“Breaking Down Autoimmune Hepatitis: A Rare Liver Disorder in Focus”

Ghaniya KC ,
Dr.Bhanu Vikraman pillai (Aims Paed Gastro)
Dr C Jayakumar 

Five years old female presented with complaints of yellow discolouration of sclera and urine since four months and high grade in intermittent fever since four days.
History of outside food intake is present
No history of similar complaints in family members.
History of consumption of complimentary and alternative system medicine present on and off since four months.
No history of cough, vomiting, abdominal pain , bleeding, altered sensorium.
No history of blood transfusions

First child of a non consanguinous marriage 
No significant antenatal , natal and post natal history.

On examination: 
Child is sick looking and icteric
No pallor, cyanosis , lymphadenopathy , edema
Auxology – normal
Head to foot – no bleeding spots

Systemic Examination : 
P/A – soft , Hepatosplenomegaly + , Bowel sounds +
Liver – Firm , rounded margins , smooth surface 
Span – 13 cm 
RS -Normal
CVS – No cardiac enlargement or muffling

Investigations : 
CBC – TC – 12K , N/L – 33/61 % , Hb- 8.4 , Plt – 233 , CRP – 15

LFT – Total bill 9.83 mg%direct DB – 7.36 , AST – 5651, ALT – 1328 , ALP – 164 , PT/INR – 2.13

Pre-op serology : Negative
Dengue panel : negative 
IgM HAV : 3.24 positive 
IgM HEV : negative 
IgM HSV 1 and 2 : negative 
DCT – 3 + 
C3, C4 – normal 
Serum Ceruloplasmin : 20.9
Urine 24 hrs copper : 46.4
Ammonia – 49.6

CT Brain – No acute intraparenchymal /extra axial bleed
CT Abdomen – Periportal and gall bladder wall edema.Trace ascites+ 

Autoimmune hepatitis panel – Positive for AMA-M2 , LKM-1, M23E , PML , GP210

Liver biopsy – shows feautures suggestive of an acute hepatitis with portal and lobular changes and periportal loss. 

Course : Child is being treated as a case of auto immune hepatitis with oral steroids and is currently improving.

Autoimmune hepatitis (AIH) in children is a rare but serious condition in which the immune system mistakenly attacks the liver, leading to inflammation and potential liver damage. While AIH can affect individuals of any age, it is often diagnosed in children and young adults. Here’s an overview of autoimmune hepatitis in children:

Types of Autoimmune Hepatitis in Children

There are two main types of autoimmune hepatitis, classified based on the pattern of antibodies present1
Type 1 (Classic): This is the most common form in children. It is associated with antibodies like antinuclear antibody (ANA) and smooth muscle antibody (SMA).
Type 2: This type is more often seen in younger children, typically under the age of 2. It is associated with liver kidney microsomal antibody (LKM1).

Symptoms

The symptoms of autoimmune hepatitis in children can vary widely, ranging from mild to severe, and may include:
• Fatigue
• Jaundice (yellowing of the skin or eyes)• Abdominal pain, particularly in the upper right side
• Loss of appetite
• Dark urine
• Pale-colored stool
• Joint pain
• Fever (in some cases)
In severe cases, children may experience liver failure, which can be life-threatening if not treated promptly.

Causes and Risk Factors

The exact cause of autoimmune hepatitis is unknown, but it is thought to involve a combination of genetic and environmental factors. Some potential risk factors include:• Genetic predisposition: Certain gene variants, such as those related to the human leukocyte antigen (HLA), may increase susceptibility.• Environmental triggers: Infections, medications, or viruses (such as Epstein-Barr virus) may trigger the onset of AIH in genetically predisposed individuals.

Diagnosis

The diagnosis of autoimmune hepatitis in children is based on:• Blood tests: Detecting liver enzymes (such as ALT and AST) elevated in liver inflammation, as well as specific antibodies (ANA, SMA, or LKM1).
• Liver biopsy: To assess the degree of liver damage and confirm the diagnosis.
• Imaging studies: To check for signs of liver damage or cirrhosis.

Treatment

The goal of treatment is to reduce inflammation and prevent further liver damage. It typically involves:1. Corticosteroids (e.g., prednisone): The first-line treatment to suppress the immune system and reduce liver inflammation.
2. Immunosuppressive drugs (e.g., azathioprine): Often used in combination with steroids to maintain remission and reduce side effects.
3. Liver transplantation: In severe cases where there is advanced liver damage or failure, a liver transplant may be necessary