Neonatal Cholestasis Flashcards
1
Q
What is the specific therapy for early surgical correction of extrahepatic biliary atresia?
A
Kasai operation.
2
Q
What is the treatment for sepsis and urinary tract infections in neonatal cholestasis?
A
Proper antibiotics.
3
Q
How is galactosemia managed in neonatal cholestasis?
A
Elimination of lactose from the diet (lactose-free diet).
4
Q
What is used to treat pruritus in neonatal cholestasis?
A
Bile acid binders like cholestyramine.
5
Q
What is the treatment for varices in neonatal cholestasis?
A
Injection sclerotherapy or band ligation.
6
Q
How is hepatic encephalopathy managed in neonatal cholestasis?
A
10% glucose infusion, enema, and oral neomycin.
7
Q
What is the replacement therapy for fats in neonatal cholestasis?
A
Medium-chain triglycerides (MCT).
8
Q
Why is Vitamin K important in neonatal cholestasis?
A
It prevents serious bleeding and intracranial hemorrhage.
9
Q
What is the daily dose of Vitamin E in neonatal cholestasis?
A
50 U/day.
10
Q
What is the daily dose of Vitamin D in neonatal cholestasis?
A
5000 U/day.
11
Q
What is the daily dose of Vitamin A in neonatal cholestasis?
A
100,000 U/day.
12
Q
What is the commonest indication for liver transplantation in neonatal cholestasis?
A
Biliary atresia if the Kasai operation fails.
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.
14
Q
What is a postoperative complication of the Kasai operation?
A
Cholangitis (fever).
15
Q
What is neonatal cholestasis?
A
Failure of normal bile to reach the duodenum due to liver or biliary disease.
16
Q
What is the definition of hyperbilirubinemia in neonatal cholestasis?
A
Conjugated (direct) bilirubin > 20% of total bilirubin.
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).
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.
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.
20
Q
What is kernicterus?
A
A condition caused by unconjugated bilirubin crossing the blood-brain barrier.
21
Q
What are the common bile duct lesions in neonatal cholestasis?
A
Extrahepatic biliary atresia (EHBA) and choledochal cyst.
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.
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.
24
Q
What is idiopathic neonatal hepatitis?
A
Neonatal hepatitis with no evident etiology, historically the commonest cause of neonatal cholestasis.
25
What are familial cholestatic syndromes?
Conditions like Alagille syndrome and progressive familial intrahepatic cholestasis (PFIC) that affect bile salt transport.
26
What is Alagille syndrome?
An autosomal dominant disorder with intrahepatic bile duct hypoplasia.
27
What is progressive familial intrahepatic cholestasis (PFIC)?
A group of autosomal recessive disorders affecting bile salt transport, leading to jaundice, pruritus, and growth failure.
28
What is the treatment for familial cholestatic syndromes?
ADEK vitamins, ileal bile acid transporter inhibitors (IBAT), and liver transplantation.
29
Why is early differentiation between idiopathic neonatal hepatitis and EHBA important?
Early surgical correction of EHBA can prevent further hepatic damage.
30
What are the diagnostic criteria for cholestasis?
Serum bilirubin (total and direct) with direct bilirubin > 20% of total, high INR, and decreased prothrombin concentration.
31
What liver enzymes are checked in cholestasis?
AST, ALT, alkaline phosphatase (ALP), and GGT.
32
What is the significance of GGT in cholestasis?
GGT is normal in PFIC type 1 and bile acid synthesis defects.
33
How is sepsis diagnosed in neonatal cholestasis?
CBC, ESR, CRP, and cultures (blood, CSF, urine).
34
How is galactosemia diagnosed?
Reducing substances in urine and enzyme assay in RBCs (galactose-1-phosphate).
35
What imaging is used to diagnose choledochal cysts?
Ultrasound or CT.
36
What metabolic screening is done for tyrosinemia?
Aminogram and succinyl acetone in urine.
37
How is cystic fibrosis diagnosed?
