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Portal hypertension children

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https://www.readbyqxmd.com/read/29209118/chronic-liver-disease-is-universal-in-children-with-biliary-atresia-living-with-native-liver
#1
Way Seah Lee, Sik Yong Ong, Hee Wei Foo, Shin Yee Wong, Chen Xi Kong, Ru Bin Seah, Ruey Terng Ng
AIM: To examine the medical status of children with biliary atresia (BA) surviving with native livers. METHODS: In this cross-sectional review, data collected included complications of chronic liver disease (CLD) (cholangitis in the preceding 12 mo, portal hypertension, variceal bleeding, fractures, hepatopulmonary syndrome, portopulmonary hypertension) and laboratory indices (white cell and platelet counts, total bilirubin, albumin, international normalized ratio, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase)...
November 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29183424/surgery-for-portal-hypertension-in-children-a-12-year-review
#2
N Patel, A Grieve, J Hiddema, J Botha, J Loveland
BACKGROUND: Portal hypertension is a common and potentially devastating condition in children. Notwithstanding advances in the nonsurgical management of portal hypertension, surgery remains an important treatment modality in select patients. We report here on our experience in the past 12 years. OBJECTIVES: To describe the profile of, indication for, and complications of shunt surgery in children with portal hypertension. METHODS: Twelve children underwent shunt surgery between 2005 and 2017...
November 6, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/29176477/noninvasive-methods-of-predicting-large-esophageal-varices-in-children-with-intrahepatic-portal-hypertension
#3
Marina R Adami, Carlos Oscar Kieling, Fernando Pereira Schwengber, Vania N Hirakata, Sandra Maria Gonçalves Vieira
OBJECTIVE: Esophageal variceal bleeding is a severe complication of portal hypertension. The standard diagnostic screening test and therapeutic procedure for esophageal varices is endoscopy, which is invasive in pediatric patients. This study aimed to evaluate the role of noninvasive parameters as predictors of large varices in children with intrahepatic portal hypertension. METHODS: Participants included in this cross-sectional study underwent a screening endoscopy...
November 22, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29176474/hepatic-parenchymal-injury-in-crigler-najjar-type-i
#4
Ellen Mitchell, Sarangarajan Ranganathan, Patrick McKiernan, Robert H Squires, Kevin Strauss, Kyle Soltys, George Mazariegos, James E Squires
BACKGROUND: Crigler-Najjar syndrome type I (CNI) arises from biallelic variants of UGT1A1 that abrogate UGT1A1 activity resulting in unconjugated hyperbilirubinemia. Historically, liver parenchyma in CNI was considered structurally and histologically normal. Recent review of CNI liver explants revealed fibrosis. Our aim was to investigate the association between hepatic histology and disease phenotype in CNI. METHODS: We extracted data from the medical record at the time of liver transplant from 22 patients with CNI at the Children's Hospital of Pittsburgh, and reviewed explant histology...
November 22, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29150327/diagnostic-accuracy-of-point-shear-wave-elastography-in-the-detection-of-portal-hypertension-in-pediatric-patients
#5
M Burak Özkan, M C Bilgici, E Eren, G Caltepe
PURPOSE: The purpose of this study was to determine the usefulness of point shear wave elastography (p-SWE) of the liver and spleen for the detection of portal hypertension in pediatric patients. MATERIALS AND METHODS: The study consisted of 38 healthy children and 56 pediatric patients with biopsy-proven liver disease who underwent splenic and liver p-SWE. The diagnostic performance of p-SWE in detecting clinically significant portal hypertension was assessed using receiver operating characteristic (ROC) curves...
November 14, 2017: Diagnostic and Interventional Imaging
https://www.readbyqxmd.com/read/29037349/the-optimal-procedure-of-modified-rex-shunt-for-the-treatment-of-extrahepatic-portal-hypertension-in-children
#6
Jin-Shan Zhang, Long Li, Wei Cheng
OBJECTIVE: Since 2008, the modified Rex shunt has been used for the treatment of extrahepatic portal venous obstruction (EHPVO) in our center, and satisfactory results were initially achieved. However, the postoperative recurrence rate gradually increased. We therefore performed a retrospective study to evaluate the outcome and identify the optimal procedure. METHODS: Between October 2008 and March 2016, 79 children with EHPVO underwent the Rex shunt in our hospital: 48 underwent the gastroportal shunt (GP), 26 underwent the portal cavernoma-Rex shunt with interposition of grafted portal vessel (PP), and 5 underwent a spleen-preserving splenoportal bypass (SP)...
November 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28986027/cystic-fibrosis-related-cirrhosis
#7
Daniel H Leung, Michael R Narkewicz
While liver involvement is common in cystic fibrosis, the major liver disorder with impact on the clinical outcome of individuals with CF is the development of multilobular cirrhosis with progression to portal hypertension. Interestingly, this is a disorder primarily of children and adolescents. We review the proposed pathogenesis, clinical presentation, diagnostic work-up, medical and surgical management, and complications of CF cirrhosis.
