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A Study to Evaluate the Safety of Rosuvastatin in Children and Adolescents With Homozygous Familial Hypercholesterolemia

Phase 3
Completed
Conditions
Homozygous Familial Hypercholesterolemia (HoFH)
Interventions
Registration Number
NCT02434497
Lead Sponsor
AstraZeneca
Brief Summary

The purpose of the study is to evaluate the safety of Rosuvastatin in Children and Adolescents with Homozygous Familial Hypercholesterolemia.

Detailed Description

This is a long-term extension (LTE) to the randomized, double-blind, cross-over study of rosuvastatin 20 mg once daily (QD) versus placebo QD in children and adolescents (aged from 6 to \<18 years) with homozygous familial hypercholesterolemia (HoFH) (Study D3561C00004).

The study is designed to assess the long-term safety and tolerability of rosuvastatin 20 mg in pediatric patients with HoFH.

In this study all patients will receive rosuvastatin 20 mg QD. Investigators will also be permitted to titrate the dose of rosuvastatin from 20 to 40 mg per day if they feel it is warranted to more aggressively treat patients' elevated LDL-C levels. This up-titration will not be permitted in Asian patients. Pharmacokinetic data of the trough plasma exposure of rosuvastatin will also be assessed for the pediatric patients with HoFH taking a daily dose of rosuvastatin 40 mg.

The primary outcome measures to be assessed include 1) Adverse events, including:

* The frequency and severity of adverse events,

* Rate of discontinuations due to adverse events,

* Abnormal serum and urine laboratory values, electrocardiograms (ECGs), physical examinations, and vital signs; and 2) Assessments of growth.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
9
Inclusion Criteria
  1. Prior to any study related procedures being performed, provision of written informed consent from a parent/both parents or guardian and statement of assent from the child or adolescent (if required by Institutional Review Board [IRB] or Independent Ethics Committee [IEC] according to local regulations and guidelines). Study D3561C00004 participants who have had their 18th birthday (adults) will be required to provide written informed consent. Communication should take place between the Investigator, patient/guardian and child/adolescent to confirm understanding and required compliance with the requirements of the study.

  2. Male and female children and adolescents who were aged 6 to <18 years at the onset of Study D3561C00004 (even if they had their 18th birthday during that study) with:

    • Documentation of genetic testing confirming 2 mutated alleles of the LDL receptor gene locus; and/or

    • Documented untreated LDL C >500 mg/dL (12.9 mmol/L) and TG <400 mg/dL (4.5 mmol/L) and at least 1 of the following criteria:

      • Tendinous and/or cutaneous xanthoma prior to 10 years of age; or
      • Documentation of HeFH in both parents by:
    • genetic and/or

    • clinical criteria

  3. Negative pregnancy test (b human chorionic gonadotropin analysis) prior to baseline in females of child bearing potential:

    • Female patients of child bearing potential must adhere to a pregnancy prevention method (abstinence, chemical, or mechanical) during the study and 3 months following the last dose;
    • Male patients should refrain from fathering a child (including sperm donation) during the study and up to 3 months following the last dose; and
  4. Were taking study drug at the end of Study D3561C00004 and are willing to follow all study procedures including adherence to dietary guidelines, study visits, fasting blood draws, and compliance with study treatment regimens.

Exclusion Criteria
  1. History of statin inducted myopathy or serious hypersensitivity reaction to other HMG CoA reductase inhibitors (statins), including rosuvastatin, at Visit 1 of Study D3561C00004.
  2. Fasting serum glucose of >9.99 mmol/L (180 mg/dL) or glycosylated hemoglobin >9% during Study D3561C00004 or patients with a history of diabetic ketoacidosis within the past year.
  3. Uncontrolled hypothyroidism defined as thyroid stimulating hormone >1.5 times the upper limit of normal (ULN) at any time during Study D3561C00004.
  4. Evidence of active liver disease or hepatic dysfunction (except a confirmed diagnosis of Gilbert's disease) as defined as non-transient elevations of ALT or AST elevations ≥3 times the ULN or non-transient total bilirubin ≥2 times the ULN during the Study D3561C00004.
  5. Definite or suspected personal history or family history of clinically significant adverse drug reactions (ADRs), or hypersensitivity to drugs with a similar chemical structure to rosuvastatin as well as other statins.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Single ArmRosuvastatin 20mgOne treatment period for all patients (\<1 year and 10 months), with the possibility to up-titrate dose to 40 mg of rosuvastatin for non-Asian patients.
Primary Outcome Measures
NameTimeMethod
The Number of Participants Who Experianced Adverse Events and Serious Adverse Events96 weeks
Safety and Tolerability in Terms of Number of Participants Who Had Adverse Events, Discontinuations Due to Adverse Events96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Basophils/Leukocytes (%) >Upper Limite of Normal (ULN)96 weeks
Safety and Tolerability in Terms of Growth, Height96 weeks
Safety and Tolerability in Terms of Abnormalitites in Sexual Maturation96 weeks
Safety and Tolerability in Terms of Growth, Height SD-score (or Z-score)96 weeks

Height z-score is a dimensionless quantity derived by subtracting the population mean from the individual raw score, and then deviding the difference by the pouulation SD of the reference population. This indicates how many SDs and observation is above or below the general population mean.

