management of neonatal cholestasis

MANAGEMENT OF NEONATAL

CHOLESTASIS

PRESENTED BY:Dr.Divya

NEONATAL CHOLESTASIS-Definition

• Prolonged elevation of conj.bilirubin beyond 14 days of life

• Serum direct bilirubin greater than 1.0mg/dl if the total serum bilirubin (TSB) is <5.0 mg/dl or

• > 20% of TSB if the TSB is >5.0 mg/dl

• Any newborn with jaundice and dark yellow urine with or without pale stools should be strongly suspected to have NC.

NEONATAL CHOLESTASIS-causes

• Infections EHBA

• Metabolic ds Choledochal cyst

• Genetic ds Cholelithiasis-TPN

• Endocrine ds Drugs

• Paucity of bile ducts

• Familial syndromes

INTRAHEPATIC EXTRAHEPATIC

INTRAHEPATIC CAUSES• Inf.-TORCHES• Hepatitis B,C• Septicemia• HIV• TB• Listeriosis• Metabolic-Galactosemia• Hereditary fructose intolerance• GSD 4• Gauchers ds• Niemann pick ds• Hereditary tyrosinemia• Alpha 1 antitrypsin def• Cystic fibrosis• NISD

INTRAHEPATIC

• Endocrine-Hypothyroidism

• Hypopituitarism

• Genetic-Down syndrome

• Edward syndrome

• Paucity of bile ducts-Watson alagille syndrome

• Familial syn-PFIC

• Aagenes syn

• Carolis ds

• BRIC

ETIOLOGICAL PROFILE OF NC IN INDIA

• Hepatocellular causes constitute 45% to 69%

• obstructive causes account for 19% to 55% of all cases

CLINICAL PRESENTATION

• Jaundice

• Hepatomegaly

• Acholic stools

• Dark urine

• Other signs and symptoms depend on specific disease process

Differentiation-BA v/s NH-AIIMS SCORE• Age - <6wks -score 2

• >6wks -score 1

• Icterus-Fluctuating,mild to mod.-score 2

• Severe >8mg% -score 1

• Urine –Normal/yellow coloured –score 2

• High coloured -score 1

• Stool – N./Light yellow -score 2

• Clay coloured -score 1

• Liver –Soft -score 4

• Firm-score 1

• Score >10 – NH

• <10 - BA

INVESTIGATIONS

• LFT-Markedly elevated S.ALP+min.elevated SGPT & SGOT s/o BA

• TFT

• SEPSIS SCREEN

Radiological evaluation

UltrasonographyFindings s/o BA

• triangular cord sign

• gallbladder not visualized

• length <1.9 cm

• To r/o choledochal cyst & cholelithiasis

• HIDA scan

• limited role in evaluation of NC

• Performing a HIDA scan is optional and one may go for a liver biopsy straightaway

Liver biopsy• Early recognition of BA by liver biopsy can avoid

unnecessary laparotomy

• F/O BA-marked bile duct proliferation

• Min. giant cell proliferation

• bile plugs in ducts

• fibrosis and lymphocytic infiltrates in the

portal tracts

• Intra-operative cholangiogram (IOC) remains the gold standard for diagnosis of BA.

Metabolic workup

• Urine exmn-Reducing sugar-Galactosemia,HFI

• Succinyl acetone-Tyrosinemia

• RBC GALT levels- Galactosemia

• AAT levels

• Sweat chloride level

• Aldolase B enz assay in liver biopsy

• S.Ferritin level

MANAGEMENT-General Management

• Nutritional support-Caloric req. 125% of RDA

• Breast feed-MCT oil @ 1-2ml/kg/d

• Veg.prot-2-3g/kg/d

• Supplementation of fat soluble vitamins

• Vitamin supplementation should be continued

till 3 months after resolution of jaundice

• Pruritus-UDCA @20mg/kg/d

• Rifampicin @ 5-10mg/kg/d

• Phenobarbitone @ 5-10mg/kg/d

SUGGESTED DAILY REQUIREMENTS

SPECIFIC TREATMENT

• Special infant formula and diets are recommended for children with specific diagnosis (galactosemia,fructosemia and tyrosinemia)

• Nitisinone (1 mg/kg/d)-Tyrosinemia

• Specific therapy -CMV (associated neurological involvement), herpes and toxoplasmosis related cholestasis.

SURGICAL

• KASAI’s Procedure

• Choledochal cyst

• (PFIC) without decompensated cirrhosis, external and internal biliary diversion

KASAI PROCEDURE

• Performed for biliary atresia that is not surgically correctable with excision of a distal atretic segment.

• Roux-en-Y portoenterostomy

• Bile flow re-established in 80-90% if performed prior to 8 weeks-old.

• Bile flow re-established in less than 20% if performed after 12 weeks-old

LIVER TRANSPLANTATION

• standard therapy for decompensated cirrhosis due to any cause

• bilirubin >6 mg/dL, three months after kasai’ssurgery

• BA who present with decompensated cirrhosis (low albumin, prolonged INR, ascites) are not likely to improve with a Kasai PE and should be referred for liver transplantation

CONCLUSIONS

• NC constitutes almost one-third of children with chronic liver disease in major hospitals in India.

• BA,NH and metabolic causes are the most important causes in India

• Early identification of the cause is essential for a favorable outcome

• Urine and stool color assessment(minimum 3 stool samples) by the mother and physician in a stool color card incorporated in all well baby cards(Indian Academy of Pediatrics and the Government of India cards)