Abstract
Summary
Porphyrin excretion in urine was studied in 18 patients with sickle cell disease, including multiple observations in 6 patients during 7 typical painful and febrile crises. With few exceptions there was moderate to marked elevation of copro-, total, and uroporphyrins at time of pain and fever.
Aminolevulinic acid, porphobilinogen, and aminoacetone during crises were within range of normal variation or only slightly increased. Following subsidence of pain and fever, total porphyrin values returned toward normal. Similarities between clinical features of painful sickle cell crisis and acute porphyria are pointed out.
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