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NCT02094222

Expanded Access Protocol for an Intermediate Size Population - RAVICTI for Byler Disease

NO LONGER AVAILABLE Last updated 18 February 2019
What this trial tests

trial testing RAVICTI in Byler Disease. No longer available.

Quick facts

Lead sponsorRobert Squires, Jr.
StatusNO LONGER AVAILABLE
Study typeEXPANDED_ACCESS

Drugs / interventions tested

Conditions studied

Sponsor

Robert Squires, Jr.

Who can join

Adults 130 Days to 21, any sex, with Byler Disease. Patients with the condition only — healthy volunteers not accepted.

Sponsor's own description

Byler Disease is the result of a homozygous missense (G308V) mutation in the ATP8B1 gene. The disease is typically manifest in the first year of life on the basis of complications of cholestasis; common presentations include jaundice, poor growth, bleeding related to vitamin K deficiency, and/or weak bones related to vitamin D deficiency. Early management of Byler Disease is directed at nutritional issues which tend to be responsive to medical intervention, unlike the pruritus/scratching which remains a devastating problem. Progressive liver disease develops in Byler Disease and can lead to cirrhosis and end-stage liver disease. This is an open label expanded access protocol of RAVICTI in children with Byler Disease. The primary hypothesis is that the administration of RAVICTI in these children is feasible, well tolerated and safe. It is also hypothesized that RAVICTI treatment leads to an improvement in biochemical markers of liver disease and it may ameliorates or prevents the development of scratching behavior as a manifestation of pruritus attributed to the liver disease.

Publications & conference data

2 peer-reviewed publications reference this trial (live from Europe PMC):

  1. Compassionate use of drugs and medical devices in the United States, the European Union and Japan.
    Tsuyuki K, Yano K, Watanabe N, Aruga A, et al · · 2016 · cited 13× · PMID 31245484 · DOI 10.1016/j.reth.2015.11.002
  2. Latent disease similarities and therapeutic repurposing possibilities uncovered by multi-modal generative topic modeling of human diseases.
    Kozawa S, Yokoyama H, Urayama K, Tejima K, et al · · 2023 · cited 1× · PMID 37123453 · DOI 10.1093/bioadv/vbad047

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