Researchers addressed scenarios that covered diagnostic and therapeutic emergencies in children with liver diseases such as Alagille syndrome (ALGS), as published in the Journal of Hepatology. These could help the early diagnosis and timely management of pediatric liver conditions.

Infants may be affected by a wide range of liver disease-causing conditions. In some instances, jaundice is the only initial symptom of such conditions. Some liver-based metabolic disorders may also lead to hyperammonaemia or hypoglycemia without any abnormality levels in liver enzymes.

As these diseases progress, however, they can cause complications that can be life-threatening. Simple interventions such as vitamin K administration could prevent neurological complications in infants with cholestasis, the authors said.

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Cholestasis could be caused by genetic conditions such as ALGS in which a genetic mutation disrupts the Notch signaling pathway and leads to bile duct paucity. This results in reduced or obstructed bile flow from the liver causing issues with fat digestion and the absorption of fat-soluble vitamins like vitamin K, resulting in malnutrition.

Read more about ALGS etiology

Infants with pediatric acute liver failure should be stabilized and investigated in conjunction with a liver transplant center, the authors said.

In case of variceal bleeding, which could be caused by portal vein thrombosis or chronic liver disease, octreotide can effectively control bleeding and stabilize the patient before endoscopy or transfer to a liver center.

Infants with liver trauma or a liver tumor should be referred to a specialist center and be managed by a multidisciplinary team.

The researchers also said that several inborn errors of metabolism can present with liver dysfunction. “Their early identification and treatment in collaboration with metabolic experts is essential to improve their outcomes,” they concluded.


Jagadisan B, Dhawan A. Emergencies in pediatric hepatology. J Hepatol. Published online January 3, 2021. doi:10.1016/j.jhep.2021.12.027