Pediatric liver and biliary diseases span a spectrum from neonatal cholestasis (e.g., biliary atresia) to inherited metabolic hepatopathies like Wilson disease and alpha-1 antitrypsin deficiency. Early recognition and individualized management are essential to support growth, prevent chronic liver injury, and improve long-term outcomes.
The liver and biliary system in children is more than “mini-adult” anatomy—it’s a dynamic organ network undergoing growth, maturation, and unique vulnerabilities. Early recognition and tailored management of liver and biliary pathology can dramatically influence growth, development, and long-term quality of life.
Pediatric liver disease encompasses a diverse array of conditions that often present differently than their adult counterparts, reflecting unique developmental and genetic influences.
In neonates, cholestatic disorders such as biliary atresia—the leading cause of infantile jaundice—demand prompt surgical referral for Kasai portoenterostomy, while genetic syndromes like Alagille disease bring with them characteristic cardiac and skeletal anomalies.
Progressive familial intrahepatic cholestasis (PFIC) subtypes illustrate how single-gene defects in bile transporters can drive early fibrosis and warrant consideration for liver transplantation.
As children grow, inherited metabolic hepatopathies emerge: glycogen storage diseases trigger hypoglycemia and hepatomegaly, Wilson disease often presents in school-aged patients with neuropsychiatric symptoms alongside hepatic copper overload, and alpha-1 antitrypsin deficiency may silently damage the liver for years.
Autoimmune hepatitis, though rarer, demands vigilance for fluctuating transaminases and can require lifelong immunosuppression.
Acute triggers such as viral infections—hepatitis A in unvaccinated populations or reactivation of hepatitis B and C—pose episodic risks, while vascular insults like Budd–Chiari syndrome and portal vein thrombosis underscore the importance of Doppler imaging in unexplained hepatomegaly.
Beyond diagnosis, pediatric hepatology revolves around tailored nutritional support, vigilant monitoring of synthetic function, and multidisciplinary collaboration to safeguard growth, optimize quality of life, and prevent progression to chronic liver failure.
Biliary atresia is a rare but devastating neonatal liver disease characterized by progressive obstruction and fibrosis of the extrahepatic bile ducts, leading to impaired bile flow, cholestasis, and eventual cirrhosis if left untreated. It typically presents in otherwise healthy infants at two to eight weeks of age with persistent jaundice, acholic (pale) stools, dark urine, and hepatomegaly. Although its precise cause remains unclear, hypotheses include viral injury, immune dysregulation, and genetic susceptibility, all converging on inflammatory destruction of bile ducts. Early diagnosis hinges on demonstrating direct hyperbilirubinemia, supportive imaging—ultrasound may reveal an absent or diminutive gallbladder, while a hepatobiliary scintigraphy (HIDA) scan shows lack of tracer excretion into the intestine—and ultimately intraoperative cholangiography. The cornerstone of therapy is the Kasai portoenterostomy, ideally performed before 60 days of life, which restores bile drainage by connecting the small intestine directly to the liver at the porta hepatis. Despite this intervention, many children develop progressive liver fibrosis, recurrent cholangitis, portal hypertension, and fat-soluble vitamin deficiencies, necessitating vigilant nutritional support and surveillance. Approximately half of patients will require liver transplantation by adolescence to achieve long-term survival. Prompt recognition, early surgical referral, and multidisciplinary management are therefore critical to improve outcomes in biliary atresia.