Sweat chloride test > 60 and genotyping.
38
What is the normal serum level of alpha-1 antitrypsin?
150-250 mg/dL.
39
What is TORCH screening?
Testing for total IgM antibodies against TORCH agents (CMV, Rubella, HSV, Toxoplasmosis).
40
What is the most important diagnostic tool for differentiating idiopathic neonatal hepatitis from EHBA?
Liver biopsy.
41
What is seen in a liver biopsy of idiopathic neonatal hepatitis?
Giant cell transformation and marked infiltration with inflammatory cells.
42
What is seen in a liver biopsy of EHBA?
Expansion of portal areas with fibrosis and bile duct proliferation.
43
What is the role of a HIDA scan in diagnosing cholestasis?
No dye excretion in biliary atresia; dye can reach the intestine in hepatitis.
44
What is a sign of biliary atresia on imaging?
Non-visualization or a micro-gallbladder after 4 hours of fasting.
45
What are the consequences of decreased bile delivery to the intestine?
Fat malabsorption (steatorrhea) and fat-soluble vitamin deficiencies (A, D, E, K).
46
What are the clinical manifestations of cholestasis?
Persistent jaundice, dark urine, pale stools, and hepatomegaly.
47
What are the manifestations of TORCH infections in neonatal cholestasis?
Cataracts, microcephaly, hepatosplenomegaly (HSM), and low birth weight.
48
What are the manifestations of galactosemia in neonatal cholestasis?
Cataracts, convulsions, and hepatomegaly.
49
What are the manifestations of Alagille syndrome?
Wide-spaced eyes, triangular face, heart defects (PS or VSD), and vertebral arch defects.
50
What are the complications of prolonged cholestasis?
Liver cirrhosis, portal hypertension, splenomegaly, and bleeding (hematemesis or intracranial hemorrhage).
51
What is the significance of pale stools in neonatal cholestasis?
Indicates decreased bile delivery to the intestine.
52
What is the significance of hepatomegaly in neonatal cholestasis?
Indicates liver involvement and possible progression to liver failure.
53
What is the role of bile in cholesterol excretion?
Bile is the body's only means of eliminating cholesterol and bile acids.
54
What is the significance of conjugated bilirubin in neonatal cholestasis?
Conjugated bilirubin is water-soluble and excreted in stools, while unconjugated bilirubin is fat-soluble and can cause kernicterus.
55
What is the significance of direct bilirubin in neonatal cholestasis?
Direct bilirubin > 20% of total bilirubin indicates cholestasis.
56
What is the significance of prothrombin time in neonatal cholestasis?
High INR and decreased prothrombin concentration indicate liver dysfunction.
57
What is the significance of AST and ALT in neonatal cholestasis?
Elevated levels indicate liver cell damage.
58
What is the significance of alkaline phosphatase (ALP) in neonatal cholestasis?
Elevated levels indicate bile duct obstruction or liver damage.
59
What is the significance of GGT in neonatal cholestasis?
Elevated levels indicate bile duct obstruction, except in PFIC type 1 and bile acid synthesis defects.
60
What is the significance of reducing substances in urine in neonatal cholestasis?
Indicates galactosemia.
61
What is the significance of succinyl acetone in urine in neonatal cholestasis?
Indicates tyrosinemia.
62
What is the significance of sweat chloride test in neonatal cholestasis?
Levels > 60 indicate cystic fibrosis.
63
What is the significance of alpha-1 antitrypsin level in neonatal cholestasis?
Deficiency (levels < 150 mg/dL) indicates alpha-1 antitrypsin deficiency.
64
What is the significance of TORCH IgM antibodies in neonatal cholestasis?
Indicates congenital infection with TORCH agents.
65
What is the significance of liver biopsy in neonatal cholestasis?
Differentiates between idiopathic neonatal hepatitis and EHBA.
66
What is the significance of HIDA scan in neonatal cholestasis?
Differentiates between biliary atresia and hepatitis based on dye excretion.
