keyword
https://read.qxmd.com/read/37651067/treatment-of-cholestasis-in-infants-and-young-children
#1
REVIEW
Nicole Heinz, Jennifer Vittorio
PURPOSE OF REVIEW: Cholestasis is characterized by a conjugated hyperbilirubinemia secondary to impaired bile synthesis, transport, or excretion from the liver. It is always pathologic and can be indicative of an underlying hepatobiliary, genetic, or metabolic disorder, several of which require timely diagnosis to ensure proper management and optimal outcomes. This review provides an overview of the evaluation of cholestasis with a focus on current and emerging treatment strategies. RECENT FINDINGS: Increased accessibility of next generation sequencing (NGS) allows for utilization of genetic testing early in the diagnostic process...
August 31, 2023: Current Gastroenterology Reports
https://read.qxmd.com/read/37299454/fat-soluble-vitamins-deficiency-in-pediatric-cholestasis-a-scoping-review
#2
REVIEW
Irene Degrassi, Ilaria Leonardi, Elisabetta Di Profio, Chiara Montanari, Gianvincenzo Zuccotti, Elvira Verduci
BACKGROUND: This review aims to identify the current indications and gaps in the management of fat-soluble vitamins in pediatric patients with cholestasis. METHODS: A comprehensive review of the literature using PubMed, Scopus, Web of Science and Embase was performed. Two authors independently identified the most relevant studies published over the past 20 years up to February 2022, including original papers, narrative reviews, observational studies, clinical trials, systematic reviews and meta-analyses...
May 26, 2023: Nutrients
https://read.qxmd.com/read/36997490/prevalence-and-risk-factors-for-pediatric-acute-and-chronic-malnutrition-a-multi-site-tertiary-medical-center-study-in-thailand
#3
JOURNAL ARTICLE
Suchaorn Saengnipanthkul, Amnuayporn Apiraksakorn, Narumon Densupsoontorn, Nalinee Chongviriyaphan
BACKGROUND AND OBJECTIVES: Malnutrition is a major public health concern that increases morbidity and mortality in hospitalized patients, particularly those in developing countries. This study aimed to investigate its prevalence, risk factors, and impact on clinical outcomes in hospitalized children and adolescents. METHODS AND STUDY DESIGN: We conducted a prospective cohort study in patients aged 1 month to 18 years who were admitted to four tertiary care hospitals between December 2018 and May 2019...
2023: Asia Pacific Journal of Clinical Nutrition
https://read.qxmd.com/read/36120654/efficacy-and-tolerance-of-enteral-nutrition-in-children-with-biliary-atresia-awaiting-liver-transplantation
#4
JOURNAL ARTICLE
Elodie Privat, Madeleine Aumar, Delphine Ley, Léa Chantal Tran, Stéphanie Coopman, Dominique Guimber, Dominique Turck, Frédéric Gottrand
OBJECTIVES: Malnutrition is common in children with biliary atresia (BA) awaiting liver transplantation (LT). Few studies have evaluated the effectiveness of enteral nutrition (EN) in these patients. The objective of this work was to assess the efficacy and tolerance of EN in children with BA awaiting LT. METHODS: A total of 31 patients with BA followed between 1995 and 2018 were retrospectively included. Anthropometric indicators (weight, length, and head circumference) and adverse effects of EN were noted at the start (T0) and the end (T1) of EN...
2022: Frontiers in Pediatrics
https://read.qxmd.com/read/35868689/fat-soluble-vitamin-assessment-and-supplementation-in-cholestasis
#5
REVIEW
Binita M Kamath, Estella M Alonso, James E Heubi, Saul J Karpen, Shikha S Sundaram, Benjamin L Shneider, Ronald J Sokol
Malnutrition in children with chronic cholestasis is a prevalent issue and a major risk factor for adverse outcomes. Fat soluble vitamin (FSV) deficiency is an integral feature of cholestatic disease in children, often occurring within the first months of life in those with neonatal cholestasis and malnutrition. This review focuses on FSVs in cholestasis, with particular emphasis on a practical approach to surveillance and supplementation that includes approaches that account for differing local resources. The overarching strategy suggested is to incorporate recognition of FSV deficiencies in cholestatic children in order to develop practical plans for close monitoring and aggressive FSV repletion...
