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Khalaf, Racha,Karjoo, Sara,Danielson, Paul,Wilsey, Michael,Shakeel, Fauzia The Korean Society of Pediatric Gastroenterology 2017 Pediatric gastroenterology, hepatology & nutrition Vol.20 No.1
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 $Omegaven^{TM}$, a fat emulsion comprised of omega-3 fatty acids. $Omegaven^{TM}$ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with $Omegaven^{TM}$ 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 $Omegaven^{TM}$. 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 $Omegaven^{TM}$.
Cholestasis beyond the Neonatal and Infancy Periods
Khalaf, Racha,Phen, Claudia,Karjoo, Sara,Wilsey, Michael The Korean Society of Pediatric Gastroenterology 2016 Pediatric gastroenterology, hepatology & nutrition Vol.19 No.1
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.
Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of OmegavenTM
Racha Khalaf,Sara Karjoo,Paul Danielson,Michael Wilsey,Fauzia Shakeel 대한소아소화기영양학회 2017 Pediatric gastroenterology, hepatology & nutrition Vol.20 No.1
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 OmegavenTM, a fat emulsion comprised of omega-3 fatty acids. OmegavenTM has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with OmegavenTM 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 OmegavenTM. No current guidelines for the diagnosis and management of hypo-ganglionosis 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 OmegavenTM.
Cholestasis beyond the Neonatal and Infancy Periods
Racha Khalaf,Claudia Phen,Sara Karjoo,Michael Wilsey 대한소아소화기영양학회 2016 Pediatric gastroenterology, hepatology & nutrition Vol.19 No.1
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 extra-hepatic 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.
Gastric Xanthoma in the Pediatric Population: A Possible Herald for Malignancy?
Joseph D. Russell,Jacquelin Peck,Claudia Phen,Janna L. Linehan,Sara Karjoo,Johnny Nguyen,Michael J. Wilsey 대한소아소화기영양학회 2020 Pediatric gastroenterology, hepatology & nutrition Vol.23 No.1
Gastric xanthoma is frequently an incidental finding on upper endoscopy in adults. Gastric xanthomas (GX) can be mistaken for malignancies and warrant prompt histologic diagnosis. The underlying etiology is not fully understood; however, it has been linked to Helicobacter pylori gastritis and gastric cancer. GX in the pediatric population is largely unreported in the literature. Because of the relative rarity, documentation with case reports are essential to provide as much data as possible to see if there is a correlation between GX and malignant potential in the pediatric population. Our group is reporting two cases, a 10-year-old male and a 7-year-old male, both who presented with chronic dysphagia, upper abdominal pain, nausea, vomiting, and loss of appetite. Upper endoscopies for both patients revealed small polypoid lesions located in the antrum with foamy histiocytes on histology, leading to the diagnosis of gastric xanthoma.
Gastric Xanthoma in the Pediatric Population: A Possible Herald for Malignancy?
Russell, Joseph D.,Peck, Jacquelin,Phen, Claudia,Linehan, Janna L.,Karjoo, Sara,Nguyen, Johnny,Wilsey, Michael J. The Korean Society of Pediatric Gastroenterology 2020 Pediatric gastroenterology, hepatology & nutrition Vol.23 No.1
Gastric xanthoma is frequently an incidental finding on upper endoscopy in adults. Gastric xanthomas (GX) can be mistaken for malignancies and warrant prompt histologic diagnosis. The underlying etiology is not fully understood; however, it has been linked to Helicobacter pylori gastritis and gastric cancer. GX in the pediatric population is largely unreported in the literature. Because of the relative rarity, documentation with case reports are essential to provide as much data as possible to see if there is a correlation between GX and malignant potential in the pediatric population. Our group is reporting two cases, a 10-year-old male and a 7-year-old male, both who presented with chronic dysphagia, upper abdominal pain, nausea, vomiting, and loss of appetite. Upper endoscopies for both patients revealed small polypoid lesions located in the antrum with foamy histiocytes on histology, leading to the diagnosis of gastric xanthoma.