العربية
English
Liver Diseases
What is liver?
Liver is an organ, which is around 1.2 kg in an adult situated on the top right quadrant of the abdomen.
What does liver do?
Liver is an important organ. Blood from stomach and intestine passes through liver , where liver extracts
Read More
nutrients from the blood and produces protein, sugar, fat etc necessary for growth and development of body. It produces substance that helps in blood clotting. It also removes drugs and toxins from blood and cleans it. It has also got immune function and protects us from harmful pathogens.
What causes Liver disease?
Inborn errors of metabolism, anatomical malformations of liver, bacterial and viral infection, tumours etc can cause liver disease.
How does children liver disease differ from adults?
Usually children are born with liver disease, which could manifest ever after few days while most of the adult disorders are aquired.
Jaundice in Newborn: Act Swiftly
Jaundice, which persists after two weeks in term babies and three weeks in preterm babes, is pathological and requires further investigation. Apart from blood group incompatablity( rhesus & ABO), lot of anatomical and functional disorders of liver could cause jaudice during neonatal period. Anatomical disorers such as biliary atresia, cysts etc, hereditary disorders such as Progressive Familial Intrahepatic cholestasis (PFIC), Alagile etc and metabolic problems such as galctosemia , tyrosenemia could cause jaundice during neonatal period.
Biliary Atresia
Biliary atresia is a condition where the bile ducts( tubes draining bile from liver to intestine) are blocked or absent. It is of utmost importance to diagnose this condition early, so that surgical treatment could be initiated. Over half of infants undergoing surgical treatment (portoenterostomy) for biliay atresia with in first 2-3 months will clear the jaundice and have a greater than 80% chance of a good quality of life, reaching adolescence without liver transplantation. Baby’s urine is colorless, if there is dark urine along with suspected pale stools (see chart) , consult your pediatrician urgently
Normal
Biliary Atresia
Acute liver failure
The liver could fail suddenly in previously well child, resulting in jaundice and synthetic failure. The cause could be due to infection, metabolic liver conditions, autoimmune disease etc. In around 30 to 40% of cases the even after exhaustive investigations the diagnosis could not be found out. This disease progresses rapidly and could cause death in few days time, unless treated appropriately. Conditions like Wilson’s disease, autoimmune liver disorder often fails to respond to medical management and require liver transplantation. Metabolic liver disease usually gets better with dietetic restriction. It is essential to find the underlying cause of liver failure to plan the type of liver transplantation. Auxiliary liver transplant, where a portion of donor liver is placed alongside of the native liver, could be done in liver failure due to hepatitis A, Hepatitis E etc. Once the acute insult is over the native liver regenerates and immunosuppression could be stopped, so that transplanted liver atrophies and the patient can live with their own liver.
Alagille syndrome
Alagille syndrome is a multisystem, genetic disorder. It is classically described as the constellation of characteristic facies (triangular face, broad forehead, pointed chin), cholestatic liver disease, cardiovascular anomalies (most commonly peripheral pulmonic stenosis), ocular abnormalities (posterior embryotoxon) and vertebral anomalies (commonly butterfly vertebrae). The liver disease could vary from mild symptomatic itching to severe jaundice and liver failure. Mild liver disease could be treated medically but severe liver disease could warrant liver transplantation.
Caroli’s disease
This is a condition where there are multiple cysts in the liver which could get infected and cause sepsis. Some newborn infants with severe autosomal-recessive polycystic kidney disease have extensive cystic bile duct changes, but renal insufficiency dominates the clinical picture. Ultrasound of the liver is often adequate for diagnosing Caroli disease; cholangiography is confirmatory. The outcome is related to the severity of renal disease, and/or the development of liver infection and fibrosis with portal hypertension.
Progressive familial intrahepatic cholestasis in children (PFIC)
Progressive familial intrahepatic cholestasis is a group of inherited disorders, which is common in India. Infants presenting with mild to severe jaundice, low GGT, elevated serum bile acids, failure to thrive and severe itching. There are three types of this disease and it could affect intestines and cause chronic diarrhoea. With mild itching these condition could be managed with medication, but in severe form of disease, “ biliary diversion” surgery could be done. In this surgery the bile is diverted either externally or internally, so that total bile acid pool is depleted, resulting in decrease of jaundice and itching. In severe cases with cirrhosis and liver failure, liver transplantation is the only option. This condition increases the risk of liver cancer in children and so these children require constant surveillance.
Non-alcoholic Fatty liver disease (NAFLD) in children
With fast growing economy, our country faces a new type of malnutrition in children, obesity. Fat deposition in liver and progression to liver cirrhosis is common in people who consume large amount of alcohol. Over end of last two decades fatty liver is being increasingly seen in children on routine ultrasound, particularly in obese children. People are aware of obesity and its complication in adults but when it comes to children, they don’t take it as a health problem. Similar to adults, children could also develop complication either during childhood itself or in their teen age due to obesity. With lot of eat outs serving fried foods and pizzas stuffed with calories and fats, prevalence of fatty liver disease is increasing in India. At present, the general prevalence of paediatric NAFLD is estimated at around 3-10 % in normal weight children, whereas this increases up to 50-70 % in obese children. The management consists of life style modification, increase in physical activities and appropriate dietetic management. The disease could gradually damage the liver and progress to liver cirrhosis requiring liver transplantation.