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Neonatal Cholestasis Flashcards

1
Q

What is the specific therapy for early surgical correction of extrahepatic biliary atresia?

A

Kasai operation.

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2
Q

What is the treatment for sepsis and urinary tract infections in neonatal cholestasis?

A

Proper antibiotics.

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3
Q

How is galactosemia managed in neonatal cholestasis?

A

Elimination of lactose from the diet (lactose-free diet).

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4
Q

What is used to treat pruritus in neonatal cholestasis?

A

Bile acid binders like cholestyramine.

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5
Q

What is the treatment for varices in neonatal cholestasis?

A

Injection sclerotherapy or band ligation.

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6
Q

How is hepatic encephalopathy managed in neonatal cholestasis?

A

10% glucose infusion, enema, and oral neomycin.

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7
Q

What is the replacement therapy for fats in neonatal cholestasis?

A

Medium-chain triglycerides (MCT).

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8
Q

Why is Vitamin K important in neonatal cholestasis?

A

It prevents serious bleeding and intracranial hemorrhage.

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9
Q

What is the daily dose of Vitamin E in neonatal cholestasis?

A

50 U/day.

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10
Q

What is the daily dose of Vitamin D in neonatal cholestasis?

A

5000 U/day.

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11
Q

What is the daily dose of Vitamin A in neonatal cholestasis?

A

100,000 U/day.

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12
Q

What is the commonest indication for liver transplantation in neonatal cholestasis?

A

Biliary atresia if the Kasai operation fails.

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13
Q

What is the Kasai operation?

A

Hepato-porto-enterostomy where the jejunum is anastomosed to patent ducts in the cut surface of the portal hepatis.

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14
Q

What is a postoperative complication of the Kasai operation?

A

Cholangitis (fever).

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15
Q

What is neonatal cholestasis?

A

Failure of normal bile to reach the duodenum due to liver or biliary disease.

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16
Q

What is the definition of hyperbilirubinemia in neonatal cholestasis?

A

Conjugated (direct) bilirubin > 20% of total bilirubin.

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17
Q

What is the function of bile in the body?

A

Digestion and absorption of lipids and fat-soluble vitamins (A, D, E, K).

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18
Q

What is the role of the liver in bilirubin metabolism?

A

The liver removes bilirubin from the blood, conjugates it with glucuronic acid, and excretes it in bile.

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19
Q

What is the difference between direct and indirect bilirubin?

A

Direct bilirubin is conjugated and water-soluble, while indirect bilirubin is unconjugated and fat-soluble.

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20
Q

What is kernicterus?

A

A condition caused by unconjugated bilirubin crossing the blood-brain barrier.

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21
Q

What are the common bile duct lesions in neonatal cholestasis?

A

Extrahepatic biliary atresia (EHBA) and choledochal cyst.

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22
Q

What infections can cause neonatal cholestasis?

A

Congenital infections like TORCH (CMV, Rubella, HSV, Toxoplasmosis) and acquired infections like neonatal sepsis or UTI.

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23
Q

What are some inborn errors of metabolism that can cause neonatal cholestasis?

A

Galactosemia, tyrosinemia, Gaucher disease, Niemann-Pick disease, alpha-1 antitrypsin deficiency, cystic fibrosis, and bile acid biosynthetic defects.

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24
Q

What is idiopathic neonatal hepatitis?

A

Neonatal hepatitis with no evident etiology, historically the commonest cause of neonatal cholestasis.

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25
Q

What are familial cholestatic syndromes?

A

Conditions like Alagille syndrome and progressive familial intrahepatic cholestasis (PFIC) that affect bile salt transport.

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26
Q

What is Alagille syndrome?

A

An autosomal dominant disorder with intrahepatic bile duct hypoplasia.

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27
Q

What is progressive familial intrahepatic cholestasis (PFIC)?

A

A group of autosomal recessive disorders affecting bile salt transport, leading to jaundice, pruritus, and growth failure.

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28
Q

What is the treatment for familial cholestatic syndromes?

A

ADEK vitamins, ileal bile acid transporter inhibitors (IBAT), and liver transplantation.

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29
Q

Why is early differentiation between idiopathic neonatal hepatitis and EHBA important?

A

Early surgical correction of EHBA can prevent further hepatic damage.

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30
Q

What are the diagnostic criteria for cholestasis?

A

Serum bilirubin (total and direct) with direct bilirubin > 20% of total, high INR, and decreased prothrombin concentration.

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31
Q

What liver enzymes are checked in cholestasis?

A

AST, ALT, alkaline phosphatase (ALP), and GGT.