Biliary atresia is a progressive fibroinflammatory disorder of unknown exact etiology, though prevailing theories invoke a combination of perinatal viral injury (for example, reovirus or rotavirus), aberrant immune responses targeting bile duct epithelium, and underlying genetic susceptibility that disrupts normal ductal plate remodeling. Infants typically manifest signs between two and eight weeks of age, most notably persistent direct‐hyperbilirubinemia that produces conjugated jaundice, acholic (pale) stools, and dark urine; hepatomegaly from accumulating bile pigments often coexists with variable pruritus and failure to thrive. Physical examination may reveal an enlarged, firm liver edge and, occasionally, splenomegaly secondary to evolving portal hypertension. Laboratory evaluation demonstrates a cholestatic pattern with elevated gamma‐glutamyl transferase and alkaline phosphatase levels alongside prolonged prothrombin time, reflecting impaired synthetic function. Diagnostic imaging begins with abdominal ultrasound to assess gallbladder morphology—absence or a small “fibrotic” gallbladder and the triangular cord sign raise suspicion—followed by hepatobiliary scintigraphy (HIDA) that typically shows lack of tracer excretion into the intestine. Magnetic resonance cholangiopancreatography can further delineate ductal anatomy, but definitive diagnosis and therapeutic planning hinge on intraoperative cholangiography during a laparotomy, which confirms extrahepatic biliary obstruction and guides timely Kasai portoenterostomy to optimize bile flow and delay cirrhosis.
Nonalcoholic fatty liver disease (NAFLD) in children represents a spectrum of liver injury driven by excessive fat accumulation in hepatocytes, independent of significant alcohol intake. Today it is the most common chronic liver disorder in pediatrics, affecting an estimated 10–20 percent of adolescents and rising in parallel with childhood obesity and metabolic syndrome. Insulin resistance appears central to its pathogenesis: excess caloric intake, particularly of fructose and saturated fats, promotes adipocyte dysfunction and lipolysis, flooding the liver with free fatty acids that overwhelm mitochondrial oxidation and lead to triglyceride deposition. While simple steatosis may remain benign, some children progress to nonalcoholic steatohepatitis (NASH), characterized by hepatocellular ballooning, inflammation, and varying degrees of fibrosis, which can culminate in cirrhosis. Clinically, pediatric NAFLD is often silent; many patients are identified through mild elevations in alanine aminotransferase discovered on routine screening or incidentally noted hepatomegaly on examination. Ultrasonography offers a noninvasive window into steatosis, though magnetic resonance imaging and elastography provide greater sensitivity for fatty infiltration and fibrosis staging. Liver biopsy remains the gold standard when diagnostic uncertainty or advanced disease is suspected. Management hinges on lifestyle intervention—structured diet modifications to reduce sugar and saturated fat alongside regular aerobic exercise—to achieve gradual weight loss, improve insulin sensitivity, and halt progression toward fibrosis and its long-term sequelae.
Pediatric hepatic, biliary, and pancreatic disorders manifest across a broad spectrum of congenital, metabolic, inflammatory, and acquired etiologies that uniquely impact growth and development. In the liver, neonatal cholestatic disorders such as biliary atresia, Alagille syndrome, and progressive familial intrahepatic cholestasis result from ductal plate malformation or transporter defects, whereas metabolic hepatopathies—including Wilson disease, alpha-1 antitrypsin deficiency, and glycogen storage diseases—unleash toxic substrate buildup and fibrosis. Infectious processes range from acute viral hepatitis to sepsis-associated cholestasis; autoimmune hepatitis demands early immunosuppression, and vascular insults like Budd–Chiari syndrome or portal vein thrombosis may present with hepatomegaly and portal hypertension. The biliary tree is affected by choledochal cysts, neonatal cholangitis, and gallstone disease, particularly in hemolytic disorders. Pediatric pancreatitis often follows trauma, viral infection, or genetic mutations in CFTR, PRSS1, or SPINK1, evolving from self-limited acute episodes to chronic inflammation with exocrine insufficiency and diabetes. Cystic fibrosis illustrates overlapping liver and pancreatic injury, as inspissated bile and mucus obstruct ductal systems. Diagnosis hinges on tailored laboratory panels, ultrasonography, MRCP, elastography, and occasional biopsy. Management requires multidisciplinary coordination—surgical interventions like Kasai portoenterostomy or choledochal cyst excision, enzyme supplementation, immunomodulation, and nutritional optimization—to preserve organ function and ensure normal childhood milestones.
