Health and Me

Ishita Roy

Father Donates Liver To Save His One-Year-Old Son From A Rare Liver Disorder

A one-year-old baby from Uzbekistan rom suffering from a rare genetic liver disorder and was saved by his father who donated a part of his liver. The child was suffering with Alagille syndrome. The timely intervention of the doctors as well as father stepping in saved the child from a life-threatening disease. 

Right after birth the child got jaundice, while it is common for newborns, and appears two to three days after birth, it often resolves in one to two weeks. However, this was not the case with him. The baby required neonatal intensive care. When he was of just two months, he was diagnosed with biliary atresia, a condition that blocks the normal flow of bile from liver. The baby underwent a Kasai procedure, which is a surgery performed in infancy to help restore bile flow.

Also Read: <a href="https://www.healthandme.com/health-news/the-great-healthcare-plan-explained-what-is-in-president-trumps-health-care-proposal-article-153455387" data-type="tilCustomLink" target="_blank">The Great Healthcare Plan Explained: What Is In President Trump's Health Care Proposal?</a> 

However, his condition continued to worsen. The baby suffered from persistent jaundice, severe itching, poor weight gain, delayed growth and pale stools. All of these symptoms indicated progressive liver failure. 

This is when the family sought help from specialized liver care in India. Upon a careful assessment, a serious heart ailment too was detected in the baby. The investigation confirmed that the baby had Alagille syndrome. 

<h2>What Happens In Alagille Syndrome?</h2>

As per the National Institute of Health, Alagille syndrome is a multisystem autosomal dominant disorder with a wide variety of clinical manifestation. Johns Hopkins Medicine says it is an inherited condition in which bile builds up in the liver because there are too few bile ducts to drain the bile. This causes liver damage. 

This is caused by JAG1 gene mutation and could be passed from parent to child. If the child has one parent with Alagille syndrome, the child has 50% chance of developing the condition. Its rarity makes it appear in one out of 70,000 babies. 

Upon the case review, it was identified that only a <span>liver transplant</span> could save the child's life, this is when his father stepped in. 

Dr Ajitabh Srivastava, Director HPB Surgery and Liver Transplant, Max Super Speciality Hospital, Patparganj, whose team led the transplant said, "“Infants with prolonged jaundice are commonly evaluated for biliary atresia, for which the Kasai procedure is an early surgical treatment aimed at restoring bile flow and delaying the need for liver transplantation." However, he noted, rare conditions like Alagille syndrome could also mimic biliary atresia in early infancy. 

"In such cases, the Kasai procedure may not provide lasting benefit because the underlying problem is genetic rather than structural and in fact the wrong procedure (Kasai) worsens the condition of the child. As the liver disease progressed despite early surgery, a timely living donor liver transplant became the only life-saving option," the doctor explains. 

The transplant has been successful, noted Dr Srivastava, and the child is on a "steady path to recovery". The doctor especially pointed out how early diagnoses could save lives even from the rarest of the rare diseases.

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A one-year-old baby from Uzbekistan rom suffering from a rare genetic liver disorder and was saved by his father who donated a part of his liver. The child was suffering with Alagille syndrome. The timely intervention of the doctors as well as father stepping in saved the child from a life-threatening disease. What Happened to The Child?Right after birth the child got jaundice, while it is common for newborns, and appears two to three days after birth, it often resolves in one to two weeks. However, this was not the case with him. The baby required neonatal intensive care. When he was of just two months, he was diagnosed with biliary atresia, a condition that blocks the normal flow of bile from liver. The baby underwent a Kasai procedure, which is a surgery performed in infancy to help restore bile flow.Also Read: The Great Healthcare Plan Explained: What Is In President Trump's Health Care Proposal? However, his condition continued to worsen. The baby suffered from persistent jaundice, severe itching, poor weight gain, delayed growth and pale stools. All of these symptoms indicated progressive liver failure. This is when the family sought help from specialized liver care in India. Upon a careful assessment, a serious heart ailment too was detected in the baby. The investigation confirmed that the baby had Alagille syndrome. What Happens In Alagille Syndrome?As per the National Institute of Health, Alagille syndrome is a multisystem autosomal dominant disorder with a wide variety of clinical manifestation. Johns Hopkins Medicine says it is an inherited condition in which bile builds up in the liver because there are too few bile ducts to drain the bile. This causes liver damage. This is caused by JAG1 gene mutation and could be passed from parent to child. If the child has one parent with Alagille syndrome, the child has 50% chance of developing the condition. Its rarity makes it appear in one out of 70,000 babies. Upon the case review, it was identified that only a liver transplant could save the child's life, this is when his father stepped in. What Does The Expert Say?Dr Ajitabh Srivastava, Director HPB Surgery and Liver Transplant, Max Super Speciality Hospital, Patparganj, whose team led the transplant said, "“Infants with prolonged jaundice are commonly evaluated for biliary atresia, for which the Kasai procedure is an early surgical treatment aimed at restoring bile flow and delaying the need for liver transplantation." However, he noted, rare conditions like Alagille syndrome could also mimic biliary atresia in early infancy. "In such cases, the Kasai procedure may not provide lasting benefit because the underlying problem is genetic rather than structural and in fact the wrong procedure (Kasai) worsens the condition of the child. As the liver disease progressed despite early surgery, a timely living donor liver transplant became the only life-saving option," the doctor explains. The transplant has been successful, noted Dr Srivastava, and the child is on a "steady path to recovery". The doctor especially pointed out how early diagnoses could save lives even from the rarest of the rare diseases.