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32
Q

What is the significance of GGT in cholestasis?

A

GGT is normal in PFIC type 1 and bile acid synthesis defects.

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33
Q

How is sepsis diagnosed in neonatal cholestasis?

A

CBC, ESR, CRP, and cultures (blood, CSF, urine).

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34
Q

How is galactosemia diagnosed?

A

Reducing substances in urine and enzyme assay in RBCs (galactose-1-phosphate).

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35
Q

What imaging is used to diagnose choledochal cysts?

A

Ultrasound or CT.

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36
Q

What metabolic screening is done for tyrosinemia?

A

Aminogram and succinyl acetone in urine.

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37
Q

How is cystic fibrosis diagnosed?

A

Sweat chloride test > 60 and genotyping.

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38
Q

What is the normal serum level of alpha-1 antitrypsin?

A

150-250 mg/dL.

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39
Q

What is TORCH screening?

A

Testing for total IgM antibodies against TORCH agents (CMV, Rubella, HSV, Toxoplasmosis).

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40
Q

What is the most important diagnostic tool for differentiating idiopathic neonatal hepatitis from EHBA?

A

Liver biopsy.

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41
Q

What is seen in a liver biopsy of idiopathic neonatal hepatitis?

A

Giant cell transformation and marked infiltration with inflammatory cells.

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42
Q

What is seen in a liver biopsy of EHBA?

A

Expansion of portal areas with fibrosis and bile duct proliferation.

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43
Q

What is the role of a HIDA scan in diagnosing cholestasis?

A

No dye excretion in biliary atresia; dye can reach the intestine in hepatitis.

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44
Q

What is a sign of biliary atresia on imaging?

A

Non-visualization or a micro-gallbladder after 4 hours of fasting.

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45
Q

What are the consequences of decreased bile delivery to the intestine?

A

Fat malabsorption (steatorrhea) and fat-soluble vitamin deficiencies (A, D, E, K).

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46
Q

What are the clinical manifestations of cholestasis?

A

Persistent jaundice, dark urine, pale stools, and hepatomegaly.

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47
Q

What are the manifestations of TORCH infections in neonatal cholestasis?

A

Cataracts, microcephaly, hepatosplenomegaly (HSM), and low birth weight.

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48
Q

What are the manifestations of galactosemia in neonatal cholestasis?

A

Cataracts, convulsions, and hepatomegaly.

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49
Q

What are the manifestations of Alagille syndrome?

A

Wide-spaced eyes, triangular face, heart defects (PS or VSD), and vertebral arch defects.

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50
Q

What are the complications of prolonged cholestasis?

A

Liver cirrhosis, portal hypertension, splenomegaly, and bleeding (hematemesis or intracranial hemorrhage).

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51
Q

What is the significance of pale stools in neonatal cholestasis?

A

Indicates decreased bile delivery to the intestine.

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52
Q

What is the significance of hepatomegaly in neonatal cholestasis?

A

Indicates liver involvement and possible progression to liver failure.

53
Q

What is the role of bile in cholesterol excretion?

A

Bile is the body’s only means of eliminating cholesterol and bile acids.

54
Q

What is the significance of conjugated bilirubin in neonatal cholestasis?

A

Conjugated bilirubin is water-soluble and excreted in stools, while unconjugated bilirubin is fat-soluble and can cause kernicterus.

55
Q

What is the significance of direct bilirubin in neonatal cholestasis?

A

Direct bilirubin > 20% of total bilirubin indicates cholestasis.

56
Q

What is the significance of prothrombin time in neonatal cholestasis?

A

High INR and decreased prothrombin concentration indicate liver dysfunction.

57
Q

What is the significance of AST and ALT in neonatal cholestasis?

A

Elevated levels indicate liver cell damage.

58
Q

What is the significance of alkaline phosphatase (ALP) in neonatal cholestasis?

A

Elevated levels indicate bile duct obstruction or liver damage.

59
Q

What is the significance of GGT in neonatal cholestasis?

A

Elevated levels indicate bile duct obstruction, except in PFIC type 1 and bile acid synthesis defects.

60
Q

What is the significance of reducing substances in urine in neonatal cholestasis?

A

Indicates galactosemia.

61
Q

What is the significance of succinyl acetone in urine in neonatal cholestasis?

A

Indicates tyrosinemia.

62
Q

What is the significance of sweat chloride test in neonatal cholestasis?

A

Levels > 60 indicate cystic fibrosis.

63
Q

What is the significance of alpha-1 antitrypsin level in neonatal cholestasis?

