Liver Transplantation

LIVER TRANSPLANTATION

Q. 1

A 51-year-old man is seen in clinic because he feels chronically ill. Physical examination is notable for a palpable, nodular liver. Serum studies for hepatitis virus markers are positive for anti-HCV and negative for HbsAg and HBcAg. Core biopsy of the liver is performed to evaluate the extent of his disease, and demonstrates chronic hepatitis with severe activity and severe fibrosis. The patient is treated with interferon for 6 months, but fails to respond. His condition continues to deteriorate, and he is treated with orthotopic liver transplantation. One year after orthotopic liver transplantation, the man develops rising transaminase and bilirubin levels. In order to minimize chronic rejection injury to hepatic endothelial cells, immunosuppressive therapy should be aimed at downregulating which of the following components of the immune response?

 A

Autoantibody production

 B

Complement protein synthesis

 C

HLA antigen expression

 D

T-lymphocyte activity

Q. 1

A 51-year-old man is seen in clinic because he feels chronically ill. Physical examination is notable for a palpable, nodular liver. Serum studies for hepatitis virus markers are positive for anti-HCV and negative for HbsAg and HBcAg. Core biopsy of the liver is performed to evaluate the extent of his disease, and demonstrates chronic hepatitis with severe activity and severe fibrosis. The patient is treated with interferon for 6 months, but fails to respond. His condition continues to deteriorate, and he is treated with orthotopic liver transplantation. One year after orthotopic liver transplantation, the man develops rising transaminase and bilirubin levels. In order to minimize chronic rejection injury to hepatic endothelial cells, immunosuppressive therapy should be aimed at downregulating which of the following components of the immune response?

 A

Autoantibody production

 B

Complement protein synthesis

 C

HLA antigen expression

 D

T-lymphocyte activity

Ans. D

Explanation:

We are presently undergoing a hidden epidemic of hepatitis C infection in this country.

While many individuals may have very slowly progressive courses with good health for decades, some develop cirrhosis relatively earlier in their disease process. These individuals are increasingly becoming candidates for liver transplantation, although the number of available livers is far less than the number of people with end-stage hepatitis C infection who might use one.

Hepatitis C can recur in the new liver, but often does not become a serious problem.

Instead, chronic rejection turns out to more often limit the longevity of the transplant.

Chronic rejection of any solid organ entails cellular injury to endothelial cells, resulting in intimal proliferation, fibrosis, and eventually, ischemic injury to the graft. Immunosuppressive therapy is directed at controlling lymphocyte activity and minimizing cellular rejection.

Autoantibodies are not involved in organ transplant rejection.
The antibodies produced are alloantibodies directed only to the graft, but not to the host.
 
Complement proteins are involved in the humoral component of acute rejection, and complement binding to alloantibodies increases graft damage.
Complement protein production, however, is not affected by immunosuppressive therapy.
 
HLA antigen expression is central to recognition of foreign cells in grafted tissue.
HLA antigens are expressed constitutively by all normal cells, and immunosuppression does not affect their production.

Q. 2

In orthotropic liver transplantation, which is the best way to get bile drainage in donor liver?

 A

Donor bile duct with recipient bile duct or Roux en Y choledochojejunostomy

 B

Donor bile duct with duodenum of recipient

 C

Donor bile duct with jejunum of recipient

 D

External drainage for few days followed by choledochojejunostomy

Q. 2

In orthotropic liver transplantation, which is the best way to get bile drainage in donor liver?

 A

Donor bile duct with recipient bile duct or Roux en Y choledochojejunostomy

 B

Donor bile duct with duodenum of recipient

 C

Donor bile duct with jejunum of recipient

 D

External drainage for few days followed by choledochojejunostomy

Ans. A

Explanation:

In orthotropic liver transplantaion, the bile duct between recipent and donor liver are repaired in end to end fasion or else  Roux en Y choledochojejunostomy.
 