Safety and Tolerability in Terms of Growth, Weight96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Alanine Aminotransferase (U/L) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Albumin (g/dL) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Aspartate Aminotransferase (U/L) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Bicarbonate (Mol/L) <LLN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Bicarbonate (Mol/L) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Ery. Mean Corpuscular HGB Concentration (g/dL) <LLN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Ery. Mean Corpuscular HGB (pg) <LLN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Ery. Mean Corpuscular Volume (fL) <LLN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Ery. Mean Corpuscular Volume (fL) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Erythrocytes (10^12/L) <LLN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Erythrocytes (10^12/L) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Hematocrit (%) <LLN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Glucose (mg/dL) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Lactate Dehydrogenase (U/L) <LLN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Phosphate (mg/dL) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Protein (g/dL) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Sodium (mmol/L) <LLN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Urate (mg/dL) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Hemoglobin (g/dL) <LLN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Leukocytes >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Lymphocytes/Leukocytes (%) <LLN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Lymphocytes/Leukocytes (%) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Monocytes/Leukocytes (%) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Platelets (10^9/L) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Blood Urea Nitrogen (mg/dL) <LLN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Chloride (mmol/L) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Serum Laboratory Values, Creatine Kinase (U/L) >ULN96 weeks
Safety and Tolerability in Terms of Abnormal Urine Laboratory Values, Urine Ketones96 weeks
Safety and Tolerability in Terms of Abnormal Urine Laboratory Values, Urine Occult Blood96 weeks
Safety and Tolerability in Terms of Abnormal Urine Laboratory Values, Urine Protein96 weeks
Safety and Tolerability in Terms of Abnormal ECG, Abnormalities96 weeks
Safety and Tolerability in Terms of Abnormal Physical Exams, Cardiovascular96 weeks
Safety and Tolerability in Terms of Abnormal Physical Exams, General Appearance96 weeks
Safety and Tolerability in Terms of Abnormal Physical Exams, Head and Neck96 weeks
Safety and Tolerability in Terms of Abnormal Physical Exams, Musculoskeletal/Extremities96 weeks
Safety and Tolerability in Terms of Abnormal Physical Exams, Skin96 weeks
Safety and Tolerability in Terms of Abnormal Vital Signs96 weeks
Secondary Outcome Measures
NameTimeMethod
Percent Change in Total Cholesterol (TC) From End of Placebo of D3561C00004 to the End of D356NC00001, Repeated Measures AnalysisUp to 22 months
Percent Change in Triglycerides (TG) From End of Placebo of D3561C00004 to the End of D356NC00001, Repeated Measures AnalysisUp to 22 months
Percent Change in Non-HDL-C From End of Placebo of D3561C00004 to the End of D356NC00001, Repeated Measures AnalysisUp to 22 months
Percent Change in LDL-C/HDL-C From End of Placebo of D3561C00004 to the End of D356NC00001, Repeated Measures AnalysisUp to 22 months
Percent Change in LDL-C From End of Placebo of D3561C00004 to the End of D356NC00001, Repeated Measures AnalysisUp to 22 months
Percent Change in HDL-C From End of Placebo of D3561C00004 to the End of D356NC00001, Repeated Measures AnalysisUp to 22 months
Percent Change in TC/HDL-C From End of Placebo of D3561C00004 to the End of D356NC00001, Repeated Measures AnalysisUp to 22 months
Percent Change in Non-HDL-C/HDL-C From End of Placebo of D3561C00004 to the End of D356NC00001, Repeated Measures AnalysisUp to 22 months
Percent Change in ApoB From End of Placebo of D3561C00004 to the End of D356NC00001, Repeated Measures AnalysisUp to 22 months
Percent Change in ApoA-1 From End of Placebo of D3561C00004 to the End of D356NC00001, Repeated Measures AnalysisUp to 22 months
Percent Change in ApoB/ApoA-1 From End of Placebo of D3561C00004 to the End of D356NC00001, Repeated Measures AnalysisUp to 22 months
Pharmacokinetic Profile in Terms of Trough Concentrations in Pediatric HoFH Taking a Daily Dose of Rosuvastatin 40mgUp to 22 months

Trial Locations

Locations (1)

Research Site

🇨🇳

Taipei City, Taiwan

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