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Study of Nutritional Factors in Porphyria

This study has been completed.
University of Texas
Information provided by:
Office of Rare Diseases (ORD) Identifier:
First received: February 24, 2000
Last updated: June 23, 2005
Last verified: December 2001

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.


Study Type: Observational
Study Design: Primary Purpose: Screening

Resource links provided by NLM:

Further study details as provided by Office of Rare Diseases (ORD):

Estimated Enrollment: 30
Study Start Date: May 1988
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.


Ages Eligible for Study:   Child, Adult, Senior
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
  • Acute intermittent porphyria
  • Variegate porphyria and hereditary coproporphyria eligible but analyzed separately
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Please refer to this study by its identifier: NCT00004788

Sponsors and Collaborators
National Center for Research Resources (NCRR)
University of Texas
Study Chair: Karl Elmo Anderson University of Texas
  More Information Identifier: NCT00004788     History of Changes
Other Study ID Numbers: 199/11886
Study First Received: February 24, 2000
Last Updated: June 23, 2005

Keywords provided by Office of Rare Diseases (ORD):
inborn errors of metabolism
rare disease

Additional relevant MeSH terms:
Porphyria, Erythropoietic
Metabolic Diseases
Skin Diseases, Genetic
Genetic Diseases, Inborn
Skin Diseases processed this record on September 18, 2017