OBSOLETE: X-linked congenital disorder of glycosylation with intellectual disability as a major feature

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ORPHA:371054
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Overview

This entry refers to a group of rare genetic conditions that are now classified under more specific diagnoses. The term 'X-linked congenital disorder of glycosylation with intellectual disability as a major feature' was previously used as an umbrella category for certain X-linked conditions where the body has trouble with a process called glycosylation — the way sugars are attached to proteins and fats in cells. When glycosylation does not work properly, it can affect many organs and body systems. The most prominent feature in this group is intellectual disability, meaning affected individuals have significant difficulties with learning, reasoning, and adaptive skills. Other symptoms can include developmental delays, seizures, abnormal muscle tone, and problems with growth. Because this is labeled as 'OBSOLETE' in medical classification systems, it means the conditions previously grouped here have been reclassified into more specific diagnoses as scientific understanding has improved. If you or a family member received this diagnosis in the past, it is important to work with a geneticist to determine the specific, updated diagnosis. Treatment is generally supportive and tailored to the individual's symptoms, as there is currently no cure for most congenital disorders of glycosylation. Management may include therapies for developmental delays, seizure medications, nutritional support, and other symptom-based interventions.

Also known as:

Key symptoms:

Intellectual disabilityDevelopmental delaysSeizures or epilepsyLow muscle tone (floppiness)Difficulty with speech and languagePoor growth or failure to thriveUnusual facial featuresProblems with coordination and balanceBehavioral difficultiesFeeding difficulties in infancy

Inheritance

X-linked recessive

Carried on the X chromosome; typically affects males more than females

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: X-linked congenital disorder of glycosylation with intellectual disability as a major feature.

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No actively recruiting trials found for OBSOLETE: X-linked congenital disorder of glycosylation with intellectual disability as a major feature at this time.

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No specialists are currently listed for OBSOLETE: X-linked congenital disorder of glycosylation with intellectual disability as a major feature.

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 OBSOLETE: X-linked congenital disorder of glycosylation with intellectual disability as a major feature.

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Community

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

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Family & Caregiver Grants

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.Has the specific gene causing this condition been identified, and what is the updated diagnosis?,What developmental therapies should we start, and how often?,Are there any specific dietary supplements or treatments that could help based on the exact genetic diagnosis?,What seizure precautions should we take, and when should we go to the emergency room?,How often should we have follow-up appointments and what monitoring tests are needed?,Are there clinical trials or research studies we should consider?,What educational and community support resources are available for our family?

Common questions about OBSOLETE: X-linked congenital disorder of glycosylation with intellectual disability as a major feature

What is OBSOLETE: X-linked congenital disorder of glycosylation with intellectual disability as a major feature?

This entry refers to a group of rare genetic conditions that are now classified under more specific diagnoses. The term 'X-linked congenital disorder of glycosylation with intellectual disability as a major feature' was previously used as an umbrella category for certain X-linked conditions where the body has trouble with a process called glycosylation — the way sugars are attached to proteins and fats in cells. When glycosylation does not work properly, it can affect many organs and body systems. The most prominent feature in this group is intellectual disability, meaning affected individuals

How is OBSOLETE: X-linked congenital disorder of glycosylation with intellectual disability as a major feature inherited?

OBSOLETE: X-linked congenital disorder of glycosylation with intellectual disability as a major feature follows a x-linked recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does OBSOLETE: X-linked congenital disorder of glycosylation with intellectual disability as a major feature typically begin?

Typical onset of OBSOLETE: X-linked congenital disorder of glycosylation with intellectual disability as a major feature is infantile. Age of onset can vary across affected individuals.