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Liver shunt children

Toshiharu Matsuura, Yoshiaki Takahashi, Yusuke Yanagi, Koichiro Yoshimaru, Kenichiro Yamamura, Eiji Morihana, Hazumu Nagata, Kiyoshi Uike, Hidetoshi Takada, Tomoaki Taguchi
BACKGROUND: Congenital portosystemic shunts (CPSS) with intrahepatic portal vein (IHPV) hypoplasia or absence cause encephalopathy or pulmonary hypertension (PH). Acute shunt closure may result in postoperative portal hypertension. The aim of this study was to propose a surgical strategy according to the anatomical types of CPSS and IHPV. METHODS: Twenty-three CPSS patients were diagnosed from1990 to 2015. All patients were evaluated by computed tomography, angiography, and PV pressure monitoring under a shunt occlusion test...
September 21, 2016: Journal of Pediatric Surgery
Sofía Verdaguer D, Juan Cristóbal Gana A
BACKGROUND: There is a paucity of good quality research about the diagnosis of esophageal varices and the prophylaxis and treatment of variceal bleeding in pediatric patients with portal hypertension There is little consensus and practically no evidence-based approach about the management of these patients. AIM: To describe the behavior and preferences of pediatric gastroenterologists in Chile in the management of portal hypertension in children. MATERIAL AND METHODS: An online survey was sent to Chilean pediatric gastroenterologists, with questions evaluating the physicians’ approaches to screening of esophageal varices in children with portal hypertension, and their preferred methods of prophylaxis and initial management of variceal bleeding...
July 2016: Revista Médica de Chile
Oliver C Burdall, Tassos Grammatikopoulos, Maria Sellars, Nedim Hadzic, Mark Davenport
A link between congenital vascular malformation (CVM) of the liver and Trisomy 21 has been suggested. We reviewed all children with Trisomy 21 referred for investigation to a specialist paediatric hepatobiliary unit (1985-2015).Forty-five children with Trisomy 21 were identified; 7 (15%) had a defined CVMs (4 female). All such infants were also diagnosed with a range of cardiac defects. CVMs were divided according to the nature of the vascular connection.Group (i) (n = 3) Abnormal veno-venous anomaly. This included porto-caval shunt and patent ductus venosus (n = 2)...
September 6, 2016: Journal of Pediatric Gastroenterology and Nutrition
Arit Parkash, Huma Arshad Cheema, Hassan Suleman Malik, Zafar Fayyaz
OBJECTIVE: To describe the clinical presentations, laboratory features and management of congenital hepatic fibrosis patients at a tertiary care hospital. METHODS: The case series was conducted at The Children Hospital and Institute of Child Health, Lahore, Pakistan, from July 2013 to June 2015, and comprised patients of congenital hepatic fibrosis diagnosed on the basis of liver biopsy. SPSS 20 was used for statistical analysis. RESULTS: The mean age of 25 patients in the study was 8...
August 2016: JPMA. the Journal of the Pakistan Medical Association
Hajime Uchida, Akinari Fukuda, Kengo Sasaki, Yoshihiro Hirata, Takanobu Shigeta, Hiroyuki Kanazawa, Atsuko Nakazawa, Osamu Miyazaki, Shunsuke Nosaka, Vidyadhar Padmakar Mali, Seisuke Sakamoto, Mureo Kasahara
BACKGROUND: Hepatoblastoma (HB) is a highly malignant primary liver tumor in children. Although liver transplantation (LT) is an effective treatment for unresectable HB with good long-term outcomes, post-transplant survival is mainly affected by recurrence, despite adjuvant chemotherapy. Novel strategies are needed to improve the outcomes in patients undergoing LT for unresectable HB. PATIENTS AND METHODS: Twelve children received LT for unresectable HB. In 9 patients, we applied early exclusion of hepatic inflow (hepatic artery and portal vein) and creation of a temporary portocaval shunt during LT...
May 31, 2016: Journal of Pediatric Surgery
Toshiharu Matsuura, Koichiro Yoshimaru, Yusuke Yanagi, Genshiro Esumi, Makoto Hayashida, Tomoaki Taguchi
BACKGROUND Liver cirrhosis is frequently accompanied by insufficient portal vein inflow (IPVF) with large portosystemic shunts. However, pediatric cases often manifested IPVF without any apparent major portosystemic shunts. Although IPVF is a very critical issue, the intraoperative assessment has not been well established. In this study, we reviewed the intraoperative approach and the outcome of the IPVF cases at our department. MATERIAL AND METHODS Eighty-three living donor liver transplantations (LDLT) were performed from 1996 to 2014...
