Four cases of acute porphyria in female patients are recorded. The acute episodes were precipitated or made worse by the administration of barbiturates in all. Death occurred in the two patients showing the most marked neurological manifestations. The post-mortem of one of them is described. The clinical and pathological features of the cases are discussed.
Liver changes in acute porphyria are considered in detail and the literature reviewed. Functional and structural alterations are generally slight in this condition in distinction to porphyria cutanea tarda, but alterations in albumin/globulin ratio and in flocculation tests are not uncommon. A peculiar fine nodularity of the liver was observed on two occasions; this has been noted by others.
Serial determinations of porphobilinogen in the urine in one case showed a diminution in excretion as the patient’s clinical state improved. Porphobilinogen estimations are related to the underlying biochemical defect and are of much greater importance than estimations of uroporphyrin. The specificity of porphobilinogenuria is discussed.
The onset of an attack may be insidious, and the Watson-Schwartz test for porphobilinogen is of the utmost value in making the diagnosis early and in identifying susceptibles.
No sharp distinction can be drawn between cases with and without neurological manifestations and although those with the worst neurological signs generally die, some severely affected patients recover.
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