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Pediatric Portal Hypertension

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https://www.readbyqxmd.com/read/29770770/adjustable-diameter-tips-in-the-pediatric-patient-the-constrained-technique
#1
Brandon C Perry, Eric J Monroe, Giridhar Shivaram
Placement of transjugular intrahepatic portosystemic shunt (TIPS) is necessary in children with portal hypertension complicated by variceal bleeding or ascites. However, placement of adult-sized endografts may be problematic due to the smaller anatomy of pediatric patients. On the other hand, placement of fixed diameter smaller stents have the corresponding problem of not accommodating future growth of the child. We describe a novel method to create an adjustable diameter TIPS as a technical solution to these problems...
May 2018: Diagnostic and Interventional Radiology: Official Journal of the Turkish Society of Radiology
https://www.readbyqxmd.com/read/29470319/splenic-rupture-in-children-with-portal-hypertension
#2
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/29377486/liver-atrophy-and-regeneration-in-noncirrhotic-portal-vein-thrombosis-effect-of-surgical-shunts
#3
Abdulrhman S Elnaggar, Adam D Griesemer, Stuart Bentley-Hibbert, Robert S Brown, Mercedes Martinez, Steven J Lobritto, Tomoaki Kato, Jean C Emond
The goal of the study is to characterize the relationship between portal vein thrombosis (PVT) and hepatic atrophy in patients without cirrhosis and the effect of various types of surgical shunts on liver regeneration and splenomegaly. Patients without cirrhosis with PVT suffer from presinusoidal portal hypertension, and often hepatic atrophy is a topic that has received little attention. We hypothesized that patients with PVT have decreased liver volumes, and shunts that preserve intrahepatic portal flow enhance liver regeneration...
January 29, 2018: Liver Transplantation
https://www.readbyqxmd.com/read/29176477/noninvasive-methods-of-predicting-large-esophageal-varices-in-children-with-intrahepatic-portal-hypertension
#4
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/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...
March 2018: Diagnostic and Interventional Imaging
https://www.readbyqxmd.com/read/29076289/orthotopic-liver-transplantation-for-sensenbrenner-syndrome
#6
Ruth Linda Ackah, Dor Yoeli, Michael Kueht, Nhu Thao Nguyen Galván, Ronald Timothy Cotton, Abbas Rana, Christine Ann O'Mahony, John Alan Goss
Sensenbrenner syndrome, or cranioectodermal dysplasia, is a rare heterogeneic autosomal recessive disorder, affecting ~1 of 1 000 000 live births. The syndrome usually manifests within the first year of life and can present with progressive liver and renal involvement. For all Sensenbrenner patients, renal and liver diseases are the main contributors of morbidity and mortality. In this report, we present the case of a 7-year-old boy with congenital liver disease progressing to liver failure secondary to Sensenbrenner syndrome...
February 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/28983709/late-complications-of-biliary-atresia-hepatopulmonary-syndrome-and-portopulmonary-hypertension
#7
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/28884036/severe-hepatopulmonary-syndrome-in-a-child-with-caroli-syndrome
#8
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
#9
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...
February 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28837442/diagnosis-follow-up-and-treatment-of-cystic-fibrosis-related-liver-disease
#10
Ivo P van de Peppel, Anna Bertolini, Johan W Jonker, Frank A J A Bodewes, Henkjan J Verkade
PURPOSE OF REVIEW: To provide an insight and overview of the challenges in the diagnosis, follow-up and treatment of cystic fibrosis-related liver disease (CFLD). RECENT FINDINGS: The variable pathophysiology of CFLD complicates its diagnosis and treatment. A 'gold standard' for CFLD diagnosis is lacking. Over the past years, new techniques to diagnose features of CFLD, such as transient elastography, have been investigated. Although most of these tests confirm cystic fibrosis-related liver involvement (CFLI), they are, however, not suitable to distinguish various phenotypical presentations or predict progression to clinically relevant cirrhosis or portal hypertension...
November 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28690995/pediatric-vascular-surgery-review-with-a-30-year-experience-in-a-tertiary-referral-center
#11
REVIEW
Seung-Kee Min, Sungsin Cho, Hyun-Young Kim, Sang Joon Kim
Pediatric vascular disease is rare, and remains a big challenge to vascular surgeons. In contrast to adults, surgery for pediatric vascular disease is complicated by issues related to small size, future growth, and availability of suitable vascular conduit. During the last 30 years, 131 major vascular operations were performed in a tertiary referral center, Seoul National University Hospital, including aortoiliac aneurysm, acute or chronic arterial occlusion, renovascular hypertension, portal venous hypertension, trauma, tumor invasion to major abdominal vessels, and others...
June 2017: Vascular Specialist International
https://www.readbyqxmd.com/read/28643816/-budd-chiari-syndrome-and-ulcerative-colitis-in-an-adolescent
#12
Federico Marchetti, Martina Mainetti, Michela Giovannini, Cristina Morelli
In patients with inflammatory bowel disease (IBD) there is an increased incidence of thromboembolic events. We report a case of a female, age 14, with a 6 months history of diarrhea with occasional presence of red blood and with a very distended abdomen with evident ascites. The diagnosis was Budd-Chiari syndrome (BCS) in ulcerative colitis (UC). Therapy with subcutaneous low molecular weight heparin, methylprednisolone, mesalazine was started. Clinical and radiological features quickly improved. No thrombophilia abnormality nor other risk factor for thrombosis were detected...
