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Article in English | WPRIM | ID: wpr-28079


Intestinal hypoganglionosis is a rare innervation disorder that provides numerous nutritional, medical and surgical challenges. In this case report, we present a case of a newborn with intestinal hypoganglionosis leading to intestinal failure and intestinal failure-associated liver disease who responded to Omegaven™, a fat emulsion comprised of omega-3 fatty acids. Omegaven™ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with Omegaven™ was seen in this patient with a clear decrease in aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and complete resolution of cholestasis with a direct bilirubin of zero within two weeks of initiation of Omegaven™. No current guidelines for the diagnosis and management of hypoganglionosis are available. We recommend a multidisciplinary approach and the use of novel therapies such as fat emulsions composed of omega-3 fatty acids for improved patient outcomes. Appropriate compassionate use protocols should be obtained from the Food and Drug Administration prior to initiation of Omegaven™.

Alanine Transaminase , Alkaline Phosphatase , Aspartate Aminotransferases , Bilirubin , Cholestasis , Compassionate Use Trials , Diagnosis , Empathy , Emulsions , Fatty Acids, Omega-3 , Hirschsprung Disease , Humans , Infant, Newborn , Liver , Liver Diseases , Parenteral Nutrition, Total , United States Food and Drug Administration
Article in English | WPRIM | ID: wpr-61676


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.

Alagille Syndrome , Bile , Bile Canaliculi , Cholelithiasis , Cholestasis , Diagnosis , Diagnosis, Differential , Hepatitis , Hepatolenticular Degeneration , Humans , Jaundice , Pruritus