Magnitude, clinical spectrum and etiology of hepatobiliary disorders in children- a tertiary care experience

Objectives: This study was undertaken to know about the magnitude, clinical spectrum and etiology of liver diseases in children in a tertiary care teaching hospital. Materials and Methods: This is a hospital based, retrospective, descriptive study. It was done on 45 children with hepatobiliary diseases admitted to pediatric department of Velammal Medical College, Madurai from Jan 2016 – Dec 2016. Results: As about 45 children out of 2259 pediatric admissions, had hepatobiliary disorders. This was contributing to 1.99% of total admissions. Liver function test was deranged in (73.3%) of children. Males (62.2%), outnumbered, females (37.7%) in our study. Children less than 5 years of age were most commonly affected (46.6%). Most common symptom in children with hepatobiliary disorders were jaundice (51.1%), nausea/vomiting (46.6%), anorexia (40%), pain abdomen (33.3%), high coloured urine (28.8%), bleeding manifestations and abdomen distension in (20%) each, fever in (17.7%), failure to thrive and irritability in (15.5%) each. The most common sign observed was icterus (51.1%), hepatomegaly (42.2%). Ascitis and splenomegaly was noted in (26.6%) each, edema in (20%) and pallor in (15.5%) of children. Acute liver diseases were more common (68.8%) than chronic liver diseases (31.1%). The most common etiology of acute liver disease was acute viral hepatitis (28.8%), cholelithiasis (22.2%), non-alcoholic fatty liver disease (6.6%), liver abscess (4.4%), acute viral hepatitis with acalculous cholecystitis in (4.4%), acute liver failure due to paracetamol poisoning in (2.2%) of children. Etiology noted for chronic liver diseases were biliary atresia (13.3%), idiopathic cirrhosis and wilsons disease in (6.6%) each, autoimmune hepatitis and chronic hepatitis in (2.2%) each. Conclusion: Since age is the single most important determinant in successful management of biliary atresia, recognition and definitive identification of the condition as the cause of neonatal cholestasis syndrome in a given case very early after the onset of symptoms is of paramount importance.


Introduction
Childhood liver disorders constitute a major proportion of hospital admissions in India. The outlook of hepatobiliary disorders has undergone tremendous change with advent of better diagnostic tools like ultrasound, radionuclear scan, viral and autoimmune markers, endoscopic retrograde cholangiography, digital substraction angiography and improved sectioning and staining techniques of liver tissue specimens [1]. Diseases of the liver contribute  [2]. Liver diseases among children include a broad spectrum of disorders such as infections, developmental abnormalities and metabolic disorders that finally result in hepatic dysfunction and cirrhosis. Acute and chronic liver diseases constitute the majority of liver disorders among children. Whereas hepatitis A and E are the common causes of acute liver disease in developing and under developed countries, metabolic causes predominate in developed countries. Certain biliary disorders such as biliary atresia present as chronic liver diseases in regions where diagnosis is delayed beyond twelve weeks. Such children often present with cirrhosis and portal hypertension [3]. Recent studies suggest that nonalcoholic fatty liver disease is an increasingly recognized condition during childhood, especially in overweight and obese children [4]. The hepatic injury in Wilsons disease is believed to be caused by excess copper, which acts as a pro oxidant and promotes the generation of free radicals [5]. The common causes for chronic liver disease in children are hepatitis B, hepatitis C, hepatitis D, autoimmune hepatitis and metabolic disorders like Wilsons disease and alpha 1 antitrypsin deficiency. In majority the etiology remains uncertain [6]. In infants, cirrhosis is most often caused by biliary atresia and genetic-metabolic disorders, while in older children, it tends to result from autoimmune hepatitis, Wilsons disease, alpha-1-antitrypsin deficiency and primary sclerosing cholangitis [7]. The need for accurate diagnosis of biliary atresia soon after clinical manifestations is important as response to surgical results will be best if the operation is performed around 8 weeks of age [8]. Diagnosis of Wilsons disease with hepatic presentation in childhood using clinical and laboratory parameters is still challenging and is often missed or delayed. Hepatic involvement is prominent during childhood and mimics the features of a variety of acute and chronic liver diseases [9,10].

Materials and Methods
Study design: Retrospective, descriptive study.
Setting: Hospital based study in a tertiary care centre in south India.
Inclusion criteria: * 3months to 15 years old children presenting to the pediatric department with liver disease * Complete patient information along with the investigation reports in the medical records.
Exclusion Criteria: *Children less than 3 months and more than 15 years of age *Medical records with incomplete information/ Evaluation done in other hospitals.
Participants: Children aged 3 months to 15 years of age admitted and treated in pediatric department from January 2016-December 2016 in a tertiary care centre, south India.

Data source:
The hospital records of the children with hepatobiliary diseases were retrieved from the medical records department following due permission.

Study size: 45 children Quantitative variable: magnitude Statistical analysis: Simple proportion test
The following data was collected from the medical records department (MRD) about the children included in this study. *Age, sex, symptoms and signs of hepatobiliary diseases. *Results of liver function tests, coagulogram, viral serology markers, autoimmune antibodies, serum ceruloplasmin, urinary copper levels, abdominal ultrasound, upper GI endoscopy findings, liver biopsy, and HIDA scan report was noted.

Results
Out of 2259 children, admitted in pediatric ward from Jan 2016-Dec 2016, about 45 children had hepatobiliary disorders, contributing to 1.99% of the total number of admissions. Liver function tests were deranged in 33 out of 45 children (73.3%). The analysis of 45 children who fulfilled the inclusion criteria is as below. According to table 1-it was seen that males 28(62.2%), outnumbered female children. Hepatobiliary disorders was most commonly seen in children less than 5 years of age 21 (46%), followed by, children of 11-15 years of age 15 (33.3%) and 6-10 years of age 9 (20%).