August 2022: Clinics in Liver Disease
https://read.qxmd.com/read/35317744/endoscopic-recanalization-for-the-complete-closure-of-long-gap-esophageal-atresia-after-reconstruction-surgery
#6
JOURNAL ARTICLE
Shin Kashima, Kentaro Moriichi, Yu Kobayashi, Yuya Sugiyama, Yuki Murakami, Takahiro Sasaki, Keitaro Takahashi, Katsuyoshi Ando, Nobuhiro Ueno, Hiroki Tanabe, Mikihiro Fujiya
BACKGROUND: Reconstruction surgery-associated stricture frequently occurs in patients with long-gap esophageal atresia (LGEA). While several endoscopic dilatation methods have been applied and would be desirable, endoscopic recanalization is very difficult in cases with complete esophageal closure. Surgical treatment has been performed for a severe stricture, which causes extensive damage to the infant. No reports have described successful endoscopic recanalization for complete closure due to scarring after surgery for LGEA...
March 22, 2022: BMC Gastroenterology
https://read.qxmd.com/read/34927330/body-composition-correlates-with-laboratory-parameters-and-disease-severity-in-infants-with-biliary-atresia
#7
JOURNAL ARTICLE
Luba Marderfeld, Orith Waisbourd-Zinman, Neta Biran, Michal Rozenfeld Bar-Lev, Ari Silbermintz, Irit Poraz, Dana Reznik, Yifat Jack, Yael Mozer Glassberg, Raanan Shamir
AIM: Infants with biliary atresia (BA) generally have chronic malnutrition. However, the best anthropometric measure to assess malnutrition and its correlation with disease severity is unknown. We aimed to assess correlations of various anthropometric measurements, including air displacement plethysmography (ADP), with laboratory parameters and with the pediatric end-stage liver disease (PELD) score in infants with BA. METHODS: Infants with BA were followed at a pediatric liver transplantation center during 2014-2018...
December 19, 2021: Pediatric Transplantation
https://read.qxmd.com/read/34869514/secondary-malnutrition-and-nutritional-intervention-in-cholestatic-liver-diseases-in-infants
#8
REVIEW
Alfredo Larrosa-Haro, Erika A Caro-Sabido
We aimed to conduct an updated review on the pathophysiology, diagnosis, and nutritional intervention of CCLD and secondary malnutrition in infants. Protein-energy malnutrition, impaired linear growth, fat-soluble vitamin deficiencies, and hepatic osteodystrophy can occur in up to 80% of cases. The proposed pathophysiological mechanisms include insufficient energy intake, lipid- and fat-soluble vitamin malabsorption, increased energy expenditure, altered intermediate metabolism, hormonal dysregulation, and systemic inflammation...
2021: Frontiers in Nutrition
https://read.qxmd.com/read/34669243/malnutrition-in-biliary-atresia-assessment-management-and-outcomes
#9
REVIEW
Julia M Boster, Amy G Feldman, Cara L Mack, Ronald J Sokol, Shikha S Sundaram
Children with biliary atresia (BA), particularly infants, are at high risk for malnutrition attributed to a multitude of factors, including poor oral intake and intolerance of enteral feeding, fat malabsorption, abnormal nutrient metabolism, and increased caloric demand. Malnutrition and sarcopenia negatively impact outcomes in BA, leading to higher pretransplant and posttransplant morbidity and mortality. This review summarizes factors contributing to nutritional deficiencies in BA and offers an organized approach to the assessment and management of malnutrition in this vulnerable population...
March 2022: Liver Transplantation
https://read.qxmd.com/read/34076201/metabolic-and-nutritional-repercussions-of-liver-disease-on-children-how-to-minimize-them
#10
JOURNAL ARTICLE
Beatriz Polisel Mazzoni, Bruna Voltani Lessa, Patricia Zamberlan
OBJECTIVE: To describe the metabolic and nutritional repercussions of chronic liver disease (CLD), proposing strategies that optimize nutritional therapy in the pre- and post-liver transplantation (LT) period, in order to promote favorable clinical outcomes and adequate growth and development, respectively. DATA SOURCES: Bibliographic search in the PubMed, Lilacs and SciELO databases of the last 12 years, in English and Portuguese; target population: children from early childhood to adolescence; keywords in Portuguese and their correlates in English: "Liver Transplant," "Biliary Atresia," "Nutrition Therapy," "Nutritional Status," and "Child"; in addition to Boolean logics "and" and "or," and the manual search of articles...
