InfantileHepatitisSyndrome
Infantile Hepatitis Syndrome
DefinitionAgroup ofsymptom complex which includeshepatocellularjaundice,pathologicphysicalsign of liver and abnornal liver functionoccurredininfancy
Defin ition □ A group of symptom complex which includes hepatocellular jaundice, pathologic physical sign of liver and abnornal liver function occurred in infancy
DefinitionAgroup ofsymptom complex which includeshepatocellular jaundice, pathologic physicalsign of liver and abnornal liver functionoccurred ininfancy
Defin ition □ A group of symptom complex which includes hepatocellular jaundice, pathologic physical sign of liver and abnornal liver function occurred in infancy
Etiology and Pathogenesis The causes arecomplicatedThepathogenesis is differentfordifferent causes
Etiology an d Pathogenesis □ The c a u s e s are complicated. □ The pathogenesis is different for different causes
1. Infection agents1)viral infection:Cytomegalovirus (CMV)Hepatitis viruses (HAV、HBV、HCV)Herps symplex virus (HSV)Rubella virusOther viruses:Varicella-Zoster virus (VZV)Epstein-Bar virus(EBV)EnterovirusHIV,etc.Pathogenesis is the virus invadedirectly or by theimmunoreactiveattack
1. Infection agents: 1) viral infection： □ Cytomegalovirus (CMV) □ Hepatitis viruses (HAV、HBV、HCV) □ Herps symplex virus (HSV) □ Rubella virus □ Other viruses: □ Varicella-Zoster virus (VZV ) □ Epstein-Bar virus (EBV) □ Enterovirus □ HIV, etc. P athogenesis is the virus invade directly or by the immunoreactive attack
2)Bacterialinfection(l)pathogenicbacteriaStaphylococcus aureus口Streptococcus口Ecoli口Salmonella口Pneumococcus口Anaerobic bacterium1Staphylococcus epidermidis, etc口(2)Pathogenesis: toxic hepatitis of sepsis(3)Clinical features:subclinical sepsis(4)Common points ofentry:oral mucosa, unbilical region,respiratorytract,urinary tract, skin, etc
2) B a c t e r i a l i n f e c t i o n ( 1 ) pathogenic bacteria □ Staphyloc occus a u reu s □ Strep tococcu s □ E coli □ Salmonella □ P n eu mococcu s □ Anaerobic bacterium □ Staphyloc occus epidermidis，etc (2) Pathogenesis： toxic hepatitis of sep sis (3) Clinical features：subclinical sep sis (4)Common points of entry： oral mucosa, unbilical region, respiratory tract, urinary tract, skin, etc
3)Otherinfectionpathogens:Toxoplasm:口Congenital ToxoplasmosisMicrospironema pallidum口Congenital syphilisFungous infection,etc
3) Other infection pathogens： □ Toxoplasm: Congenital Toxoplasmosis □ Microspironema pallidum: Congenital syphilis □ Fungous infection, etc
TORCH:TToxoplasmaOOthers R-Rubella virusCytomegalovirusHHerps symplex virus
TORCH： T—Toxoplasma O—Others R— Rubella virus C— Cytomega lovirus H—Herps symplex virus
2.Hereditary andgenetic metabolic disorders1)Carbohydratedysmetabolism Galactosemisgalactose-l-phosphate uridyltransferase deficiency deficiency Fructoseintolerancefructose bisphosphatealdolase Pompe's syndrome1)Aminoacid dysmetabolismTyrosinemia3)LipiddysmetabolismNiemann-Pick ‘sdisease, Gaucher disease or Wolman' s disease4)Otherdysmetabolism
2.Hereditary and genetic metabolic disorders 1)Carbohydrate dysmetabolism □ Galactosemis galactose-1-phosphate uridyltransferase deficiency deficiency □ Fructose intolerance fructose bisphosphate aldolase □ Pompe's syndrome 1)Amino acid dysmetabolism Tyrosinemia 3)Lipid dysmetabolism Niemann-Pick‘s disease, Gaucher disease or Wolman’s disease 4)Other dysmetabolism
Galactosemislactosegalactose +glucosegalactokinasel-galactosephosphategalactose-l-phosphate uridyltransferase1-glucosephosphateClinical features:Vomiting, difficult feeding, hypoglycemia, alack of energy,failure to thrive, cataract, jaundice, hepatomegaly , liver cirrhosis,mental retardation after several days ofmilk feedingBiochemistryexaminationblood glucosel, blood galactoset, reducing sugar test +Diagnos is : enzymatic determination
lactose □ Dia gn osis: enzymatic determination Galactosemis galactos e + glu cose galactokinase 1-galactose phosphate galactose -1-phos phate uridyltransferase 1-glucose phosphate □ Clinical features： Vomiting, difficult feeding, hypoglycemia, a lack of energy, failure to thrive, cataract, jaundice, hepatomegaly , liver cirrhosis, mental retardation after several days of milk feeding. □ Biochemistry examination: blood glucose↓, blood galactose↑, reducing sugar test +