[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-study-detail:100554341":3},{"organization":4,"outcomesModule":7,"designInfo":19,"detailedDescription":25,"studyPopulation":18,"armGroups":26,"interventions":33,"overallOfficials":39,"centralContacts":43,"locations":53,"responsibleParty":70,"collaborators":73,"id":76,"slug":18,"hasResults":77,"nctId":78,"briefTitle":79,"officialTitle":80,"acronym":18,"eligibilityCriteria":81,"healthyVolunteers":82,"sex":83,"minAge":84,"maxAge":18,"enrollmentInfo":85,"targetDuration":18,"studyType":88,"phases":89,"briefSummary":90,"conditions":91,"keywords":93,"overallStatus":55,"whyStopped":18,"lastUpdateSubmitDate":97,"lastUpdatePostDateStruct":98,"startDateStruct":101,"completionDateStruct":103,"leadSponsor":105,"locationsCount":106},{"fullName":5,"class":6},"University Hospital, Motol","OTHER",{"primaryOutcomes":8,"secondaryOutcomes":13,"otherOutcomes":18},[9],{"measure":10,"description":11,"timeFrame":12},"Change in inflammatory parameters","Monitoring inflammatory markers before and after intervention - CRP (C reactive protein) and SAA (serum amyloid A) levels will be measured before and after the intervention (time 0 and time 3 months of GG use).\n\nThese values tend to be elevated in patients with MVK deficiency due to their inflammatory status compared to normal values defined in clinical laboratories (UHMotol CRP \\\u003C 8 mg\u002Fl, SAA \\\u003C 10 mg\u002Fl).\n\nThe expected outcome would result in a decrease in inflammatory markers.","3 monhts of intervention",[14],{"measure":15,"description":16,"timeFrame":17},"Change in the clinical status of patients","Monitoring of general clinical status and defined parameters - general condition, fatigue, fever, skin manifestations, abdominal pain, and headache using a questionnaire. The questionnaire is designed for daily monitoring of the parameters mentioned above. The scale of the questionnaire is from 1 to 10, with 1 being the best, normal condition, and 10 being the worst degree of disability.","3 months",null,{"allocation":20,"interventionModel":21,"interventionModelDescription":22,"primaryPurpose":6,"observationalModel":18,"timePerspective":18,"maskingInfo":23},"NA","SINGLE_GROUP","Patients with MVK deficiency compared to healthy controls and to subjects on statin therapy (statins target the same metabolic pathway)",{"masking":24,"maskingDescription":18,"whoMasked":18},"NONE","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).\n\nThis study evaluates the safety and efficacy of dietary geranylgeraniol (GG) supplementation in patients with genetically confirmed mevalonate kinase deficiency.\n\nThe pilot study consists of a month of a pre-supplementation period to monitor the level of inflammation and clinical status in patients with MVK deficiency(HIDS).\n\nThis period is followed by 3 months of supplementation of 150mg of GeranylGraniol (GG Pure (Extendlife Natural Products) containing GG gold®30 Annatto Extract, 500 mg capsule (30% geranylgeraniol, 150 mg per capsule).",[27],{"label":28,"type":29,"description":30,"interventionNames":31},"patients with MVK deficiency","EXPERIMENTAL","patients fulfilling inclusion criteria",[32],"Dietary Supplement: geranylgeraniol",[34],{"type":35,"name":36,"description":37,"armGroupLabels":38,"otherNames":18},"DIETARY_SUPPLEMENT","geranylgeraniol","GG Pure (Extendlife Natural Products) containing GG gold®30 Annatto Extract, 500 mg capsule, use for 3 months, once daily",[28],[40],{"name":41,"affiliation":5,"role":42},"Anna Šedivá, Mgr","PRINCIPAL_INVESTIGATOR",[44,49],{"name":45,"role":46,"phone":47,"phoneExt":18,"email":48},"Anna Šedivá, Prof","CONTACT","+420603166112","anna.sediva@fnmotol.cz",{"name":50,"role":46,"phone":51,"phoneExt":18,"email":52},"Eva Aljamal, Mgr","+420601354311","eva.aljamal@fnmotol.cz",[54],{"facility":5,"status":55,"city":56,"state":18,"zip":57,"country":58,"countryCode":18,"cosmosGeoPoint":59,"geoPoint":64,"contacts":65},"RECRUITING","Prague","15006","Czechia",{"type":60,"coordinates":61},"Point",[62,63],14.42076,50.08804,{"lat":63,"lon":62},[66,68],{"name":45,"role":46,"phone":67,"phoneExt":18,"email":48},"+420 603166112",{"name":50,"role":46,"phone":69,"phoneExt":18,"email":52},"+420 601354311",{"type":42,"investigatorFullName":71,"investigatorTitle":72,"investigatorAffiliation":5,"oldNameTitle":18,"oldOrganization":18},"Anna Sediva","Prof. Anna Sediva, MD, PhD",[74],{"name":75,"class":6},"Washington University School of Medicine","100554341",false,"NCT06497829","Geranylgeraniol Supplementation in Patients With Mevalonate Kinase Deficiency","Geranylgeraniol Supplementation in Patients With Mevalonate Kinase Deficiency, MVK Deficiency (hyperIgD Syndrome)","Inclusion Criteria:\n\n* Patients older than 12 years with genetically confirmed MVK deficiency\n* Overall good clinical status apart from the clinical presentation of the MVK deficiency\n* Normal liver tests\n* Compliance with the study protocol\n\nExclusion Criteria:\n\n• Children below 12 years",true,"ALL","12 Years",{"count":86,"type":87},6,"ESTIMATED","INTERVENTIONAL",[20],"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.\n\nA 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.",[92],"Mevalonate Kinase Deficiency",[94,95,96,36],"immunoglobulin D","mevalonate kinase","isoprenoids","2024-07-09",{"date":99,"type":100},"2024-07-12","ACTUAL",{"date":102,"type":100},"2022-01-25",{"date":104,"type":87},"2025-12-31",{"name":5,"class":6},1]