May 2017: Recenti Progressi in Medicina
https://www.readbyqxmd.com/read/28640110/case-report-of-a-modified-meso-rex-bypass-as-a-treatment-technique-for-late-onset-portal-vein-cavernous-transformation-with-portal-hypertension-after-adult-deceased-donor-liver-transplantation
#13
Dongdong Han, Rui Tang, Liang Wang, Ang Li, Xin Huang, Shan Shen, Jiahong Dong
RATIONALE: Portal vein thrombosis is a complication after liver transplantation and cavernous transformation of the portal vein (CTPV) is a result of portal vein thrombosis, with symptoms of portal hypertension revealed by an enhanced CT scan. Meso-Rex bypass is an artificial shunt connecting the left portal vein to the superior mesenteric vein and is mainly used for idiopathic cavernomas. This technique is also used for post-transplant portal vein thrombosis in pediatric patients thereby bypassing obstructed sites of the extrahepatic portal vein...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28588171/clinical-and-laboratory-predictors-of-esophageal-varices-in-children-with-chronic-liver-disease
#14
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
https://www.readbyqxmd.com/read/28583542/splenectomy-for-severe-intestinal-bleeding-caused-by-portal-hypertensive-enteropathy-after-pediatric-living-donor-liver-transplantation-a-report-of-three-cases
#15
K Tokodai, N Kawagishi, S Miyagi, C Nakanishi, Y Hara, W Nakanishi, T Kamei, N Ohuchi
BACKGROUND: The incidence of portal vein thrombosis after pediatric living-donor liver transplantation (LDLT) is reported to be higher than that after deceased-donor or adult liver transplantation. Portal vein thrombosis can cause portal hypertension and related complications, including portal hypertensive gastropathy or portal hypertensive enteropathy (PHE). PHE, in particular, can lead to severe intestinal bleeding, which is extremely difficult to treat. However, the pathogenesis of and appropriate treatment for PHE are not clearly defined, especially after pediatric LDLT...
June 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28556542/long-term-outcomes-of-de-novo-autoimmune-hepatitis-in-pediatric-liver-transplant-recipients
#16
Udeme D Ekong, Patrick McKiernan, Mercedes Martinez, Steven Lobritto, Deirdre Kelly, Vicky L Ng, Estella M Alonso, Yaron Avitzur
The long-term course and outcome of DAIH is unknown. A retrospective multicenter study assessing associations and long-term consequences of DAIH developing in a transplanted allograft is presented. Children with DAIH were followed from diagnosis until death, re-LT, or transfer of care and for a minimum of 1 year. A total of 31 patients of 1833 (1.7%) LT were identified; 29 followed for a median of 7.1 years (range, 1.6-15); 52% had no rejection preceding diagnosis of DAIH. Transaminases fell following treatment with steroids and antimetabolites (ALT 108 vs 39 U/L (P=...
September 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28494529/nash-therapy-omega-3-supplementation-vitamin-e-insulin-sensitizers-and-statin-drugs
#17
REVIEW
Stephen Caldwell
Non-alcoholic steatohepatitis (NASH) is the more aggressive form of non-alcoholic fatty liver disease (NAFLD). NASH can progress to hepatic fibrosis, cirrhosis, portal hypertension and primary liver cancer. Therapy is evolving with a substantial number of trials of promising new agents now in progress. In this article however, we will examine data for several older forms of therapy which have been fairly extensively studied over the years: Polyunsaturated Fatty Acid (PUFA) supplements, vitamin E, insulin sensitizing agents with a focus on pioglitazone and statin agents...
June 2017: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/28487619/one-step-minilaparotomy-assisted-transmesenteric-portal-vein-recanalization-combined-with-transjugular-intrahepatic-portosystemic-shunt-placement-a-novel-surgical-proposal-in-pediatrics
#18
Gloria Pelizzo, Pietro Quaretti, Lorenzo Paolo Moramarco, Riccardo Corti, Marcello Maestri, Giulio Iacob, Valeria Calcaterra
Transjugular intrahepatic portosystemic shunt (TIPS) placement is a standard procedure for the treatment of portal hypertension complications. When this conventional approach is not feasible, alternative procedures for systemic diversion of portal blood have been proposed. A one-step interventional approach, combining minilaparotomy-assisted transmesenteric (MAT) antegrade portal recanalization and TIPS, is described in an adolescent with recurrent esophageal varice bleeding and portal cavernoma (PC). A 16-year-old girl was admitted to our Unit because of repeated bleeding episodes over a short period of time due to esophageal varices in the context of a PC...
April 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28437324/noncirrhotic-portal-fibrosis-in-pediatric-population
#19
Vikrant Sood, Bikrant B Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari, Seema Alam
OBJECTIVES: Noncirrhotic portal fibrosis (NCPF) has been classically described as a disease of young to middle age with limited literature regarding its occurrence, onset, or clinical presentation in children. We hereby present a series of 19 patients diagnosed and managed as NCPF in pediatric age group. METHODS: A retrospective review of all the patients presenting to the pediatric hepatology department (age <18 years) and diagnosed as NCPF was done and data were evaluated...
May 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28429043/transsplenic-endovascular-recanalization-and-stenting-of-a-completely-occluded-portal-vein-with-jejunal-variceal-embolization-in-a-pediatric-liver-transplant-recipient
#20
Jeffrey Forris Beecham Chick, Alexandria Jo, Narasimham Dasika, Wael E Saad, Ravi Nara Srinivasa
Portal vein thrombosis occurs in 1.4% of pediatric liver transplant candidates and 3.7% of liver transplant recipients. While portal vein recanalization without and with portal vein stenting has been described in adult transplant candidates and recipients, it has never been described in the pediatric transplant population. This report presents a pediatric liver transplant recipient with portal hypertension secondary to portal vein thrombosis successfully managed with transsplenic access and subsequent portal vein recanalization and stenting...
July 2017: Pediatric Radiology
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