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RAD001 Therapy of Angiomyolipomata in Patients With TS Complex and Sporadic LAM

Phase 1
Completed
Conditions
Tuberous Sclerosis
Lymphangioleiomyomatosis
Interventions
Registration Number
NCT00457964
Lead Sponsor
Children's Hospital Medical Center, Cincinnati
Brief Summary

The purpose of this research study is to find out what effects RAD001 has on angiomyolipomas of a person with Tuberous Sclerosis Complex and to determine the safe dose of RAD001 without toxicity.

The hypothesis is that the drug will inhibit the growth of the angiomyolipomas and possibly even cause regression.

Detailed Description

Tuberous sclerosis complex is a genetic disorder with a birth incidence of approximately one in six thousand. Angiomyolipomas occur in eighty percent of persons with TS, and in up to 60% of persons with LAM. They are fatty tumors that often occur in kidney or liver as well as other parts of the body. They can grow and cause damage to surrounding kidney tissue and even renal failure. They may also leak blood causing potentially life-threatening hemorrhage.

Laboratory studies have shown that RAD001 suppresses the chemical that cause tumors to grow in tuberous sclerosis and sporadic LAM.

The primary goal of this study is to evaluate the clinical effectiveness of RAD001 in a 24 month trial. Although the primary goal is to determine if the drug RAD001 has effects on angiomyolipomas, other diseases associated with tuberous sclerosis will be monitored too, specifically any change in involvement of your brain or lungs with tuberous sclerosis. The use of RAD001 to treat angiomyolipomas in tuberous sclerosis or sporadic LAM is considered experimental

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
36
Inclusion Criteria
  • Diagnosed with angiomyolipomas and have either Tuberous Sclerosis Complex or lymphangioleiomyomatosis
  • Angiomyolipoma one centimeter or greater in largest diameter
  • Between the ages of 18 and 65 years old.
  • If female, documentation of negative pregnancy test prior to enrollment and, where applicable, use of appropriate non-estrogen containing birth control contraceptive regimen while on study.
  • Adequate renal function (creatinine < 3 mg/dl)
Exclusion Criteria
  • Pregnant or lactating women
  • Continuous requirement for supplemental oxygen
  • Surgery within past 2 months
  • Use of an investigational drug within last 30 days.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Administration of RAD001RAD001-
Primary Outcome Measures
NameTimeMethod
Patients will be considered responders if their angiomyolipoma volume decreases by thirty percent or more from baseline12 months
Secondary Outcome Measures
NameTimeMethod

Trial Locations

Locations (1)

Cincinnati Children's Hospital Medical Center

🇺🇸

Cincinnati, Ohio, United States

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