67
What is the significance of non-visualization of the gallbladder in neonatal cholestasis?
Indicates biliary atresia.
68
What is the significance of fat malabsorption in neonatal cholestasis?
Leads to steatorrhea and fat-soluble vitamin deficiencies.
69
What is the significance of fat-soluble vitamin deficiencies in neonatal cholestasis?
Leads to deficiencies in vitamins A, D, E, and K, causing various complications.
70
What is the significance of pruritus in neonatal cholestasis?
Caused by retained bile acids.
71
What is the significance of dark urine in neonatal cholestasis?
Indicates conjugated hyperbilirubinemia.
72
What is the significance of splenomegaly in neonatal cholestasis?
Indicates portal hypertension.
73
What is the significance of bleeding in neonatal cholestasis?
Indicates vitamin K deficiency and possible intracranial hemorrhage.
74
What is the significance of butterfly vertebrae in neonatal cholestasis?
Indicates Alagille syndrome, a condition associated with vertebral arch defects.
75
What is the significance of heart defects in neonatal cholestasis?
Indicates Alagille syndrome, which can include pulmonary stenosis (PS) or ventricular septal defect (VSD).
76
What is the significance of eye anomalies in neonatal cholestasis?
Indicates Alagille syndrome, which can include wide-spaced eyes and other eye abnormalities.
77
What is the significance of faltering growth in neonatal cholestasis?
Indicates chronic liver disease and malnutrition, often seen in progressive familial intrahepatic cholestasis (PFIC).
78
What is the significance of rickets in neonatal cholestasis?
Indicates vitamin D deficiency due to fat malabsorption.
79
What is the significance of intracranial hemorrhage in neonatal cholestasis?
Indicates vitamin K deficiency, which can lead to serious bleeding complications.
80
What is the significance of hematemesis in neonatal cholestasis?
Indicates portal hypertension and variceal bleeding.
81
What is the significance of splenomegaly in neonatal cholestasis?
Indicates portal hypertension, often due to liver cirrhosis.
82
What is the significance of liver cirrhosis in neonatal cholestasis?
Indicates end-stage liver disease, requiring liver transplantation.
83
What is the significance of portal hypertension in neonatal cholestasis?
Leads to complications like varices, splenomegaly, and bleeding.
84
What is the significance of variceal bleeding in neonatal cholestasis?
A serious complication of portal hypertension, requiring urgent treatment.
85
What is the significance of cholangitis in neonatal cholestasis?
A postoperative complication of the Kasai operation, indicated by fever.
86
What is the significance of biliary sludge in neonatal cholestasis?
Can be caused by certain antibiotics like cefoperazone, leading to bile flow obstruction.
87
What is the significance of liver enzymes in neonatal cholestasis?
Elevated levels indicate liver cell damage or bile duct obstruction.
88
What is the significance of elevated GGT in neonatal cholestasis?
Indicates bile duct obstruction, except in PFIC type 1 and bile acid synthesis defects.
89
What is the significance of normal GGT in neonatal cholestasis?
Suggests PFIC type 1 or bile acid synthesis defects.
90
What is the significance of elevated alkaline phosphatase (ALP) in neonatal cholestasis?
Indicates bile duct obstruction or liver damage.
91
What is the significance of elevated AST and ALT in neonatal cholestasis?
Indicates liver cell damage.
92
What is the significance of high INR in neonatal cholestasis?
Indicates liver dysfunction and impaired clotting factor synthesis.
93
What is the significance of decreased prothrombin concentration in neonatal cholestasis?
Indicates liver dysfunction and impaired clotting factor synthesis.
94
What is the significance of reducing substances in urine in neonatal cholestasis?
Indicates galactosemia, a treatable cause of cholestasis.
95
What is the significance of succinyl acetone in urine in neonatal cholestasis?
Indicates tyrosinemia, a metabolic disorder causing liver failure.
96
What is the significance of sweat chloride test in neonatal cholestasis?
