Study of Nutritional Factors in Porphyria

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.

Completed

Looking for future studies?

Notify Me

Key information

Conditions

Porphyria Congenital, Hereditary, and Neonatal Diseases and Abnormalities Disease Attributes Genetic Diseases, Inborn Metabolic Diseases Metabolism, Inborn Errors Nutritional and Metabolic Diseases Pathologic Processes Pathological Conditions, Signs and Symptoms Porphyrias Rare Diseases

Age range

0 year and older

Sex eligibility

All sexes

Study type

Observational

About this study

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.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Acute intermittent porphyria
Variegate porphyria and hereditary coproporphyria eligible but analyzed separately

Sponsors and collaborators

Lead sponsor

National Center for Research Resources (NCRR)

Nih

Collaborators

University of Texas

Registry information

Important dates

Study start: 1988
First posted: Feb 25, 2000
Registry last updated: Jun 24, 2005

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

Published trials that share one or more normalized conditions with this study.

Completed