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French Registry of Children Treated With Norditropin® for Short Stature Associated With Noonan Syndrome

Conditions
Noonan Syndrome
Interventions
Registration Number
NCT05308927
Lead Sponsor
Novo Nordisk A/S
Brief Summary

This is a non-interventional registry of children treated with Norditropin® for short stature due to Noonan Syndrome (NS). This study aims to provide data on long-term growth evolution and safety of Norditropin® as well as Health Related Quality of Life (HRQoL) data. This registry will include the entirety of children treated with Norditropin® for short stature due to NS over the inclusion period. The decision to initiate treatment with commercially available Norditropin® is made by the patient/parents/Legally Acceptable Representative (LAR) and the treating physician before and independently from the decision to include the patient in this study.

Detailed Description

Not available

Recruitment & Eligibility

Status
ENROLLING_BY_INVITATION
Sex
All
Target Recruitment
221
Inclusion Criteria
  • Patients with a clinical and/or genetic diagnosis of NS
  • Patients who are treated with Norditropin® (already treated or initiating) and who are followed in a participating center
  • The decision to initiate treatment with commercially available Norditropin® has been made by the patient/parents/Legally Acceptable Representative (LAR) and the treating physician before and independently from the decision to include the patient in this study
Exclusion Criteria
  • Patients/Parents/LAR opposed to the collection and processing of their children's medical data
  • Mental incapacity, unwillingness or language barriers precluding adequate understanding or cooperation

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Arm && Interventions
GroupInterventionDescription
Incident patientsNorditropinChildren initiated Norditropin® upon their inclusion in the study but independently from the decision to participate in this study
Prevalent patients - finished growth upon inclusionNorditropinChildren were already treated with Norditropin® before their inclusion in the study and finished their growth upon their inclusion. Data collected retrospectively from medical records
Prevalent patients - not finished growth upon inclusionNorditropinChildren were already treated with Norditropin® before their inclusion in the study and did not finish their growth upon inclusion. Data collected both retrospectively and prospectively
Primary Outcome Measures
NameTimeMethod
Change in height standard deviation scoreFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in standard deviation scores (SDS) ranging from -10 to +10

Secondary Outcome Measures
NameTimeMethod
Change in diastolic blood pressureFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in millimeters of Mercury (mm Hg)

Number of adverse drug reactions (ADR)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Count of events

Number of serious adverse events (SAE)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Count of events

Change in Haemoglobin A1c (HbA1c) levelFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in %

Change in fasting total cholesterolFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in mg/dl or g/L or mmol/L

Change in fasting triglyceridesFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in mg/dL or g/L or mmol/L

Number of serious adverse drug reactions (SADR)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Count of events

Number of adverse events (AE)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Count of events

EducationFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Number of participants is measured under the categorical variables - standard education, education with assistance and educational measures

School levelFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Number of participants is measured under the variables - preschool, elementary school, secondary school and high school

Educational measuresFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Number of participants is measured under the categorical variables - physiotherapy, psychomotricity, occupational therapy and speech therapy

Tanner stageFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured as units on scale. 3 sub-scales used - Pubic hair scale, Female breast development scale and Male external genitalia scale. Tanner stages range from Stage 1 to 5 with Tanner Stage 1 corresponding to the pre-pubertal form and Tanner Stage 5 corresponding to the final adult form

Change in pulse rateFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in beats per minute

Change in systolic blood pressureFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in mm Hg

Change in echocardiogram parameters (cm/s)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Change in Diastolic function (Ea and Aa) measured in centimeters per second (cm/s)

Change in fasting low-density lipoproteins (LDL) cholesterolFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in mg/dL or g/L or mmol/L

Change in Follicle-Stimulating Hormone (FSH) (female from 8 years old and male from 9 years old)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in International units per litre (UI/L) or mUI/mL

Frequency of injections of Norditropin® treatment per weekFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Count of events

Reasons for treatment modificationsFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in number of participants under categorical variable - safety (occurrence of ARs or AEs), growth too fast, growth too slow, patient's or parents/LAR choice; and other

Type and number of concomitant treatments linked to an adverse eventFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Count of events

Change in body mass index (BMI)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in kilogram per square metre (kg/m\^2)

Change in corrected QT-interval (Electrocardiogram)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in millisecond (msec)

Change in echocardiogram parameters (mm)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Change in Left ventricular interventricular septal maximum thickness in diastole and Left ventricular end diastolic diameter is measured in millimeter (mm)

Change in echocardiogram parameters (%)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Change in Left Ventricular Fractional Shortening (LVFS) and Left Ventricular Ejection Fraction (LVEF) is measured in percentage (%)

Change in complete blood count (CBC) (g/dL)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Change in Haemoglobin (Hb) measured in grams per deciLiter (g/dL)

Change in CBC (G/L)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Change in white blood cells, lymphocytes, monocytes and platelets measured in grams per liter (G/L)

Change in Insulin-like growth factor 1 (IGF-1) level (ng/ml)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in nanograms per milliliter (ng/ml)

Change in IGF-1 level (SDS)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in SDS

Change in fasting insulin levelFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in milligrams per deciliter (mg/dL) or milli-international units per litre (mUI/L)

Change in fasting blood glucose levelFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in mg/dL or g/L or millimoles per litre (mmol/L)

Change in fasting high-density lipoproteins (HDL) cholesterolFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in mg/dL or g/L or mmol/L

Change in Luteinizing Hormone (LH) (female from 8 years old and male from 9 years old)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in mg/mL

Change in oestradiol (female from 8 years old and male from 9 years old)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in pg/mL

Change in testosterone (female from 8 years old and male from 9 years old)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in ng/mL

Change in weightFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in SDS

Change in fasting total cholesterol/HDL ratioFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Ratio of fasting total cholesterol and HDL

Total duration of Norditropin® treatmentFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in years

Total cumulative dose of Norditropin® treatmentFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in mg/kg

Change in bone ageFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in year

Change in height velocity (cm/year)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in cm/year

Change in height velocity (SDS/year)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in SDS/year

Change in inhibin BFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in picograms per milliliter (pg/mL)

Change in Anti-Müllerian Hormone (AMH)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in milligrams per milliliter (mg/mL)

Change in dose of Norditropin® treatmentFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in milligram per kilogram per day (mg/kg/day)

Number of modifications (change in dosage/ temporary stop)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Count of events

Duration of temporary stopFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Measured in months

Type and number of concomitant treatmentsFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Count of events

Type and number of NS comorbiditiesFrom start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

Number of participants measured under categorical variables - Cardiac defects including pulmonary valve stenosis and hypertrophic cardiomyopathy, Orthopaedic issues (pectus, scoliosis) and Anorchia in males

Change in Health-Related Quality of Life (HRQoL) score (PedsQL)From start of Norditropin® treatment (day 0) until end of follow-up (up to 6 years)

PedsQL score used to assess the HRQoL, ranges from 1 to 100 (higher scores indicate better HRQoL)

Trial Locations

Locations (1)

Novo Nordisk Investigational Site

🇫🇷

Toulouse cedex 9, France

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