Hypocalcemic vitamin D-dependent rickets

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ORPHA:289157OMIM:264700E55.0
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Overview

Hypocalcemic vitamin D-dependent rickets, also known as vitamin D-dependent rickets (VDDR), is a group of rare inherited disorders that affect how the body processes vitamin D. Vitamin D is essential for absorbing calcium from food and keeping bones strong. In this condition, the body either cannot properly convert vitamin D into its active form or cannot respond to active vitamin D normally. This leads to low calcium levels in the blood (hypocalcemia), which causes the bones to become soft and weak — a condition called rickets in children. The main symptoms usually appear in infancy or early childhood and include bowed legs, delayed growth, muscle weakness, bone pain, and dental problems. Children may have trouble walking or experience frequent fractures. Seizures can occur due to very low calcium levels. Without treatment, the disease can lead to significant skeletal deformities. Treatment depends on the specific type of vitamin D-dependent rickets. Type 1 (also called 1-alpha-hydroxylase deficiency or VDDR type 1A) is treated with active vitamin D (calcitriol), which bypasses the enzyme deficiency. Type 2 (also called hereditary vitamin D-resistant rickets or VDDR type 2A) involves resistance to vitamin D at the receptor level and may require very high doses of calcium, sometimes given intravenously. With early diagnosis and proper treatment, many patients can achieve significant improvement in bone health and growth, though lifelong therapy is usually needed.

Also known as:

Key symptoms:

Bowed legs or knock kneesDelayed growth and short statureMuscle weaknessBone pain and tendernessFrequent bone fracturesDelayed walking or waddling gaitSeizures from low calciumDental problems such as delayed tooth eruption or enamel defectsWidening of the wrists and anklesSoft skull bones in infantsIrritability and fussiness in babiesNumbness or tingling in hands and feetMuscle cramps or spasmsHair loss (in type 2 cases)

Clinical phenotype terms (46)— hover any for plain English
RicketsHP:0002748Low serum calcitriolHP:0012052Secondary hyperparathyroidismHP:0000867Deformed rib cageHP:0000886Rachitic rosaryHP:0000897Enlargement of the costochondral junctionHP:0000920TetanyHP:0001281Increased susceptibility to fracturesHP:0002659Delayed epiphyseal ossificationHP:0002663OsteomalaciaHP:0002749Sparse bone trabeculaeHP:0002752Thin bony cortexHP:0002753Generalized aminoaciduriaHP:0002909
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Hypocalcemic vitamin D-dependent rickets.

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No actively recruiting trials found for Hypocalcemic vitamin D-dependent rickets at this time.

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No specialists are currently listed for Hypocalcemic vitamin D-dependent rickets.

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 Hypocalcemic vitamin D-dependent rickets.

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Community

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific type of vitamin D-dependent rickets does my child have, and which gene is affected?,What are the target blood calcium and vitamin D levels we should aim for with treatment?,How often will blood tests and monitoring be needed?,What are the signs that the medication dose needs to be adjusted?,Are there any activities or sports my child should avoid?,What should I do if my child has a seizure or shows signs of very low calcium?,Should other family members be tested for this condition?

Common questions about Hypocalcemic vitamin D-dependent rickets

What is Hypocalcemic vitamin D-dependent rickets?

Hypocalcemic vitamin D-dependent rickets, also known as vitamin D-dependent rickets (VDDR), is a group of rare inherited disorders that affect how the body processes vitamin D. Vitamin D is essential for absorbing calcium from food and keeping bones strong. In this condition, the body either cannot properly convert vitamin D into its active form or cannot respond to active vitamin D normally. This leads to low calcium levels in the blood (hypocalcemia), which causes the bones to become soft and weak — a condition called rickets in children. The main symptoms usually appear in infancy or early

How is Hypocalcemic vitamin D-dependent rickets inherited?

Hypocalcemic vitamin D-dependent rickets follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Hypocalcemic vitamin D-dependent rickets typically begin?

Typical onset of Hypocalcemic vitamin D-dependent rickets is infantile. Age of onset can vary across affected individuals.