November 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28983709/late-complications-of-biliary-atresia-hepatopulmonary-syndrome-and-portopulmonary-hypertension
#8
Frederick M Karrer, Bradley J Wallace, Arturo E Estrada
Children with biliary atresia (BA) following Kasai portoenterostomy have a high risk for portal hypertension, however, while variceal and hemorrhagic complications have been more commonly studied, less frequent but no less possibly devastating complications of hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH) remain less well understood. HPS and PPH both occur in a setting of portal hypertension, however, paradoxically patients with HPS develop pulmonic vasculature dilation leading to shunting and hypoxia, while those with PPH develop an opposite progression of pulmonary vasoconstriction eventually leading to cor pulmonale and decompensation...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28967497/pulmonary-artery-hypertension-in-childhood-the-transforming-growth-factor-%C3%AE-superfamily-related-genes
#9
REVIEW
Shi-Min Yuan
Pulmonary artery hypertension (PAH) is very rare in childhood, and it can be divided into heritable, idiopathic drug- and toxin-induced and other disease (connective tissue disease, human immunodeficiency virus infection, portal hypertension, congenital heart disease, or schistosomiasis)-associated types. PAH could not be interpreted solely by pathophysiological theories. The impact of the transforming growth factor-β superfamily-related genes on the development of PAH in children remains to be clarified. Pertinent literature on the transforming growth factor-β superfamily-related genes in relation to PAH in children published after the year 2000 was reviewed and analyzed...
August 12, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28887821/serpina1-and-man1b1-polymorphisms-are-not-linked-to-severe-liver-disease-in-a-french-cohort-of-alpha-1-antitrypsin-deficiency-children
#10
Philippe Joly, Alain Lachaux, Mathias Ruiz, Lioara Restier, Abdelhouaed Belmalih, Colette Chapuis-Cellier, Alain Francina, Céline Renoux, Marion Bouchecareilh
BACKGROUND & AIMS: Fifteen to twenty percent of alpha-1 antitrypsin deficiency patients (A1ATD) have a severe liver outcome (portal hypertension - PHT) during childhood. Since they all share the same ZZSERPINA1 genotype and that environmental factors such as alcohol cannot be advanced, the presence of modifier genes is now well recognized. SNPs located on the SERPINA1 and MAN1B1 genes have already been tested in very few studies with contradictory or not replicated results. METHODS: Our genotype-phenotype correlation study, performed on 92 ZZ children, aimed at determining once and for all if SERPINA1 and MAN1B1 polymorphisms may be implied in the onset of PHT...
November 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28884036/severe-hepatopulmonary-syndrome-in-a-child-with-caroli-syndrome
#11
W De Jesus-Rojas, K McBeth, A Yadav, J M Stark, R A Mosquera, C Jon
Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexplained, hypoxemia. The presence of HPS can improve patient priority on the liver transplantation wait list, despite their Pediatric End-Stage Liver Disease (PELD) score...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28842757/successful-long-term-outcome-of-pediatric-liver-kidney-transplantation-a-single-center-study
#12
Jesús Quintero Bernabeu, Javier Juamperez, Marina Muñoz, Olalla Rodriguez, Ramon Vilalta, José A Molino, Marino Asensio, Itxarone Bilbao, Gema Ariceta, Carlos Rodrigo, Ramón Charco
INTRODUCTION: Liver-kidney transplantation is a rare procedure in children, with just ten to 30 cases performed annually worldwide. The main indications are autosomal recessive polycystic liver-kidney disease and primary hyperoxaluria. This study aimed to report outcomes of liver-kidney transplantation in a cohort of pediatric patients. METHODS: We retrospectively analyzed all pediatric liver-kidney transplantations performed in our center between September 2000 and August 2015...
August 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28814423/portal-hypertension-and-its-management-in-children
#13
REVIEW
Tassos Grammatikopoulos, Patrick James McKiernan, Anil Dhawan
Portal hypertension (PHT), defined as raised intravascular pressure in the portal system, is a complication of chronic liver disease or liver vascular occlusion. Advances in our ability to diagnose and monitor the condition but also predict the risk of gastrointestinal bleeding have enabled us to optimise the management of children with PHT either at a surveillance or at a postbleeding stage. A consensus among paediatric centres in the classification of varices can be beneficial in streamlining future paediatric studies...
August 16, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28782299/continuous-erythropoiesis-receptor-activator-cera-for-the-anaemia-of-chronic-kidney-disease
#14
REVIEW
Valeria M Saglimbene, Suetonia C Palmer, Marinella Ruospo, Patrizia Natale, Jonathan C Craig, Giovanni Fm Strippoli
BACKGROUND: Continuous erythropoiesis receptor activator (CERA) is a newer, longer acting ESA which might be preferred to other ESAs (epoetin or darbepoetin) based on its lower frequency of administration. Different dosing requirements and molecular characteristics of CERA compared with other ESAs may lead to different health outcomes (mortality, cardiovascular events, quality of life) in people with anaemia and chronic kidney disease (CKD). OBJECTIVES: To assess benefits and harms of CERA compared with other epoetins (darbepoetin alfa and epoetin alfa or beta) or placebo/no treatment or CERA with differing strategy of administration for anaemia in individuals with CKD...
