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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.
Racha Khalaf,Carmen Cuffari 대한소아소화기영양학회 2018 Pediatric gastroenterology, hepatology & nutrition Vol.21 No.1
Androgen therapy has proven efficacy in treating patients with bone marrow failure who are not candidates for bone marrow transplantation. Herein, we report on a case of colonic angioectasia secondary to oxymetholone use in an adolescent patient with Hoyeraal-Hreidarsson syndrome (HHS). A 13-year-old Caucasian male with HHS charac-terized by cerebellar hypoplasia, developmental delay, microcephaly, esophageal strictures and myelodysplasia presented with severe hematochezia from colonic angioectasia secondary to long-term oxymetholone therapy. These vascular lesions resolved spontaneously once this anabolic steroid was discontinued. While androgen therapy is often recommended for certain anemias and myelodysplastic syndromes, clinicians should be aware of the poten-tial complication in developing these perceived uncommon colonic angioectasias. Moreover, pediatric gastro-enterologists should familiarize themselves in identifying these vascular lesions by colonoscopy, especially among the high risk groups on long-term anabolic steroid therapy.
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.
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.
Khalaf, Racha,Cuffari, Carmen The Korean Society of Pediatric Gastroenterology 2018 Pediatric gastroenterology, hepatology & nutrition Vol.21 No.1
Androgen therapy has proven efficacy in treating patients with bone marrow failure who are not candidates for bone marrow transplantation. Herein, we report on a case of colonic angioectasia secondary to oxymetholone use in an adolescent patient with Hoyeraal-Hreidarsson syndrome (HHS). A 13-year-old Caucasian male with HHS characterized by cerebellar hypoplasia, developmental delay, microcephaly, esophageal strictures and myelodysplasia presented with severe hematochezia from colonic angioectasia secondary to long-term oxymetholone therapy. These vascular lesions resolved spontaneously once this anabolic steroid was discontinued. While androgen therapy is often recommended for certain anemias and myelodysplastic syndromes, clinicians should be aware of the potential complication in developing these perceived uncommon colonic angioectasias. Moreover, pediatric gastroenterologists should familiarize themselves in identifying these vascular lesions by colonoscopy, especially among the high risk groups on long-term anabolic steroid therapy.
Endoscopic Balloon Dilation for Treatment of Congenital AntralWeb
Jacquelin Peck,Racha Khalaf,Ryan Marth,Claudia Phen,Roberto Sosa,Francisco Balsells Cordero,Michael Wilsey 대한소아소화기영양학회 2018 Pediatric gastroenterology, hepatology & nutrition Vol.21 No.4
Congenital antral webs are a rare but relevant cause of gastric outlet obstruction in infants and children. The condition may lead to feeding refusal, vomiting, and poor growth. Due to the relative rarity of the disease, cases of congenital antral web are frequently misdiagnosed or diagnosed with significant delay as physicians favorably pursue diagnoses of pyloric stenosis and gastric ulcer disease, which are more prevalent. We report a case of an eight-month-old female who presented with persistent non-bilious emesis, feeding difficulties, and failure to thrive and was discovered to have an antral web. The web was successfully treated with endoscopic balloon dilation, which resolved her symptoms. Two years later, the patient remains asymptomatic and is thriving with weight at the 75th percentile for her age.
Endoscopic Balloon Dilation for Treatment of Congenital Antral Web
Peck, Jacquelin,Khalaf, Racha,Marth, Ryan,Phen, Claudia,Sosa, Roberto,Cordero, Francisco Balsells,Wilsey, Michael The Korean Society of Pediatric Gastroenterology 2018 Pediatric gastroenterology, hepatology & nutrition Vol.21 No.4
Congenital antral webs are a rare but relevant cause of gastric outlet obstruction in infants and children. The condition may lead to feeding refusal, vomiting, and poor growth. Due to the relative rarity of the disease, cases of congenital antral web are frequently misdiagnosed or diagnosed with significant delay as physicians favorably pursue diagnoses of pyloric stenosis and gastric ulcer disease, which are more prevalent. We report a case of an eight-month-old female who presented with persistent non-bilious emesis, feeding difficulties, and failure to thrive and was discovered to have an antral web. The web was successfully treated with endoscopic balloon dilation, which resolved her symptoms. Two years later, the patient remains asymptomatic and is thriving with weight at the 75th percentile for her age.
Suriyan Supapvanich,Preyanuch Mitsang,Pannipa Youryon,Chairat Techavuthiporn,Panida Boonyaritthongchai,Racha Tepsorn 한국원예학회 2018 Horticulture, Environment, and Biotechnology Vol.59 No.3
Physicochemical quality and bioactive compounds of ‘Taaptipjaan’ wax apple (Syzygium samarangenese) that were immersed in 0, 0.5 and 1.0 mM salicylic acid (SA) for 30 min and then stored at 12 ± 1 °C for 9 days were investigated. SA had no effect on the colour of fruit skin, total soluble solids, total acidity, total sugars, ascorbic acid (AsA) and anthocyanin contents but could help maintaining the firmness of the fruit. Antioxidant activity, total phenols, total flavonoids and antioxidant enzymes activities such as peroxidase and catalase were enhanced by SA. The SA immersion at the concentration of 0.5 mM effectively maintained firmness and visual appearance without causing any shrinkage on the fruit top and skin pitting and enhanced the bioactive compounds greater than 1.0 mM SA immersion. The evidences suggest that 0.5 mM SA immersion is an effective approach of maintaining quality and enhancing bioactive compounds including the activities of antioxidant enzymes in the fruit during short-term storage.
Iluyshechkin, Alex,Agranovski, Igor E,Altman, Igor S,Racha, Naresh,Choi, Mansoo IOP Publishing Ltd 2005 Superconductor science & technology Vol.18 No.8
<P>Bi-2212/Ag tapes are fabricated by a combination of dip-coating and partial melt processing. We investigate the effect of addition of MgO particles obtained by Mg combustion. Two different methods of MgO particle embedment in the Bi-2212 matrix are applied. Microstructural studies of processed tapes show that the distribution of MgO particles in the Bi-2212 matrix depends on the methods of tape preparation. If particles were added to the slurry, MgO formed clusters, which depressed Bi-2212 grain alignment and led to degradation of the tape’s transport properties.</P><P> MgO particles, introduced in the Bi-2212 matrix by direct deposition during Mg combustion, significantly enhance the transport properties of the tapes. This improvement also depends on the magnitude of the magnetic field and operating temperatures. At 25 K, the Bi-2212 tape with the MgO added by direct deposition has 2.5 times higher <I>J</I><SUB>c</SUB> value compared to the tapes without MgO doping.</P>