Our results suggest that airway vascular leakage is a major pathophysiologic feature of early asthma deterioration, occurring before recrudescence of cellular inflammation.

In the eighth week of pregnancy the medical indication for induced abortion was established due to an exacerbating acute intermittent porphyria with life-threatening neurological symptoms. delta-aminolaevulinic acid and porphobilinogen were excessively increased in urine prior to the operation. Anaesthesia was induced with a bolus of propofol, alfentanil and droperidol, maintained by 67% of nitrous oxide and small bolus injections of the three drugs. The patient regained consciousness immediately after the operation, and reached full orientation and cooperativeness within five minutes. The postoperative period remained uneventful and the neurological and psychological symptoms returned to the pre-exacerbation status. Chorion gonadotropins and porphyria markers decreased within the next four weeks accompanied by a simultaneously progressing clinical improvement. The use of propofol and the other drugs appears justified even in exacerbated porphyria.

The influence of hormonal oral contraceptives on the urinary porphyrin excretion of 40 healthy females has been studied. Two different hormonal oral contraceptives (combinations of gestoden or desogestrel, respectively, and ethinylestradiol) were applied for half a year. In each case twenty women received one of these two combinations. Porphyrin precursors delta-aminolevulinic acid and porphobilinogen were normal in all subjects as well as the mean of uroporphyrin and coproporphyrin. One healthy female developed a mild secondary coproporphyrinuria. In this case coproporphyrin isomer I was slightly enhanced and isomer III slightly lowered. Furthermore it could be shown that three females with repeated premenstrual clinical expression of an acute hepatic porphyria (acute intermittent porphyria and hereditary coproporphyria) could be treated successfully with a hormonal oral contraceptive or other exogenous hormones to stabilize the latent, subclinical phase of the disease.

A 49-year-old man, known to have had an increased light sensitivity since childhood, was admitted to hospital because of jaundice. Biochemical and morphological examination revealed cirrhosis of the liver with cholestasis. There was a 70-fold increase of protoporphyrin content in the erythrocytes, increased fecal protoporphyrin excretion as well as secondary coproporphyrinuria. Despite symptomatic treatment with ursodeoxycholic acid and cholestyramine hepatic failure ensued for which orthotopic liver transplantation was performed five months after the diagnosis had been made. The patient died two months later of treatment-resistant septicaemia and multiorgan failure. This case demonstrates the need for annual monitoring of liver functions and porphyrin parameters to ensure earliest possible diagnosis of hepatic involvement in erythrohepatic protoporphyria.

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