August 7, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28694570/extrahepatic-portal-vein-obstruction-in-children-role-of-preoperative-imaging
#15
Shashidhar Achar, Hemonta Kumar Dutta, Rudra Kanta Gogoi
AIM: Extrahepatic portal vein obstruction (EHPVO) is characterized by features of recent thrombosis or portal hypertension with portal cavernoma as a sequel of portal vein obstruction. Imaging of spleno-portal axis is the mainstay for the diagnosis of EHPVO. The aim of this study is to analyze the role of imaging in the preoperative assessment of the portal venous system in children with EHPVO. MATERIALS AND METHODS: A hospital-based cross-sectional study was conducted on twenty children with EHPVO aged between 1 and 18 years over a period of 1 year...
July 2017: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/28685316/-liver-biopsy-in-children-and-adolescents-preliminary-morphological-examinations-in-diffuse-liver-disease
#16
REVIEW
C Lackner, A S Knisely
BACKGROUND: Frequencies of types of liver disease differ between adults and nonadults (NA). OBJECTIVES: The particular problems encountered in interpreting liver-biopsy findings in NA often require referral in consultation.  To permit this efficiently, we recommend specific approaches to light microscopy, with special stains and immunohistochemistry, as well as to ultrastructural study. METHODS: Prosection and the choice of special stains are described, and are discussed in relation to clinical questions...
July 2017: Der Pathologe
https://www.readbyqxmd.com/read/28638943/elevated-serum-heat-shock-protein-70-and-liver-stiffness-reflect-hepatic-dysfunction-and-severity-in-postoperative-biliary-atresia
#17
Sittisak Honsawek, Wanvisa Udomsinprasert, Napaphat Jirathanathornnukul, Voranush Chongsrisawat, Yong Poovorawan
BACKGROUND: Biliary atresia (BA) is a severe chronic liver disease characterized by progressive obstructive cholangiopathy of biliary tract. Heat shock protein 70 (HSP70) is involved in protecting cells against a wide variety of stress and plays a protective role in tissue damage. The purpose of this study was to investigate serum HSP70 and liver stiffness in BA and determine the association of serum HSP70, liver stiffness, and outcome parameters in post-Kasai BA patients. METHODS: One hundred post-Kasai BA patients and 40 controls were enrolled...
August 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28611971/diagnosis-and-management-of-hepatobiliary-complications-in-autosomal-recessive-polycystic-kidney-disease
#18
REVIEW
Andrew Wehrman, Alyssa Kriegermeier, Jessica Wen
Autosomal recessive polycystic kidney disease (ARPKD) is a congenital hepatorenal fibrocystic disease. The hepatic manifestations of ARPKD can range from asymptomatic to portal hypertension and massively dilated biliary system that results in liver transplantation. Hepatic complications of ARPKD typically present with signs of portal hypertension (splenomegaly and thrombocytopenia) or cholangitis. Liver disease in ARPKD does not always correlate with severity of renal disease. Management of ARPKD-related liver disease is largely treating specific symptoms, such as antibiotics for cholangitis or endoscopic treatment for variceal bleeding...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28588179/childhood-wilson-disease-bangladesh-perspective
#19
M Rukunuzzaman, A B Karim, M Nurullah, F Sultana, M W Mazumder, M A Rahman, S B Billah, F Begum, M Oliullah
Wilson's disease (WD) is an autosomal recessive disorder affecting copper metabolism causing copper induced damage to various organs. In children liver is commonly involved. Central nervous system, eyes, RBC, kidneys, brain and bones may also be affected. Aim of the study is to evaluate clinical & laboratory profile of Wilson's disease in children. This cross sectional descriptive study was conducted at the department of Paediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh, from January 2011 to December, 2013...
April 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28588171/clinical-and-laboratory-predictors-of-esophageal-varices-in-children-with-chronic-liver-disease
#20
M I Hasan, M Rukunuzzaman, M Nurullah, F Sultana
Esophageal varices are a serious consequence of portal hypertension in patients with chronic liver diseases. Several studies have evaluated possible noninvasive markers of esophageal varices to reduce the number of unnecessary endoscopies in patients with chronic liver disease. Aim of this study was to identify clinical and laboratory predictors of esophageal varices in children with chronic liver disease. This cross sectional observational study was done at Pediatric Gastroenterology and Nutrition Department of Bangabandhu Sheikh Mujib Medical University, Dhaka, over a period of 6 months...
April 2017: Mymensingh Medical Journal: MMJ
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