Pediatric liver pathology—most often in the form of percutaneous or trans-jugular liver biopsy—is reserved for situations in which noninvasive testing has failed to yield a definitive diagnosis or when histologic grading and staging will directly impact treatment decisions. Typical indications include chronic elevation of aminotransferases or cholestatic enzymes persisting beyond three to six months without an identified cause; prolonged conjugated hyperbilirubinemia in the neonate or infant; assessment of fibrosis and inflammatory activity in suspected autoimmune hepatitis, nonalcoholic steatohepatitis, or metabolic hepatopathies such as Wilson disease and alpha-1 antitrypsin deficiency; and differentiation of overlapping conditions—such as distinguishing between acute viral hepatitis and drug-induced liver injury. Biopsy also plays a key role when imaging uncovers focal hepatic lesions, vascular anomalies, or unexplained biliary tract abnormalities, and in pre-transplant evaluation to establish baseline histology. It can guide therapy by confirming cirrhosis or by quantifying steatosis, iron overload, or storage material in disorders like hemochromatosis or glycogen storage disease. Prior to biopsy, thorough history, laboratory panels, and imaging studies (ultrasound, elastography, MRI) should be completed to minimize risk. Ultimately, pediatric liver pathology is performed only when the benefits of obtaining tissue—clarifying etiology, refining prognosis, and tailoring management—outweigh procedural risks.
Treatment of pediatric liver diseases requires tailored interventions across medical, surgical, nutritional, and transplant strategies. Biliary atresia demands early Kasai portoenterostomy before 60 days to restore bile flow and delay cirrhosis; unsuccessful cases progress to transplant. PFIC and Alagille syndrome benefit from ursodeoxycholic acid and external biliary drainage, with transplant for refractory cholestasis. Wilson disease is managed with chelators like penicillamine or trientine plus zinc to reduce copper accumulation, while alpha-1 antitrypsin deficiency focuses on supportive care and transplantation if synthetic failure ensues. Glycogen storage diseases require frequent cornstarch feeds or gastric infusions to maintain euglycemia and a diet tailored to limit glycogen buildup. NAFLD in children relies on lifestyle modification—dietary sugar restriction and exercise—to improve insulin sensitivity. Autoimmune hepatitis mandates induction with steroids and azathioprine, then maintenance immunosuppression. Acute viral hepatitis often resolves with supportive care, although hepatitis B or C may need antivirals. Vascular disorders such as Budd–Chiari and portal vein thrombosis are treated with anticoagulation, angioplasty, or shunting. Enzyme replacement and pancreatic supplementation address cystic fibrosis–related liver and pancreatic injury. Across all conditions, multidisciplinary coordination and growth monitoring optimize outcomes, with liver transplantation reserved for decompensated cirrhosis or treatment-refractory disease.
Treatment of biliary atresia revolves around timely surgical, medical, and nutritional interventions to optimize bile flow, support growth, and minimize complications. The primary procedure is the Kasai portoenterostomy—ideally performed before 60 days of age—which entails excision of fibrotic extrahepatic bile ducts and anastomosis of a Roux-en-Y jejunal loop to the porta hepatis. Early Kasai success correlates with clearance of jaundice, improved cholestasis markers, and delayed cirrhosis, although up to half of patients still progress to end‐stage liver disease. Postoperatively, adjuvant therapies often include ursodeoxycholic acid to enhance bile secretion, short courses of corticosteroids to modulate inflammation, and prophylactic antibiotics (for example, trimethoprim-sulfamethoxazole) to prevent recurrent cholangitis. Nutritional strategies emphasize high-calorie, medium-chain triglyceride–enriched formulas alongside fat-soluble vitamin supplementation and close monitoring of growth parameters. Longitudinal follow-up with serial liver function tests, abdominal ultrasound for portal hypertension, and endoscopic screening for varices guides ongoing care. Children with Kasai failure, decompensated cirrhosis, or refractory cholangitis require prompt referral for living or deceased donor liver transplantation, which offers definitive treatment and excellent long-term survival. A coordinated, multidisciplinary team—pediatric hepatology, surgery, nutrition, and transplant specialists—is essential to tailor care pathways. Emerging research into bile duct regeneration, immunomodulatory agents, and potential gene therapies promises to refine future management guidelines.
