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Published on: August 23, 2022
Cholestasis beyond the Neonatal and Infancy Periods
Racha Khalaf1, Claudia Phen1, Sara Karjoo2
1Department of Medical Education, Johns Hopkins All Children's Hospital, St. Petersburg, FL, United States.
Insights
Cholestasis, a bile excretion disorder, requires prompt diagnosis and treatment. This review covers the pathophysiology, diagnosis, and management of pediatric cholestasis beyond infancy.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
Background:
- Cholestasis stems from impaired bile excretion, either via mechanical obstruction or defective canalicular excretion.
- Prompt diagnosis and treatment are crucial for cholestasis management.
- The differential diagnosis is broad, encompassing congenital and acquired causes, necessitating differentiation between intrahepatic and extrahepatic origins.
Purpose of the Study:
- To provide a comprehensive overview of cholestasis in pediatric patients beyond the neonatal and infancy periods.
- To discuss the pathophysiology, diagnostic strategies, and management approaches for cholestasis.
- To highlight the importance of nutritional and medical support in chronic cholestasis.
Main Methods:
- Literature review focusing on cholestasis in pediatric populations beyond infancy.
- Synthesis of information on cholestasis pathophysiology, diagnosis, and treatment.
- Emphasis on differentiating intrahepatic from extrahepatic causes.
Main Results:
- Cholestasis management is etiology-dependent, ranging from supportive care to curative interventions.
- Optimal nutritional and medical support are integral to managing chronic pediatric cholestasis.
- Accurate differentiation between intrahepatic and extrahepatic cholestasis is clinically imperative.
Conclusions:
- Effective management of pediatric cholestasis requires a thorough understanding of its diverse etiologies and origins.
- A multidisciplinary approach integrating diagnosis, treatment, and supportive care is essential.
- This review serves as a guide for clinicians managing cholestasis in older children.
Abstract:
Cholestasis results from impairment in the excretion of bile, which may be due to mechanical obstruction of bile flow or impairment of excretion of bile components into the bile canaliculus. When present, cholestasis warrants prompt diagnosis and treatment. The differential diagnosis of cholestasis beyond the neonatal period is broad and includes congenital and acquired etiologies. It is imperative that the clinician differentiates between intrahepatic and extrahepatic origin of cholestasis. Treatment may be supportive or curative and depends on the etiology. Recent literature shows that optimal nutritional and medical support also plays an integral role in the management of pediatric patients with chronic cholestasis. This review will provide a broad overview of the pathophysiology, diagnostic approach, and management of cholestasis beyond the neonatal and infancy periods.
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