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7/28/2019 Neonatal Hepatitis Dr. Montes 5.10.2013
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Morning ReportMolly Montes MD PGY2 May 10, 2013
7/28/2019 Neonatal Hepatitis Dr. Montes 5.10.2013
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2mo ex-36week preemie female presents withjaundice and abnormal lab values
Family member noted yellow skin 2 wks PTA
Feeding well 3-4oz Neosure Q3H PO
very green stools 4-5x/day
Seen by PCP for 2mos WCC
Conjugated bilirubin 2.7
CMP notable for Hyperkalemia - K 6.1
metabolic acidosis - CO2 18
BUN 32 Cr 0.68
Alk Phos 558
ALT 251 AST 264
Presentation
7/28/2019 Neonatal Hepatitis Dr. Montes 5.10.2013
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? Echogenic bowel on fetal ultrasound
Born vaginally at 36wks for pre-eclampsia
BW 1932g
NICU stay x5-6days for feeding/growing
normal bilirubin at discharge
No meds
No allergies
Mom, Aunt, MGM with splenomegaly andprogressive vision loss
Brother with large liver
New pet dog at home No smoke exposure
History
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T-afebrile HR 136 BP 79/36 RR 42 SaO2 >94% inRA
Petite, jaundiced infant in no acute distress NCAT, AFOFS, triangular chin red reflex present bilaterally, mild scleral icterus TMs clear, MMM, normal palate No neck masses or LAD Lungs clear, normal WOB RRR, no murmur, normal pulses Liver 1cm BCM, no splenomegaly No sacral dimple or tuft Normal GU exam Green-pale colored stool in diaper
Normal extremities/digits Moves all limbs, normal tone and behavior
Physical Exam
7/28/2019 Neonatal Hepatitis Dr. Montes 5.10.2013
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Neonatal HepatitisAKA Neonatal Cholestasis
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Structural Defects Biliary Atresia Choledochal cysts Alagille syndrome
Caroli Disease Congenital hepatic fibrosis Neonatal sclerosing cholangitis Bile Acid Synthesis/Transport
Disorder CF, Wilsons Dubin-Johnson PFIC
Polycystic liver/kidney disease
Toxic TPN-induced Drugs
Endocrinopathies Hypothyroid Hypopituitarism
Metabolic Tyrosinemia Galactosemia Lipid Metabolism disorders
Mitochondrial disorders Neonatal Citrullinemia A1AT Deficiency Peroxisomal disorders Urea cycle disorders
Idiopathic
Infectious sepsis Congenital syphilis Echovirus, CMV, EBV
Rarely Hepatitis A/B/C toxoplasmosis
Differential
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CMP Hyperkalemia Metabolic acidosis: bicarb 16 Decreased renal function: BUN 26 Cr 0.73 Elevated LFTs: ALT 251, AST 264 Elevated Alk Phos 454
GGT 1164
Urine Studies
UA with micro WNL Urine culture NG Urine succinylacetone normal (test for
tyrosinemia)
CBC with differential WNL
Coagulation studies WNL
Work-Up
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Abdominal Ultrasound
Normal liver
Tubular gallbladder
Common bile duct not visualized
No dilation of intrahepatic bile ducts
Mild b/l pelviectasis
Kidneys borderline small Normal size and position of spleen
Normal hepatic Doppler
Work-Up Imaging
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Brother with Hepatomegaly Diagnosed with A1AT deficiency at age 2
Patients A1AT 38 (normal 100-200), ZZ phenotype
A1AT is antiprotease Deficiency leads to accumulation of often abnormal
proteins Lung disease arises from increased amount of elastase
which degrades elastin in lung parenchyma leading toemphysema
Liver disease attributed to accumulation of abnormalA1AT in hepatocytes Skin findings include panniculitis, urticaria Associated with vasculitis and IBD Occasionally associated with glomerulonephritis and
IgA nephropathy 1 in 2000-5000
Alpha-1 Antitrypsin
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Progressive destruction and obliteration of
extrahepatic biliary tree Unknown Etiology
