Tall stature-intellectual disability-renal anomalies syndrome

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ORPHA:500095OMIM:617107Q87.3
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8Treatment centers

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Overview

Tall stature-intellectual disability-renal anomalies syndrome is a rare genetic condition that affects multiple parts of the body. It is sometimes referred to by its Orphanet code ORPHA:500095. People with this syndrome are typically much taller than average for their age, have some degree of intellectual disability or learning difficulties, and have structural problems with one or both kidneys. The kidney problems can range from mild differences in kidney shape or position to more serious issues that affect how well the kidneys work. This condition is present from birth, though some features may become more noticeable as a child grows. Intellectual disability can range from mild to moderate, meaning some children may need extra support at school and in daily life. Tall stature is often one of the first things noticed, sometimes even before birth during prenatal ultrasound. Kidney anomalies may be found during routine imaging or when investigating other symptoms. There is currently no cure for this syndrome. Treatment focuses on managing each symptom individually. This may include educational support, speech and occupational therapy, and regular monitoring of kidney health. A team of specialists is usually needed to provide the best care.

Also known as:

Key symptoms:

Unusually tall height for ageIntellectual disability or learning difficultiesKidney structural abnormalities (such as missing, misshapen, or misplaced kidneys)Delayed speech and language developmentDelayed developmental milestones (such as sitting or walking later than expected)Behavioral challengesPossible facial features that look different from other family membersReduced kidney function in some cases

Clinical phenotype terms (47)— hover any for plain English
Retinal colobomaHP:0000480Renal malrotationHP:0004712Proportionate tall statureHP:0011407CamptodactylyHP:0012385Bifid ureterHP:0030037Persistently decreased total neutrophil countHP:0410252Transiently decreased total neutrophil countHP:0410255
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 Tall stature-intellectual disability-renal anomalies syndrome.

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No actively recruiting trials found for Tall stature-intellectual disability-renal anomalies syndrome at this time.

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No specialists are currently listed for Tall stature-intellectual disability-renal anomalies syndrome.

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 Tall stature-intellectual disability-renal anomalies syndrome.

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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

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.What genetic tests should my child have to confirm this diagnosis?,How serious are my child's kidney problems, and what do we need to monitor?,What therapies or early intervention programs do you recommend?,Are there any dietary changes we should make to protect kidney health?,What signs should prompt us to seek emergency care?,Are there any clinical trials or research studies we could participate in?,What support services are available for children with intellectual disability in our area?

Common questions about Tall stature-intellectual disability-renal anomalies syndrome

What is Tall stature-intellectual disability-renal anomalies syndrome?

Tall stature-intellectual disability-renal anomalies syndrome is a rare genetic condition that affects multiple parts of the body. It is sometimes referred to by its Orphanet code ORPHA:500095. People with this syndrome are typically much taller than average for their age, have some degree of intellectual disability or learning difficulties, and have structural problems with one or both kidneys. The kidney problems can range from mild differences in kidney shape or position to more serious issues that affect how well the kidneys work. This condition is present from birth, though some features

How is Tall stature-intellectual disability-renal anomalies syndrome inherited?

Tall stature-intellectual disability-renal anomalies syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Tall stature-intellectual disability-renal anomalies syndrome typically begin?

Typical onset of Tall stature-intellectual disability-renal anomalies syndrome is neonatal. Age of onset can vary across affected individuals.