Neonatal Hepatitis Dr. Montes 5.10.2013

7/28/2019 Neonatal Hepatitis Dr. Montes 5.10.2013

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Morning ReportMolly Montes MD PGY2 May 10, 2013


2/18

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


3/18

? 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


4/18

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


5/18

Neonatal HepatitisAKA Neonatal Cholestasis


6/18

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


7/18

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


8/18

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


9/18

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


10/18

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


11/18

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


12/18

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


13/18

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


14/18

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


15/18Complications

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


16/18

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


17/18

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


18/18

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

Neonatal Hepatitis Dr. Montes 5.10.2013

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