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Phase 2 N=5 Treatment

Safety, Pharmacokinetic (PK) and Pharmacodynamic (PD) Study of Repeat Doses of Inhaled Nemiralisib in Patients With APDS/PASLI

Activated PI3K-delta Syndrome

Enrolled (actual)
5
Serious AEs
0.0%
Results posted
Jun 2021
Primary outcome: Primary: Number of Participants With Any Serious Adverse Events (SAEs) and Any Non-serious Adverse Events (Non-SAEs) — 5; 0 Participants

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
Nemiralisib (Drug)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
GlaxoSmithKline
Primary completion
Jun 2020

Outcome Measures

OutcomeResultp-value
PRIMARY
Number of Participants With Any Serious Adverse Events (SAEs) and Any Non-serious Adverse Events (Non-SAEs)
5; 0
PRIMARY
Change From Baseline in Diastolic Blood Pressure (DBP) and Systolic Blood Pressure (SBP)
1.0; 4.0; 8.5; 2.0; 17.0; -1.0
PRIMARY
Change From Baseline in Pulse Rate
3.2; 2.0; 6.8; 4.8; 7.0
PRIMARY
Change From Baseline in Respiratory Rate
0.2; 0.8; 1.5; 1.0; 2.0
PRIMARY
Change From Baseline in Body Temperature
0.10; 0.17; 0.07; 0.17; 0.50
PRIMARY
Change From Baseline in Electrocardiogram (ECG) Mean Heart Rate
-1.0; -0.8; 5.3; 1.0
PRIMARY
Change From Baseline in PR Interval, QRS Duration, Uncorrected QT Interval, QT Corrected Interval-Fredericia Interval (QTcF) and QTc Corrected by Bazett's Formula (QTcB)
-2.2; -4.8; -3.3; -6.0; -5.6; -4.5
PRIMARY
Change From Baseline in Clinical Chemistry Parameters: Alanine Aminotransferase (ALT), Alkaline Phosphatase (ALP) and Aspartate Aminotransferase (AST)
1.0; 0.8; 0.0; 1.3; -0.8; 0.8
PRIMARY
Change From Baseline in Clinical Chemistry Parameters : Albumin and Total Protein
2.6; 1.3; 2.8; 2.0; 4.0; 1.3
PRIMARY
Change From Baseline Values in Clinical Chemistry Parameters: Sodium, Potassium, Calcium, Glucose and Urea
-0.2; 0.5; -0.8; 0.0; 0.24; 0.30
PRIMARY
Change From Baseline Values in Clinical Chemistry Parameters: Direct Bilirubin, Total Bilirubin and Creatinine
-1.0; -1.8; -0.5; -0.5; -1.8; -3.0
PRIMARY
Change From Baseline Values in Clinical Chemistry Parameter: C-Reactive Protein
0.56; 0.75; 2.75; 3.90
PRIMARY
Change From Baseline for Hematology Parameters: Basophil, Eosinophils, White Blood Cells (WBC), Lymphocytes, Neutrophils, Monocytes and Platelets
-0.002; 0.008; 0.008; 0.013; 0.108; 0.088
PRIMARY
Change From Baseline for Hematology Parameter: Hemoglobin
10.0; 8.3; 13.5; 13.3
PRIMARY
Change From Baseline for Hematology Parameter: Hematocrit
0.0320; 0.0333; 0.0518; 0.0465
PRIMARY
Change From Baseline in Hematology Parameter: Mean Corpuscular Volume (MCV)
1.00; 2.33; 2.02; 1.03
PRIMARY
Change From Baseline in Hematology Parameter: Mean Corpuscular Hemoglobin (MCH)
0.10; -0.15; -0.43; -0.47
PRIMARY
Change From Baseline for Hematology Parameter: Red Blood Cell Count
0.304; 0.280; 0.488; 0.485
PRIMARY
Change From Baseline in Forced Expiratory Volume in 1 Second (FEV1)
0.226; 0.290; -0.165; 0.016; -0.002
SECONDARY
Plasma Concentration Following Administration of NEMI
0.00; 0.00; 0.00; 467.00; 1524.70; 658.27