2021: Revista Paulista de Pediatria: Orgão Oficial da Sociedade de Pediatria de São Paulo
https://read.qxmd.com/read/32925558/unusual-clinical-course-for-untreated-malformative-biliary-atresia-infant-is-portal-hypertension-an-important-driver-of-liver-fibrosis
#11
JOURNAL ARTICLE
Fabiola Di Dato, Giulia Ranucci, Jean de Ville de Goyet, Daniele Alberti, Raffaele Iorio
In biliary atresia, infants left untreated, and in those with unsuccessful porto-enterostomy, hepatic condition and function worsen rapidly towards cirrhosis, malnutrition, portal hypertension with ascites, and variceal haemorrhage; many die within the first 3 years of life unless they benefit from liver replacement. We describe a girl with biliary atresia splenic malformation syndrome, who had portal vein cavernoma and microsplenia; she did not undergo porto-enterostomy. She survived with her native liver over the age of 3 years...
February 1, 2021: Journal of Pediatric Gastroenterology and Nutrition
https://read.qxmd.com/read/32432119/pediatric-cholestatic-liver-disease-review-of-bile-acid-metabolism-and-discussion-of-current-and-emerging-therapies
#12
REVIEW
Alyssa Kriegermeier, Richard Green
Cholestatic liver diseases are a significant cause of morbidity and mortality and the leading indication for pediatric liver transplant. These include diseases such as biliary atresia, Alagille syndrome, progressive intrahepatic cholestasis entities, ductal plate abnormalities including Caroli syndrome and congenital hepatic fibrosis, primary sclerosing cholangitis, bile acid synthesis defects, and certain metabolic disease. Medical management of these patients typically includes supportive care for complications of chronic cholestasis including malnutrition, pruritus, and portal hypertension...
2020: Frontiers in Medicine
https://read.qxmd.com/read/32068547/pediatric-screening-tools-for-malnutrition-an-update
#13
REVIEW
Jessie M Hulst, Koen Huysentruyt, Koen F Joosten
PURPOSE OF REVIEW: There is ongoing interest in nutritional screening tools in pediatrics to facilitate the identification of children at risk for malnutrition who need further assessment and possible nutritional intervention. The choice for a specific tool depends on various factors. This review aims to provide an overview of recent progress in pediatric nutritional screening methods. RECENT FINDINGS: We present recent studies about newly developed or adjusted tools, the applicability of nutritional screening tools in specific populations, and how to implement screening in the overall process of improving nutritional care in the pediatric hospital setting...
May 2020: Current Opinion in Clinical Nutrition and Metabolic Care
https://read.qxmd.com/read/29356765/resolving-malnutrition-with-parenteral-nutrition-before-liver-transplant-in-biliary-atresia
#14
JOURNAL ARTICLE
Danielle Wendel, Melissa Mortensen, Alisha Harmeson, Michele L Shaffer, Evelyn Hsu, Simon Horslen
OBJECTIVE: Malnutrition is a common complication of end-stage liver disease (ESLD) associated with poor liver transplant outcomes. Nasogastric feeds are used for nutritional supplementation, but some patients remain malnourished. Parenteral nutrition (PN) can be effective, but has potential complications. The primary objective was to evaluate the effect of PN on anthropometric measures in children with ESLD awaiting liver transplant. Secondary objectives were evaluation of PN-associated complications, liver function tests, pediatric end-stage liver disease scores, waitlist time, and post-transplant length of stay (total and time in the intensive care unit)...
