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Liver Flashcards

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1
Q

Features hepatic dysfunction

A 
Encephalopathy
Jaundice
Epistaxis
Cholestasis (pale stools, dark urine, deficiency fat soluble vit)
Ascites
Hypotonia
Peripheral neuropathy
Rickets (Vit D deficiency)
Varices with portal hypertension
Spider Naevi
Muscle wasting (malnutrition)
Bruising and petechia
Splenomegaly with portal hypertension
Hepatorenal failure
Palmar erythema
Clubbing
Loss of fat stores (malnutrition)
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2
Q

Physiological jaundice

A

Common
90% resolve by 2 w
- 3w if preterm

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3
Q

Feature Bile Duct Atresia

A 
Prolonged neonatal jaundice
Progressive fibrosis and obliteration of the biliary tree
Chronic liver failure and death within 2y
Mild Jaundice
Pale stools
Faltering growth
Hepatomegaly
Splenomegaly
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4
Q

Ix Biliary atresia

A

Raised conjugated bilirubin
Abnormal LFTs
Fasting abdo US: contracted or absent gall bladder
Dx by ERCP: failure to outline normal biliary tree
Liver biopsy: neonatal hepatitis w extrahepatic biliary obstructive features

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5
Q

Mx Biliary Atresia

A

Palliative surgery:
-Kasai hepatoportoenterostomy (jejenum anastamoses with porta hepatis)
-fibrotic ducts bypassed to allow biliary drainage
Early surgery increases success rate
Fat soluble vitamin supplementation
Liver transplant

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6
Q

Cx Biliary Atresia

A

Cholangitis
Cirrhosis
Malnutrition

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7
Q

Causes of prolonged neonatal jaundice

A 
Breast milk jaundice
Infection (esp UTI)
Haemolytic anaemia
Hypothyroidism
High GI obstruction
Critter-Najjar syndrome
Biliary Atresia
Choledochal cysts
Neonatal hepatitis syndrome
Intrahepatic biliary hypoplasia (alagille syndrome)
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8
Q

Causes of neonatal hepatitis syndrome

A 
Congenital infection
Inborn errors of metabolism
alpha1-Antitripsin deficiency
Galactosaemia
Tryosinaemia type 1
Errors of bile acid synthesis
Progressive familial intrahepatic cholestasis
Cystic fibrosis
Intestinal failure associated liver disease (parental nutrition)
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9
Q

Features Alagille syndrome

A

AD inheritance
Characteristic triangular face
Skeletal abnormality .e.g. Butterfly vertebrae
Congenital heart disease: peripheral pulmonary stenosis
Renal tubular disorders
Eye defects
Profoundly choleostatic with pruritis and faltering growth

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10
Q

Mx Alagille Syndrome

A 
Confirmed by genetic test
Nutrition and fat soluble vitamins
Pruritis difficult to manage
Liver transplant
Death by cardiac cause
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11
Q

Features Progressive familial intrahepatic cholestasis

A 
AR disorder affecting bile salt transport
Jaundice
Intense pruritis
Faltering growth
Rickets
Diarrhoea
Hearing loss
Gallstones
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12
Q

Mx Progressive familial intrahepatic cholestasis

A

Dx by gene mutation in bile salt transporter genes
Nutritional support and fat soluble vitamins
Liver transplant due to progression of fibrosis
Pruritis difficult to manage

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13
Q

Features alpha1-Antitypsin deficiency

A

AR disorder (protease inhibitor PiZZ chromosome 14)
Protein accumulation within hepatocytes
Liver disease in infants and children
Lung disease in adulthood

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14
Q

Presentation alpha1-Antitrypsin deficiency

A 
Prolonged neonatal jaundice
Bleeding due to Vit K defieincy
Hepatomegaly
Splenomegaly
Cirrhosis
Portal hypertension
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15
Q

Mx alpha1-Antitrypsin Deifiency

A

Dx on enzyme level in plasma and protein phenotype
50% have good prognosis
Liver transplant
Avoid smoking: risk of pulmonary disease

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16
Q

Features Galactosaeia

A 
Rare
Poor feeding
Vomiting
Jaundice
Hepatomegaly 
Symptomatic when fed milk
Untreated leads to cataracts and developmental delay
Late features: ovarian failure, learning difficulty
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17
Q

Galactosaemia fatal course

A

Usually caused by Gram negative sepsis
Shock
Haemorrhage
DIC

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18
Q

Ix Galactosaemia

A

Detecting galactose in urine
Enzyme galactose-1-phosphate-uridyl transferase in rbc
Can be masked by recent blood transfusion

