Trial Outcomes & Findings for Collection and Study of Cerebrospinal Fluid in Patients With Hunter Syndrome (NCT NCT01449240)

NCT ID: NCT01449240

Last Updated: 2021-06-09

Results Overview

The concentration of total GAG, including heparan sulfate (HS) and dermatan sulfate (DS) oligosaccharides, in CSF was measured using an enzymatic assay.

Recruitment status

COMPLETED

Target enrollment

10 participants

Primary outcome timeframe

Day 1

Results posted on

2021-06-09

Participant Flow

Participant milestones

Participant milestones
Measure
No Investigational Treatment or Control Group
This was an observational study for the collection and study of cerebrospinal fluid (CSF) in patients with Hunter syndrome. No investigational treatment was given.
Overall Study
STARTED
10
Overall Study
COMPLETED
10
Overall Study
NOT COMPLETED
0

Reasons for withdrawal

Withdrawal data not reported

Baseline Characteristics

Collection and Study of Cerebrospinal Fluid in Patients With Hunter Syndrome

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
No Investigational Treatment or Control Group
n=9 Participants
This was an observational study for the collection and study of CSF in patients with Hunter syndrome. No investigational treatment was given.
Age, Categorical
<=18 years
4 Participants
n=5 Participants
Age, Categorical
Between 18 and 65 years
5 Participants
n=5 Participants
Age, Categorical
>=65 years
0 Participants
n=5 Participants
Age, Continuous
19.36 years
n=5 Participants
Sex: Female, Male
Female
0 Participants
n=5 Participants
Sex: Female, Male
Male
9 Participants
n=5 Participants
Region of Enrollment
United States
8 Participants
n=5 Participants
Region of Enrollment
United Kingdom
1 Participants
n=5 Participants

PRIMARY outcome

Timeframe: Day 1

Population: Pharmacodynamic Population: All patients for which an evaluable CSF sample was collected. This included a pediatric patient who was consented to provide a retrospective CSF sample.

The concentration of total GAG, including heparan sulfate (HS) and dermatan sulfate (DS) oligosaccharides, in CSF was measured using an enzymatic assay.

Outcome measures

Outcome measures
Measure
No Investigational Treatment or Control Group
n=9 Participants
This was an observational study for the collection and study of CSF in patients with Hunter syndrome. No investigational treatment was given.
Levels of Total Glycosaminoglycan (GAG) in CSF
816.750 ng/mL
Interval 316.45 to 1317.05

SECONDARY outcome

Timeframe: Day 1

Population: Pharmacodynamic Population: All patients for which an evaluable CSF sample was collected. Urinary GAG was not measured in 2 patients: 1 pediatric patient who provided a retrospective CSF sample only (no urine sample was collected) and 1 adult patient whose CSF sample was not considered evaluable and therefore their urinary GAG was not measured.

The levels of GAG (including sulfated DS/HS oligosaccharides) in urine were determined by the Blyscan sulfated GAG assay kit. The concentration of GAG in urine was normalized to the urine creatinine value and reported as mg GAG/mmol creatinine.

Outcome measures

Outcome measures
Measure
No Investigational Treatment or Control Group
n=8 Participants
This was an observational study for the collection and study of CSF in patients with Hunter syndrome. No investigational treatment was given.
Levels of GAG in Urine
12.458 mg GAG/mmol Creatinine
Interval 3.282 to 21.633

Adverse Events

No Investigational Treatment or Control Group

Serious events: 1 serious events
Other events: 4 other events
Deaths: 0 deaths

Serious adverse events

Serious adverse events
Measure
No Investigational Treatment or Control Group
n=9 participants at risk
This was an observational study for the collection and study of CSF in patients with Hunter syndrome. No investigational treatment was given. Safety analyses were performed in the Safety Population, which was defined as all patients who had undergone a procedure for CSF sample collection. This included a patient who underwent unsuccessful CSF sample collection; no CSF or urine GAG data were available for this adult patient.
Nervous system disorders
Headache
11.1%
1/9 • Number of events 1

Other adverse events

Other adverse events
Measure
No Investigational Treatment or Control Group
n=9 participants at risk
This was an observational study for the collection and study of CSF in patients with Hunter syndrome. No investigational treatment was given. Safety analyses were performed in the Safety Population, which was defined as all patients who had undergone a procedure for CSF sample collection. This included a patient who underwent unsuccessful CSF sample collection; no CSF or urine GAG data were available for this adult patient.
Musculoskeletal and connective tissue disorders
Stiff Neck
11.1%
1/9 • Number of events 1
Nervous system disorders
Headache
11.1%
1/9 • Number of events 1
Injury, poisoning and procedural complications
Pain Secondary to Surgical Procedure
11.1%
1/9 • Number of events 1
Injury, poisoning and procedural complications
Difficulty Hearing in Right Ear Secondary to Neurosurgery
11.1%
1/9 • Number of events 1
Injury, poisoning and procedural complications
Intermittent Headaches Post-Neurosurgery
11.1%
1/9 • Number of events 1
Gastrointestinal disorders
Intermittent Lower Abdomen Spasms
11.1%
1/9 • Number of events 1
Injury, poisoning and procedural complications
Intermittent Soreness Post-Surgery
11.1%
1/9 • Number of events 1

Additional Information

Study Director

Shire

Phone: +1 866 842 5335

Results disclosure agreements

  • Principal investigator is a sponsor employee If a multicenter publication is not submitted within twelve (12) months after conclusion, abandonment or termination of the Study at all sites, or after Sponsor confirms there shall be no multicenter Study publication, the Institution and/or such Principal Investigator may publish the results from the Institution site individually.
  • Publication restrictions are in place

Restriction type: OTHER