Study of Nutritional Factors in Porphyria
Primary Purpose
Porphyria
Status
Completed
Phase
Locations
Study Type
Observational
Intervention
Sponsored by
About this trial
This is an observational trial for Porphyria focused on measuring inborn errors of metabolism, porphyria, rare disease
Eligibility Criteria
Acute intermittent porphyria Variegate porphyria and hereditary coproporphyria eligible but analyzed separately
Sites / Locations
Outcomes
Primary Outcome Measures
Secondary Outcome Measures
Full Information
NCT ID
NCT00004788
First Posted
February 24, 2000
Last Updated
June 23, 2005
Sponsor
National Center for Research Resources (NCRR)
Collaborators
University of Texas
1. Study Identification
Unique Protocol Identification Number
NCT00004788
Brief Title
Study of Nutritional Factors in Porphyria
Study Type
Observational
2. Study Status
Record Verification Date
December 2001
Overall Recruitment Status
Completed
Study Start Date
May 1988 (undefined)
Primary Completion Date
undefined (undefined)
Study Completion Date
undefined (undefined)
3. Sponsor/Collaborators
Name of the Sponsor
National Center for Research Resources (NCRR)
Collaborators
University of Texas
4. Oversight
5. Study Description
Brief Summary
OBJECTIVES: I. Determine whether differences in dietary habits are associated with disease activity in patients with acute intermittent porphyria.
II. Determine whether premenstrual porphyria attacks are associated with increased luteal phase energy requirements.
III. Determine whether energy requirements are higher than intakes in men with unexplained frequent porphyria attacks.
IV. Assess the nutritional status of women with acute intermittent porphyria using a comprehensive series of laboratory methods, including zinc and pyridoxine status.
V. Determine whether the frequency of disease exacerbations decreases when dietary and nutritional abnormalities are corrected in these patients.
VI. Evaluate the safety and efficacy of a parenteral nutrition regimen for patients with acute porphyria attacks.
Detailed Description
PROTOCOL OUTLINE: Women record diet intake for at least 1 cycle (28 days), then undergo a comprehensive nutritional assessment at least once during the follicular and luteal phases of the cycle.
Men also record diet intake for at least 1 month and undergo indirect calorimetry and other studies.
All patients receive a comprehensive analysis of diet and energy metabolism. Energy expenditure, resting metabolic rates, and basal metabolic rates are determined with indirect calorimetry, including measurements taken during the follicular and luteal phases of the cycle. Other testing includes hormone assays, serology for nutritional studies, and a urinary metabolite profile. Zinc, lead, and other metals are measured by atomic absorption spectroscopy.
The diagnosis of acute intermittent porphyria is confirmed by erythrocyte porphobilinogen deaminase; urinary aminolevulinic acid, porphobilinogen, and porphyrins; and total fecal porphyrins.
Patients hospitalized for acute attacks of porphyria during the study will receive standard treatment, including intravenous hematin and parenteral support.
6. Conditions and Keywords
Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Porphyria
Keywords
inborn errors of metabolism, porphyria, rare disease
7. Study Design
Enrollment
30 (false)
10. Eligibility
Sex
All
Minimum Age & Unit of Time
0 Years
Accepts Healthy Volunteers
No
Eligibility Criteria
Acute intermittent porphyria
Variegate porphyria and hereditary coproporphyria eligible but analyzed separately
Overall Study Officials:
First Name & Middle Initial & Last Name & Degree
Karl Elmo Anderson
Organizational Affiliation
University of Texas
Official's Role
Study Chair
12. IPD Sharing Statement
Learn more about this trial
Study of Nutritional Factors in Porphyria
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