Khalaf Racha, Phen Claudia, Karjoo Sara, Wilsey Michael
Department of Medical Education, Johns Hopkins All Children's Hospital, St. Petersburg, FL, United States.
Department of Gastroenterology and Nutrition, Johns Hopkins All Children's Hospital, St. Petersburg, FL, United States.
Pediatr Gastroenterol Hepatol Nutr. 2016 Mar;19(1):1-11. doi: 10.5223/pghn.2016.19.1.1. Epub 2016 Mar 22.
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.
胆汁淤积是由于胆汁排泄受损所致，这可能是由于胆汁流动的机械性梗阻或胆汁成分向胆小管排泄受损引起的。一旦出现胆汁淤积，就需要及时诊断和治疗。新生儿期以后胆汁淤积的鉴别诊断范围广泛，包括先天性和后天性病因。临床医生必须区分胆汁淤积的肝内和肝外起源。治疗可能是支持性的或治愈性的，这取决于病因。最近的文献表明，最佳的营养和医疗支持在慢性胆汁淤积患儿的管理中也起着不可或缺的作用。本综述将对新生儿期和婴儿期以后胆汁淤积的病理生理学、诊断方法和管理进行广泛概述。
