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Characterization of the Long-term Safety, Efficacy, and Pharmacodynamics Revestive® in the Management of Short Bowel Syndrome Pediatric Patients

Phase 4
Completed
Conditions
Short Bowel Syndrome
Interventions
Registration Number
NCT03562130
Lead Sponsor
Assistance Publique - Hôpitaux de Paris
Brief Summary

The purpose of this study is to evaluate if the treatment could maximize intestinal absorption, minimize the inconvenience of diarrhea, and avoid, reduce or eliminate the need for parenteral support (PS) to achieve normal growth, to avoid parenteral nutrition complications and to achieve the best possible quality of life for the patient

Detailed Description

The short bowel syndrome (SBS) may be defined as a severe malabsorption caused by reduction of intestinal absorptive surface following massive resection of the small intestine. Teduglutide (Revestive®) is an analog of glucagon-like peptide 2 (GLP-2), a naturally occurring hormone that regulates the functional and structural integrity of the cells lining the gastrointestinal tract. The aim of the treatment is to maximize intestinal absorption, minimize the inconvenience of diarrhea, and avoid, reduce or eliminate the need for parenteral support (PS) to achieve the best possible quality of life for the patient. The rationale for the use of Revestive® is based on data obtained, especially in the trial in SBS patients.

Treatment with 0.05 mg/kg/day was safe and well tolerated (no recorded side effects).

Patients remained stable despite substantial reduction in parenteral nutrition (PN) supply as evidenced by stable body weight and height, serum electrolytes, pancreatic enzymes and renal function tests.

Treatment was associated with:

* Reduced PN volume and calories delivered by 25 and 45% respectively with 20% of patients weaned off PN during the study period

* Increased Enteral Nutrition (EN) supply in volume and calories by 40 and 62% respectively

* Increased in plasma citrulline during the treatment period, but decreased after Teduglutide discontinuation The recommended dose of Revestive® in children and adolescents (aged 1 to 17 years) is the same as for adults (0.05 mg/kg body weight once daily).

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
25
Inclusion Criteria
  • Being aged from 2 to 18 years old included ;
  • Presenting less than 80 cm of residual small intestine with or without the terminal ileum, ileocecal valve and right colon or having less than 120 cm in case of Short Bowel Syndrome (SBS) caused by Hirschsprung disease;
  • Being stable on PN support (inability to significantly reduce PN intake for the last six months before inclusion) ;
  • Being dependent on PN for at least 2 years and enterally fed (oral or tube feeding) ;
  • Having a normal colonoscopy in the 12 months before screening for children with maintained colon (=SBS type 2 or 3) older than 12 years ;
  • Having signed the Informed consent form (or parents or legal representative for minor patients).
Exclusion Criteria
  • Having a major gastrointestinal surgical intervention like serial transverse enteroplasty or any other bowel lengthening procedure performed within 6 months of screening ;
  • Having a clinically significant untreated intestinal obstruction or active stenosis ;
  • Having an unstable absorption due to cystic fibrosis or known DNA abnormalities ;
  • Presenting a radiographic or manometric evidence of pseudo-obstruction or severe known dysmotility syndrome, including persistent, severe gastroschisis-related motility disorders ;
  • Having an unstable cardiac disease, congenital heart disease or cyanotic disease, with the exception of patients who had undergone ventricular or atrial septal defect repair ;
  • Having a history of cancer or clinically significant lymphoproliferative disease; excepted resected cutaneous basal or squamous cell carcinoma, or in situ non-aggressive and surgically resected cancer ;
  • Having participated in a clinical study using an experimental drug within 1 month or an experimental antibody treatment within 3 months prior to screening, or concurrent participation in any clinical study using an experimental drug that would affect the safety of teduglutide ;
  • Having already used native GLP-2 and glucagon-like peptide-1 analog or human growth hormone within 3 months prior to screening ;
  • Having already used oral or IV glutamine, octreotide, or dipeptidyl peptidase IV (DPP-IV) inhibitors within 3 months prior to screening ;
  • Having an active Crohn's disease which has been treated with biological therapy within the 6 months prior to screening ;
  • Having an intestinal polyposis;
  • Being, for female patient, both lactating and breast-feeding or having a positive pregnancy test during the screening period;
  • Refusing the follow the protocol requirements in terms of birth control ;
  • Being unable to follow the study procedures for any reason: psychological, geographical...
  • Hypersensitivity to the active substance or to any of the excipients listed in section 6.1 of Summary of Product Characteristics (SPC), or trace residues of tetracycline.
  • Active or suspected malignancy.
  • Patients with a history of malignancies in the gastrointestinal tract including the hepatobiliary system within the last five years.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
RevestiveTeduglutideRevestive® (teduglutide)is administered in children sub cutaneous injection at 0.05 mg/kg body weight once daily
Primary Outcome Measures
NameTimeMethod
Decrease in parenteral nutrition: Parenteral Nutrition/Resting Energy Expenditure (PN/REE)At week 24

Evaluate the efficacy of Revestive® treatment

Secondary Outcome Measures
NameTimeMethod
Stool weight/24hEvery 4 weeks up to week 48

to evaluate the impact of Revestive on Intestinal absorption

Ostomy output defined as stool balance testing, urine output and plasma citrullineup to week 48

Evaluate the impact of Revestive on ostomy flow

Change in stools consistency (Bristol stool chart)up to week 48

to evaluate the impact of Revestive on diarrhea

Percentage of lipid in stoolEvery 4 weeks up to week 48

to evaluate the impact of Revestive on Intestinal absorption

Change in days per week of Parenteral Nutrition (PN)up to week 48

Quantify the impact of Revestive on the number of perfusion in a week

Percentage of sodium in stoolEvery 4 weeks up to week 48

to evaluate the impact of Revestive on Intestinal absorption

Number of adverse eventsAt week 48

to evaluate the long term safety of Revestive

Change in body weightAt baseline, then at 6 and 12 months
Change in blood pressureAt baseline, then at 6 and 12 months
Change in number of stool per dayup to week 48

to evaluate the impact of Revestive on diarrhea

Ingesta (calorimetric measure)Every 4 weeks up to week 48

to evaluate the impact of Revestive on Intestinal absorption

Percentage of carbohydrate in stoolEvery 4 weeks up to week 48

to evaluate the impact of Revestive on Intestinal absorption

Change in heart rateAt baseline, then at 6 and 12 months
Percentage of nitrogen in stoolEvery 4 weeks up to week 48

to evaluate the impact of Revestive on Intestinal absorption

Endogenous GLP-2 rates (antibody ELISA)up to week 48

to evaluate the response rate of Revestive

Trial Locations

Locations (1)

Hôpital Necker - Enfants malades

🇫🇷

Paris, France

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