Erythropoietic protoporphyria: pitfalls and proposed solutions for patient communication about the disease, a survey of parents and adult patients.

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Summary of "Erythropoietic protoporphyria: pitfalls and proposed solutions for patient communication about the disease, a survey of parents and adult patients."

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Name: Journal of the American Academy of Dermatology
ISSN: 1097-6787


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Medical and Biotech [MESH] Definitions

A carotenoid that is a precursor of VITAMIN A. It is administered to reduce the severity of photosensitivity reactions in patients with erythropoietic protoporphyria (PORPHYRIA, ERYTHROPOIETIC). (From Reynolds JEF(Ed): Martindale: The Extra Pharmacopoeia (electronic version). Micromedex, Inc, Engewood, CO, 1995.)

An autosomal dominant porphyria that is due to a deficiency of FERROCHELATASE (heme synthetase) in both the LIVER and the BONE MARROW, the last enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features include mainly neurological symptoms, rarely cutaneous lesions, and elevated levels of protoporphyrin and COPROPORPHYRINS in the feces.

A mitochondrial enzyme found in a wide variety of cells and tissues. It is the final enzyme in the 8-enzyme biosynthetic pathway of HEME. Ferrochelatase catalyzes ferrous insertion into protoporphyrin IX to form protoheme or heme. Deficiency in this enzyme results in ERYTHROPOIETIC PROTOPORPHYRIA.

Sterile solutions, essentially free from foreign particles and suitably compounded and dispensed, for instillation into the eye. It does not include solutions for cleaning eyeglasses or CONTACT LENS SOLUTIONS. (From Dorland, 27th ed)

Providing the patient, family or others information, and then allowing them to take an action or restate the information in their own words. The clinician prefaces communications with a statement framed to show the clinician has the burden of effective communication rather than the patient.

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