Trial Outcomes & Findings for Study of ORGN001 (Formerly ALXN1101) in Neonates, Infants and Children With Molybdenum Cofactor Deficiency (MOCD) Type A (NCT NCT02629393)

NCT ID: NCT02629393

Last Updated: 2023-10-17

Results Overview

Patients with a confirmed diagnosis of MOCD Type A, treated with ORGN001 and still alive at last observation.

Recruitment status

COMPLETED

Study phase

PHASE2/PHASE3

Target enrollment

5 participants

Primary outcome timeframe

Through last observation (average of 24 months)

Results posted on

2023-10-17

Participant Flow

Participant milestones

Participant milestones
Measure
Patients Treated With ORGN001 (Formerly ALXN1101)
All patients who received at least one dose of ORGN001 (formerly ALXN1101)
Overall Study
STARTED
5
Overall Study
COMPLETED
2
Overall Study
NOT COMPLETED
3

Reasons for withdrawal

Reasons for withdrawal
Measure
Patients Treated With ORGN001 (Formerly ALXN1101)
All patients who received at least one dose of ORGN001 (formerly ALXN1101)
Overall Study
MOCD Type A not genetically confirmed
2
Overall Study
Physician Decision
1

Baseline Characteristics

Study of ORGN001 (Formerly ALXN1101) in Neonates, Infants and Children With Molybdenum Cofactor Deficiency (MOCD) Type A

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
Patients Treated With ORGN001 (Formerly ALXN1101)
n=3 Participants
All patients who received at least one dose of ORGN001 (formerly ALXN1101)
Age, Categorical
<=18 years
3 Participants
n=93 Participants
Age, Categorical
Between 18 and 65 years
0 Participants
n=93 Participants
Age, Categorical
>=65 years
0 Participants
n=93 Participants
Age, Continuous
1 days old
n=93 Participants
Sex: Female, Male
Female
2 Participants
n=93 Participants
Sex: Female, Male
Male
1 Participants
n=93 Participants
Race (NIH/OMB)
American Indian or Alaska Native
0 Participants
n=93 Participants
Race (NIH/OMB)
Asian
2 Participants
n=93 Participants
Race (NIH/OMB)
Native Hawaiian or Other Pacific Islander
0 Participants
n=93 Participants
Race (NIH/OMB)
Black or African American
0 Participants
n=93 Participants
Race (NIH/OMB)
White
1 Participants
n=93 Participants
Race (NIH/OMB)
More than one race
0 Participants
n=93 Participants
Race (NIH/OMB)
Unknown or Not Reported
0 Participants
n=93 Participants
Region of Enrollment
Norway
1 participants
n=93 Participants
Region of Enrollment
United Kingdom
1 participants
n=93 Participants
Region of Enrollment
Israel
1 participants
n=93 Participants

PRIMARY outcome

Timeframe: Through last observation (average of 24 months)

Patients with a confirmed diagnosis of MOCD Type A, treated with ORGN001 and still alive at last observation.

Outcome measures

Outcome measures
Measure
ORGN001 (Formerly ALXN1101)
n=3 Participants
ORGN001 (formerly ALXN1101)
Overall Survival
3 Participants

SECONDARY outcome

Timeframe: At Month 12 visit

Number of patients who can feed orally

Outcome measures

Outcome measures
Measure
ORGN001 (Formerly ALXN1101)
n=3 Participants
ORGN001 (formerly ALXN1101)
Feeding Pattern
Oral feeding at Baseline · Able to feed orally
2 Participants
Feeding Pattern
Oral feeding at Baseline · Not able to feed orally
1 Participants
Feeding Pattern
Oral feeding at Month 12 · Able to feed orally
3 Participants
Feeding Pattern
Oral feeding at Month 12 · Not able to feed orally
0 Participants

Adverse Events

Patients Treated With ORGN001 (Formerly ALXN1101)

Serious events: 4 serious events
Other events: 4 other events
Deaths: 0 deaths

Serious adverse events

Serious adverse events
Measure
Patients Treated With ORGN001 (Formerly ALXN1101)
n=5 participants at risk
All patients who received at least one dose of ORGN001 (formerly ALXN1101)
Respiratory, thoracic and mediastinal disorders
Apneoa
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
General disorders
Complication associated with device
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Bacteraemia
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Gastroenteritis
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Pneumonia
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
General disorders
Catheter site swelling
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Viral tonsillitis
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Gastrointestinal disorders
Vomiting
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Viral infection
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Gastrointestinal viral
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Surgical and medical procedures
Central venous catheterisation
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Product Issues
Device leakage
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Cardiac disorders
Cardiac failure
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Nervous system disorders
Seizures
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Respiratory syncytial virus infection
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Device related sepsis
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Device related infection
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.

Other adverse events

Other adverse events
Measure
Patients Treated With ORGN001 (Formerly ALXN1101)
n=5 participants at risk
All patients who received at least one dose of ORGN001 (formerly ALXN1101)
Renal and urinary disorders
Haematuria
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Nasopharyngitis
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Oral herpes
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Conjunctivitis
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Respiratory, thoracic and mediastinal disorders
Cough
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Skin and subcutaneous tissue disorders
Eczema
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Pneumonia
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Metabolism and nutrition disorders
Hypoglycaemia
40.0%
2/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Hepatobiliary disorders
Hyperbilirubinaemia
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Metabolism and nutrition disorders
Hypocalcaemia
40.0%
2/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Gastrointestinal disorders
Anal fissure
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Eye disorders
Conjunctival haemorrhage
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Pathogen resistance
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Blood and lymphatic system disorders
Anaemia
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
General disorders
Pyrexia
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Catheter site infection
40.0%
2/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Respiratory, thoracic and mediastinal disorders
Rhinorrhea
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Gastrointestinal disorders
Diarrhoea
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Congenital, familial and genetic disorders
Chiari network
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Gastroenteritis
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Gastrointestinal disorders
Vomiting
40.0%
2/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Skin and subcutaneous tissue disorders
Dermatitis
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Investigations
Staphylococcus test positive
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Injury, poisoning and procedural complications
Fall
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
General disorders
Catheter site rash
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Injury, poisoning and procedural complications
Contusion
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Otitis media acute
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
General disorders
Catheter site erythema
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
General disorders
Catheter site haemorrhage
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
General disorders
Catheter site swelling
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Injury, poisoning and procedural complications
Skin laceration
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Viral infection
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Tonsillitis
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
COVID-19
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Cardiac disorders
Cardiac failure
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Congenital, familial and genetic disorders
Ventricular septal defect
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Eye disorders
Eye discharge
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Seizure
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.
Infections and infestations
Bronchitis
20.0%
1/5 • Adverse Event data were collected from Day 1 with ORGN001 treatment until study completion, up to Month 36 or study termination or completion.
All-cause mortality is zero in this study because there were no deaths that occurred in patients with confirmed MOCD Type A. All SAE data presented.

Additional Information

Business Development and Operations

Origin Biosciences (affiliate of BridgeBio)

Phone: 650-391-9740

Results disclosure agreements

  • Principal investigator is a sponsor employee Institution/Investigator shall have the right to publish or present the results of Institution's and Investigator's activities including that Study Data which was obtained or derived at Institution. Institution/Investigator agree to submit any proposed publication/presentation to Sponsor for review at least 60 days prior to submitting any such proposed publication. Within 30 days of its receipt, Sponsor shall advise if any changes are needed to protect confidentiality.
  • Publication restrictions are in place

Restriction type: OTHER