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Pediatric cholestasis

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https://www.readbyqxmd.com/read/28947328/outcomes-of-biliary-atresia-in-the-nordic-countries-a-multicenter-study-of-158-patients-during-2005-2016
#1
Mikko P Pakarinen, Lars Søndergaard Johansen, Jan F Svensson, Kristin Bjørnland, Vladimir Gatzinsky, Pernilla Stenström, Antti Koivusalo, Nina Kvist, Markus Almström, Ragnhild Emblem, Sigurdur Björnsson, Torbjörn Backman, Runar Almaas, Hannu Jalanko, Björn Fischler, Jørgen Thorup
BACKGROUND/PURPOSE: Biliary atresia is the most common reason for newborn cholestasis and pediatric liver transplantation. Even after normalization of serum bilirubin after portoenterostomy, most patients require liver transplantation by adulthood due to expanding fibrosis. We addressed contemporary outcomes of biliary atresia in the Nordic countries. METHODS: Data on center and patients characteristics, diagnostic practices, surgical treatment, adjuvant medical therapy after portoenterostomy, follow-up and outcomes were collected from all the Nordic centers involved with biliary atresia care during 2005-2016...
September 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28947327/understanding-the-relationship-between-hospital-volume-and-patient-outcomes-for-infants-with-gastroschisis
#2
Genia Dubrovsky, Greg D Sacks, Scott Friedlander, Steven Lee
BACKGROUND: For many surgical operations, there is a well-established relationship between surgical volume and outcome. We investigated whether this relationship exists for infants with gastroschisis. METHODS: Using the Kids' Inpatient Database for years 2003, 2006, 2009, and 2012, we identified all patients undergoing gastroschisis repair. Controlling for patient characteristics and complexity of disease (comorbid intestinal atresia/perforation, necrotizing enterocolitis, and respiratory distress syndrome), we compared surgical outcomes (mortality, length of stay, and incidence of TPN cholestasis) by hospital volume based on quartile for gastroschisis cases treated per year...
September 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28838453/cholestasis-after-pediatric-liver-transplantation-recurrence-of-a-progressive-familial-intrahepatic-cholestasis-phenotype-as-a-rare-differential-diagnosis-a-case-report
#3
B Prusinskas, S Kathemann, D Pilic, B Hegen, P Küster, V Keitel, D Häussinger, R Büscher, H A Baba, P F Hoyer, E Lainka
INTRODUCTION: Nonobstructive cholestasis after pediatric liver transplantation is a common diagnostic and therapeutic dilemma. We describe a girl with neonatal cholestasis because of progressive familial intrahepatic cholestasis 2 (PFIC-2) and presence of a homozygous splice site mutation in the ABCB11 gene. Liver transplantation was performed because of end-stage liver disease at the age of 6. Cholestasis with normal gamma-glutamyl transferase (GGT) developed 8 years after liver transplantation...
September 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28809725/clinical-characteristics-and-complications-of-pediatric-liver-biopsy-a-single-centre-experience
#4
Patricia Almeida, Richard A Schreiber, Jennifer Liang, Quais Mujawar, Orlee R Guttman
INTRODUCTION: Percutaneous liver biopsy (LB) is the gold standard method for evaluation and management of patients with liver disease. The purpose of this study was to characterize pediatric patients undergoing LB at British Columbia Children's Hospital, and to determine the rate and timing of complications following the procedure. MATERIAL AND METHODS: The medical records of all pediatric patients who underwent LB during a six-year retrospective study were reviewed to collect demographic and procedure-related data...
September 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28731841/survey-of-nutrition-management-practices-in-centers-for-pediatric-intestinal-rehabilitation
#5
Anita M Nucci, Kipp Ellsworth, Austin Michalski, Emily Nagel, Jackie Wessel
BACKGROUND: Nutrition management of pediatric intestinal failure (IF) requires interdisciplinary coordination of parenteral nutrition (PN) and enteral nutrition (EN) support. Nutrition strategies used by specialists in pediatric intestinal rehabilitation to promote gut adaptation and manage complications have not been previously summarized. METHODS: A practice survey was distributed to members of the dietitian subgroup of the American Society for Parenteral and Enteral Nutrition Pediatric Intestinal Failure Section...
