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Dental Health, Diet, Inflammation and Biomarkers in Patients With Acute Intermittent Porphyria(AIP)

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ClinicalTrials.gov Identifier: NCT01617642
Recruitment Status : Active, not recruiting
First Posted : June 12, 2012
Last Update Posted : May 14, 2019
Sponsor:
Collaborator:
The Royal Norwegian Ministry of Health
Information provided by (Responsible Party):
Nordlandssykehuset HF

Tracking Information
First Submitted Date June 5, 2012
First Posted Date June 12, 2012
Last Update Posted Date May 14, 2019
Study Start Date July 2012
Estimated Primary Completion Date December 2021   (Final data collection date for primary outcome measure)
Current Primary Outcome Measures
 (submitted: June 8, 2012)
  • Blood pressure [ Time Frame: Within 2 months after inclusion ]
    Resting systolic and diastolic blood pressure, a number of inflammatory parameters, serum markers for iron status and inflammation
  • Diet registration [ Time Frame: Within 2 months after inclusion ]
    Dietary registration during one week
  • Iron status [ Time Frame: Within 2 months after inclusion ]
    Blood samples for evaluation of iron status
  • Inflammatory status [ Time Frame: Within 2 months after inclusion ]
    Blood samples (cytokines etc) for evaluation of inflammation
Original Primary Outcome Measures Same as current
Change History Complete list of historical versions of study NCT01617642 on ClinicalTrials.gov Archive Site
Current Secondary Outcome Measures
 (submitted: June 8, 2012)
Dental health [ Time Frame: Within two months after inclusion ]
Evaluate dental health through clinical examination
Original Secondary Outcome Measures Same as current
Current Other Pre-specified Outcome Measures Not Provided
Original Other Pre-specified Outcome Measures Not Provided
 
Descriptive Information
Brief Title Dental Health, Diet, Inflammation and Biomarkers in Patients With Acute Intermittent Porphyria(AIP)
Official Title Dental Health, Diet, Inflammation and Biomarkers in Patients With Acute Intermittent Porphyria(AIP)
Brief Summary Acute intermittent porphyria (AIP) is an autosomal dominant inherited disease, which is relatively prevalent in northern Norway with a total of around 90 patients. This provides us with a special opportunity to study AIP. AIP is caused by a mutation in the porphobilinogen deaminase, an enzyme in the haem synthesis. AIP presents symptoms, particularly among fertile women and older men. Typical symptoms are abdominal pain and dark red urine, nausea, vomiting, constipation, muscle weakness and nerve damage including paraesthesia and even paresis. This is known as symptomatic or manifest AIP (MAIP). Others do not display symptoms, so-called latent AIP (LAIP). AIP attacks may be triggered by a host of medicaments which affect the haem synthesis, infections, alcohol and stress. Treatments of manifestations include high sugar intake (4 sugar lumps/hour), alternatively administer glucose and Normosang (synthetic haem arginate) by intravenous injection and removing triggering factors. Diet, glucose intake, dental health and inflammatory parameters will be examined. This study can provide new knowledge about why only some people develop symptoms of AIP. Main hypothesis: There are differences in the diet, iron status, inflammation and glucose metabolism of the MAIP group vs. the LAIP group and the control group.
Detailed Description

In a group of people with proven acute intermittent porphyria (AIP) mutation, some will remain asymptomatic, while others have repeated periods of porphyria symptoms. Glucose inhibits ALA synthetase (ALAS), the first rate-limiting enzyme in the haem synthesis. Studies of individual patients point to the fact that increased glucose and/or fructose content in the diet inhibits porphyria attacks. A high sugar intake can reduce the disease activity in patients with AIP. The diet and related biomarkers of those with latent and manifest AIP will therefore be mapped to explain why some have latent and others have manifest acute intermittent porphyria. Other studies point to the fact that people with manifest AIP who have later developed diabetes type 2 no longer have porphyria symptoms. Dental health will also be examined.

Inflammation also affects the haem synthesis. Infections and/or inflammation are known to trigger AIP attacks. The disease activity in patients with acute intermittent porphyria in relation to inflammatory status, iron status, glucose metabolism and diet will therefore be examined.

The iron metabolism is interesting to study because it is believed that the overstimulation of the haem synthesis is what triggers porphyria attacks. Haem consists of iron and protoporphyrin IX, and it is therefore possible that iron supplements in cases of iron deficiency can induce increased haem synthesis and by doing so trigger and/or aggravate AIP.

Kidney failure is a serious secondary complication in some patients with MAIP. Protein markers for kidney injury in urine will be examined.

Study Type Observational
Study Design Observational Model: Case-Control
Time Perspective: Cross-Sectional
Target Follow-Up Duration Not Provided
Biospecimen Retention:   Samples With DNA
Description:
Serum and plasma from cases With acute intermittent porphyria and age, sex and place of recidence matched controls. Urine samples. Pax tubes for RNA/DNA samples.
Sampling Method Non-Probability Sample
Study Population

The group of patients with acute intermittent porphyria will be recruited from primary care clinics and patient organizations.

The control group will be selected randomly from the same geographical area, matched for gender and age

Condition Acute Intermittent Porphyria
Intervention Not Provided
Study Groups/Cohorts
  • Control group
    Healthy control group, matched for age and gender
  • Acute intermittent porphyria
    Patients with acute intermittent porphyria.
Publications *

*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Recruitment Information
Recruitment Status Active, not recruiting
Estimated Enrollment
 (submitted: June 8, 2012)
150
Original Estimated Enrollment Same as current
Estimated Study Completion Date December 2021
Estimated Primary Completion Date December 2021   (Final data collection date for primary outcome measure)
Eligibility Criteria

Inclusion Criteria:

  • Diagnosed acute intermittent porphyria

Exclusion Criteria:

  • Regulatory use of antiinflammatory drugs including steroids and NSAIDS
  • Lacking consent competence
Sex/Gender
Sexes Eligible for Study: All
Ages 18 Years and older   (Adult, Older Adult)
Accepts Healthy Volunteers Yes
Contacts Contact information is only displayed when the study is recruiting subjects
Listed Location Countries Norway
Removed Location Countries  
 
Administrative Information
NCT Number NCT01617642
Other Study ID Numbers 2011/2197/REK
ID/7462 SFP 1068-12 ( Other Grant/Funding Number: Northern Norway Regional Health Authority )
Has Data Monitoring Committee No
U.S. FDA-regulated Product Not Provided
IPD Sharing Statement Not Provided
Responsible Party Nordlandssykehuset HF
Study Sponsor Nordlandssykehuset HF
Collaborators The Royal Norwegian Ministry of Health
Investigators
Principal Investigator: Ole L Brekke, MD, PhD University of Tromsø, Norway
PRS Account Nordlandssykehuset HF
Verification Date May 2019