February 2018: Journal of Pediatric Gastroenterology and Nutrition
https://read.qxmd.com/read/28699603/infants-with-extrahepatic-biliary-atresia-effect-of-follow-up-on-the-survival-rate-at-ege-university-medical-school-transplantation-center
#15
JOURNAL ARTICLE
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://read.qxmd.com/read/27989363/complications-in-pediatric-hepatobiliary-surgery
#16
REVIEW
Gabriella Grisotti, Robert A Cowles
This review highlights the complications and their risk factors encountered in pediatric hepatobiliary surgery, specifically in the context of pediatric hepatic resection, excision of choledochal cyst, and the Kasai hepatoportoenterostomy procedure for biliary atresia as well as other procedures potentially affecting the biliary tree. With the understanding that these are relatively rare procedures, case reports and small case series are included in addition to larger series when available. The review focuses on publications in English over the past 15 years...
December 2016: Seminars in Pediatric Surgery
https://read.qxmd.com/read/27957786/impaired-intention-to-treat-survival-after-listing-for-liver-transplantation-in-children-with-biliary-atresia-compared-to-other-chronic-liver-diseases-20%C3%A2-years-experience-from-the-nordic-countries
#17
JOURNAL ARTICLE
S Malenicka, B-G Ericzon, M H Jørgensen, H Isoniemi, T H Karlsen, M Krantz, V Naeser, M Olausson, A Rasmussen, K Rönnholm, T Sanengen, T Scholz, B Fischler, A Nemeth
Biliary atresia (BA) is the most common indication for LT in children. We investigated whether this diagnosis per se, compared to other chronic liver diseases (OCLD), had an influence on patient survival. Data from 421 Scandinavian children, 194 with BA and 227 with OCLD, listed for LT between 1990 and 2010 were analyzed. The intention-to-treat survival and influencing risk factors were studied. Patients with BA had higher risk of death after listing than patients with OCLD. The youngest (<1 year) and smallest (<10 kg) children with the highest bilirubin (>510 μmol/L), highest INR (>1...
March 2017: Pediatric Transplantation
https://read.qxmd.com/read/27650268/biliary-atresia-indications-and-timing-of-liver-transplantation-and-optimization-of-pretransplant-care
#18
REVIEW
Shikha S Sundaram, Cara L Mack, Amy G Feldman, Ronald J Sokol
Biliary atresia (BA) is a progressive, fibro-obliterative disorder of the intrahepatic and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation (LT). Indications for LT in BA include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extrahepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for LT...
January 2017: Liver Transplantation
https://read.qxmd.com/read/26269683/serum-proinflammatory-cytokines-and-nutritional-status-in-pediatric-chronic-liver-disease
#19
JOURNAL ARTICLE
Daniele Santetti, Maria Inês de Albuquerque Wilasco, Cristina Toscani Leal Dornelles, Isabel Cristina Ribas Werlang, Fernanda Urruth Fontella, Carlos Oscar Kieling, Jorge Luiz Dos Santos, Sandra Maria Gonçalves Vieira, Helena Ayako Sueno Goldani
AIM: To evaluate the nutritional status and its association with proinflammatory cytokines in children with chronic liver disease. METHODS: We performed a cross-sectional study with 43 children and adolescents, aged 0 to 17 years, diagnosed with chronic liver disease. All patients regularly attended the Pediatric Hepatology Unit and were under nutritional follow up. The exclusion criteria were fever from any etiology at the time of enrollment, inborn errors of the metabolism and any chronic illness...
August 7, 2015: World Journal of Gastroenterology: WJG
https://read.qxmd.com/read/26205222/predictive-equations-are-inaccurate-in-the-estimation-of-the-resting-energy-expenditure-of-children-with-end-stage-liver-disease
#20
JOURNAL ARTICLE
Andrea Carpenter, Vicky Lee Ng, Karen Chapman, Simon C Ling, Marialena Mouzaki
BACKGROUND AND OBJECTIVES: Malnutrition is common in children with end-stage liver disease (ESLD) and is associated with increased morbidity and mortality. The inability to accurately estimate energy needs of these patients may contribute to their poor nutrition status. In clinical practice, predictive equations are used to calculate resting energy expenditure (cREE). The objective of this study is to assess the accuracy of commonly used equations in pediatric patients with ESLD. METHODS: Retrospective study performed at the Hospital for Sick Children...
March 2017: JPEN. Journal of Parenteral and Enteral Nutrition
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