Ref: Atlas of Upper Gastrointestinal and Hepato-Pancreato-Biliary Surgery By Pierre-Alain Clavien, 2007, Page 475

 


Q. 3

Indications of Liver transplantation are All/Except

 A

Biliary atresia

 B

Sclerosing cholangitis

 C

Hepatitis A

 D

Cirrhosis

Q. 3

Indications of Liver transplantation are All/Except

 A

Biliary atresia

 B

Sclerosing cholangitis

 C

Hepatitis A

 D

Cirrhosis

Ans. C

Explanation:

Ans. is ‘c’ i.e. Hepatitis A 

Liver transplantation is indicated for those children and adults, who in the absence of contraindications suffer from severe, irreversible liver disease for which alternative medical or surgical treatments have been exhausted or are unavailable.

  • Most common indication

in children ________  Biliary atresia

in adults  –> Cirrhosis

Harrison 17/e writes- “Currently, chronic hepatitis C and alcoholic liver disease are the most common indications for liver transplantation, accounting for over 40% of all adult candidates who undergo the procedure.”

Indications for         ver Transplantation

Adults

Children

Biliary atresia

Primary biliary cirrhosis

Neonatal hepatitis

Secondary biliary cirrhosis

Congenital hepatic fibrosis

Primary sclerosing cholangitis

Alagille’s disease

Autoimmune hepatitis

Byler’s disease

Caroli’s disease

al- antitrypsin deficiency

Cryptogenic cirrhosis

Inherited disorders of metabolism

Chronic hepatitis with cirrhosis

Wilson’s disease

Hepatic vein thrombosis

Tyrosinemia

Fulminant hepatitis

Glycogen storage diseases

Alcoholic cirrhosis

Lysosomal storage diseases

Chronic viral hepatitis

Protoporphyria

Primary hepatocellular malignancies

Crigler-Najjar disease type I

Hepatic adenomas

Familial hypercholesterolemia

Nonalcoholic steatohepatitis

Primary hyperoxaluria type I

Familial amyloid polyneuropathy

Hemophilia

 

 

Contraindications to Liver Transplantation

Absolute

Relative

Uncontrolled extrahepatobiliary infection

Age >70

Active, untreated sepsis

Prior extensive hepatobiliary surgery

Uncorrectable, life-limiting congenital anomalies

Portal vein thrombosis

Active substance or alcohol abuse

Renal failure

Advanced cardiopulmonary disease

Previous extrahepatic malignancy (not

including nonmelanoma skin cancer)

Extrahepatobiliary malignancy (not including

nonmelanoma skin cancer)

Severe obesity

Metastatic malignancy to the liver

Severe malnutrition/wasting

Cholangiocarcinoma

Medical noncompliance

AIDS

HIV seropositivity

Life-threatening systemic diseases

Intrahepatic sepsis

Severe hypoxemia secondary to right-to-left

intrapulmonary shunts (P02 < 50 mmHg)

Severe pulmonary hypertension (mean PA

pressure >35 mmHg)

Uncontrolled psychiatric disorder


Q. 4

In orthotopic liver transplantation, which is the best way to get bile drainage in donor liver:

 A

Anastomose donor bile duct with the duodenum of recipient

 B

Anastomose donor bile duct with the jejunum of recipient

 C

Anastomose donor bile duct with recipient bile duct or Roux-en-Y choledochojejunostomy

 D

External drainage for few days followed by choledochojejunostomy

Q. 4

In orthotopic liver transplantation, which is the best way to get bile drainage in donor liver:

 A

Anastomose donor bile duct with the duodenum of recipient

 B

Anastomose donor bile duct with the jejunum of recipient

 C

Anastomose donor bile duct with recipient bile duct or Roux-en-Y choledochojejunostomy

 D

External drainage for few days followed by choledochojejunostomy

Ans. C

Explanation:

Ans is ‘c’ i.e. Anastomose donor bile duct with recipient bile duct or Roux-en-Y choledochojejunostomy

“Caval, portal vein, hepatic artery, and bile duct anastomoses are performed in succession, the last by end-to-end suturing of the donor and recipient common bile ducts or by choledochojejunostomy to a Roux-en- Y loop if the recipient common bile duct cannot be used for reconstruction (e.g., in sclerosing cholangitis).”- Harrison 17/e p

Bile duct anastomosis in orthotopic liver transplant is done by

  1. Choledochocholedochostomy or
  2. Roux-en-Y choledochojejunostomy

Choledochocholedochostomy

end to end anastomosis between donor CBD and the recipient CBD

used when the recipient bile duct is not diseased

preferred method of bile duct anastomoses

Roux-en-Y choledochojejunostomy

alternative bile duct anastomosis when choledochocholedocostomy is not possible, that is when recipient extrahepatic bile duct is diseased (eg. sclerosing cholangitis), or small or when there is significant recipient donor duct size mismatch.


