Status:

UNKNOWN

Efficacy of Rapamycin (Sirolimus) in the Treatment of Peutz-Jeghers Syndrome

Lead Sponsor:

Peking Union Medical College Hospital

Collaborating Sponsors:

Air Force General Hospital of the PLA

Cancer Institute and Hospital, Chinese Academy of Medical Sciences

Conditions:

Peutz-Jeghers Syndrome

Eligibility:

All Genders

Phase:

PHASE4

Brief Summary

A prospective non-randomized open label single arm clinical trial to examine the efficacy and safety of sirolimus in patients with Peutz-Jeghers Syndrome.

Detailed Description

PJS (Peutz-Jeghers Syndrome) is mostly caused by mutations in Lkb1 gene, which leads to an increased activity of mTOR pathway, thus making mTOR inhibitor (sirolimus) a promising candidate in treatment...

Eligibility Criteria

Inclusion

  • Patients are diagnosed with PJS.
  • Patients have gastrointestinal polyps related syndromes, including abdominal pain, abdominal distension, gastrointestinal bleeding, etc, with imageological examination suggesting intestinal obstruction or intussusception; or whose symptoms recur after previous digestive endoscopic treatment and surgery; or who are inappropriate or unwilling to accept the above treatment again and wish to receive pharmacotherapy.
  • Conventional treatment didn't work well in patients combined with PJS-related tumors.
  • Physical condition (ECGO): 0\~3
  • Organ function is good and biochemical indices meet the following conditions:
  • AST≤2.5×upper limit of normal value (ULN),
  • ALT≤2.5×upper limit of normal value (ULN),
  • Serum total bilirubin (TSB)≤1.5×upper limit of normal value (ULN),
  • Creatinine≤1.5×upper limit of normal value (ULN).
  • No other medications have been received for intestinal polyps within 3 months prior to the clinical trial.
  • Patients participate in the trial voluntarily and have signed the informed consent by the participant or his/her legal guardian.

Exclusion

  • Patients underwent a surgery within 2 weeks.
  • Patients may need emergency surgery in the near future.
  • Patients are allergic to any ingredient of rapamycin.
  • Patients suffer from a disease requiring immediate blood transfusion.
  • Patients suffer from any disease or condition that may impact implementation of the study or interpretation of the results. This type of diseases includes:
  • Known severe blood coagulation disorders
  • Known anemia that is not caused by intestinal polyps
  • Known hemoglobinopathy
  • Other gastrointestinal infectious diseases
  • Serious heart, liver, kidney and other concomitant diseases that may endanger lives
  • Patients are in pregnancy and lactation.
  • Alcohol or drug (such as aperient) abuse
  • Patients took part in another clinical trial that may influence this study.
  • The researchers believe that there are other unfavorable reasons for the patient to become a subject.

Key Trial Info

Start Date :

September 16 2018

Trial Type :

INTERVENTIONAL

Allocation :

ESTIMATED

End Date :

July 1 2022

Estimated Enrollment :

10 Patients enrolled

Trial Details

Trial ID

NCT03781050

Start Date

September 16 2018

End Date

July 1 2022

Last Update

January 23 2019

Active Locations (1)

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Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences

Beijing, China/Beijing, China, 100000