Shunt hyperbilirubinemia syndrome
Introduction
Introduction to shunt hyperbilirubinemia syndrome The shunt hyperbilirubinemia syndrome, Israel syndrome, is caused by excessive production of bilirubin by bone marrow red blood cells or precursors, or directly by the destruction and production of erythroline or tetrapyrrole precursors. Both men and women can suffer from the disease, 10 to 20 years old, with jaundice and splenomegaly, blood examination for spherical red blood cells and increased erythrocyte fragility, reticulocyte red increase, indirect hyperbilirubinemia, increased urinary biliary excretion, Free dissolution and mechanical fragility, normal liver function. basic knowledge The proportion of illness: 0.003% Susceptible people: no special people Mode of infection: non-infectious complication:
Cause
The cause of shunt hyperbilirubinemia syndrome
Excessive bilirubin produced by bone marrow red blood cells or precursors, or directly caused by destruction and production of erythroline or tetrapyrrole precursors.
Prevention
Divided hyperbilirubinemia syndrome prevention
Active prevention of hepatobiliary diseases is the basic preventive measure of this disease.
Complication
Divided complications of hyperbilirubinemia syndrome Complication
Biliary malformations, central nervous system damage, bilirubin encephalopathy, etc.
Symptom
Symptoms of shunt hyperbilirubinemia syndrome Common symptoms Urinary biliary excretion increases diarrhea, bloating, nausea, jaundice, indigestion
Both men and women can suffer from the disease, 10 to 20 years old, with jaundice and splenomegaly, blood examination for spherical red blood cells and increased erythrocyte fragility, reticulocyte red increase, indirect hyperbilirubinemia, increased urinary biliary excretion, Free dissolution and mechanical fragility, normal liver function.
According to clinical features and laboratory tests can be diagnosed, but liver biopsy is required to confirm the exclusion of other types of congenital jaundice.
Examine
Examination of shunt hyperbilirubinemia syndrome
Blood tests showed increased spherocytosis of red blood cells and red blood cells, moderate increase in reticulocytes, indirect hyperbilirubinemia, increased urinary excretion, free dissolution and normal mechanical fragility, and normal liver function.
Blood tests, liver tissue biopsy.
Diagnosis
Diagnosis and diagnosis of shunt hyperbilirubinemia syndrome
Need to be distinguished from other types of jaundice.
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