masalah gastrointestinal kolestasis
DESCRIPTION
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CholestasisMuzal Kadim-Aryono Hendarto
Gastrohepatology DivisionChild Health DepartmentMedical Faculty University of Indonesia
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DefinitionNeonatal cholestasis is defined as conjugated hyperbilirubinemia developing within the first 90 days of extrauterine lifeConjugated bilirubin > 1.0 mg/dlConjugated bilirubin > 20% of the total bilirubin.
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Cholestatic jaundiceAffects 1 : 2,500 Infants and is thus infrequently seen by mostproviders of medical care to infants
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BUT
Many infants withCholestatic appear otherwise healthy and grow normally Misleading physician into believing that is physiologic or caused by breast-feeding, when infact it may be caused by biliary atresia
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PresentationJaundice - conjugated bilirubinPale stools Dark urineAbnormal LFTs - high or low GGTBleedingHepatosplenomegalyAbdominal massFailure to thrive
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Differential Diagnosis
Anatomy1. Biliary atrresia2. Choledochal cystInfectious3. Toxoplasmosis4. Rubella5. CMV6. Herpes simplex7. Syphilis8. Bacterial sepsis9. Urinary tract infectionMetabolic10. Tyrosinemia11. GalactosemiaEndocrine12. Hypothyroidism13. HypocortisolismGenetic14. Alagille syndrome15. PFIC
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Cholestasis at Ciptomangunk usumo Hospital (2000-2003)N = 162 ptIdiopathic NNH28%EHBA23%UTI17%Sepsis14%CMV5,5%Alagille2 %Choledochal Cyst1%Miscellaneous 9,5% (metabolic, PFIC etc)
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Clnical historyDetail about mothers pregnancyBirth weigh and gestational ageVit K administrationFamily historyConsanguinityHistory of present illnessDate of jaundiceColor of stools and urineDrug history, parenteral nuitritionBleedingFeeding histroyDiarrhea and vomiting
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Clinical Clues
Clinical featureSuggestingFamily history, consanguinity, dysmorphic featureMetabolic/inherited diseaseBruising, petechiae or bleedingVit K deficiencyHypoglycemiaSecondary to metabolic diseaseHypopituitarismAcute liver failureSplenomegalyIntrauterine infectionInborn error of metabolismAdvance liver diseaseAscitesIntrauterine infectionInborn error of metabolism
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Clinical Clues...
Clinical featureSuggestingDysmorphicTrisomies, AlagillesCardiac MurmurAlagilles, EHBASick InfantSepsis, HLH,cong infecMicropenisPanhypopituitarismCataractsRubella, GalactosaemiaSitus InversusEHBARetinal probsTORCHS, AlagillesMassCholedochal CystCutan HaemangiomaLiver HaemangiomaWhite HairHLHBile Stained HerniaSpont Perforation of Bile Duct
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Initial investigationFractioned serum bilirubin analysisSGPT, SGPT, GGTTest for liver function: PT, aPTT, albumin, cholesterol, glucoseCBCBacterial culture: urine and or blood if indicatedUrinalysis including reducing sugarFT4 and TSH if screening was not performed
- Pale StoolsWarrants urgent referral to exclude biliary atresia- surgery
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JaundiceAcutely ill? Manage the acute illness Consider: Sepsis, UTIHypopitutarismIron storage disease Metabolic disordersHemolysisYesCholestasis?
No
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Cholestasis?YesNoHistoryPhysical examUrinalysisUrine cultureFT4,TSHFinding spesific disease?Unconjugated hyperbilirubinemiaEvaluate further
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Finding spesific disease?YesNoTreat&Evaluate furtherDoes Bilirubin normalize by 4-6 weeks of age?No
Consult Pediatric GI
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Thank you
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