Urea Cycle Disorder

Last reviewed

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Who is this for?
Show terms as
6FDA treatments3Active trials17Specialists8Treatment centers5Financial resources

Where are you in your journey?

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

Urea Cycle Disorder is treated with 11 medications in our database, including sodium phenylbutyrate, Sodium phenylacetate and Sodium benzoate, Carbaglu, SODIUM PHENYLACETATE AND SODIUM BENZOATE, Orapaz, and 6 more. 11 of these have manufacturer assistance programs available to help reduce out-of-pocket costs. Medications are manufactured by Horizon, Bausch Health, Recordati, Horizon No punctuation., Amgen and others. Patients and caregivers can find copay cards, patient assistance programs, and travel grants for Urea Cycle Disorder treatment below.

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

NORD ↗

FDA & Trial Timeline

4 events
Jul 2020Systemic Biomarkers of Brain Injury From Hyperammonemia

Children's National Research Institute

TrialRECRUITING
Oct 2019Ureagenesis Analysis in Healthy Subjects and in Urea Cycle Disorder Patients

University Children's Hospital, Zurich — NA

TrialRECRUITING
Aug 2016Neuroimaging and Neuropsychological Outcomes in Urea Cycle Disorders

Children's National Research Institute

TrialRECRUITING
Apr 2012Orphan Europe Carbaglu® Surveillance Protocol

Nicholas Ah Mew

TrialRECRUITING

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

Treatments

6 available

Sodium Phenylbutyrate

SODIUM PHENYLBUTYRATE TABLETS, 500 MG· ENDO USA, Inc.

indicated in all patients with neonatal-onset deficiency (complete enzymatic deficiency, presenting within the first 28 days of life)

Ravicti

GLYCEROL PHENYLBUTYRATE· Horizon Therapeutics USA, Inc.
RAVICTI is indicated for use as a nitrogen-binding agent for chronic management of patients with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or amino acid supp

RAVICTI is indicated for use as a nitrogen-binding agent for chronic management of patients with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone.

Glycerol Phenylbutyrate

GLYCEROL PHENYLBUTYRATE ORAL· Par Health USA, LLC
indicated for use as a nitrogen-binding agent for chronic management of patients with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or amino acid supplementation

indicated for use as a nitrogen-binding agent for chronic management of patients with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone

Orapaz

Cephalexin, Ibuprofen, Chlorhexidine Gluconate· CTS Chemical Industries

Detailed copay and financial assistance information is not publicly available for this medication at this time. Please consult your pharmacist or the manufacturer's official patient support program fo

BUPHENYL, Buphenyl

· Horizon No punctuation.

Detailed copay and financial assistance information is not publicly available for this medication at this time. Please consult your pharmacist or the manufacturer's official patient support program fo

Carbaglu

Carglumic Acid· Recordati

* Patient Copay Amount: $0 copay * Maximum Annual Benefit Limit: Not Publicly Available * Core Eligibility Restrictions: Must be a qualified patient with commercial insurance. Patient Assistance Progr

Clinical Trials

3 recruitingView all trials with filters →
N/A1 trial
Ureagenesis Analysis in Healthy Subjects and in Urea Cycle Disorder Patients
N/A
Actively Recruiting
· Sites: Zurich, Canton of Zurich
Other2 trials
Neuroimaging and Neuropsychological Outcomes in Urea Cycle Disorders
Actively Recruiting
PI: Andrea L. Gropman, M.D. (Children's National Research Institute) · Sites: Washington D.C., District of Columbia · Age: 750 yrs
Systemic Biomarkers of Brain Injury From Hyperammonemia
Actively Recruiting
PI: Nicholas Ah Mew, MD (Children's National Research Institute) · Sites: Washington D.C., District of Columbia · Age: 718 yrs

