Diaphanospondylodysostosis

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ORPHA:66637OMIM:608022Q78.8
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2Active trials3Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Diaphanospondylodysostosis (also sometimes called DSD) is an extremely rare and severe bone disorder that affects the spine and other parts of the skeleton before and after birth. The name comes from Greek and Latin words meaning 'transparent vertebrae,' which refers to how the bones of the spine appear unusually thin or underdeveloped on imaging. This condition is caused by changes (mutations) in the BMPER gene, which plays an important role in how bones form during early development. Babies born with this condition typically have very serious problems with their spine, including missing or poorly formed vertebrae (the small bones that make up the backbone). Many also have underdeveloped or absent sacrum (the bone at the base of the spine), kidney problems, and abnormalities of the ribs and other bones. Because the spine and surrounding structures are so severely affected, this condition often impacts breathing and organ function. Unfortunately, diaphanospondylodysostosis is considered a lethal or near-lethal condition in most cases, meaning many affected babies do not survive beyond the newborn period. There is currently no cure, and treatment focuses on comfort and supportive care. Families dealing with this diagnosis benefit greatly from a team of specialists including geneticists, neonatologists, and palliative care providers.

Also known as:

Key symptoms:

Missing or severely underdeveloped vertebrae (backbone bones)Absent or underdeveloped sacrum (the bone at the base of the spine)Abnormally shaped or missing ribsKidney abnormalities or absent kidneysVery small or narrow chestBreathing difficulties due to chest and rib problemsShort trunk (short body length)Abnormal appearance of the spine on X-ray or ultrasoundLow birth weight or small sizePossible limb abnormalities

Clinical phenotype terms (11)— hover any for plain English
Missing ribsHP:0000921MyelomeningoceleHP:0002475Narrow pelvis boneHP:0003275Absent or minimally ossified vertebral bodiesHP:0004599Multiple renal cystsHP:0005562Abnormal vertebral segmentation and fusionHP:0005640Enlarged thoraxHP:0100625
Inheritance

Autosomal recessive

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

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

6 events
Oct 2025Breaking Silence Through Story: A Narrative Medicine Intervention for Parents of Children With Urogenital Conditions

Sarah Schlegel — NA

TrialRECRUITING
Jun 2024Integrating Hypertension Management in DSD for HIV

Infectious Diseases Research Collaboration, Uganda

TrialNOT YET RECRUITING
Jun 2021DSD Models at Zambia Sentinel Sites (SENTINEL 2)

Boston University

TrialACTIVE NOT RECRUITING
Jun 2021DSD Models at Malawi Sentinel Sites (SENTINEL 2-Malawi)

Boston University

TrialACTIVE NOT RECRUITING
Jun 2021DSD Models at South Africa Sentinel Sites

Boston University

TrialACTIVE NOT RECRUITING
Jan 2017Minipuberty in Infants Born With Potential Hypogonadism Hypogonadotrope

Centre Hospitalier Universitaire Vaudois

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Diaphanospondylodysostosis.

2 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

2 recruitingView all trials with filters →
N/A1 trial
Breaking Silence Through Story: A Narrative Medicine Intervention for Parents of Children With Urogenital Conditions
N/A
Actively Recruiting
· Sites: Boston, Massachusetts · Age: 1899 yrs
Other1 trial
Minipuberty in Infants Born With Potential Hypogonadism Hypogonadotrope
Actively Recruiting
PI: Nelly Pitteloud, MD (CHUV) · Sites: Lausanne, Canton of Vaud

Specialists

3 foundView all specialists →
VM
Veronica Gomez-Lobo, M.D.
WASHINGTON, DC
Specialist
PI on 6 active trials
SP
Shanlee M Davis, MD, PhD
AURORA, CO
Specialist
PI on 3 active trials
BB
Barbara B Biesecker
CLARKS SUMMIT, PA
Specialist
PI on 16 active trials

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 Diaphanospondylodysostosis.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Diaphanospondylodysostosis

Disease timeline:

New recruiting trial: Breaking Silence Through Story: A Narrative Medicine Intervention for Parents of Children With Urogenital Conditions

A new clinical trial is recruiting patients for Diaphanospondylodysostosis

New recruiting trial: Sex Steroids Balance for Metabolic and Reproductive Health in Klinefelter Syndrome

A new clinical trial is recruiting patients for Diaphanospondylodysostosis

New recruiting trial: Differentiated Service Delivery (DSD) for People Living with HIV (PLHIV) and Comorbidities

A new clinical trial is recruiting patients for Diaphanospondylodysostosis

New recruiting trial: Identifying New Genetic Causes to Development Disorders

A new clinical trial is recruiting patients for Diaphanospondylodysostosis

New recruiting trial: Minipuberty in Infants Born With Potential Hypogonadism Hypogonadotrope

A new clinical trial is recruiting patients for Diaphanospondylodysostosis

New recruiting trial: Data Collection Study of Pediatric and Adolescent Gynecology Conditions

A new clinical trial is recruiting patients for Diaphanospondylodysostosis

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 does my child's specific genetic mutation in the BMPER gene mean for their prognosis?,What supportive care options are available for my baby after birth?,What is the chance that a future pregnancy would be affected by this condition?,Can prenatal testing be done in future pregnancies to check for this condition?,Are there any clinical trials or research studies we should know about?,What palliative care and emotional support resources are available for our family?,Should other family members be tested to see if they are carriers of the BMPER gene change?

Common questions about Diaphanospondylodysostosis

What is Diaphanospondylodysostosis?

Diaphanospondylodysostosis (also sometimes called DSD) is an extremely rare and severe bone disorder that affects the spine and other parts of the skeleton before and after birth. The name comes from Greek and Latin words meaning 'transparent vertebrae,' which refers to how the bones of the spine appear unusually thin or underdeveloped on imaging. This condition is caused by changes (mutations) in the BMPER gene, which plays an important role in how bones form during early development. Babies born with this condition typically have very serious problems with their spine, including missing or

How is Diaphanospondylodysostosis inherited?

Diaphanospondylodysostosis follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Diaphanospondylodysostosis typically begin?

Typical onset of Diaphanospondylodysostosis is neonatal. Age of onset can vary across affected individuals.

Are there clinical trials for Diaphanospondylodysostosis?

Yes — 2 recruiting clinical trials are currently listed for Diaphanospondylodysostosis on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Diaphanospondylodysostosis?

3 specialists and care centers treating Diaphanospondylodysostosis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.