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Portal hypertension children

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https://www.readbyqxmd.com/read/29622386/long-term-outcome-of-transjugular-intrahepatic-portosystemic-shunt-for-portal-hypertension-in-autosomal-recessive-polycystic-kidney-disease
#1
Sarah Verbeeck, Djalila Mekhali, David Cassiman, Geert Maleux, Peter Witters
BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) with congenital hepatic fibrosis (CHF) causes portal hypertension and its complications. A transjugular intrahepatic portosystemic shunt (TIPSS) could serve as a symptomatic treatment for portal hypertension-related symptoms in these children. AIMS: To study the effect of TIPSS on portal hypertension, liver and kidney function and the long term complications. MATERIALS AND METHODS: We report on 5 children with CHF treated with a TIPSS to manage severe portal hypertension related symptoms...
March 15, 2018: Digestive and Liver Disease
https://www.readbyqxmd.com/read/29604222/biliary-atresia-clinical-and-research-challenges-for-the-21-st-century
#2
Jorge A Bezerra, Rebecca G Wells, Cara L Mack, Saul J Karpen, Jay H Hoofnagle, Edward Doo, Ronald J Sokol
Biliary atresia (BA) is a fibroinflammatory disease of the intra- and extrahepatic biliary tree. Without medical treatment, surgical hepatic portoenterosmy (HPE) may restore bile drainage, but progression of the intrahepatic disease results in complications of portal hypertension and advanced cirrhosis in most children. Recognizing that further progress in the field is unlikely without a better understanding of the underlying cause(s) and pathogenesis of the disease, the National Institutes of Diabetes and Digestive and Kidney Diseases sponsored a research workshop focused on innovative and promising approaches and on identifying future areas of research...
March 31, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29515345/adult-presentation-of-noncirrhotic-portal-hypertension-and-ascites-following-treatment-for-wilms-tumor-in-childhood
#3
Linda Kievit, Pia Kræmer, Stephen Hamilton-Dutoit, Henning Grønbæk
A 37-year-old male, who at the age of 8 years had been treated for right-sided Wilms' tumor with nephrectomy, radiotherapy, and chemotherapy, presented with noncirrhotic portal hypertension (NCPH), grade 2 esophageal varices, and ascites. A CT scan demonstrated hypoplasia of liver segments 2 and 3. A liver biopsy showed portal tract fibrosis without cirrhosis, with histological features of NCPH. Liver vein catheterization showed a normal portal pressure gradient of 5 mm Hg while spleen to hepatic vein pressure was 29 mm Hg...
January 2018: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/29470319/splenic-rupture-in-children-with-portal-hypertension
#4
Orith Waisbourd-Zinman, Amit Shah, Henry C Lin, Elizabeth B Rand
INTRODUCTION: Massive splenomegaly from portal hypertension (PHTN) in children raises the specter of splenic rupture; however, the incidence, etiology, and risk of rupture have not been studied, nor have existing practices to reduce risk. We therefore performed an international survey to describe the splenic rupture cases in PHTN and to describe the existing empirical practice among hepatologists. METHODS: A questionnaire was constructed to elicit cases of splenic rupture and collect hepatologists' common practices for prevention of splenic rupture...
March 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29464506/idiopathic-portal-hypertension-and-extrahepatic-portal-venous-obstruction
#5
REVIEW
Rajeev Khanna, Shiv Kumar Sarin
BACKGROUND: Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are the commonest presentations. AIM: The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO. RESULTS: IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions...
February 20, 2018: Hepatology International
https://www.readbyqxmd.com/read/29427256/laparoscopic-cholangiogram-in-biliary-atresia-a-refinement-in-the-gallbladder-hitch-technique
#6
Ravi Kishore, Sundeep M C Kisku, Reju Joseph Thomas, Srinivasa Kishore Jeenipalli
INTRODUCTION: The study describes a refinement in the gallbladder hitch stitch and assesses the value of the laparoscopic cholangiogram in children with suspected biliary atresia. METHODS: Twenty children with neonatal jaundice and no drainage as shown on the HIDA scan underwent a diagnostic laparoscopy through an umbilical 5 mm port. A 3 mm laparoscopic needle holder inserted through a 3.5 mm port to the left of the umbilicus was used to hitch the gallbladder to the abdominal wall...
February 9, 2018: Pediatric Surgery International
https://www.readbyqxmd.com/read/29297940/oral-mucosa-lesions-and-gingival-bleeding-can-indicate-the-progression-of-liver-disease-in-children-and-adolescents-aged-two-to-18-years
#7
Dorota Olczak-Kowalczyk, Ewa Krasuska-Sławińska, Dariusz Gozdowski, Wojciech Kowalczyk, Joanna Pawłowska
AIM: This study assessed correlations between systemic disturbances of paediatric chronic liver diseases (CLD) and oral symptoms in subjects aged 2-18 years. METHODS: It was carried out during outpatient appointments at the Children's Memorial Health Institute, Warsaw, Poland, from 2010 to 2015 and comprised 52 CLD patients with a mean age of 12.3 ± 4.6. We also recruited 54 generally healthy controls with a mean age of 12.0 ± 3.7 from the Department of Paediatric Dentistry at the Medical University of Warsaw...
January 3, 2018: Acta Paediatrica
https://www.readbyqxmd.com/read/29287004/non-invasive-markers-of-portal-hypertension-appraisal-of-adult-experience-and-potential-utilisation-in-children
#8
Harry Sutton, Anil Dhawan, Tassos Grammatikopoulos
Portal hypertension (PHT) is a significant cause of morbidity and mortality in children with chronic liver disease and portal vein obstruction. Increased portal pressure results in variceal formation along the gastrointestinal (GI) tract resulting in major bleeding. Identifying children with significant PHT who are more likely to suffer GI bleeding has been challenging and the role of surveillance upper GI endoscopy has been debated. This review analyses research done on serum biomarkers and imaging techniques as possible predictors of significant PHT...
April 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29243189/congenital-portosystemic-venous-shunt
#9
REVIEW
M Papamichail, M Pizanias, N Heaton
Congenital portosystemic venous shunts are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation and have been divided into extra- and intrahepatic shunts. They occur during liver and systemic venous vascular embryogenesis and are associated with other congenital abnormalities. They carry a higher risk of benign and malignant liver tumors and, if left untreated, can result in significant medical complications including systemic encephalopathy and pulmonary hypertension...
March 2018: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29209118/chronic-liver-disease-is-universal-in-children-with-biliary-atresia-living-with-native-liver
#10
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
#11
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
#12
Marina Rossato 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 (EV) 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...
March 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29176474/hepatic-parenchymal-injury-in-crigler-najjar-type-i
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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