Follow-up of Prader Willi Syndrome Infants Treated by Oxytocin and Comparison With Not-treated Infants.

Study Overview
=================
Brief Summary
-----------------
The objective of this study is to collect data on tolerance and effects of early treatment with oxytocin in children with Prader Willi Syndrome aged from 3 to 4 years and to compare these infants with not treated age-matched infants with Prader Willi Syndrome.

Detailed Description
-----------------
In accordance with recommendations of regulatory authorities, we want to collect long term data of patients treated with oxytocin before the age of 6 months. Moreover clinical observations of these infants support long term effects on communication skills, global development and behaviour.

Official Title
-----------------
Long Term Evaluation of Infants Aged From 3 to 4 Years Old Included in the Ancient Study (Repeated Administrations of Oxytocin in Infants With Prader Willi Syndrome Aged From 0 to 6 Months) and Comparison With Not Treated and Age-matched Prader Willi Syndrome Infants (OT2SUITE)

Conditions
-----------------
Prader-Willi Syndrome

Intervention / Treatment
-----------------
* Drug: Oxytocin
* Other: Control


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Infants with Prader Willi Syndrome (genetic diagnosis confirmed) For treated group : infant included in the ancient study For not treated group: infant never treated with oxytocin Exclusion Criteria: Subject involved in another search including an exclusion period still in progress at the time of inclusion. Impossibility to give parents or legal guardian informed information No coverage by a Social Security scheme Refusal of parents or legal representative to sign consent. If a patient has a contraindication to Magnetic resonance imaging, it may be included in the study but Magnetic resonance imaging will not be performed.

Ages Eligible for Study
-----------------
Minimum Age: 3 Years
Maximum Age: 4 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Design Details

Primary Purpose: Treatment
Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Oxytocin<br>Groups of children with Prader Willi Syndrome treated by oxytocin for 7 days during their first 6 months of life. | Drug: Oxytocin<br>* Infant included in the ancient study (repeated administrations of oxytocin in infants with Prader Willi Syndrome aged from 0 to 6 months)<br>|
| Experimental: Control<br>Groups of children with Prader Willi Syndrome not treated by oxytocin for 7 days during their first 6 months of life. | Other: Control<br>* Not treated.<br>|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Evaluation of communication skills. | Assessed by Vineland-II scale. | Day 1 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Evaluation of adaptative behavior composite and 3 domains : Daily living skills, Socialization, Motor skills. | Assessed by Vineland-II scale. | Day 1 | 
| Evaluation of behavioral troubles. | Assessed by Child Behaviour Check List questionnaire. | Day 1 | 
| Evaluation of global development. | Assessed by Bayley Scales of Infant and Toddler Development. | Day 2 and 3 | 
| Evaluation of orality and eating behaviour. | Assessed by: A questionnaire on eating behavior. An oral evaluation, which combines a clinical examination carried out by the reference center physician, and the assessment of eating behavior during the meal. The fluoroscopy of swallowing. | Day 2 | 
| Evaluation of brain activity. | Assessed by a morphological Magnetic resonance imaging, a resting functional Magnetic resonance imaging. | Day 3 | 
| Evaluation of plasma levels of ghrelin and other peptides involved in feeding behaviour or energy metabolism. | Circulating levels of acylated and non-acylated ghrelin and some peptides and neuropeptides involved in appetite regulation (leptin, cortisol, insulin, Glucagon like peptide-1, pancreatic polypeptide, orexin A, alpha-melanocyte stimulating hormone...). | Day 1 | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Prader-Willi, Oxytocin, Communication skills, Oral skills, Brain activity, Hormonal disease