July 1, 2017: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/28710680/development-of-a-novel-tool-to-assess-the-impact-of-itching-in-pediatric-cholestasis
#6
Binita M Kamath, Linda Abetz-Webb, Ciara Kennedy, Bonnie Hepburn, Martha Gauthier, Nathan Johnson, Sharon Medendorp, Alejandro Dorenbaum, Lora Todorova, Benjamin L Shneider
OBJECTIVES: The aim was to develop a clinical outcome assessment (COA) for itching in children with cholestatic pruritus. METHODS: This prospective study aimed to enroll patients aged 4-30 years with Alagille syndrome (ALGS) or progressive familial intrahepatic cholestasis type 1 and caregivers of patients aged 5 months to 14 years. Eligible patients experienced itching during ≥3 of the 7 days before enrollment and had not undergone liver transplant or surgical interruption of the enterohepatic circulation...
July 14, 2017: Patient
https://www.readbyqxmd.com/read/28699603/infants-with-extrahepatic-biliary-atresia-effect-of-follow-up-on-the-survival-rate-at-ege-university-medical-school-transplantation-center
#7
Miray Karakoyun, Maşallah Baran, Caner Turan, Murat Kılıç, Orkan Ergun, Sema Aydoğdu
BACKGROUND/AIMS: Biliary atresia (BA) is the main cause of neonatal cholestasis and the primary reason for infant liver transplants worldwide. It is an obliterative cholangiopathy observed only in children and caused by progressive inflammation and fibrosis of the bile duct. We collaborated with a liver transplantation center to investigate the effects of follow-up in patients with BA. MATERIALS AND METHODS: Medical records of 99 patients who were diagnosed with BA and monitored at our center from 1990 to 2002 (27 patients) and from 2003 to 2015 (72 patients) were analyzed retrospectively...
July 2017: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/28683534/a-case-of-alagille-syndrome-presenting-with-chronic-cholestasis-in-an-adult
#8
Jihye Kim, Bumhee Yang, Namyoung Paik, Yon Ho Choe, Yong-Han Paik
Alagille syndrome (AGS) is a complex multisystem disorder that involves mainly the liver, heart, eyes, face, and skeleton. The main associated clinical features are chronic cholestasis due to a paucity of intrahepatic bile ducts, congenital heart disease primarily affecting pulmonary arteries, vertebral abnormalities, ocular embryotoxon, and peculiar facies. The manifestations generally become evident at a pediatric age. AGS is caused by defects in the Notch signaling pathway due to mutations in JAG1 or NOTCH2...
September 2017: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/28665446/first-experience-on-bilirubin-removal-with-a-hemoadsorption-column-lixelle%C3%A2-in-a-child-with-cardiogenic-liver-injury
#9
Gabriella Bottari, Andrea Moscatelli, Enrico E Verrina, Franco Lerzo, Fabio S Taccone
INTRODUCTION: Hyperbilirubinemia may have deleterious effects on many organs, even after the neonatal age. Blood purification is effective in the treatment of hyperbilirubinemia. Recently some reports suggest the potential role of hemoadsorption columns in this setting. METHODS: We present the case of a 6-year-old child with severe hyperbilirubinemia due to congestive liver dysfunction, complicated by persistent inflammation, immunosuppression and catabolism syndrome (PICS)...
June 20, 2017: International Journal of Artificial Organs
https://www.readbyqxmd.com/read/28521607/intravenous-fish-oil-and-pediatric-intestinal-failure-associated-liver-disease-changes-in-plasma-phytosterols-cytokines-and-bile-acids-and-erythrocyte-fatty-acids
#10
Kara L Calkins, Andrea DeBarber, Robert D Steiner, Martiniano J Flores, Tristan R Grogan, Susanne M Henning, Laurie Reyen, Robert S Venick
BACKGROUND: Soybean oil (SO) emulsions are associated with intestinal failure-associated liver disease (IFALD); fish oil (FO) emulsions are used to treat IFALD. SO and FO differ with respect to their fatty acid and phytosterol content. In children with IFALD whose SO was replaced with FO, we aimed to (1) quantify changes in erythrocyte fatty acids and plasma phytosterols, cytokines, and bile acids and (2) correlate these changes with direct bilirubin (DB). DESIGN: This study enrolled IFALD children who received 6 months of FO...