Levels > 60 indicate cystic fibrosis, a cause of cholestasis.
97
What is the significance of alpha-1 antitrypsin level in neonatal cholestasis?
Deficiency (levels < 150 mg/dL) indicates alpha-1 antitrypsin deficiency, a cause of liver disease.
98
What is the significance of TORCH IgM antibodies in neonatal cholestasis?
Indicates congenital infection with TORCH agents, a cause of cholestasis.
99
What is the significance of liver biopsy in neonatal cholestasis?
Differentiates between idiopathic neonatal hepatitis and EHBA based on histology.
100
What is the significance of HIDA scan in neonatal cholestasis?
Differentiates between biliary atresia and hepatitis based on dye excretion.
101
What is the significance of non-visualization of the gallbladder in neonatal cholestasis?
Indicates biliary atresia, a common cause of neonatal cholestasis.
102
What is the significance of fat malabsorption in neonatal cholestasis?
Leads to steatorrhea and fat-soluble vitamin deficiencies.
103
What is the significance of fat-soluble vitamin deficiencies in neonatal cholestasis?
Leads to deficiencies in vitamins A, D, E, and K, causing various complications.
104
What is the significance of pruritus in neonatal cholestasis?
Caused by retained bile acids, leading to intense itching.
105
What is the significance of dark urine in neonatal cholestasis?
Indicates conjugated hyperbilirubinemia, a hallmark of cholestasis.
106
What is the significance of pale stools in neonatal cholestasis?
Indicates decreased bile delivery to the intestine, a sign of cholestasis.
107
What is the significance of hepatomegaly in neonatal cholestasis?
Indicates liver involvement and possible progression to liver failure.
108
What is the significance of bleeding in neonatal cholestasis?
Indicates vitamin K deficiency and possible intracranial hemorrhage.
109
What is the significance of varices in neonatal cholestasis?
Risk of bleeding and requires treatment like sclerotherapy or band ligation.
110
What is the significance of hepatic encephalopathy in neonatal cholestasis?
Indicates severe liver dysfunction and requires glucose infusion and neomycin.
111
What is the significance of Kasai operation in neonatal cholestasis?
Early surgical correction of EHBA to prevent further liver damage.
112
What is the significance of liver transplantation in neonatal cholestasis?
Required for end-stage liver disease or failed Kasai operation.
113
What is the significance of medium-chain triglycerides (MCT) in neonatal cholestasis?
Used as a fat replacement therapy due to better absorption.
114
What is the significance of ADEK vitamins in neonatal cholestasis?
Prevents deficiencies in fat-soluble vitamins.
115
What is the significance of ileal bile acid transporter inhibitors (IBAT) in neonatal cholestasis?
Reduces pruritus and improves bile acid transport.
116
What is the significance of cholestyramine in neonatal cholestasis?
Bile acid binder used to treat pruritus.
117
What is the significance of sclerotherapy in neonatal cholestasis?
Treatment for varices to prevent bleeding.
118
What is the significance of band ligation in neonatal cholestasis?
Treatment for varices to prevent bleeding.
119
What is the significance of glucose infusion in neonatal cholestasis?
Treatment for hepatic encephalopathy.
120
What is the significance of neomycin in neonatal cholestasis?
Reduces ammonia levels in hepatic encephalopathy.
121
How to differentiate at first look of jaundiced neonate
Olive green , normal birth w , clay colored stool from begin is atresia
Lbw giant cell ساعه تروح و ساعه تيجي
122
Colesstasis+mr+conv+cataract=
Galactosomia
123
Cholesat+murmur=
Algaille syndrome
124
Chole+wheezes
Alpha -1 anti trypsin , cf
125
Chole +catarct _pda +microcephaly
Cong rubella
126
Chole+albensim
Tyrsoinemia
127
Chole+mass +abdominal pain
Choledochal cyst
128
Largest HSM
Neiman gaucer
129
At which trim cong rubella
1