Post-treatment care for a child with biliary disease requires a multidisciplinary approach that addresses nutrition, medication adherence, monitoring, and psychosocial support. Following Kasai portoenterostomy or other surgical interventions, it’s essential to ensure high-calorie intake with medium-chain triglyceride–enriched formulas to support growth and prevent fat-soluble vitamin deficiencies; supplement vitamins A, D, E, and K as directed by a pediatric hepatologist. Caregivers should administer ursodeoxycholic acid and prophylactic antibiotics using pill organizers or dosing apps to maintain consistency. Regular follow-up appointments including liver function tests, abdominal ultrasounds, and endoscopic surveillance allow early detection of cholangitis, portal hypertension, or progressive fibrosis. Parents need education on recognizing signs of infection—fever, acholic stools, irritability—and when to seek urgent care. Maintaining an immunization schedule, with particular attention to pneumococcal and rotavirus vaccines, reduces infection risks. Avoidance of hepatotoxic medications and exposure to environmental toxins prevents liver injury. Psychosocial support, through play therapy, support groups, or mental health referrals, helps families adapt to disease management. Coordination between primary care, nutritionists, surgeons, and transplant teams ensures cohesive care. By emphasizing consistent monitoring, nutritional optimization, medication adherence, and family education, caregivers can enhance their child’s growth, minimize complications, and improve long-term outcomes following biliary disease treatments.
Equipped hospitals, and advanced specialized centers with experienced doctors and specialists are available in all medical treatment areas in Iran. Also, good hotels and entertainment centers have made Iran an appropriate choice for patients who need Pediatric Liver & Biliary Disease.
In Iran, the cost of pediatric liver and biliary interventions is considerably lower than in Western countries, yet varies by procedure and facility. Kasai portoenterostomy for biliary atresia typically runs between $3,000 and $5,000, covering surgeon fees, anesthesia, and a 10- to 14-day hospital stay. Laparoscopic cholecystectomy—often indicated for choledochal cysts or gallstone disease—costs roughly $1,600–$3,000. Endoscopic retrograde cholangiopancreatography (ERCP) for biliary decompression or stent placement in infants with cholangitis is priced at about $450–$1,100.
Look for persistent jaundice (yellowing of skin or eyes) beyond two weeks in newborns, pale or clay-colored stools, dark urine, poor weight gain, abdominal swelling, or unexplained itching. Early recognition and referral to a pediatric hepatologist can make a critical difference in outcomes.
Diagnosis combines detailed history and exam with targeted lab tests (liver enzymes, bilirubin fractions, autoantibodies, metabolic panels) plus imaging. Ultrasound with Doppler is first-line, often followed by HIDA scan in cholestasis or MRCP for ductal anatomy.
Therapies range from medical (ursodeoxycholic acid, immunosuppressants, nutritional support, enzyme replacement) to interventional/surgical (Kasai portoenterostomy, ERCP, cyst excision) and, when indicated, liver transplantation.
Consistently ranked as one of the Top Hospitals in Iran
Bahman Super Speciality Hospital has 200 official beds
Ranked the biggest and most equipped private treatment center in Iran
The first private hospital in Iran to get the Green Award International Certification
The first private PET scan center equipped with PET-CT, PET-MAMMO, Cyclotron, and Conventional Nuclear Medicine
The first private super specialty ophthalmology hospital in Iran.
Considering the increasing expansion of Mashhad city and medical needs in 1973.
One of the best-equipped diagnostic and therapeutic cardiology centers in the region.