's Medical Ctr. and Univ. of C) · Sites: Hartford, Connecticut · Age: 089 yrs

Specialists

21 foundView all specialists →
PM
Patrick McMullan
JACKSONVILLE, FL
Specialist
4 Albright hereditary osteodystrophy publications
NH
Nasrin Hakimzada
Specialist
1 Albright hereditary osteodystrophy publication
NS
Nijatullah Safi
Specialist
1 Albright hereditary osteodystrophy publication
SA
Sultan Mahmood Alikozai
Specialist
1 Albright hereditary osteodystrophy publication
AR
Abdul Jamil Rasooli
Specialist
1 Albright hereditary osteodystrophy publication
TJ
Tooryalai Jalalzai
Specialist
1 Albright hereditary osteodystrophy publication
QS
Qais Siddiqui
VALLEY STREAM, NY
Specialist
1 Albright hereditary osteodystrophy publication
EG
Emily L Germain-Lee
FARMINGTON, CT
Specialist
4 Albright hereditary osteodystrophy publications
PM
Peter Maye
Specialist
3 Albright hereditary osteodystrophy publications
QY
Qingfen Yang
Specialist
3 Albright hereditary osteodystrophy publications
SR
Sierra H Root
Specialist
2 Albright hereditary osteodystrophy publications
DR
David Rowe
Specialist
2 Albright hereditary osteodystrophy publications
IK
Ivo Kalajzic
Specialist
2 Albright hereditary osteodystrophy publications
SN
Sahar Noor
Specialist
1 Albright hereditary osteodystrophy publication
AM
Ashley Shoemaker, MD
Nashville, Tennessee
Specialist

Rare Disease Specialist

PI on 4 active trials
SE
Sarah Edie
Specialist
2 Albright hereditary osteodystrophy publications

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 Albright hereditary osteodystrophy.

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Community

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Latest news about Albright hereditary osteodystrophy

Disease timeline:

New recruiting trial: Effect of Theophylline in Pseudohypoparathyroidism

A new clinical trial is recruiting patients for Albright hereditary osteodystrophy

New recruiting trial: Albright Hereditary Osteodystrophy: Natural History, Growth, and Cognitive/Behavioral Assessments

A new clinical trial is recruiting patients for Albright hereditary osteodystrophy

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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.

Common questions about Albright hereditary osteodystrophy

What is Albright hereditary osteodystrophy?

Albright hereditary osteodystrophy (AHO) is a rare genetic disorder characterized by a distinctive constellation of physical features including short stature, obesity, round face, subcutaneous ossifications (calcium deposits under the skin), brachydactyly (shortening of the fingers and toes, particularly the fourth and fifth metacarpals), and often mild intellectual disability. The condition is caused by heterozygous loss-of-function mutations in the GNAS gene, which encodes the alpha subunit of the stimulatory G protein (Gsα), a critical signaling molecule involved in the response of many tis

How is Albright hereditary osteodystrophy inherited?

Albright hereditary osteodystrophy follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Albright hereditary osteodystrophy typically begin?

Typical onset of Albright hereditary osteodystrophy is childhood. Age of onset can vary across affected individuals.

Are there clinical trials for Albright hereditary osteodystrophy?

Yes — 3 recruiting clinical trials are currently listed for Albright hereditary osteodystrophy on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Albright hereditary osteodystrophy?

21 specialists and care centers treating Albright hereditary osteodystrophy are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.