May 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28497648/intrahepatic-cholangiojejunostomy-for-complex-biliary-stenosis-after-pediatric-living-donor-liver-transplantation
#11
Fernando A Alvarez, Rodrigo Sanchez Claria, Juan Glinka, Martin de Santibañes, Juan Pekolj, Eduardo de Santibañes, Miguel A Ciardullo
The treatment of biliary stenosis after pediatric LDLT is challenging. We describe an innovative technique of peripheral IHCJ for the treatment of patients with complex biliary stenosis after pediatric LDLT in whom percutaneous treatment failed. During surgery, the percutaneous biliary drainage is removed and a flexible metal stylet is introduced trough the tract. Subsequently, the most superficial aspect of the biliary tree is recognized by palpation of the stylet's round tip in the liver surface. The liver parenchyma is then transected until the bile duct is reached...
August 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28483736/child-with-pancreatic-hydatid-cyst-presenting-with-cholestasis
#12
Nagehan Aslan, Tuğba Koca, Aykut Recep Aktaş, Füsun Zeynep Akçam, Mustafa Akçam
Although hydatid cysts are often seen in the liver and lungs, they may be present in many organs. Even in countries where hydatid cyst disease is endemic, the occurrence of pancreatic hydatid cysts is rare. Pancreatic hydatid cysts are important for the differential diagnosis of patients with pancreatic pseudocysts and cystic carcinomas. We could not find cystic echinococcosis cases which are kept together pancreas and liver in PubMed. In this article, we highlight the fact that pancreatic cystic echinococcosis may play a role in the etiology of cholestasis and that cysts may not be isolated in the pancreas in a pediatric population...
March 2017: Türkiye Parazitolojii Dergisi
https://www.readbyqxmd.com/read/28434083/predictors-of-a-successful-outcome-for-infants-with-short-bowel-syndrome-a-30-year-single-institution-experience
#13
Tatsuru Kaji, Kazuhiko Nakame, Seiro Machigashira, Takafumi Kawano, Ryuta Masuya, Waka Yamada, Koji Yamada, Shun Onishi, Tomoe Moriguchi, Koshiro Sugita, Motoi Mukai, Satoshi Ieiri
PURPOSE: Short-bowel syndrome (SBS) is associated with high morbidity and mortality. We conducted this study to establish the predictors of survival and weaning off parenteral nutrition (PN). METHODS: We reviewed the medical records of 16 SBS infants treated at our institution within a 30-year period. SBS was defined as a residual small-bowel length (RSBL) of <75 cm. Loss of the ileocecal valve (ICV), cholestasis (D-Bil >2.0 mg/dl), enterostomy, and RSBL were all evaluated...
April 22, 2017: Surgery Today
https://www.readbyqxmd.com/read/28426902/hepatic-steatosis-after-pediatric-liver-transplant
#14
Emily R Perito, Tabitha Vase, Rageshree Ramachandran, Andrew Phelps, Kuang-Yu Jen, Robert H Lustig, Vickie A Feldstein, Philip Rosenthal
Hepatic steatosis develops after liver transplantation (LT) in 30% of adults, and nonalcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in nontransplanted children. However, posttransplant steatosis has been minimally studied in pediatric LT recipients. We explored the prevalence, persistence, and association with chronic liver damage of hepatic steatosis in these children. In this single-center study of pediatric patients transplanted 1988-2015 (n = 318), 31% of those with any posttransplant biopsy (n = 271) had ≥ 1 biopsy with steatosis...
July 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28397065/referral-patterns-and-factors-influencing-age-at-admission-of-infants-with-cholestasis-in-india
#15
Gopinathan Mathiyazhagan, Barath Jagadisan
OBJECTIVES: To define the recognition, age at admission, referral time and referral pattern of neonatal cholestasis in India. METHODS: This prospective, observational study was conducted from February 2015 through March 2016 in the Pediatric gastroenterology unit of JIPMER, Pondicherry in infants with cholestasis < 6 mo of age. RESULTS: Among 64 infants, median age of admission was 52 d (IQR 28-63 d). Fifty of sixty four infants (78.1%) came with parent-reported cholestasis-related symptoms of either jaundice alone (57...
