| disease | Concentrated Bile Syndrome |
| alias | Bile Concentration Syndrome, Biliary Cast Syndrome, Congenital Immune-mediated Hemolytic Hepatitis, Bile-Plug Syndrome, Congenital-Immunological Hemolytic Hepatitis |
Concentrated gall fel syndrome, also known as Congenital-Immunological Hemolytic Hepatitis, Bile-Plug Syndrome, or Concentrated Bile Syndrome, refers to the occurrence of significant obstructive jaundice following neonatal hemolytic disease.
bubble_chart Pathogenesis
Various types of neonatal hemolytic disease can cause this condition. Due to excessive hemolysis, the bilirubin in the gallbladder increases, leading to a rise in gallbladder concentration, followed by gallbladder thickening, forming bile plugs. These bile plugs aggregate to form gallbladder plugs, thereby causing obstructive jaundice. Additionally, high concentrations of bilirubin can also lead to swelling of liver cells, resulting in secondary intrahepatic gallbladder stasis. This condition differs in nature from extrahepatic obstruction caused by bilirubin stones.
bubble_chart Clinical Manifestations
1. Hemolytic symptoms: Anemia often gradually worsens within 1 to 2 weeks after birth, disproportionate to the degree of jaundice, with hepatosplenomegaly, reticulocytosis, and some patients testing positive for the Coombs test or showing Rh incompatibility between mother and infant.
2. Obstructive jaundice: Typically appears 2 days after birth, lasting for 3 weeks, with varying severity and obstructive characteristics; stools are clay-colored.
bubble_chart Auxiliary Examination
Positive urine bilirubin, negative urobilinogen, elevated serum bilirubin concentration, predominantly conjugated bilirubin.
1. Jaundice appears within a few hours to 48 hours after birth.
2. Jaundice persists for more than 3 weeks.
3. Serum bilirubin is elevated and exhibits the characteristics of obstructive jaundice.
4. Flocculation test is negative.5. Incompatible maternal and infant blood types, with the newborn showing clinical manifestations of hemolytic disease.
bubble_chart Treatment Measures
Exchange blood therapy and ultraviolet phototherapy can be used to prevent the occurrence of kernicterus. Patients with liver function impairment should receive liver-protective treatment.
This sign should be differentiated from organic extrahepatic biliary obstruction such as neonatal hepatitis and congenital biliary atresia.