Which child with liver disease needs liver transplantation?

Liver transplantation is sometimes the only treatment for children  with acute or chronic liver disease that is progressive, life-threatening and unable to be successfully treated with other therapies such as medications and surgery. In children the most common indication is failed surgery for biliary atresia, a congenital condition. Liver transplantation has about a 90% one-year survival rate and thousands of people have benefited from this remarkable surgery all over the world and now in India.

When should you seek an opinion on liver transplantation?

In children with chronic liver disease of any etiology when there are signs & symptoms of advanced liver disease like uncontrolled ascites(fluid in the abdomen), encephalopathy ( mental changes due to liver disease), growth retardation, recurrent infections etc opinion should be sought for liver transplantation. It is important to remember that the results of this procedure are best when performed in time i.e neither early nor late. Of cpurse your family physician or paediatrician needs to be consulted on this but since liver transplantation is a relatively new field in India there is limited awareness even amongst medical professionals on this issue.

Where does one get a liver from?

There are two types of liver donors. The first type is a cadaveric donor i.e. an individual whose brain is irreversibly damaged and heart is about to stop and whose family members agree for donation of organs. Such donation is legal in India and several such transplants have been performed.The other type of liver donor is a live donor i.e. when a part of the liver of a close relative is removed and transplanted.  For a child this is usually one of the parents.

Who can be a liver donor?

For living donation, the person must be a close relative aged between 18-55 years, in perfect health and have a matching blood group. There is a series of tests that are performed on the donor to ascertain that he or she is fit for donation since it is a major operation for the donor also.

Is live donation safe?

Live  liver donation is safe since the liver has great reserve and regenerates to its original size quickly (within weeks) after a part of it is removed. Also for a child only a small part of the donors liver is removed.The donor suffers from no long-term effects, does not have to take any medication beyond 2-3 weeks, and is back to normalcy in a month. However there is a very low rate of a major complication associated with risk to life for the donor(0.01-0.05%).

What are the results of liver transplantation?

Liver transplantation is successful in more than 90% of patients . It success rate partly depends on the stage of liver disease as well as the cause but in most established centres survival after liver transplantation exceeds 70% at five years. More importantly it gives an excellent quality of life with a normal life expectancy for age. Besides relief from the symptoms of liver disease after transplantation children have a growth spurt. They attend regular school and take part in all activities including sport like any health growing child.

After a liver transplant, does a child have to take life long medicines and lead a very restricted life?

Yes, but the number of medicines and the doses decrease with passage of time. Most patients are only on small doses of 1 or 2 medicines at about a year after transplant, and down to a single medicine in 2-4 years which needs to be taken life-long. Although such persons do have a higher susceptibility to infection than usual, this rapidly diminishes by 3-6 months after transplant when the doses of the immunosuppressive medicine are reduced. So they have to be a little cautious initially, but later, even though the infection rate remains a little higher, this does not interfere with normal daily activities.

Which are the diseases in children which may need a Liver transplantation?

1. Diseases which are chronic and not salvageable with medical therapy (as these are advanced)

2. Autoimmune

3. Budd-Chiari syndrome

4. Wilson Disease

5.   Hepatitis B & C

6. Neonatal Hemochromatosis

7. Galactosemia

8. Metabolic: Tyrosinemia, Specific few Glycogen storage disorders, Familial Hypercholesterolemia,

                     Primary Oxaluria (Liver and Kidney transplant), Criggler Najjar Syndrome type I,

                     Mitochondrial disorders without neurological involvement,

                      Alpha -1- Antitrypsin deficiency (not seen in India), Methyl aminoaciduria

9. Failed Kasai surgery for Biliary Atresia beyond 3 months of age

10. Cholestatic disorder: Alagille's syndrome

                                                PFIC (progressive familial intrahepatic cholestasis),

                                      PSC (Primary Sclerosis colangitis)

11. Tumours: Hepatoblastoma, Hepatocellular carcinoma

12. Hepatopulmonry syndrome due to any cause

13. Acute Liver failure due to any cause satisfying King’s College Hospital Criteria.

      Ex. Hepatitis A and Hepatitis E in India,

      Drug induced liver failure, eg. anti-tubercular drugs.

Which are the disorders which are not cured by Liver transplantation?

1. Niemann-pick disease type C

2. Leukemia

3. Lymphoma

4. Hemophagocytic Lymphohistiocytosis

5. Giant cell hepatitis with Comb’s positive haemolytic anaemia

6. Mitochondrial disorders with neurological involvement.

Important articles on Pediatric Liver Transplantation in India

1. Cost and efficacy of immunosuppression using generic products following living donor liver

   Transplantation in India.

    Sudhindran S, Aboobacker S, Menon RN, Unnikrishnan G, Sudheer OV, Dhar P.

    Indian J Gastroenterol. 2012 Jan;31(1):20-3. doi: 10.1007/s12664-011-0138-0. Epub 2011 Dec 23.

    PMID: 22194185 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed?term=Cost%20and%20efficacy%20of%20imm...

2. Pediatric liver transplantation in India: The complete picture?

   Arora RS.

   J Indian Assoc Pediatr Surg. 2011 Jul;16(3):120. doi: 10.4103/0971-9261.83494. No abstract available.

   PMID: 21897580 [PubMed] Free PMC Article

http://www.ncbi.nlm.nih.gov/pubmed?term=Pediatric%20liver%20transplantat...

