Of 50 infants with persistent signs of biliary obstruction, not atresia, seen in the past 7 years, 16 had preceding hemolytic disease from ABO or Rh-incompatibility and 3 had acquired hemolytic anemia. One infant had probable serum hepatitis and 30 others had hepatitis of unknown etiology. All had a patent external biliary tract.
Clinically these patients had jaundice, light stools and dark urine. All appeared in a good state of nutrition except those with hepatitis of unknown cause. The results of laboratory studies have been similar in these patients and have included a high direct serum bilirubin, usually negative cephalin flocculation, frequently elevated zinc sulfate turbidity and low cholesterol esters. The urine urobilin may be absent or increased in amount.
Histologic studies of liver have shown the presence of multinucleated giant liver cells, much myelopoiesis and erythropoiesis, plugging of bile canaliculi and mild bile duct proliferation. Large amounts of yellow pigment have been found within the liver cells but this pigment has not taken the usual iron stains. Livers from patients with hepatitis of unknown etiology have shown more evidence of inflammation, cell necrosis and portal fibrosis than the others.
Pertinent laboratory studies to help differentiate these cases from biliary atresia have included determinations of serum bilirubin, zinc sulfate turbidity, cholesterol and cholesterol esters and urine urobilin. Studies of maternal and infant blood factors and search for evidence of agglutination and increased production of erythrocytes are also important. Tests not found to be useful in differentiation are cholesterol esterase, alkaline phosphatase and prothrombin time.
If diagnosis is impossible from clinical and laboratory information, exploration of the biliary tract with liver biopsy and diodrast cholangiography may be necessary. This procedure should be delayed until an adequate period of observation has elapsed and until the infant is over 4 months of age. Such a delay may result in spontaneous clearing of the jaundice with complete recovery.
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