新生儿和婴儿胆汁淤积（英文版）

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1、NeonatalandInfantileCholestasisYing-kitLeung,MD,FAAPPresident,HongKongSocietyofPaediatricGastroenterology,HepatologyandNutrition,Yantai,Shandong,July2006DEFINITIONNeonatalcholestasisisdefinedasconjugatedhyperbilirubinemiadevelopingwithinthefirst90daysofextrauterinelife.Conjugatedbi

2、lirubinexceeds1.5to2.0mg/dl.Conjugatedbilirubingenerallyexceeds20%ofthetotalbilirubin.BilirubinProductionBiliverdinBilirubinerythrocytehemoglobinmusclemyoglobincytochromescatalaseshemeoxygenasebiliverdinreductasereticulo-endothelialcellBilirubinAlbuminliverCO+FeHemeGSTBilirubinUpta

3、ke,Conjugation,ExcretionBBAlbGBGGBRUDPGUDPGLUCURONYLTRANSFERASEHEPATOCYTESINUSOIDDISSEBILECANALICULUS2.UPTAKE3.CONJUGATION4.EXCRETIONE.R.B-GB-GB-B-GB-GB-GBuBgsBuBgB-GdarkurineacholicstoolsConjugatedHyperbilirubinemiaConjugatedhyperbilirubinemiaextrahepaticintrahepaticNeonatalCholes

4、tasisEXTRAHEPATICETIOLOGIESExtrahepaticbiliaryatresiaCholedochalcystBileductstenosisSpontaneousperforationofthebileductCholelithiasisInspissatedbile/mucusplugExtrinsiccompressionofthebileductINTRAHEPATICETIOLOGIESIdiopathicToxicGenetic/ChromosomalInfectiousMetabolicMiscellaneousINT

5、RAHEPATICETIOLOGIESIdiopathicNeonatalHepatitisToxicTPN-associatedcholestasisDrug-inducedcholestasisGenetic/ChromosomalTrisomy18Trisomy21INTRAHEPATICETIOLOGIESInfectiousBacterialsepsis(E.coli,Listeriosis,Staph.aureus)TORCHESHepatitisBandCVaricellaCoxsackievirusEchovirusTuberculosisI

6、NTRAHEPATICETIOLOGIESMetabolicDisordersofCarbohydrateMetabolismGalactosemiaFructosemiaGlycogenStorageDiseaseTypeIVDisordersofAminoAcidMetabolismTyrosinemiaHypermethioninemiaINTRAHEPATICETIOLOGIESMetabolic(cont.)DisordersofLipidMetabolismNiemann-PickdiseaseWolmandiseaseGaucherdiseas

7、eCholesterolesterstoragediseaseDisordersofBileAcidMetabolism3B-hydroxysteroiddehydrogenase/isomeraseTrihydroxycoprostanicacidemiaINTRAHEPATICETIOLOGIESMetabolic(cont.)PeroxisomalDisordersZellwegersyndromeAdrenoleukodystrophyEndocrineDisordersHypothyroidismIdiopathichypopituitarismI

8、NTRAHEPATICETIOLOGIESMetab