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A NEW FORM OF CONGENITAL HEMATOPORPHYRIA: OLIGOCHROMEMIA PORPHYRINURIA (MEGALOSPLENICA CONGENITA)

AKIRA SATO, M.D.; HIROSHI TAKAHASHI, M.D.
Am J Dis Child. 1926;32(3):325-333. doi:10.1001/archpedi.1926.04130090002001.
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In this paper, we report a peculiar form of congenital hematoporphyria which hitherto has been undescribed. The name "oligochromemia porphyrinuria (megalosplenica congenita)" is suggested. The characteristic features of the conditions are:

1. Congenital hematoporphyria, that is, congenital porphyrinuria 1 with cutaneous sensitivity to light as its sequelum; (2) onset at birth; (3) enlarged spleen at birth with subsequent splenomegaly; (4) chlorotic type of anemia; (5) fatal termination; (6) familial factor (presence of entire syndrome in each of the affected members).

The cases described here represent the earliest form of congenital porphyria on record. The onset at birth would tend to support the theory of an endogenous disorder of metabolism. The excretion of an intensely red urine, rich in porphyrins and the sensitivity to light manifested by the typical hydroa lesions, appear shortly after birth. The complete symptom triad of congenital porphyria includes the purplish pigmentation of the milk teeth, although

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