2016: Annals of Transplantation: Quarterly of the Polish Transplantation Society
Michail Papamichail, Amir Ali, Alberto Quaglia, John Karani, Nigel Heaton
Intrahepatic portosystemic shunts (IPSS) are rare congenital anomalies arising from disordered portal vein embryogenesis. It has been described in both children and adults and may be asymptomatic or be associated with a variety of neurophysiological and pulmonary complications. When recognized, early intervention to occlude the shunt will reverse the associated complications. Literature review reports of surgical and radiological occlusion of the shunt, but due to its rarity, a standard therapeutic protocol has not been established...
June 2016: Hepatobiliary & Pancreatic Diseases International: HBPD INT
Ashish Bavdekar, Nitin Thakur
Ascites is an accumulation of serous fluid within the peritoneal cavity. It is the most common complication of liver cirrhosis. In children, hepatic, renal and cardiac disorders are the most common causes. Portal hypertension and sodium and fluid retention are key factors in the pathophysiology of ascites. Peripheral arterial vasodilatation hypothesis is the most accepted mechanism for inappropriate sodium retention and formation of ascites. Diagnostic paracentesis is indicated in children with newly diagnosed ascites and in children with suspected complications of ascites...
June 9, 2016: Indian Journal of Pediatrics
Varun K Sharma, Prajakta R Ranade, Shaji Marar, Fazal Nabi, Aabha Nagral
OBJECTIVES: Budd-Chiari syndrome (BCS) is an uncommon cause of chronic liver disease in children. The literature on the management of pediatric BCS is scarce. Our aim was to determine the long-term outcome of patients undergoing a radiological intervention for the treatment of BCS. METHODS: Thirty-two children diagnosed with BCS between 2004 and 2014 were included. Data on the course of disease, medical management, response, and complications related to radiological interventions and outcome were collected...
May 2016: European Journal of Gastroenterology & Hepatology
Jung-Bin Park, In-Kyung Song, Ji-Hyun Lee, Eun-Hee Kim, Hee-Soo Kim, Jin-Tae Kim
OBJECTIVES: Few studies have examined cardiopulmonary resuscitation for patients with congenital heart disease, although they are at a high risk of cardiac arrest. Therefore, this study investigated the optimal chest compression position in patients with a single ventricle while providing them with basic life support. DESIGN: This is a retrospective study of patients with a single ventricle who are undergoing chest CT. SETTING: Tertiary teaching children's hospital...
April 2016: Pediatric Critical Care Medicine
Lin Wu, Lu Zhao, Ying Lu, Lan He, Xihong Hu
BACKGROUND: Congenital intrahepatic shunts in children are rare and precise treatment strategies have not been established. OBJECTIVE: The purpose of this study was to present our experience with transcatheter embolization in a descriptive case series of congenital intrahepatic shunts in children. MATERIALS AND METHODS: We retrospectively studied 10 children with symptomatic congenital intrahepatic shunts who were treated with embolization at our institute between January 2008 and December 2014...
April 2016: Pediatric Radiology
Gloria Chocarro, María Virginia Amesty, Jose Luis Encinas, Alejandra Vilanova Sánchez, Francisco Hernandez, Ane M Andres, Manolo Gamez, Juan Antonio Tovar, Manuel Lopez Santamaria
INTRODUCTION: Congenital portosystemic shunt (CPSS) is a rare entity without insufficiency in treatment issues. The aim of this article is to show our experience in the heterogeneity of this condition. MATERIAL AND METHODS: A retrospective study of 25 CPSS in the period 1995 to 2014 was conducted. Description of the morphology, clinical impact, and treatment is given. RESULTS: According to the imaging techniques (IT), the shunt was apparently intrahepatic in 14 patients, extrahepatic in 10 patients, and mixed in 1 patient...
February 2016: European Journal of Pediatric Surgery
Gloria Chocarro, Paloma Triana Junco, Eva Dominguez, María Virginia Amesty, Vanesa Nuñez Cerezo, Francisco Hernandez, Javier Murcia, Juan Antonio Tovar, Manuel Lopez Santamaria
OBJECTIVE: The mesoportal shunt (MPS) and liver transplantation (LT) have changed the scenario of extrahepatic portal vein obstruction (EHPVO) since the MPS, the only "curative" technique, can now be offered in asymptomatic patients and also thrombotic complications of LT have increased the incidence of EHPVO. MATERIAL AND METHODS: A retrospective study of patients undergoing surgery for EHPVO was conducted between 1990 and 2015. An analysis was done for the shunt permeability and clinical evolution over time...
