Congenital Erythropoietic Protoporphyria

Porter, F. Stanley; Lowe, Bruce A.

doi:10.1182/blood.v22.5.521.521

Cited by 66 publications

(3 citation statements)

References 2 publications

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“…Also to be considered are the liver changes in erythropoietic protoporphyria. Porter and Lowe (5) reported two cases of erythropoietic protoporphyria and one of them was diagnosed as having portal cirrhosis. They described two possible explanations for the portal cirrhosis, one was precipitation of porphyrins, the other was the hepatotoxic effect of porphyrin.…”

Section: Commentmentioning

confidence: 99%

Six Cases of Erythropoietic Protoporphyria

Nonaka

Nishimoto

Hirowatari

et al. 1975

The Journal of Dermatology

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Six patients with erythropoietic protoporphyria; the most common of the porphyrias, were seen at Nagasaki University Hospital and studied clinically and histopathologically. Cutaneous changes were observed in all six patients and are considered to be the result of both protoporphyrin and sunlight. These changes were classified as either acute or chronic, and the chronic changes were examined histologically and revealed a perivascular accumulation of PAS positive material in the upper dermis.Slight aberrations of liver function were found in three of our six cases, and we suggest that the liver cells were involved by a combination of protoporphyrin and sunlight.

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Section: Commentmentioning

confidence: 99%

Six Cases of Erythropoietic Protoporphyria

Nonaka

Nishimoto

Hirowatari

et al. 1975

The Journal of Dermatology

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“…The methods used to measure the in vitro synthesis of protoporphyrin and haeme by bone marrow have been reported in detail (Porter 1963). Bone marrow samples were obtained by aspiration from tibias and femurs.…”

Section: E T H O D Smentioning

confidence: 99%

“…To further identify the porphyrins they were extracted into ethyl acetate, dried, esterified (Schwartz et al 1960), and chromatographed (Chu et al 1951) with protoporphyrin IX and coproporphyrin 111 standards. The preparation of the standards is described elsewhere (Porter 1963).…”

Section: E T H O D Smentioning

confidence: 99%

Vitamin E Deficiency in The Monkey X. Protoporphyrin and Haeme Synthesis by Peripheral Blood and Bone Marrow

Porter¹,

Fitch²

1966

Scandinavian Journal of Haematology

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In vitro studies of porphyrin and haeme synthesis by bone marrow cells from vitamin E‐deficient monkeys indicate that vitamin E is required for the normal synthesis of these compounds. The defect in porphyrin synthesis apparently results from abnormal delta‐aminolaevulinic acid synthesis. This defect was not found in the reticulocytes of peripheral blood. The administration of vitamin E corrected the abnormality in porphyrin synthesis, but the addition of vitamin E in vitro did not. Coenzyme Q10 administration also resulted in increased porphyrin synthesis by the bone marrow cells of vitamin E‐deficient monkeys.

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