neonatal cholestasis 10.14.2011

8/3/2019 Neonatal Cholestasis 10.14.2011

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Morning Report10/14/11

Katy Piercy


2/18

2-Month Well Child Check

2-mo girl Born at 39 weeks, C/S for breech,

uncomplicated pregnancy

Feeding well, no illnesses, no meds

Skin has appeared more yellow for 1 week

Stools are light yellow No fever, emesis, diarrhea, constipation


3/18

Physical Exam

T 36, HR 139, RR 56, BP 89/49, SpO2 100% on RAWeight 4.11 kg (7%ile), Height 53 cm (3%ile)Gen: Awake, alert, interactive, no distress.HEENT: NCAT, +scleral icterus, no oral lesions, MMM, nodysmorphic features.

Neck: Supple, no thyromegaly, no LAD.Resp: CTA bilaterally, no retractions.CV: RRR, normal S1 S2, no murmur/gallop, CR


4/18

Initial Labs

Na 138, K 4.7, Cl 104, CO2 21, BUN 6, Cr 0.21, Glu 70, Ca 10

WBC 10, Hgb 9.8, Hct 28.9, Plt 399INR 1.0, PTT 46

Total bili 7.8Conjugated 3.6, unconjugated 1.9

Alk phos 521ALT 111AST 129GGT 519Prot 6.7Alb 4.1


5/18

DDx Neonatal Cholestasis

Idiopathic neonatal hepatitis

Extrahepatic biliary atresia Alpha-1 antitrypsin deficiency Intrahepatic cholestasis syndromes

Alagille Byler

Bacterial sepsis Hepatitis: CMV

Rubella, Herpes Endocrine

Hypothyroidism, panhypopituitarism

Galactosemia Inborn errors of bile acid biosynthesis

30-35%

25-30%7-10%

5-6%

2%3-5%

1%1%

1%2-5%


6/18

Other Extrahepatic Causes

Biliary ductal hypoplasia

Cholelithiasis

Choledochal cyst

Spontaneous perforation of CBD


7/18

...More Labs

TSH 2.56

Alpha-1 antitrypsin: normal level and phenotype

Urine organic acids, succinyl acetoneCortisolSweat chlorideViral serology (hepatitis, TORCH, HIV)Viral/bacterial cultures


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Other Studies

Abdominal ultrasound:-Mild hepatomegaly with nonvisualization of the gallbladder.-Fibrous triangular cord.-Prominence of the hepatic artery.

-Prominent peripheral hepatic artery vascularity by Doppler.

Liver biopsy: Cholestatic hepatopathy.Portal triads are expanded by fibrosis and increased numbers of bile

ductules. There is mild mixed inflammation in the triads. Bile plugging is occasionally

seen within bile ductules. There is moderate hepatocellular and canalicularcholestasis. There is hepatocyte unrest, with hepatocyte ballooning and numerousmultinucleated hepatocytes. No viral inclusions are seen.


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Biliary Atresia

1 in ~15,000 live births

Isolated (70%), lateralization d/o (10-15%), othermalformations (10-15%)

Most common reason for pediatric liver transplant in US Etiology unclear--infection, metabolic, environmental?

Progressive destruction of bile ducts (extra- to intrahepatic) Fibrosis --> cirrhosis --> liver failure Presentation usually after 2-3 weeks of life

o Jaundice, clay-colored stools (acholic), dark urine


10/18

Clay-colored?


11/18


12/18

Biliary Atresia

1 in ~15,000 live births

Isolated (70%), lateralization d/o (10-15%), othermalformations (10-15%)

Most common reason for pediatric liver transplant in US Etiology unclear--infection, metabolic, environmental?

Progressive destruction of bile ducts (extra- to intrahepatic) Fibrosis --> cirrhosis --> liver failure Presentation usually after 2-3 weeks of life

o Jaundice, clay-colored stools (acholic), dark urineo Hepatomegalyo Conjugated hyperbili, elevated alk phos, GGT, AST/ALTo Gallbladder often not seen on U/So No excretion of radioisotope on radionuclide scanso Liver biopsy if not ruled out by the other studies


13/18

Management

Surgical exploration- diagnostic and therapeutic

Intraoperative cholangiography may be performedo Classic: nondilated, proximally obliterated ductal

system Kasai: hepatoportoenterostomy

o Roux-en-Y limb of jejunum sewn around thetransected porta hepatis

o More successful when done earlier--best before3 months of age!!!


14/18


15/18

Management

Meds:o Glucocorticoids after Kasai (inconsistent

study results)o Choleretics (utility not established)o Antibiotics as prophylaxis for cholangitis

Nutrition: Need 150% normal caloric needs Fat-soluble vitamin supplementation


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Prognosis

No surgery: Mean survival 8 months, Max 2 years. S/p Kasai:o Overall: 25-35% 10-yr survival w/o transplant.o No bile flow: hepatic failure by 1 year.

o Bile flow (50-70%): 66% 10-yr survivalw/o transplant.

o ~80% eventually need transplant by adulthood. Indications for transplant:

o primary failure of Kasaio complications of portal hypertensiono growth failure despite NG feedso progressive liver dysfunction


17/18

Back to the Case...

Kasai failed Now 8 months old Growth failure (5 kg) Portal hypertension

o Asciteso Splenomegalyo Thrombocytopenia

Cholangitis

Bili 25, INR 1.9, Plt 60 Awaiting transplant

Picture from Dr. Jacksons powerpoint


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References

Zallen GS, et al. Biliary atresia. Pediatr Rev.2006;27;243-248.

Haber BA, Erlichman J. Biliary atresia. In:UpToDate, Basow, DS (Ed), UpToDate,Waltham, MA, 2011.

Jackson, WD. The yellow infant: a cliniciansapproach to diagnosis. PowerPointpresentation on TeamSpace.

neonatal cholestasis 10.14.2011

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