A

Deficiency (levels < 150 mg/dL) indicates alpha-1 antitrypsin deficiency.

64
Q

What is the significance of TORCH IgM antibodies in neonatal cholestasis?

A

Indicates congenital infection with TORCH agents.

65
Q

What is the significance of liver biopsy in neonatal cholestasis?

A

Differentiates between idiopathic neonatal hepatitis and EHBA.

66
Q

What is the significance of HIDA scan in neonatal cholestasis?

A

Differentiates between biliary atresia and hepatitis based on dye excretion.

67
Q

What is the significance of non-visualization of the gallbladder in neonatal cholestasis?

A

Indicates biliary atresia.

68
Q

What is the significance of fat malabsorption in neonatal cholestasis?

A

Leads to steatorrhea and fat-soluble vitamin deficiencies.

69
Q

What is the significance of fat-soluble vitamin deficiencies in neonatal cholestasis?

A

Leads to deficiencies in vitamins A, D, E, and K, causing various complications.

70
Q

What is the significance of pruritus in neonatal cholestasis?

A

Caused by retained bile acids.

71
Q

What is the significance of dark urine in neonatal cholestasis?

A

Indicates conjugated hyperbilirubinemia.

72
Q

What is the significance of splenomegaly in neonatal cholestasis?

A

Indicates portal hypertension.

73
Q

What is the significance of bleeding in neonatal cholestasis?

A

Indicates vitamin K deficiency and possible intracranial hemorrhage.

74
Q

What is the significance of butterfly vertebrae in neonatal cholestasis?

A

Indicates Alagille syndrome, a condition associated with vertebral arch defects.

75
Q

What is the significance of heart defects in neonatal cholestasis?

A

Indicates Alagille syndrome, which can include pulmonary stenosis (PS) or ventricular septal defect (VSD).

76
Q

What is the significance of eye anomalies in neonatal cholestasis?

A

Indicates Alagille syndrome, which can include wide-spaced eyes and other eye abnormalities.

77
Q

What is the significance of faltering growth in neonatal cholestasis?

A

Indicates chronic liver disease and malnutrition, often seen in progressive familial intrahepatic cholestasis (PFIC).

78
Q

What is the significance of rickets in neonatal cholestasis?

A

Indicates vitamin D deficiency due to fat malabsorption.

79
Q

What is the significance of intracranial hemorrhage in neonatal cholestasis?

A

Indicates vitamin K deficiency, which can lead to serious bleeding complications.

80
Q

What is the significance of hematemesis in neonatal cholestasis?

A

Indicates portal hypertension and variceal bleeding.

81
Q

What is the significance of splenomegaly in neonatal cholestasis?

A

Indicates portal hypertension, often due to liver cirrhosis.

82
Q

What is the significance of liver cirrhosis in neonatal cholestasis?

A

Indicates end-stage liver disease, requiring liver transplantation.

83
Q

What is the significance of portal hypertension in neonatal cholestasis?

A

Leads to complications like varices, splenomegaly, and bleeding.

84
Q

What is the significance of variceal bleeding in neonatal cholestasis?

A

A serious complication of portal hypertension, requiring urgent treatment.

85
Q

What is the significance of cholangitis in neonatal cholestasis?

A

A postoperative complication of the Kasai operation, indicated by fever.

86
Q

What is the significance of biliary sludge in neonatal cholestasis?

A

Can be caused by certain antibiotics like cefoperazone, leading to bile flow obstruction.

87
Q

What is the significance of liver enzymes in neonatal cholestasis?

A

Elevated levels indicate liver cell damage or bile duct obstruction.

88
Q

What is the significance of elevated GGT in neonatal cholestasis?

A

Indicates bile duct obstruction, except in PFIC type 1 and bile acid synthesis defects.

89
Q

What is the significance of normal GGT in neonatal cholestasis?

A

Suggests PFIC type 1 or bile acid synthesis defects.

90
Q

What is the significance of elevated alkaline phosphatase (ALP) in neonatal cholestasis?

A

Indicates bile duct obstruction or liver damage.

91
Q

What is the significance of elevated AST and ALT in neonatal cholestasis?

A

Indicates liver cell damage.

92
Q

What is the significance of high INR in neonatal cholestasis?

A

Indicates liver dysfunction and impaired clotting factor synthesis.

93
Q

What is the significance of decreased prothrombin concentration in neonatal cholestasis?

A

Indicates liver dysfunction and impaired clotting factor synthesis.

94
Q

What is the significance of reducing substances in urine in neonatal cholestasis?

A

Indicates galactosemia, a treatable cause of cholestasis.

