- 14-yr-old suffering from Crigler–Najjar Syndrome (CNS) successfully operated upon
- Liver transplant gives a new lease of life to CNS type I patients
- CNS is a very rare disease estimated at 0.6-1.0 per million
Mumbai June 12, 2015: Global
Hospitals Mumbai - one of the fastest growing chains of tertiary care,
multi-super specialty and multi-organ transplant hospitals in India, has
added one more feather to its cap, by performing Western India’s first
auxiliary liver transplant on a 14 year old patient from Mumbai
suffering from Crigler–Najjar Syndrome (CNS). The liver was donated by Yash’s aunt.
Crigler-Najjar
Syndrome Type I is a very rare inherited disorder in which bilirubin (a
substance made by the liver) cannot be broken down. It occurs when the
enzyme that normally converts bilirubin into a form that can easily be
removed from the body does not work correctly. Without this enzyme,
bilirubin can build up in the body and lead to jaundice (yellow
discoloration of skin and eyes) and damage to the brain, muscles, nerves
and loss of life in extreme conditions. This is a very rare disease
(estimated at 0.6–1.0 per million live births).
The syndrome runs
in families (inherited). A child must receive a copy of the defective
gene from both parents to develop the severe form of the condition.
Parents who are carriers (with just one defective gene) have about half
the enzyme activity of a normal adult, but do not have symptoms.
Lifelong light treatment (photo therapy), blood transfusion and liver transplant are used for CNS treatment.
The 14 year old
resident of Oshiwara, Yash Ranjit was detected with CNS, 14 days after
he was born and was on photo therapy since then. Yash had to undergo
daily photo therapy for 6 hours a day which increased to 12-14 hours a
day as he grew up, to keep his bilirubin under control; else he would
have suffered brain damage.
Yash’s parents
were very disturbed with his condition, but were reluctant to undergo a
liver transplant due to various reasons and fear. They imported a photo
therapy machine to keep Yash under light. Due to this condition, Yash
could not lead a normal life like other children of his age, as he had
to compulsorily take photo therapy, and was confined to the four walls
of his home.
During this
course, one day Yash got seizure and his parents could not see the
plight of their son. That’s when they decided to go for liver
transplant.
It was not an easy choice for the parents. Both
his parents turned out to be unfit for a liver donation. They were in a
state of flux, and then his aunt came forward and agreed to donate part
of her liver to Yash. Yash came to
Global Hospital and the team of experts at Global Hospital decided to
perform the rare complex auxiliary liver transplant on him. The
transplant was successfully performed and Yash has been discharged from
the hospital and now can lead a normal life, but will have to be on
immunosuppressant medications.
Prof. Mohamed Rela, Group Director, Institute of Liver Diseases, HPB Surgery and Transplantation said,
“Auxiliary liver transplant is a complex procedure, unlike a normal
liver transplant, wherein a complete liver is transplanted. The
procedure is a technically demanding one. In this we remove a part of
the liver and replace it with a part of the donor liver. Once the
patient's own liver regenerates, then the need for donor liver no more
exists. In most cases, the donor liver shrinks and becomes a small
tissue. In some, another surgery is needed to remove it.”
Dr. Ravi Mohanka - Chief Surgeon and HOD, HPB Surgery and Liver Transplantation Global Hospitals, Mumbai said,
“Yash is suffering from a rare genetic disorder due to which, his
liver was not able to produce a certain enzyme, which is needed for
detoxification, due to which his bilirubin was always shooting up and he
had to be under photo therapy constantly. However, Phototherapy does
not work after an age because the thickened skin blocks the light. With
the successful transplant, the other part of his liver has started
producing desired enzyme and his liver function has become normal. ”
Dr. Parijat
Gupte, Consultant Hepatologist, Hepatology, Liver Intensive Care &
Transplant Hepatology Deaprtment, Global Hospitals, Mumbai said,
“The patient was on high risk of getting severe brain damage due to the
high bilirubin levels, had he been not operated upon quickly. As soon
as Yash came to Global Hospitals, the doctors decided to perform
auxiliary liver transplant, as it was the only option to save him. The
patient is doing good and has been discharged from the hospital and will
be able to lead a normal life hereafter.”
Yash’s liver transplant took place on 28th
May. During the surgery a portion of Yash’s liver was removed and
replaced with a small portion of his aunt’s liver. Post – operative
complications were managed by liver intensive care team. He got
discharged on 10th June with a normally functioning liver.
Also known as
Auxiliary Partial Orthotopic Liver Transplantation (APOLT); the
procedure entails attaching part of a donor liver to the failing liver
in the recipient, where it supports the patient, clears toxins, and
prevents brain injury during recovery. After the patient's native liver
recovers, the donor liver withers in most patients and the majority of
patients are able to withdraw from immunosuppressant medications.
The expert team of
doctors comprised Dr. Samir Shah, Dr. Guruprasad Shetty, Dr. Somnath
Chattopadhyay and Dr. Vishal Sachde. Yash’s parents are extremely
thankful and relieved post the surgery and are very happy with their
decision. Global Hospital
Mumbai has already established itself in the transplant space and has
handled many complex liver surgeries in the past, including the
successful Dual Lobe Living Donor Liver Transplant in the past. With the
first ever auxiliary liver transplant in Global Hospital Mumbai, the
hospital as yet again reiterated its commitment to provide world class
health care facilities to the patients.
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