Is There a Sensibility Increased in the Growth Hormone at Child With Prader-Willi Syndrome?
- Conditions
- Prader-Willi SyndromeGrowth Hormone Deficiency
- Interventions
- Drug: Growth hormone (Genotonorm® or Omnitrope®)Procedure: biopsyProcedure: DEXA, blood tests, H.G.P.O, osseous age.
- Registration Number
- NCT01298180
- Lead Sponsor
- University Hospital, Toulouse
- Brief Summary
The purpose of this study is to estimate the sensibility at the growth hormone in vivo at the children presenting a Prader-Willi syndrome (SPW) in comparison with children presenting a deficit in growth hormone (GHD).
- Detailed Description
Estimate the sensibility at the growth hormone in vivo at the children presenting a Prader-Willi syndrome (SPW) in comparison with children presenting a deficit in growth hormone (GHD) by the measure of the circulating rates of IGF-I under treatment.
Recruitment & Eligibility
- Status
- COMPLETED
- Sex
- All
- Target Recruitment
- 111
-
SPW and SPW-B :
- Female or male child of age > or = 1 year
- Child naïve of treatment by GH and that must begin a treatment with GH
- Child covered by a national insurance scheme or an equivalent
- Signature of the informed consent by one of both holders of the parental authority
-
GHD :
- Female or male child of age > or = 1 year
- Child paired for the age (+/-on 1 year) and for the sex with regard to the group SWP
- Child presenting a GH* deficiency defined by :
Growth criteria of size (size) < 2 DS) Criteria of speed of growth (speed of growth < 1 DS over the last year) 2 tests of pharmacological stimulation of GH with peak GH max < 20 mUI
- Child naïve of treatment by GH and that must begin a treatment with GH
- Child covered by a national insurance scheme or an equivalent
- Signature of the informed consent by one of both holders of the parental authority * The deficit in GH can be isolated or associated with one or several other hormonal deficits: deficit in TSH, deficit in ACTH, deficit in LH-FSH, deficit in prolactin. The child GHD can thus receive other treatments associated with the growth hormone.
-
T : controls
- Female or male child of age > or = 1 year
- Child paired for the age (+/-on 1 year) and for the sex with regard to the group SWP
- Child hospitalized at the hospital of the children of the University Hospital of Toulouse for a programmed surgical operation
- Child covered by a national insurance scheme or an equivalent
- Signature of the informed consent by one of both holders of the parental authority
-
SPW-GH-B :
- Female or male child of age > or = 1 year
- Child hospitalized for a programmed surgical operation
- Child covered by a national insurance scheme or an equivalent
- Child treated with GH for at least 3 month
- Signature of the informed consent by one of both holders of the parental authority
-
SPW and GHD
- Child presenting a contraindication to the taking of growth hormone :
- Growth cartilage welded
- Tumoral pathology in process of evolution
- Corticosteroid therapy (not substitute)
- Allergy known about solvent
- Badly balanced diabetes
- Child presenting a hypersensitivity to the active principle or to one of the excipients of Genotonorm ® or Omnitrope ®
- Child presenting a severe obesity (defined by a report weight / size > 200 %)
- Child presenting clinical signs ENT (snores associated with a hypertrophy of the adenoids vegetations and\or the tonsils)
- Child presenting clinical signs evoking a respiratory illness of the sleep (night-respiratory snores, respiratory breaks during the sleep)
-
SPW-B:
- Child presenting a hypersensitivity to the local anaesthetic with amide connecion
- Child presenting a hypersensitivity to the components of the bandage Emlapatch®
- Child presenting a hypersensitivity to one of the components of the lidocaïne aguettant without conservative®
- Child presenting a porphyria
- Child presenting a congenital methemoglobinemia
