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NCT01307098

Safety, Tolerability and Pharmacokinetics of SBC-102 (Sebelipase Alfa) in Adult Participants With Lysosomal Acid Lipase Deficiency

Completed Phase 1, PHASE2 Results posted Last updated 11 December 2018
What this trial tests

Phase 1, PHASE2 trial testing Sebelipase alfa 0.35 mg/kg in Cholesterol Ester Storage Disease(CESD) in 9 participants. Completed in 6 January 2012.

Timeline
25 April 2011
Primary endpoint
6 January 2012
6 January 2012

Quick facts

Lead sponsorAlexion Pharmaceuticals, Inc.
PhasePhase 1, PHASE2
StatusCompleted
Study typeINTERVENTIONAL
Allocationnon randomized
Designsingle group
Maskingnone
Primary purposetreatment
Enrollment9
Start date25 April 2011
Primary completion6 January 2012
Estimated completion6 January 2012
Sites7 locations across France, United Kingdom, United States, Czechia

Drugs / interventions tested

Conditions studied

Sponsor

Alexion Pharmaceuticals, Inc. — full company profile →

Who can join

Adults 18 to 65, any sex, with Cholesterol Ester Storage Disease(CESD) or Lysosomal Acid Lipase Deficiency. Patients with the condition only — healthy volunteers not accepted.

Results — posted to ClinicalTrials.gov

Per-arm endpoint measurements with 95% confidence intervals where reported. Source: trial results section.

Number Of Participants Reporting TEAEs And Infusion-Related Reactions (IRRs) Primary · Screening up to Day 52

Safety and tolerability of sebelipase alfa was primarily assessed by monitoring the number of participants reporting treatment-emergent adverse events (TEAEs), including serious adverse events, and infusion-related reactions (IRRs). The number of participants who discontinued from the study due to a TEAE is also presented. An IRR was defined as any adverse event that occurred between the start of the infusion and 4 hours after completion of the infusion and was assessed by the Investigator as at least possibly related to study drug. A summary of serious and all other non-serious adverse events

TEAEs
GroupValue95% CI
Sebelipase Alfa 0.35 mg/kg1
Sebelipase Alfa 1 mg/kg3
Sebelipase Alfa 3 mg/kg3
SAEs
GroupValue95% CI
Sebelipase Alfa 0.35 mg/kg0
Sebelipase Alfa 1 mg/kg0
Sebelipase Alfa 3 mg/kg0
IRRs
GroupValue95% CI
Sebelipase Alfa 0.35 mg/kg0
Sebelipase Alfa 1 mg/kg0
Sebelipase Alfa 3 mg/kg0
TEAEs Leading to Study Discontinuation
GroupValue95% CI
Sebelipase Alfa 0.35 mg/kg0
Sebelipase Alfa 1 mg/kg0
Sebelipase Alfa 3 mg/kg0

Adverse events — posted to ClinicalTrials.gov

Time frame: Screening up to Day 52. Reporting threshold: 0%. Adverse-event reports describe events observed during the trial — not all are caused by the drug.

Sebelipase Alfa 0.35 mg/kg
Serious: 0/3 (0%)
Deaths:
Sebelipase Alfa 1 mg/kg
Serious: 0/3 (0%)
Deaths:
Sebelipase Alfa 3 mg/kg
Serious: 0/3 (0%)
Deaths:
Other adverse events (27 terms — click to expand)

ReactionSystemSebelipase Alfa 0.35 mg/kgSebelipase Alfa 1 mg/kgSebelipase Alfa 3 mg/kg
DiarrhoeaGastrointestinal disorders
HeadacheNervous system disorders
Abdominal discomfortGastrointestinal disorders
Abdominal distensionGastrointestinal disorders
Abdominal painGastrointestinal disorders
Abdominal pain lowerGastrointestinal disorders
DyspepsiaGastrointestinal disorders
FlatulenceGastrointestinal disorders
NauseaGastrointestinal disorders
Rectal haemorrhageGastrointestinal disorders
Catheter site related reactionGeneral disorders
Feeling hotGeneral disorders
Oedema peripheralGeneral disorders
Upper respiratory tract infectionInfections and infestations
Viral upper respiratory tract infectionInfections and infestations
HypercholesterolaemiaMetabolism and nutrition disorders
HypertriglyceridaemiaMetabolism and nutrition disorders
Bone painMusculoskeletal and connective tissue disorders
Musculoskeletal painMusculoskeletal and connective tissue disorders
MyalgiaMusculoskeletal and connective tissue disorders
PresyncopeNervous system disorders
SyncopeNervous system disorders
Urine odour abnormalRenal and urinary disorders
Breast swellingReproductive system and breast disorders
Vaginal haemorrhageReproductive system and breast disorders
Dermatitis contactSkin and subcutaneous tissue disorders
Night sweatsSkin and subcutaneous tissue disorders

Data from ClinicalTrials.gov NCT01307098 adverse events section.

Sponsor's own description

This was the first clinical study of SBC-102 (sebelipase alfa) for the treatment of Lysosomal Acid Lipase (LAL) Deficiency. It was an open-label dose escalation study in adult participants with liver dysfunction due to LAL Deficiency and was designed to examine 3 doses of sebelipase alfa. The targeted number for this study was 9 evaluable participants.

Publications & conference data

5 peer-reviewed publications reference this trial (live from Europe PMC):

  1. Clinical effect and safety profile of recombinant human lysosomal acid lipase in patients with cholesteryl ester storage disease.
    Balwani M, Breen C, Enns GM, Deegan PB, et al · · 2013 · cited 80× · PMID 23348766 · DOI 10.1002/hep.26289
  2. Sebelipase alfa over 52 weeks reduces serum transaminases, liver volume and improves serum lipids in patients with lysosomal acid lipase deficiency.
    Valayannopoulos V, Malinova V, Honzík T, Balwani M, et al · · 2014 · cited 61× · PMID 24993530 · DOI 10.1016/j.jhep.2014.06.022
  3. The role of sebelipase alfa in the treatment of lysosomal acid lipase deficiency.
    Erwin AL. · · 2017 · cited 18× · PMID 28804516 · DOI 10.1177/1756283x17705775
  4. Enzyme replacement therapy in lysosomal acid lipase deficiency (LAL-D): a systematic literature review.
    Bashir A, Tiwari P, Duseja A. · · 2021 · cited 5× · PMID 37181111 · DOI 10.1177/26330040211026928
  5. Annual Meeting of the Canadian Association for the Study of the Liver (CASL), the Canadian Network on Hepatitis C (CANHEPC) and the Canadian Association of Hepatology Nurses (CAHN) 2021 Abstracts.
    · 2021 · cited 1× · PMID 35991765 · DOI 10.3138/canlivj.4.2.abst

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