Rare Liver Diseases
June 18, 2019
Apart from known liver diseases like cirrhosis, hepatitis and jaundice, there are numerous rare liver diseases too. When children are struck with such diseases, the solution is usually a liver transplant, where an unhealthy liver is replaced by a healthy one.
Here are a few rare liver diseases that benefit by liver transplants:
Alpha-1 Antitrypsin Deficiency
Alpha-1 Antitrypsin Deficiency is a hereditary disorder of the genes that lead to lung and liver disease. Among children, this is the most common gene-related cause of liver disease. This disease can begin in late childhood and early adulthood. A small percentage of adults with this disease usually end up with liver disease, often leading to liver failure.
The ratio of people to this disease worldwide is an estimated 1: 2,500. It is common across all ethnicities but is more commonly found among Europeans. When a child contracts this disease, it means that both parents carry this genetic trait.
Alpha-1 Antitrypsin is a protein made organically within the liver. It plays a stellar role in preventing the enzymes in several organs from breaking down. An inability to create a sufficient amount of this protein within the body typifies patients of Alpha-1 Antitrypsin.
- Swollen legs or abdomen
- Lack of appetite
- Abnormal liver tests
Alpha-1 Antitrypsin is detected by doing blood tests.
There is no known cure for Alpha-1 Antitrypsin. However, treating it involves an injecting synthetic alpha-1 antitrypsin, which helps prevent the lungs from deteriorating.
- Patients must ensure that their daily intake comprises essential nutrients.
- Along with this, a daily dose of Vitamins E, D and K should be taken.
A genetic disorder, Alagille Syndrome has the potential to affect the heart, liver and other organs. It causes liver damage due to defects in the bile ducts. These ducts are instrumental in transporting bile from the liver to the gall bladder and on to the small intestine.
This rare liver disease is congenital and affects babies and little children. The ratio of those who suffer from it is 1: 70,000.
Children who suffer from this rare liver disease show an increasing amount of loss of bile ducts by the age of one year. They also show up bile ducts narrowing outside the liver, causing damage o the liver cells. This could lead to cirrhosis which becomes chronic so that the liver does not perform well normally and a liver transplantation is the solution.
- Loose and pale stools
- Itchy skin
- Low growth from birth to three months
- Defective kidneys
- Problems in the spine, eyes and cardiovascular system
- Heart murmurs
- Family history
- Physical exam
- Eye tests
- Blood tests
- Imaging tests
- Liver biopsy
Treating this disease begins with increasing bile flow from the liver and normalizing the child’s physical and mental growth. It also includes setting right any nutritional shortcomings and taking medication to increase bile flow. Vitamins A, D, E and K should be taken regularly.
Why Rela Hospital
No matter how complicated and hopeless these cases may seem, our sensitive surgeons can treat them with the finest medication, surgery and post-operative observation. By being under the care of the best paediatric liver transplantation surgeon hospital in India, children with such rare liver diseases can get a new lease of life.
Welcome to the Dr Rela Hospital, Chennai, a unique and modern hospital for liver diseases which is a coming together of the finest minds in liver disease. This 450-bedded hospital stands testimony to the successful treatment of many children and adults who needlessly suffer from chronic liver diseases.
Dr Rela Hospital, Chennai is a liver diseases treatment hospital in Chennai which uses modern techniques and treatment methods for liver disease.
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