95
Q

What is the significance of succinyl acetone in urine in neonatal cholestasis?

A

Indicates tyrosinemia, a metabolic disorder causing liver failure.

96
Q

What is the significance of sweat chloride test in neonatal cholestasis?

A

Levels > 60 indicate cystic fibrosis, a cause of cholestasis.

97
Q

What is the significance of alpha-1 antitrypsin level in neonatal cholestasis?

A

Deficiency (levels < 150 mg/dL) indicates alpha-1 antitrypsin deficiency, a cause of liver disease.

98
Q

What is the significance of TORCH IgM antibodies in neonatal cholestasis?

A

Indicates congenital infection with TORCH agents, a cause of cholestasis.

99
Q

What is the significance of liver biopsy in neonatal cholestasis?

A

Differentiates between idiopathic neonatal hepatitis and EHBA based on histology.

100
Q

What is the significance of HIDA scan in neonatal cholestasis?

A

Differentiates between biliary atresia and hepatitis based on dye excretion.

101
Q

What is the significance of non-visualization of the gallbladder in neonatal cholestasis?

A

Indicates biliary atresia, a common cause of neonatal cholestasis.

102
Q

What is the significance of fat malabsorption in neonatal cholestasis?

A

Leads to steatorrhea and fat-soluble vitamin deficiencies.

103
Q

What is the significance of fat-soluble vitamin deficiencies in neonatal cholestasis?

A

Leads to deficiencies in vitamins A, D, E, and K, causing various complications.

104
Q

What is the significance of pruritus in neonatal cholestasis?

A

Caused by retained bile acids, leading to intense itching.

105
Q

What is the significance of dark urine in neonatal cholestasis?

A

Indicates conjugated hyperbilirubinemia, a hallmark of cholestasis.

106
Q

What is the significance of pale stools in neonatal cholestasis?

A

Indicates decreased bile delivery to the intestine, a sign of cholestasis.

107
Q

What is the significance of hepatomegaly in neonatal cholestasis?

A

Indicates liver involvement and possible progression to liver failure.

108
Q

What is the significance of bleeding in neonatal cholestasis?

A

Indicates vitamin K deficiency and possible intracranial hemorrhage.

109
Q

What is the significance of varices in neonatal cholestasis?

A

Risk of bleeding and requires treatment like sclerotherapy or band ligation.

110
Q

What is the significance of hepatic encephalopathy in neonatal cholestasis?

A

Indicates severe liver dysfunction and requires glucose infusion and neomycin.

111
Q

What is the significance of Kasai operation in neonatal cholestasis?

A

Early surgical correction of EHBA to prevent further liver damage.

112
Q

What is the significance of liver transplantation in neonatal cholestasis?

A

Required for end-stage liver disease or failed Kasai operation.

113
Q

What is the significance of medium-chain triglycerides (MCT) in neonatal cholestasis?

A

Used as a fat replacement therapy due to better absorption.

114
Q

What is the significance of ADEK vitamins in neonatal cholestasis?

A

Prevents deficiencies in fat-soluble vitamins.

115
Q

What is the significance of ileal bile acid transporter inhibitors (IBAT) in neonatal cholestasis?

A

Reduces pruritus and improves bile acid transport.

116
Q

What is the significance of cholestyramine in neonatal cholestasis?

A

Bile acid binder used to treat pruritus.

117
Q

What is the significance of sclerotherapy in neonatal cholestasis?

A

Treatment for varices to prevent bleeding.

118
Q

What is the significance of band ligation in neonatal cholestasis?

A

Treatment for varices to prevent bleeding.

119
Q

What is the significance of glucose infusion in neonatal cholestasis?

A

Treatment for hepatic encephalopathy.

120
Q

What is the significance of neomycin in neonatal cholestasis?

A

Reduces ammonia levels in hepatic encephalopathy.

121
Q

How to differentiate at first look of jaundiced neonate

A

Olive green , normal birth w , clay colored stool from begin is atresia
Lbw giant cell ساعه تروح و ساعه تيجي

122
Q

Colesstasis+mr+conv+cataract=

A

Galactosomia

123
Q

Cholesat+murmur=

A

Algaille syndrome

124
Q

Chole+wheezes

A

Alpha -1 anti trypsin , cf

125
Q

Chole +catarct _pda +microcephaly

A

Cong rubella

126
Q

Chole+albensim

A

Tyrsoinemia

127
Q

Chole+mass +abdominal pain

A

Choledochal cyst

128
Q

Largest HSM

A

Neiman gaucer

129
Q

At which trim cong rubella

Hepatology (5 decks)

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