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1、Case(3):progressivefamilialintrahepaticcholestasisMedicalNumber:1003027882GeneralinformationName:xiaobojunAge:2month17daysSex:maleRace:HanOccupation:unemployedmaritalstatus:unmarriedID:UnknownNationality:ChinaAddress:guangchangCommunity,hanyangRoad,caidian
2、,wuhan,Hubei.Tel:13871107167Historyofthepresenter:MotherHujingyanReliability:ReliableDateofadmission:2014-02-0714:47pmDateofrecord:2014-02-0716:45pmChiefcomplaint:yellowingoftheskinandeyesfor2months.Presentillness:Thepatientwithmanifestationoftheyellowingo
3、fskinandSclerathenextdayafterthechildwasborn.Notassociatedwithfever、vomit、diarrhea,Yellowstool,withoutWhiteclaystools.HospitalizedHubeimaternalandchildhealthcaredueto“respiratorydistresssyndromeofnewborn”、“RespiratoryFailure”“ventilator-associatcdpneumonia
4、”“bronchopulmonarydysplasia”“cholestaticsyndrome”“anemiaofprematurity”“verylowbirthweightinfant”from2013-11to2014-01-26.Respiratorassistedventilationfor7daysandcontinuouspositiveairwaypressure(CPAP)assistedventilation33dayswhilehospitalized.Thechaildstarte
5、danti-infectivetherapy(Meropenem、Sulperazon),plasmatransfusion,redcelltransfusions,blue-rayradiationtherapy,withthesetreatmentthesignsandsymptomalleviateandleavehospital.JaundicewassignificantlyincreasedwithoutanyinducementvisitedHubeimaternalandchildhealt
6、hcareat2014-02-03,Bloodbiochemicalitem:totalbilirubin206.6umol/L,conjugatedbilirubin130.4umol/L,indirectbilirubin76.2umol/L,ALT34.1U/L,AST132.6U/L,r-GT72U/L,totalbileacid89.2umol/L,totalprotein45g/L,albumin37.1g/L,ALP717U/L.Thenthechildvisitedourhospitalcl
7、inicservice,Hospitalizedwith“jaundiceofunknownorigin”.Sinceonset,bothhisspiritednessandphysicalenergywarenormal,buthisappetitewasnotgood,sleepnormal,Defecationandurinationarenormal.physicalstrengthandbodyweightnosignificantchange.Pasthistory1、Generalhealth
8、:ThepatientHospitalizedHubeimaternalandchildhealthcaredueto“respiratorydistresssyndromeofnewborn”、“RespiratoryFailure”“ventilator-associatcdpneumonia”“bronchopulmonarydysplasia”“cholestaticsyndrome”“anemiaofp