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19
Q

Mx Galactosaemia

A

Galactose free diet prevent liver disease progression

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20
Q

Features Fulimant Hepatitis

A 
Development of hepatic necrosis with subsequent loss of liver function
\+/- liver encephalopathy
Due to infection or metabolic conditions
Jaundice
Coagulopathy
Hypoglycaemia
Electrolyte disturbance
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21
Q

Early signs hepatic encephalopathy

A

Alternate periods of irritability and confusion with drowsiness
Aggressive and difficult behaviour

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22
Q

Cx fulminant hepatitis

A

Cerebral oedema
Haemorrhage
Sepsis
Pancreatisis

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23
Q

Dx fulminant hepatitis

A 
Bilirubin normal in early stages
Transaminases elevated 10-100x normal level
Alkaline phosphatase increased
Abnormal coagulation
Plasma ammonia increased
EEG shows hepatic encephalopathy 
CT cerebral oedema
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24
Q

Mx fulminant hepatitis

A

Maintain blood glucose >4mmol/L: IV dextrose
Broad spectrum Abx : sepsis prevention
Antifungals
IV vitamin K
H2 blocking/PPIs: prevent GI haemorrhage
Fluid restriction and mannitol in cerebral oedema

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25
Poor prognostic factors fulminant hepatitis
``` Shrinking liver Rising bilirubin Falling transaminases Worsening coagulopathy Progression to coma -Without liver transplant ~ 70% in coma will die ```
26
Causes of acute liver failure <2y
``` Infection .e.g. Herpes simplex Metabolic disease Seronegative hepatitis Drug induced Neonatal haemochromatosis ```
27
Causes liver failure >2y
``` Seronegative hepatitis Paracetamol OD Mitochondrial disease Wilson disease Autoimmune hepatitis ```
28
Causes chronic liver disease
``` Post viral hepatitis B/C Autoimmune hepatitis Sclerosing cholangitis Drug induced liver disease Cystic fibrosis Wilson disease Fibropolycystic liver disease Non-alcoholic fatty liver disease Alpha1-antitrypsin deficiency ```
29
Features Viral hepatitis
``` Nausea Vomiting Abdominal pain Lethargy Jaundice Large tender liver Splenomegaly Raised transaminases Normal coagulation ```
30
Features Hepatitis A
Mild illness 2-4w | Self limiting prolonged cholestatic hepatitis and fuliminant hepatitis
31
Mx Hepatitis A
IgM antibody Dx No treatment Vaccination of close contacts within 2w of onset Human normal immunoglobulin in those at high risk
32
Transmission of hepatitis B
Perinatal from mother to baby Horizontal spread within families Inoculation with infected blood via needles, dialysis etc. Sexual transmission
33
Outcome for infants with HBV
Usually asymptomatic | 90% become chronic carriers
34
Outcomes for older children with HBV
Usually asymptomatic Majority resolve spontaneously 1-2% develop acute liver failure 5-10% chronic carriers
35
Dx Hepatitis B
HBV antigens and antibodies IgM core antibodies in acute infection HBV surface antigen ongoing infectivity
36
Cx chronic hepatitis
Cirrhosis | Hepatocellular carcinoma
37
Mx chronic HBV
Poor efficacy Interferon or Pegylated interferon successful in 30-50% Antiviral .e.g. Lamivudine, adefovir effective in 25%
38
Prevention hepatitis B
Antenatal maternal screening Surface antigen positive: babes receive vaccination from birth E Antigen positive: hepatitis B immunoglobulin Check antibody response to vaccination at 12m -further vaccination required in 5% Rest of family vaccinated
39
Features Hepatitis C
IVDU Vertical transmission greater in HIV co-infection Majority become chronic carriers: cirrhosis and hepatocellular carcinoma risk
40
Mx hepatitis C
Pegylated interferon and ribavirin is curative | Treatment >3y old: may resolve with viral infections
41
Features hepatitis D
Cannot replicate without HBV Causes acute exacerbation of chronic HBV Cirrhosis in chronic infection
42
Features Hepatitis E
Mild self limiting illness in most Associated with water, infected pork, blood transfusion During pregnancy: fulminant hepatic failure with high mortality
43
Features autoimmune hepatitis and sclerosing cholangitis
Presents 7-10y More common in girls Acute hepatitis, fulminant hepatitis, or chronic liver disease Autoimmune features: skin rash, arthritis, haemolytic anaemia, nephritis Association with IBD, coeliac, other autoimmune disease
44