Abnormal Fetal development
Splenic anomalies
Obstructive cholangiopathy
Viral infections Abnormal immune response
Findings cholestasis, HSM, acholic stools
May have absent or irregular gallbladder on U/S
Treat early with Kasai
Biliary Atresia
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Portoenterostomy
Roux-en-Y loop of jejunum usedto drain bile either from patentcommon bile duct or directlyfrom liver
Delays progression of cirrhosis ,
portal HTN, and liver failure Risk of cholangitis
Kasai Procedure
Childrennetwork.org
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Autosomal Dominant Mutation of Jagged 1 gene on 20p12 Paucity of intrahepatic bile ducts with syndromic
features
Facies deep set eyes, mild hypertelorism,overhanging forehead, long flat nose, small pointedchin
MSK short stature, butterfly or fused vertebrae, ribanomalies, spina bifida occulta
Cardiac PPS, TOF, VSD, ASD, Coarct, PA
Renal renal artery stenosis, tubulopathies High pitched cry, microcolon, pancreatic
insufficiency, multiple ophthalmologic anomalies
Alagille Syndrome
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H&P
Family History
Exposures infectious,idiopathic
Stool assessment
Micro Blood and urine cultures
Viral studies
Laboratory
Fractionated bilirubin (conj bili >20% total or
>2mg/dL)
LFTs Transaminitis => Primary
hepatocellular injury
GGT and alkalinephosphatase Biliary tract injury
Coagulation studies Metabolic liver disease
Metabolic screen Urine/serum amino acids
Urine for succinylacetone Serum iron and ferritin
AFP
Bile acids in urine/serum
Thyroid studies
CF test
A1AT level and phenotype
Neonatal Hepatitis Work-Up
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Abdominal Ultrasound Liver size and composition Gallbladder and biliary system
Ascites, portal venous flow, spleen
HIDA Scan Hepatobiliary scintigraphy Radioactive tracer injected into vein Tracer circulates to liver and gets excreted by biliary
system and taken into the gallbladder
Cholangiography Percutaneous transhepatic or via ex-lap Injection of contrast into GB or CBD
MR cholangiography No radiation exposure
Liver Biopsy
Neonatal Hepatitis Imaging
7/28/2019 Neonatal Hepatitis Dr. Montes 5.10.2013
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Cholestasis
Retention/Regurgitation Reduced bile delivery to gut
Bile acidCholesterolBilirubin
PruritisHepatotoxicityXanthomatosis
Jaundice
Decreased luminal bile salts
Malabsorption
MalnutritionGrowth Retardation
Fat-soluble vitamindeficiency
Diarrhea
Calcium deficiency
ANight blindnessDmetabolic bone disease
K hypoprothobinemiaE neuromuscular degeneration
Progressive
biliarycirrhosis
Portal HTN
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Treat underlying illness
Nutritional support
Growth monitoring
Supplemental feeding/TPN
MCT, fatty acids
ADEK
Pruritis
Ursodeoxycholic acid
Anti-histamines
Rifampin
Cholestyramine
Phenobarbital
Ascites
Diuretics
Sodium restriction Paracentesis
Portal HTN with varices
Propanolol, octreotide Endoscopy
TIPS
Liver transplant
Management
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Patient seen by genetics and nephrology
Started on actigall for improved bile flow
Started on sodium bicitrate to managemetabolic acidosis
2wks later in clinic
Still vigorously feeding/stooling
Increase in HSM
Stable LFTs, GGT
Low vitamins A&D
Started on ADEK
Nutritional supplement
Follow-Up
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UptoDate. Neonatal Hepatitis
Neonatal Cholestasis Nelson Textbook of
Pediatrics, 19th ed. Kliegman et al. 1281-1388. e12011.
Wyllie, Robert and Jeffrey Hyams. PediatricGastrointestinal and Liver Disease 3rd ed. 2006.
Donia et al. Predictive value of assessment ofdifferent modalities in the diagnosis of infantilecholestasis.J Internal Medicine Research.2010;38(6):2100-16).
References