Sagliker syndrome

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Overview

Sagliker syndrome is a very rare condition that develops as a complication of long-standing chronic kidney disease (also called chronic renal failure) and the severe secondary hyperparathyroidism that comes with it. Secondary hyperparathyroidism means the parathyroid glands in the neck become overactive because the kidneys can no longer properly balance calcium and phosphorus in the blood. Over time, this leads to dramatic changes in the bones of the face and skull, causing a characteristic ugly facial appearance that includes a short and flattened nose, enlarged jaw, and misshapen skull. The syndrome was first described by Dr. Yahya Sagliker in 2004. Patients with Sagliker syndrome typically have been on dialysis or have had poorly controlled kidney disease for years. The excess parathyroid hormone causes the bones to weaken and reshape, particularly in the face and skull. Other features can include short stature, hearing loss, vision problems, dental abnormalities, and fingertip changes. The facial deformity can be quite severe and affects quality of life significantly. Treatment focuses on controlling the underlying kidney disease and hyperparathyroidism. This may include medications to lower parathyroid hormone levels, phosphate binders, vitamin D supplements, and in many cases surgical removal of the parathyroid glands (parathyroidectomy). Some patients may also need corrective facial or jaw surgery. Early and aggressive management of secondary hyperparathyroidism in kidney disease patients is key to preventing this syndrome from developing.

Key symptoms:

Severe changes in facial appearance (ugly face deformity)Flattened or shortened noseEnlarged or protruding jawMisshapen skullShort statureHearing lossVision problemsDental abnormalities or tooth lossBone painWeakened and fragile bonesSwollen or clubbed fingertipsDifficulty breathing through the noseFatigue and weaknessPale skin from anemia

Clinical phenotype terms (13)— hover any for plain English
Elevated circulating parathyroid hormone levelHP:0003165High-frequency hearing impairmentHP:0005101Neoplasm of the oral cavityHP:0100649
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Sagliker syndrome.

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No actively recruiting trials found for Sagliker syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Sagliker syndrome community →

No specialists are currently listed for Sagliker 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 Sagliker syndrome.

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Community

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Latest news about Sagliker syndrome

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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.

Questions for your doctor

Bring these to your next appointment

  • Q1.What is my current parathyroid hormone level, and what is the target we are aiming for?,Would parathyroidectomy (surgery to remove the parathyroid glands) be beneficial in my case?,Am I a candidate for kidney transplantation, and how would that affect this condition?,Are there surgical options to correct the facial bone changes?,What dietary changes should I make to help control my phosphorus and calcium levels?,How often should my blood levels and bone health be monitored?,Can you refer me to a mental health professional to help me cope with the emotional impact of this condition?

Common questions about Sagliker syndrome

What is Sagliker syndrome?

Sagliker syndrome is a very rare condition that develops as a complication of long-standing chronic kidney disease (also called chronic renal failure) and the severe secondary hyperparathyroidism that comes with it. Secondary hyperparathyroidism means the parathyroid glands in the neck become overactive because the kidneys can no longer properly balance calcium and phosphorus in the blood. Over time, this leads to dramatic changes in the bones of the face and skull, causing a characteristic ugly facial appearance that includes a short and flattened nose, enlarged jaw, and misshapen skull. The

How is Sagliker syndrome inherited?

Sagliker syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Sagliker syndrome typically begin?

Typical onset of Sagliker syndrome is adult. Age of onset can vary across affected individuals.