- Child presenting a contraindication to Meopa : patients requiring a ventilation in pure oxygen, intracranial High blood pressure, Any change of the state of consciousness, preventing the cooperation of the patient, Pneumothorax, Bubbles of emphysema, Gaseous embolism, Accident of dive, abdominal gaseous Distension, Patient having received recently an ophthalmic gas (SF6, C3F8, C2F6) used in the eye surgery as long as persists a bubble of gas inside the eye and at least during a period of 3 months. Grave postoperative complications can arise in touch with the increase of the pressure intraocular, facial Traumatism interesting the region of application of the mask
-
T : controls
- Chronicle pathology in which an abnormality of growth would be involved
- Other hormonal abnormalities
- Children receiving a treatment on the long range, corticosteroid therapy in particular, being able to interfere with the sensibility to GH or to the insulin
- Holder of the parental authority under supervision, guardianship or under protection of justice
- Participation in another study simultaneously at this one
Study & Design
- Study Type
- INTERVENTIONAL
- Study Design
- PARALLEL
- Arm && Interventions
Group Intervention Description SPW Growth hormone (Genotonorm® or Omnitrope®) Children presenting a Prader-Willi Syndrome SPW DEXA, blood tests, H.G.P.O, osseous age. Children presenting a Prader-Willi Syndrome GHD Growth hormone (Genotonorm® or Omnitrope®) Patient deficient in Growth Hormone GHD DEXA, blood tests, H.G.P.O, osseous age. Patient deficient in Growth Hormone SPW-B Growth hormone (Genotonorm® or Omnitrope®) Patient with Prader-Willi Syndrome who has Biopsy SPW-B DEXA, blood tests, H.G.P.O, osseous age. Patient with Prader-Willi Syndrome who has Biopsy T biopsy Patient Control SPW-GH-B biopsy Patient with Prader-Willi Syndrome taking growth Hormone and who has biopsy SPW-B biopsy Patient with Prader-Willi Syndrome who has Biopsy
- Primary Outcome Measures
Name Time Method Measure of the circulating rates of IGF-I under treatment. 1 year (M12)
- Secondary Outcome Measures
Name Time Method Measure of the circulating rate of IGFBP-3, GHBP, ghrelin and apelin. 1 year (M12) Measure of physical composition's variation. 1 year (M12) Measure of blood sugar level, H.G.P.O., and hyperglycaemia. 1 year (M12) Measure of the sensibility at the growth hormone in vitro, on fibroblasts and adipocytes obtained by biopsy. 1 year (M12)
Trial Locations
- Locations (26)
CHU Nice Hôpital Archet 2 - 151 route Saint Antoine de Ginestière
🇫🇷Nice, France
CHU La Rochelle Service de Pédiatrie - Rue du Dr Schweitzer
🇫🇷La Rochelle, France
CHU Saint-Etienne Hôpital Nord Service de Pédiatrie
🇫🇷Saint-etienne, France
CHU Montpellier Hôpital Arnaud de Villeneuve - 371 ave du doyen Gaston Giraud
🇫🇷Montpellier, France
CHU Strasbourg Hôpital Haute-Pierre - Avenue Molière
🇫🇷Strasbourg, France
CHU Limoges Hôpital Mère Enfant Service Pédiatrie - 8, ave du Larrey
🇫🇷Limoges, France
CHG Avignon - 305, rue Raoul Follereau
🇫🇷Avignon, France
CHU Lyon Hôpital Debrousse service Pédiatrie
🇫🇷Lyon, France
CHU Lorient Hôpital du Scorff Pôle Femme Mère Enfant - Rue Guiguen
🇫🇷Lorient, France
AP-HM Hôptal La Timone Service de Pédiatrie Mutidisciplinaire
🇫🇷Marseille, France
CHU Nantes Hôpital Mère Enfant Service de Pédiatrie
🇫🇷Nantes, France
CHU Poitiers Service de Pédiatrie - Rue de la Miléterie
🇫🇷Poitiers, France
CHU Reims Service de Pédiatrie - 47, rue Cognacq-Jay
🇫🇷Reims, France
CHU Besançon Hôpital Saint Jacques - 2 Place Saint Jacques
🇫🇷Besancon, France
CHU Angers - 4 rue Larrey
🇫🇷Angers, France
CHU Amiens Hôpital Nord Service Pédiatrie - Place Victor Pauchet
🇫🇷Amiens, France
CHU Bordeaux Hôpital Pellegrin Service endocrinologie de l'enfant - Place Amélie Raba Léon
🇫🇷Bordeaux, France
CHU Dijon Service de pédiatrie - 2, Bd Maréchal de lattre de Tassigny
🇫🇷Dijon, France
CHU Brest Département de Pédiatrie - 5, ave Foch
🇫🇷Brest, France
CHU Grenoble Service de pédiatrie - BP 217
🇫🇷Grenoble, France
CHRU Lille Hôpital Jeanne de Flandre service de Pédiatrie
🇫🇷Lille, France
AP-HP Hôpital Necker Enfants Malades Service d'endocrinologie pédiatrique - 149 route de Sèvres
🇫🇷Paris, France
CHRU Tours Centre de Pédiatrie Gatien de Clocheville
🇫🇷Tours, France
Hôpital d'Enfants - Rue Morvan
🇫🇷Vandoeuvre Les Nancy, France
CHU Rouen Hôpital Nicolle - 1, rue de Germont
🇫🇷Rouen, France
CHU Toulouse Hôpital des Enfants Service d'endocrinologie - 330 ave de Grande Bretagne
🇫🇷Toulouse, France