3. Reversal of severe Wilson arthropathy by liver transplantation.

    Nagral A, Sathe K.

    Indian Pediatr. 2011 May;48(5):406-7.

    PMID: 21654009 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed?term=Reversal%20of%20severe%20Wilson%....

4. Portal biliopathy treated with living-donor liver transplant: index case.

    Gupta S, Singhal A, Goyal N, Vij V, Wadhawan M.

    Exp Clin Transplant. 2011 Apr;9(2):145-9.

    PMID: 21453234 [PubMed - indexed for MEDLINE] Free Article

http://www.ncbi.nlm.nih.gov/pubmed?term=Portal%20biliopathy%20treated%20....

5. Pediatric liver transplantation: A report from a pediatric surgical unit.

    Rao S, D'Cruz AL, Aggarwal R, Chandrashekar S, Chetan G, Gopalakrishnan G, Dunn S.

    J Indian Assoc Pediatr Surg. 2011 Jan;16(1):2-7. doi: 10.4103/0971-9261.74512.

   PMID: 21430839 [PubMed] Free PMC Article

http://www.ncbi.nlm.nih.gov/pubmed/21430839

 

6. Pediatric liver transplantation in India: Its time has come.

    D'Cruz AL.

    J Indian Assoc Pediatr Surg. 2011 Jan;16(1):1. doi: 10.4103/0971-9261.74510. No abstract available.

    PMID: 21430838 [PubMed] Free PMC Article      http://www.ncbi.nlm.nih.gov/pubmed?term=Pediatric%20liver%20transplantat...

7. Outcome of live donor liver transplantation in Indian children with bodyweight < 7.5 kg.

    Kaur S, Wadhwa N, Sibal A, Jerath N, Sasturkar S.

    Indian Pediatr. 2011 Jan;48(1):51-4. Epub 2011 Aug 1.

    PMID: 20972305 [PubMed - indexed for MEDLINE] Free Article

http://www.ncbi.nlm.nih.gov/pubmed?term=Outcome%20of%20live%20donor%20li....

8. Successful living donor liver transplant in a very small child.

    Kumar V, Raina R.

    Indian J Anaesth. 2010 Jul;54(4):347-9. doi: 10.4103/0019-5049.68392.

    PMID: 20882182 [PubMed] Free PMC Article

http://www.ncbi.nlm.nih.gov/pubmed?term=Successful%20living%20donor%20li....

9. Living donor liver transplantation for giant cavernous hemangioma of liver in a child.

    Sood D, Mohan N, Singh A, Buxi TB, Nundy S, Soin AS.

    Pediatr Transplant. 2011 Nov;15(7):E135-8. doi: 10.1111/j.1399-3046.2010.01320.x. Epub 2010 Jun 27.

    PMID: 20598089 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed?term=Living%20donor%20liver%20transpl....

10. Reversal of nail changes after liver transplantation in a child.

      Gandhi V, Nagral A, Philip S, Malkani RH, Pimputkar R.

      Indian J Gastroenterol. 2009 Jul-Aug;28(4):154-6. doi: 10.1007/s12664-009-0054-8. Epub2009Nov24.

      PMID: 19937415 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed?term=Reversal%20of%20nail%20changes%2...

11. Reversal of severe hepato-pulmonary syndrome in congenital hepatic fibrosis after living-related liver

      transplantation.

      Nagral A, Nabi F, Humar A, Nagral S, Doctor N, Khubchandani SR, Amdekar YK.

      Indian J Gastroenterol. 2007 Mar-Apr;26(2):88-9.

      PMID: 17558074 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed?term=reversal%20of%20severe%20hepato-...

12. Liver transplantation in children.

      Kelly DA, Sibal A.

      Indian Pediatr. 2006 May;43(5):389-91. No abstract available.

      PMID: 16735759 [PubMed - indexed for MEDLINE] Free Article

http://www.ncbi.nlm.nih.gov/pubmed/16735759

13. India's first successful pediatric liver transplant.

      Poonacha P, Sibal A, Soin AS, Rajashekar MR, Rajakumari DV.

      Indian Pediatr. 2001 Mar;38(3):287-91. No abstract available.

      PMID: 11255309 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed?term=India's%20first%20successful%20p....

14. Need for liver transplantation in Indian children.

      Mehrotra P, Yachha SK.

      Indian Pediatr. 1999 Apr;36(4):356-61.

      PMID: 10717692 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed/10717692

15. Liver transplantation in children.

      Kelly DA, Sibal A.

      Indian Pediatr. 1999 Apr;36(4):353-5. Review. No abstract available.

      PMID: 10717691 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed/10717691

16. Selection of donors and organization of a donor system for liver transplantation in India.

      Patnaik PK.

      Trop Gastroenterol. 1997 Jul-Sep;18(3):92-4.

      PMID: 9385847 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed?term=Selection%20of%20donors%20and%20....

17. Pediatric liver transplantation: the Indian perspective.

      Bhatnagar V.

      Indian J Pediatr. 1996 Nov-Dec;63(6):751-60.

      PMID: 10830056 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed?term=Pediatric%20liver%20transplantation%3A%20the%20Indian%20perspective.%20%20%20%20%20%20%20Bhatnagar%20V..

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