Ignite Creation Date:
2024-07-17 @ 11:44 AM
Last Modification Date:
2024-10-26 @ 3:34 PM
Study NCT ID:
NCT06497829
Status:
RECRUITING
Last Update Posted:
2024-07-12
First Post:
2024-07-01
Brief Title:
Geranylgeraniol Supplementation in Patients With Mevalonate Kinase Deficiency
Sponsor:
University Hospital Motol
Organization:
University Hospital Motol
Study Overview
Official Title:
Geranylgeraniol Supplementation in Patients With Mevalonate Kinase Deficiency MVK Deficiency hyperIgD Syndrome
Status:
RECRUITING
Status Verified Date:
2024-07
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
Brief Summary:
Mevalonate kinase deficiency HyperIgD syndrome HIDS is an inborn error of immunity caused by a block in the mevalonate pathway The subsequent lack of isoprenoids with antiinflammatory properties might contribute to the autoinflammatory nature of the disease
A pilot study aims to verify the safety and efficacy of the dietary supplement Geranylgeraniol aiming at a mitigation of isoprenoids deficiency caused by the above-mentioned block in the MVK pathway
A pilot study aims to verify the safety and efficacy of the dietary supplement Geranylgeraniol aiming at a mitigation of isoprenoids deficiency caused by the above-mentioned block in the MVK pathway
Detailed Description:
Mevalonate kinase MVK deficiency a rare autosomal recessive disease significantly impacts metabolism and immunity leading to mevalonic aciduria in severe cases and hyper-IgD syndrome HIDS in partial deficiency These conditions arise due to disruptions in the mevalonate pathway which is an essential metabolic pathway responsible for the synthesis of non-sterol isoprenoids and other molecules The resulting metabolic blockade triggers autoinflammatory responses primarily due to deficient isoprenoid intermediates such as geranylgeranyl pyrophosphate GGPP
This study evaluates the safety and efficacy of dietary geranylgeraniol GG supplementation in patients with genetically confirmed mevalonate kinase deficiency
The pilot study consists of a month of a pre-supplementation period to monitor the level of inflammation and clinical status in patients with MVK deficiencyHIDS
This period is followed by 3 months of supplementation of 150mg of GeranylGraniol GG Pure Extendlife Natural Products containing GG gold30 Annatto Extract 500 mg capsule 30 geranylgeraniol 150 mg per capsule
This study evaluates the safety and efficacy of dietary geranylgeraniol GG supplementation in patients with genetically confirmed mevalonate kinase deficiency
The pilot study consists of a month of a pre-supplementation period to monitor the level of inflammation and clinical status in patients with MVK deficiencyHIDS
This period is followed by 3 months of supplementation of 150mg of GeranylGraniol GG Pure Extendlife Natural Products containing GG gold30 Annatto Extract 500 mg capsule 30 geranylgeraniol 150 mg per capsule
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None