August 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28355202/microrna-155-modulates-bile-duct-inflammation-via-targeting-suppressor-of-cytokine-signaling-1-in-biliary-atresia
#16
Rui Zhao, Rui Dong, Yifan Yang, Yuqing Wang, Jin Ma, Jiang Wang, Hao Li, Shan Zheng
BACKGROUND: Biliary atresia (BA) is an etiologically perplexing disease, manifested by neonatal cholestasis, repeated cholangitis, and progressive biliary fibrosis. MiR-155 has been implicated to modulate the immune response, which contributes to biliary injury. However, its potential role in the pathogenesis of BA has not been addressed so far. METHODS: The microRNA changes from BA patients and controls were identified via microarray. The immunomodulatory function of miR-155 was investigated via cell transfection and reporter assay system...
March 29, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28343205/-biliary-atresia-signs-and-symptoms-diagnosis-clinical-management
#17
REVIEW
Ewa Orłowska, Piotr Czubkowski, Piotr Socha
Biliary atresia is a chronic cholangiopathy leading to progressive fibrosis of both intra- and extrahepatic bile ducts. The cause of the condition is unknown. Fundamental management of biliary atresia is surgical intervention and the outcomes of the treatment depend on the child's age with best results when performed within the first 2 months of life. Thus, the main role of pediatric healthcare is an urgent differential diagnosis and prompt qualification for the surgery, optimal postoperative management and early qualification for the liver transplantation in patients with persistent cholestasis...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28333693/pediatric-intestinal-failure-associated-liver-disease
#18
Cathleen M Courtney, Brad W Warner
PURPOSE OF REVIEW: The goal of this review is to provide updates on the definition, pathophysiology, treatment, and prevention of intestinal failure-associated liver disease (IFALD) that are relevant to care of pediatric patients. RECENT FINDINGS: Current literature emphasizes the multifactorial nature of IFALD. The pathogenesis is still largely unknown; however, molecular pathways have been identified. Key to these pathways are proinflammatory cytokines involved in hepatic inflammation and bile acids synthesis such as Toll-like receptor 4 and farnesoid X receptor, respectively...
June 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28326955/a-challenging-case-of-severe-infantile-cholestasis-in-alpha-1-antitrypsin-deficiency
#19
Zahida Khan, Veena L Venkat, Kyle A Soltys, Donna B Stolz, Sarangarajan Ranganathan
Jaundice in the newborn period can be physiologic and is often due to benign causes. Jaundice due to conjugated hyperbilirubinemia extending beyond the second week of life may be an early sign of several cholestatic or metabolic liver diseases, and it requires logical and timely analysis so that specific treatments can be initiated. Alpha-1 antitrypsin deficiency is the most common genetic cause of pediatric liver disease and transplantation, and it must be considered when evaluating cholestatic infants. Here, we present an unusual case of alpha-1 antitrypsin deficiency with severe infantile cholestasis and rapid decompensation in the first 4 months of life, where in-depth but timely diagnosis was crucial for the appropriate intervention to take place...
March 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28149148/pediatric-liver-transplantation-our-experiences
#20
Ahmet Basturk, Aygen Yılmaz, Ersin Sayar, Ayhan Dinçhan, İbrahim Aliosmanoğlu, Halil Erbiş, Bülent Aydınlı, Reha Artan
OBJECTIVE: The aim of our study was to evaluate our liver transplant pediatric patients and to report our experience in the complications and the long-term follow-up results. MATERIALS AND METHODS: Patients between the ages of 0 and 18 years, who had liver transplantation in the organ transplantation center of our university hospital between 1997 and 2016, were included in the study. The age, sex, indications for the liver transplantation, complications after the transplantation, and long-term follow-up findings were retrospectively evaluated...
October 2016: Eurasian Journal of Medicine
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