OBSOLETE: Craniosynostosis-synostoses-hypertensive nephropathy syndrome

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Overview

Craniosynostosis-synostoses-hypertensive nephropathy syndrome is an extremely rare genetic condition that has been described in medical literature but is now considered an obsolete or reclassified diagnosis in some disease databases. This condition was characterized by a combination of three main problems: craniosynostosis (early fusion of the skull bones in infants, which can affect head shape and brain growth), synostoses (abnormal fusion of other bones in the body, particularly joints), and hypertensive nephropathy (kidney disease that leads to high blood pressure). Affected individuals may have an unusually shaped head due to premature skull bone fusion, stiff or immovable joints from bone fusions elsewhere in the body, and progressive kidney problems that cause dangerously high blood pressure. Because this condition is so rare and has been reclassified, very limited information is available about its full clinical spectrum. Treatment has generally been supportive, focusing on surgical correction of skull abnormalities when needed, management of joint problems, and control of blood pressure and kidney disease. Patients with this diagnosis should work closely with a team of specialists to address each aspect of the condition. The obsolete status of this entry suggests that the condition may now be classified under a different or updated disease name, and patients should discuss current classification with their geneticist.

Also known as:

Key symptoms:

Abnormal head shape from early skull bone fusionStiff or fused jointsHigh blood pressureKidney problemsLimited range of motion in fingers or toesShort statureFused bones in the hands or feetHeadaches related to high blood pressureSwelling due to kidney dysfunctionFacial asymmetry or unusual facial features

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: Craniosynostosis-synostoses-hypertensive nephropathy syndrome.

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No actively recruiting trials found for OBSOLETE: Craniosynostosis-synostoses-hypertensive nephropathy syndrome at this time.

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No specialists are currently listed for OBSOLETE: Craniosynostosis-synostoses-hypertensive nephropathy 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 OBSOLETE: Craniosynostosis-synostoses-hypertensive nephropathy syndrome.

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.Has this diagnosis been reclassified, and is there a more current name for this condition?,What genetic testing should be done to confirm the diagnosis?,How often should my child's kidney function and blood pressure be checked?,When is surgery recommended for the skull bone fusion, and what are the risks?,What can be done to help with joint stiffness and mobility?,Are there any clinical trials or research studies we should know about?,What is the long-term outlook for kidney function, and when might dialysis or transplant be needed?

Common questions about OBSOLETE: Craniosynostosis-synostoses-hypertensive nephropathy syndrome

What is OBSOLETE: Craniosynostosis-synostoses-hypertensive nephropathy syndrome?

Craniosynostosis-synostoses-hypertensive nephropathy syndrome is an extremely rare genetic condition that has been described in medical literature but is now considered an obsolete or reclassified diagnosis in some disease databases. This condition was characterized by a combination of three main problems: craniosynostosis (early fusion of the skull bones in infants, which can affect head shape and brain growth), synostoses (abnormal fusion of other bones in the body, particularly joints), and hypertensive nephropathy (kidney disease that leads to high blood pressure). Affected individuals may

How is OBSOLETE: Craniosynostosis-synostoses-hypertensive nephropathy syndrome inherited?

OBSOLETE: Craniosynostosis-synostoses-hypertensive nephropathy syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does OBSOLETE: Craniosynostosis-synostoses-hypertensive nephropathy syndrome typically begin?

Typical onset of OBSOLETE: Craniosynostosis-synostoses-hypertensive nephropathy syndrome is neonatal. Age of onset can vary across affected individuals.