The nicotinic acid test in the evaluation of unconjugated hyperbilirubinemia.

In 42 young adults with Gilbert's syndrome and in 9 patients with inactive, well-compensated liver cirrhosis the peroral nicotinic acid test was performed. Of the 13 parameters tested, the groups differed significantly in the sum of total and unconjugated serum bilirubin concentrations, respectively, during the test; in the difference between the maximal and initial concentrations of total and unconjugated bilirubin; in the retention of total bilirubin at the third, fourth and fifth hour of the test; and in the size of the area under the curve of unconjugated bilirubin increase. However, due to considerable overlap, no single parameter could reliably differentiate among all persons with Gilbert's syndrome and cirrhotics. The nicotinic acid test is neither necessary for the diagnosis of Gilbert's syndrome nor can it reliably differentiate between this condition and severe organic liver disease. Yet, it does supply valuable information about the readiness of the organism to develop or enhance hyperbilirubinemia following a defined stimulus.

[Ultrasonic examination of the epigastrium in persons with Gilbert's syndrome]. / Ultrazvukové vysetrení nadbrisku u osob s Gilbertovým syndromem.

In 29 subjects with Gilbert's syndrome during ultrasonographic examination of the epigastrium in real time a significantly larger anteroposterior dimension of the right liver lobe (by cca 11%] was found. In the other investigated dimensions no significant deviations were found, not even a significantly higher echogeneity of the liver parenchyma. In two instances multiple cholecystolithiasis was detected. Sonography, a non-invasive method with a high yield, is an important link in the examination programme of subjects with Gilbert's syndrome.

Resin hemoperfusion in hepatic porphyrias.

In three types of hepatic porphyrias, 32 hemoperfusions over polyhema-coated Amberlite XAD-2 resin were performed. An attack of acute intermittent porphyria subsided after a 6-h hemoperfusion. During the procedure, 13 L of plasma was completely cleared of porphyrins. In a child with variegate porphyria, the laboratory changes were slight and no clinical effect of hemoperfusion was seen. In a patient suffering from porphyria cutanea tarda resistant to standard therapy, 30 hemoperfusions were performed during a period of 10 months. The total plasma porphyrin concentration increased sharply following the start of the treatment, and preceding the fifth procedure it reached a peak of four times the initial value. Since then, the plasma porphyrin level decreased, eventually to one-half the starting value. The mean drop in porphyrin level during each perfusion was 22%. Similar changes were observed in the amount of porphyrins excreted into the urine, which, following an early rise, decreased to 25% of the initial value. However, the patient's skin vulnerability improved only slightly. There were no untoward effects of the treatment.

Severe liver injuries due to sulfamethoxazole-trimethoprim and sulfamethoxydiazine.

Two cases of severe sulfonamide liver injury are described. In one patient, fulminant liver failure developed after two days of taking sulfamethoxazole-trimethoprim. The patient was treated with resin hemoperfusion and recovered completely. Another patient became jaundiced after using sulfamethoxydiazine for ten days. Very severe intrahepatic cholestasis developed and cleared up only after high-dose prednison treatment. Marked hyperbilirubinemia persisted for four months and aminotransferase activity had not normalized ten months after the onset of the disease.

Long-term support of hepatic excretory function by perfusion of plasma and blood through synthetic resin.

In a patient with advanced primary biliary cirrhosis, 55 resin perfusions were performed which were accompanied by marked improvement in the patient's condition and laboratory findings.