Dx Autoimmune hepatitis and sclerosing cholangitis
``` Elevated total protein Hyperglammaglobulinaemia IgG >20g/L Positive autoantibodies Low serum complement C4 Typical histology ```
45
Mx Autoimmune hepatitis and sclerosing cholangitis
Prednisone Azathioprine Urosodeoxycholic acid- sclerosing cholangitis
46
Liver disease in CF
Second most common cause of death Most common abnormality steatosis Other cx; progressive biliary fibrosis from thick tenacious bile, cirrhosis, portal hypertension Histology: fatty liver, focal biliary fibrosis, focal modular cirrhosis
47
Mx Liver disease in CF
``` Supportive therapy Endoscopic treatement of varices Nutritional therapy Ursodeoxycholic acid Liver transplantation/ heart-lung transplantation ```
48
Features Wilson disease
AR mutation in caeruloplasmin Defective excretion of copper in the bile Accumulation of copper in brain, liver, kidney, cornea Presents >3y
49
Presentation Wilson disease
``` Young children: liver disease Older children: neuropsychiatric disease (extrapyrimidal signs) Renal tubular dysfunction Vitamin D resistant rickets Haemolytic anaemia Kayser-fleischer rings develop >7y ```
50
Ix Wilson disease
Low serum caeruloplasmin and copper Increased urinary copper excretion -further increased by cheating agent penicillamie Dx hepatic copper on biopsy or gene mutation
51
Mx Wilson disease
``` Penicillamine or trientine -promote urinary copper excretion -reduce hepatic and CNS copper Zinc: reduces copper absorption Pyridoxine: to prevent peripheral neuropathy ```
52
Features Fibroplastic Liver Disease
Inherited condition affecting biliary tree development Liver cystic disease Fibrosis Renal disease
53
Features congenital hepatic fibrosis
``` Children >2y Hepatosplenomegaly Abdominal distension Portal hypertension Normal LFTs Coexisting cystic renal disease: hypertension and renal dysfunction Histology: bands of hepatic fibrosis ```
54
Cx congenital hepatic fibrosis
Portal hypertension Varices Cholangitis
55
Features non-alcoholic fatty liver disease
Fatty deposits and inflammation, fibrosis, cirrhosis and liver failure Associated with metabolic syndrome and obesity Usually asymptomatic RUQ pain, lethargy
56
Ix Non-alcoholic fatty liver disease
Echogenic liver on US Mildly elevated transaminases Biopsy: marked steatosis +/- inflammation and fibrosis
57
Cx chronic liver disease
``` Nutrition: fat malabsorption (long chain), protein malnutrition (high liver catabolism), anorexia (NG feed) Fat soluble vitamin (oral or IM) Pruritus Encephalopathy Cirrhosis Portal hypertension Oesophageal varices Ascites Spontaneous bacterial peritonitis Renal failure ```
58
Indications for liver transplant
Severe malnutrition unresponsive to therapy Cx refractory management: bleeding, varices, ascites Poor quality of life Failure of growth and development
59
Post transplanation Cx Liver
``` Primary non function Hepatic artery thrombosis Biliary leaks and strictures Rejection Sepsis Death occurs within first 3m 1y survival ~ 90% 20y survival >80% ```
60
Mx Pruritis in liver disease
``` Loose cotton clothing Avoid overheating Cut nails Emollients Phenobarbital: stimulate bile flow Cholestyramine: absorb bile salts Ursodeoxycholic acid: solubise bile Rifampicin: enzyme inducer ```
61
Fat soluble vitamins defiencies
K: bleeding A: retinal changes and night blindness E: peripheral neuropathy, haemolysis, ataxia D: Rickets and fractures
62
Features Cirrhosis
Extensive fibrosis with regenerative nodules 2' to hepatocellular disease/chronic bile duct obstruction Diminished hepatic function Portal hypertension Splenomegaly, varices, ascites Risk of hepatocellular carcinoma
63
Signs of cirrhosis
``` Jaundice Palmar erythema Plantar erythema Telangiectasia Spider navaei Malnutrition Hypotonia Dilated abdominal veins Splenomegaly Shrunken and impalpable liver ```
64
Screening in cirrhosis
Cause of chronic liver disease Upper GI endoscopy: varices and erosive gastritis Abdominal US: liver, spleen, varices Biopsy
65
Ix decompensated cirrhosis
Elevated aminotransferases and alkaline phosphatase Decreased plasma albumin Prolonged PT
66
Mx oesophageal varices
Upper GI endoscopy Blood transfusion and H2 antagonist in acute bleeds Octreotide infusion, vasopressin analogues, endoscopic band ligation and sclerotherapy in persistent bleeds Portocaval shunting Stent placement between hepatic and portal vein

Paediatrics (22 decks)

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