Q. 5

Commonest indication for….. liver transplantation  in infant is –

 A

Alcoholic cirrhosis

 B

Biliary cirrhosis

 C

Primary hemochromatosis

 D

Biliary atresia

Q. 5

Commonest indication for….. liver transplantation  in infant is –

 A

Alcoholic cirrhosis

 B

Biliary cirrhosis

 C

Primary hemochromatosis

 D

Biliary atresia

Ans. D

Explanation:

Ans is d i.e.; Biliary atresia


Q. 6

Liver transplantation was first done by ‑

 A

Starzl

 B

Huggins

 C

Carrel

 D

Christian Benard

Q. 6

Liver transplantation was first done by ‑

 A

Starzl

 B

Huggins

 C

Carrel

 D

Christian Benard

Ans. A

Explanation:

Ans. is ‘a’ i.e., Starzl 

The first human liver transplant was performed in 1963 by Dr. Thomas Starzl.


Q. 7

Following liver transplantation, recurrence of primary disease in the liver most likely occures in:

 A

Wilson disease

 B

Autoimmune hepatitis

 C

Alpha – 1 antitrypsin deficiency

 D

Primary biliary cirrhosis

Q. 7

Following liver transplantation, recurrence of primary disease in the liver most likely occures in:

 A

Wilson disease

 B

Autoimmune hepatitis

 C

Alpha – 1 antitrypsin deficiency

 D

Primary biliary cirrhosis

Ans. B

Explanation:

Answer is B (Autoimmune hepatitis):

Amongst the options provided, recurrence of primary disease in the liver following liver transplantation is most common with auto immune hepatitis.

‘Autoimmune hepatitis has been recognized to recur in upto 40% of transplanted livers as immuno supression is reduced.’ – CMDT’ 2006 / 663


Q. 8

Indications for Liver Transplantation include:

 A

Hemochromatosis

 B

Primary Biliary Cirrhosis

 C

Sclerosing cholangitis with ulcerative colitis

 D

All

Q. 8

Indications for Liver Transplantation include:

 A

Hemochromatosis

 B

Primary Biliary Cirrhosis

 C

Sclerosing cholangitis with ulcerative colitis

 D

All

Ans. D

Explanation:

Answer is D All (All of the above) :

Biliary atresia is an indication for Liver transplantation in children while primary Biliary Cirrhosis, Sclerosing cholangitis and Hematochromatosis an established indications for Liver transplantation in adults

Indication for Liver Transplantation:

Children

  • Biliary atresia
  • Neonatal hepatitis
  • Congenital hepatic fibrosis
  • Alagille’s disease”
  • Byler’s disease
  • a 1 -Antitrypsin deficiency
  • Inherited disorders of metabolism
  • Wilson’s disease
  • Tyrosinemia
  • Glycogen storage diseases
  • Lysosomal storage diseases
  • Protoporphyria
  • Crigler-Najjar disease type I
  • Familial hypercholesterolemia
  • Primary hyperoxaluria type I
  • Hemophilia

Aduts

  • Primary biliary cirrhosis
  • Secondary biliary cirrhosis
  • Primary sclerosing cholangitis
  • Autoimmune hepatitis
  • Carob’s disease’
  • Cryptogenic cirrhosis
  • Chronic hepatitis with cirrhosis
  • Hepatic vein thrombosis
  • Fulminant hepatitis
  • Alcoholic cirrhosis
  • Chronic viral hepatitis
  • Primary hepatocellular malignancies
  • Hepatic adenomas
  • Nonalcoholic steatohepatitis
  • Familial amyloid polyneuropathy

“Arteriohepatic dysplasia, with paucity of bile ducts, and congenital malformations, including pulmonary stenosis.

Intrahepatic cholestasis, progressive liver failure, mental and growth retardation

`Multiple cystic dilatations of the intrahepatic biliary tree.

Hematochromatosis is also an indication for Liver Transplantation

‘Among genetic and metabolic conditions, Hematachroatosis is the most common reason for Liver Transplantation’



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