Hypospadias-intellectual disability, Goldblatt type syndrome

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ORPHA:2261OMIM:241760Q87.8
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Overview

Hypospadias-intellectual disability, Goldblatt type syndrome (also sometimes called Goldblatt syndrome) is a very rare genetic condition that affects males. It combines two main features: hypospadias and intellectual disability. Hypospadias is a birth difference where the opening of the urethra (the tube that carries urine out of the body) is not at the tip of the penis but somewhere along the underside. This can affect urination and, later in life, fertility. The intellectual disability in this syndrome ranges from mild to moderate, meaning affected individuals may have some difficulty with learning, communication, and daily tasks, but the degree varies from person to person. This condition is present from birth and is thought to be caused by changes in a person's genes, though the exact gene responsible has not been clearly identified in all cases. Because it is so rare, most of what doctors know comes from a very small number of reported families and individuals. There is no cure for Goldblatt syndrome. Treatment focuses on managing the individual symptoms. Surgery can correct hypospadias in most cases, and educational support, speech therapy, and occupational therapy can help people with intellectual disability reach their full potential. Early diagnosis and a team of specialists working together give the best outcomes.

Also known as:

Key symptoms:

Hypospadias — the urinary opening is on the underside of the penis rather than at the tipIntellectual disability — difficulty with learning, reasoning, and problem-solvingDelayed speech and language developmentDelayed developmental milestones such as sitting, walking, or talking later than expectedShort stature — being shorter than average for ageAbnormal positioning or appearance of the genitalsPossible behavioral challenges

Clinical phenotype terms (15)— hover any for plain English
Inheritance

X-linked recessive

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

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 Hypospadias-intellectual disability, Goldblatt type syndrome.

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No specialists are currently listed for Hypospadias-intellectual disability, Goldblatt type syndrome.

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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 Hypospadias-intellectual disability, Goldblatt type syndrome.

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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.What genetic tests should we do to confirm the diagnosis and find the gene responsible?,What is the best timing and approach for hypospadias surgery for my child?,What early intervention services should we start right away to support development?,What is the likely degree of intellectual disability, and how will it affect my child's future independence?,What is the chance that other children in our family could be affected?,Are there any clinical trials or research studies we could participate in?,What specialists should be part of our care team, and how often should we see them?

Common questions about Hypospadias-intellectual disability, Goldblatt type syndrome

What is Hypospadias-intellectual disability, Goldblatt type syndrome?

Hypospadias-intellectual disability, Goldblatt type syndrome (also sometimes called Goldblatt syndrome) is a very rare genetic condition that affects males. It combines two main features: hypospadias and intellectual disability. Hypospadias is a birth difference where the opening of the urethra (the tube that carries urine out of the body) is not at the tip of the penis but somewhere along the underside. This can affect urination and, later in life, fertility. The intellectual disability in this syndrome ranges from mild to moderate, meaning affected individuals may have some difficulty with l

How is Hypospadias-intellectual disability, Goldblatt type syndrome inherited?

Hypospadias-intellectual disability, Goldblatt type syndrome follows a x-linked recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Hypospadias-intellectual disability, Goldblatt type syndrome typically begin?

Typical onset of Hypospadias-intellectual disability, Goldblatt type syndrome is neonatal. Age of onset can vary across affected individuals.