Summary

This is an open-label study conducted to investigate safety, pharmacokinetics and pharmacodynamics of repeat doses of inhaled nemiralisib (NEMI) in participants with activated phosphoinositide 3-kinase (PI3K) delta syndrome /p110 delta-activating mutation causing senescent T Cells, lymphadenopathy and immunodeficiency (APDS/PASLI)

Eligibility Criteria

Inclusion Criteria

  • Male and female subjects aged 18 or older at the time of signing the informed consent.
  • Patients with a clinical phenotype consistent with APDS, including a history of recurrent (frequency greater than would be expected in an immunocompetent individual) ear, sinus or pulmonary infections, and who have a known type 1 APDS-associated genetic PI3K delta mutation (i.e. E1021K, N334K, E525K and C416R).
  • Body weight >=45 kilograms (kg) and body mass index (BMI) >=18 kg/square meter (m^2) (inclusive)
  • Male subject. Male subjects with female partners of child bearing potential must comply with the following contraception requirements from the time of first dose of study medication until completion of the follow-up telephone call at 1-2 weeks from last dose. Vasectomy with documentation of azoospermia. Male condom plus partner use of one of the following contraceptive options: 1. Contraceptive subdermal implant 2. Intrauterine device or intrauterine system 3. Combined estrogen and progestogen oral contraceptive, 4. Injectable progestogen 5. Contraceptive vaginal ring 6. Percutaneous contraceptive patches. This is an all inclusive list of those methods that meet the GSK definition of highly effective: having a failure rate of less than 1% per year when used consistently and, correctly and, when applicable, in accordance with the product label. For non-product methods (e.g. male sterility), the investigator determines what is consistent and correct use. The GlaxoSmithKline (GSK) definition is based on the definition provided by International Conference on Harmonisation (ICH).
  • Female subject. A female subject is eligible to participate if she is not pregnant (as confirmed by a negative urine human chorionic gonadotrophin [hCG] test), not lactating, and at least one of the following conditions applies: 1. Non-reproductive potential defined as: Pre-menopausal females with one of the following: Documented tubal ligation, Documented hysteroscopic tubal occlusion procedure with follow-up confirmation of bilateral tubal occlusion, Hysterectomy, Documented Bilateral Oophorectomy. Postmenopausal defined as 12 months of spontaneous amenorrhea (in questionable cases a blood sample with simultaneous follicle stimulating hormone [FSH] and estradiol levels consistent with menopause). Females on hormone replacement therapy (HRT) and whose menopausal status is in doubt will be required to use one of the highly effective contraception methods if they wish to continue their HRT during the study. Otherwise, they must discontinue HRT to allow confirmation of post-menopausal status prior to study enrolment. 2. Reproductive potential and agrees to follow one of the options listed in the Modified List of Highly Effective Methods for Avoiding Pregnancy in Females of Reproductive Potential (FRP) from 30 days prior to the first dose of study medication and until completion of the follow-up telephone call at 1-2 weeks from last dose.
  • Capable of giving signed informed consent as described in protocol which includes compliance with the requirements and restrictions listed in the consent form and in the protocol.

Exclusion Criteria

  • Alanine aminotransferase (ALT) >2xupper limit normal (ULN) and bilirubin >1.5xULN (isolated bilirubin >1.5xULN is acceptable if bilirubin is fractionated and direct bilirubin 450 milliseconds (msec) or QTc > 480 msec in subjects with Bundle Branch Block
  • A subject with a clinical abnormality or laboratory parameter(s) which is/are not specifically listed in the inclusion or exclusion criteria, outside the reference range for the population being studied may be included only if the investigator, in consultation with the Medical Monitor if required, agree and document that the finding is unlikely to introduce additional risk factors and will not interfere with the study procedures.
  • Regular or chronic treatment with strong inhibitors of Cytochrome P450 (CYP) 3A4 and/or CYP2D6 (this includes some anti-epil
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT02593539). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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