Quercetin in Patients With XIAP (X-linked Inhibitor of Apoptosis) Deficiency
A Pilot Study to Evaluate Safety and Tolerability, Pharmacokinetics, and Preliminary Efficacy of Quercetin in Patients With XIAP (X-linked Inhibitor of Apoptosis) Deficiency
Children's Hospital Medical Center, Cincinnati
12 participants
Feb 26, 2026
INTERVENTIONAL
Conditions
Summary
The purpose of the study is to evaluate safety and tolerability of oral quercetin in reducing inflammation in male patients with XIAP deficiency. Quercetin is a naturally occurring antioxidant that has many properties including the ability to decrease inflammation in other diseases.
Eligibility
Inclusion Criteria5
- Male patients
- Diagnosis of XIAP deficiency
- Patients ≥2 years of age
- Serum IL-18 ≥600 pg/mL (approximately 25% above the lab-defined upper limit of normal of 477 pg/mL)
- Able to take enteral medication
Exclusion Criteria8
- Renal failure requiring dialysis
- Total bilirubin >3 mg/dl and/or SGPT >300 at time of enrollment
- Patients receiving digoxin therapy, who are unable to discontinue treatment due to medical reasons
- Patients receiving fluoroquinolone therapy, who are unable to discontinue treatment due to medical reasons
- Patients who are at risk of pregnancy or fathering a child and are unable to use acceptable methods of birth control during the length of the study
- Patients who have received quercetin or any over the counter anti-oxidant supplementation within last 1 month
- Patients with unstable disease status or other medical issues requiring hospitalization or rapid escalation of medical care
- Participating in another therapeutic study for XIAP deficiency
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Interventions
Quercetin will be administered orally at a weight adjusted dose for a maximum total daily dose of 4000mg/day, divided twice a day. If the patient is 70 kg or more, the dose will automatically be assigned at the maximum dose of 4000mg/day. Patients will be instructed to mix quercetin with a small amount of yogurt or other preferred food for ingestion.
Locations(1)
View Full Details on ClinicalTrials.gov
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NCT07433621