Neonatal cholestasis is defined as prolonged conjugated hyperbilirubinemia that occurs in the newborn period. It results from a diminished bile flow and/or excretion of conjugated bilirubin from the hepatocyte into the duodenum. It is manifested by a conjugated bilirubin level over 2.0 ml/dl. The incidence of neonatal cholestasis is approximately 1:2500 live births. Cholestasis must always be considered in newborns with prolonged jaundice lasting more than 14-21 days.
In this case you need to contact a doctor urgently, because in 1 in 3 such cases have biliary atresia, a condition where in the bile duct which drains bile from the liver is not formed and its important / rather emergency and offer them surgeryCauses of cholestatic jaundice
Neonatal Hepatitis – abnormality arising in the liver from a variety of causes ranging from infection, genetic / metabolic conditions, parenteral nutrition, endocrinal disorders etc.
Paucity of bile ducts – There is deficiency in the no of intrahepatic ducts. This could be non syndrome or syndromic such as Alagille’s syndrome where besides jaundice there is involvement of skeletal system, heart, eyes, typical facies etc
Biliary atresia - here the bile duct is fibrotic congenitally
Biliary atresia needs to be diagnosed as early as possible as it requires Urgent Surgery called Kasai’s Portoenterostomy. The results of the surgery are better if its done before 2 months of age. The ultimate outcome post surgery depends on the status of liver at the time of surgery – (fibrosis / cirrhosis / not). If the bilirubin continues to be high post surgery then these babies eventually require liver transplant – the timing of which depends on the rapidity of progress of the liver disease in an individual. It could vary from as early as few months to several years post surgery.
Also its important to identify treatable causes of cholestasis such as infection, hypothyroidism, galactosemia etc. Its always advisable to give your baby 1 mg/kg of vitamin K in cases of cholestatic jaundice to prevent bleeding / bruising. Doctor may prescribe ursodeoxycholic, a naturally occurring dihydroxy bile salt that comprises 1% of the total bile pool, desaturates bile, stimulates bile flow, and thereby facilitates canaclicular secretion of the accumulated atypical bile acids. It can reduce direct hyperbilirubinemia significantly.
Viral Hepatitis A
The Hepatitis A is the commonest cause of infective jaundice in children in India.How do you get hepatitis A infection ?
It’s a food born infection, that is results due to contamination food and water.What’s the symptoms ?
It could be asymptomatic or have mild symptoms or end up with liver failure. Usually 1 in 1000 children and 1 in 100 develop liver failure. Patients with chronic liver disease also have a more serious outcome. Once liver failure develops it very critical. Even at the best of centre which are experts in the management of liver failure, one can salvage only 50% -60% of the cases. Rest cannont be saved without a liver transplant.Prevention ? Symptoms can be vague and mimic the flu. Most people with hepatitis A usually have : Several days later, symptoms of liver problems will occur. Children with hepatitis A may also have the following symptoms : What are the symptoms of liver failure ? People over age 50 and those with chronic liver disease may have a more severe of hepatitis A called fulminant hepatitis A infection. Symptoms can include : What is the outcome of liver transplant in acute liver failure ?
In western world the results are poorer to liver transplant in non fulminant probably due to the waiting time for cadaveric graft. In our experience in living related liver transplant outcome is excellent with 95% survival.
Hepatitis B & Hepatitis C
Hepatitis B and Hepatitis C are viruses that infect the liver and gradually damage it leading to cirrhosis of liver and / or hepatocellular carcinoma.
Once the marker is positive that is HbsAg positive or HCV Ab positive then the doctor will carry out further tests to identify the infectivety of the virus and determine when and how to treat it. Test that are usually carried out are blood tests , ultrasound and liver biopsy.
Treatment is usually with interferon injections (Peg interferon preferably), oral drugs (Lamuvidine, Adefovir, Ribavirin) are usually combined with these injections.
There is risk of progression to cirrhosis of liver or hepatocellular carcinoma with chronic hepatitis B and chronic hepatitis C infections.
Metabolic Liver Diseases
We all have genes inherited from our parents which decide whether we are tall, short, fair, dark, etc. Some genes we inherit are “recessive”, that is, we carry the gene but it does not show up in us anywhere. When we have children, if our partner carries exactly the same recessive gene, there is a one in four chance in every child we have that these recessive genes will match up and become “dominant” showing in the child. Often the matching of recessive genes leads to nothing more extraordinary than, perhaps, a red-haired child of dark haired parents. However, if the recessive genes are carriers of a metabolic disorder, the child will have a metabolic disease although his/her parents do not.
Other metabolic diseases usually affect only boys and are carried by the mother. These are known as X linked, being carried on the sex chromosome. If the child is male there is a 1 in 2 chance that he will be affected. Climb strongly recommends that expert genetic advice is given when a diagnosis is made
Common metabolic liver disease : Galactosemia, Tyrosinemia, Leukodystrophies, Glycogen Storage Diseases, Wilson’s disease, Hyperammonemia, organic acidaemias and other like Gaucher’s, Leigh’s, Tay Sachs, Cystinosis, Batten’s, Schilders, Sandhoff’s, Alpha One Antitrypsin Deficiency, and the optic acidaemias.
Treatment : Much progress has been made, to treat metabolic diseases with diet or drugs to restore the chemical balance or through organ transplantation. Bone marrow or liver transplantation in some cases means that the donor marrow or liver can introduce the enzyme missing in the recipient but this form of therapy is available for a very limited number of disorders and is not without risk. Direct infusions of the missing enzyme is another method of treatment that has been used successfully in one or two disorders such as Gaucher’s disease, Nieman Pick’s.
With improvement in economic status, food availability and increased western dietary habits, obesity is being increasingly recognized in Indian children.Liver is involved in 10-20% of children with obesity and severity of involvement increases with degree of obesity. Liver involvement in obese children includes :
Cirrhosis of Liver
Cirrhosis means there is extensive scarring of the liver tissue. Scarred areas are not working. Cirrhosis of liver results from a variety of conditions. Common causes include cholestatic liver diseases such as biliary atresia, metabolic liver diseases such as Wilson’s, tyrosinemia, glycogen storage disorders, other etiologies such as chronic hepatitis B/C etc.In this condition you need to be consulting a Pediatric hepatologist that is a liver specialist, to manage various complications associated with cirrhosis. Most of these children would eventually require liver transplantation though some of the metabolic disorders like Wilson’s may respond well to medical treatment provided an early diagnosis is made.