Specialists

17 foundView all specialists →
TT
Tommaso Tabaglio
Specialist
1 Urea Cycle Disorder publication
HN
Hikaru Nishida
Specialist
1 Urea Cycle Disorder publication
TT
Toshiki Tsunogai
Specialist
1 Urea Cycle Disorder publication
MY
Masahide Yazaki
Specialist
1 Urea Cycle Disorder publication
GC
Gaik Siew Ch'ng
Specialist
1 Urea Cycle Disorder publication
ML
Manikandan Lakshmanan
Specialist
1 Urea Cycle Disorder publication
SL
Su Seong Lee
Specialist
1 Urea Cycle Disorder publication
JY
Jackie Y Ying
Specialist
1 Urea Cycle Disorder publication
JO
Jin Rong Ow
Specialist
1 Urea Cycle Disorder publication
EI
Eri Imagawa
Specialist
1 Urea Cycle Disorder publication
WC
Wei Yuan Cher
Specialist
1 Urea Cycle Disorder publication
SC
Shermin Yu Tung Chan
Specialist
1 Urea Cycle Disorder publication
RG
Rajasekhar Reddy Gurrampati
Specialist
1 Urea Cycle Disorder publication
VR
Venkataramanan Ramadass
Specialist
1 Urea Cycle Disorder publication
JP
Jeffrey Krischer, PhD
Specialist
PI on 3 active trials
ML
Mun Fai Loke
Specialist
1 Urea Cycle Disorder publication

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

Financial Resources

2 resources
Ravicti(GLYCEROL PHENYLBUTYRATE)Horizon Therapeutics USA, Inc.
Glycerol Phenylbutyrate(GLYCEROL PHENYLBUTYRATE ORAL)Par Health USA, LLC

3 travel grants are also available for Urea Cycle Disorder patients — see Travel Grants below ↓

Travel Grants

3 grants

Ravicti Patient Access Liaisons (PALs) Travel Assistance

Apply ↗

HealthWell Foundation Urea Cycle Disorders Fund

HealthWell Foundation

Applicants must be receiving treatment for Urea Cycle Disorders within the United States and possess health insurance that covers their medication. Eligibility also requires a household income at or below 500% of the Federal Poverty Level, adjusted for household size and cost of living.

Apply ↗Up to $4,500

Urea Cycle Disorders Financial Assistance Program

Applicants must be U.S. citizens or permanent residents diagnosed with a urea cycle disorder and prescribed an FDA-approved treatment with existing prescription coverage. Eligibility also requires meeting financial criteria based on household income and size to receive assistance with treatment-related costs and travel expenses.

Apply ↗

Community

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Latest news about Urea Cycle Disorder

Disease timeline:

New recruiting trial: Ureagenesis Analysis in Healthy Subjects and in Urea Cycle Disorder Patients

A new clinical trial is recruiting patients for Urea Cycle Disorder

New recruiting trial: Neuroimaging and Neuropsychological Outcomes in Urea Cycle Disorders

A new clinical trial is recruiting patients for Urea Cycle Disorder

New recruiting trial: Orphan Europe Carbaglu® Surveillance Protocol

A new clinical trial is recruiting patients for Urea Cycle Disorder

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.

Common questions about Urea Cycle Disorder

What is Urea Cycle Disorder?

Urea Cycle Disorder is treated with 11 medications in our database, including sodium phenylbutyrate, Sodium phenylacetate and Sodium benzoate, Carbaglu, SODIUM PHENYLACETATE AND SODIUM BENZOATE, Orapaz, and 6 more. 11 of these have manufacturer assistance programs available to help reduce out-of-pocket costs. Medications are manufactured by Horizon, Bausch Health, Recordati, Horizon No punctuation., Amgen and others. Patients and caregivers can find copay cards, patient assistance programs, and travel grants for Urea Cycle Disorder treatment below.

Are there clinical trials for Urea Cycle Disorder?

Yes — 3 recruiting clinical trials are currently listed for Urea Cycle Disorder on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Urea Cycle Disorder?

17 specialists and care centers treating Urea Cycle Disorder are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.

What treatment and support options exist for Urea Cycle Disorder?

8 FDA-approved treatments and 2 patient support programs are currently tracked on UniteRare for Urea Cycle Disorder. See the treatments and support programs sections for copay assistance, eligibility, and contact details.