February 2016: European Journal of Pediatric Surgery
Erin R Lane, Evelyn K Hsu, Karen F Murray
Ascites is the pathologic accumulation of fluid within the peritoneal cavity. There are many causes of fetal, neonatal and pediatric ascites; however, chronic liver disease and subsequent cirrhosis remain the most common. The medical and surgical management of ascites in children is dependent on targeting the underlying etiology. Broad categories of management strategies include: sodium restriction, diuresis, paracentesis, intravenous albumin, prevention and treatment of infection, surgical and endovascular shunts and liver transplantation...
2015: Expert Review of Gastroenterology & Hepatology
Emmanuelle Ecochard-Dugelay, Virginie Lambert, Jean-Marc Schleich, Mathieu Duché, Emmanuel Jacquemin, Olivier Bernard
OBJECTIVE: Portopulmonary hypertension (POPH) is a known complication of cirrhosis in adults, but there is little information on its incidence and outcome in children with liver disease. We report 14 patients with POPH and present a synthesis of the medical literature. METHODS: Diagnosis of POPH in the 14 patients was based on right-sided heart catheterization displaying mean pulmonary artery pressure (mPAP) >25 mmHg, indexed pulmonary vascular resistances >3 Wood units · m, and pulmonary wedge pressure <15 mmHg...
September 2015: Journal of Pediatric Gastroenterology and Nutrition
S Jayakumar, A Odulaja, S Patel, M Davenport, N Ade-Ajayi
AIM OF THE STUDY: To report the outcomes of children who underwent Sengstaken-Blakemore tube (SBT) insertion for life-threatening haemetemesis. METHODS: Single institution retrospective review (1997-2012) of children managed with SBT insertion. Patient demographics, diagnosis and outcomes were noted. Data are expressed as median (range). MAIN RESULTS: 19 children [10 male, age 1 (0.4-16) yr] were identified; 18 had gastro-oesophageal varices and 1 aorto-oesophageal fistula...
July 2015: Journal of Pediatric Surgery
Serpil Bas, Tulay Guran, Zeynep Atay, Belma Haliloglu, Saygın Abalı, Serap Turan, Abdullah Bereket
Congenital portosystemic shunt (CPSS) is persistence of an anomalous embryological connection of the portal vein with a large vein of the vena cava system. Clinical presentations include neonatal cholestasis, liver tumors, and encephalopathy, but can be variable in timing and symptomatology. We report 2 girls who presented 10 years apart with the same complaint of early pubarche at age 7 years, with inappropriately low DHEAS levels. In addition to hyperandrogenemia (elevated testosterone and androstenedione) and advanced bone age, both had hyperinsulinemia, and hypothyroxinemia...
2015: Hormone Research in Pædiatrics
Lisa M Guay-Woodford
Autosomal recessive polycystic kidney disease (ARPKD) is a severe, typically early onset form of renal cystic disease. The care of ARPKD patients has traditionally been the purview of pediatric nephrologists for management of systemic hypertension and progressive renal insufficiency. However, the disease has multisystem manifestations and a comprehensive care strategy frequently requires a multidisciplinary team. In severely affected infants, the diagnosis often is first suspected by obstetricians when enlarged, echogenic kidneys and oligohydramnios are detected on prenatal ultrasounds...
2014: Journal of Pediatric Genetics
Duygu Erge, Mukadder Ayşe Selimoğlu, Cemşit Karakurt, Hamza Karabiber, Özlem Elkıran, Ferat Çatal, Reyhan Koroğlu
Hepatopulmonary syndrome is an important pulmonary vascular complication of liver disease. Its diagnosis is based on the presence of hypoxaemia and the demonstration of intrapulmonary shunting by contrast-enhanced echocardiography or perfusion lung scanning. Awareness of this condition is critical to improve the outcomes of patients with chronic liver disease and/or portal hypertension because hepatopulmonary syndrome receives additional priority on the waiting list for transplantation. A non-invasive measurement of the blood oxygen saturation with pulse oximetry is recommended as a screening tool for this syndrome...
December 2014: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
Vibhor V Borkar, Ujjal Poddar, Aditya Kapoor, Srivatsa Ns, Anshu Srivastava, Surender K Yachha
BACKGROUND & AIMS: Hepatopulmonary syndrome in children has not been extensively studied particularly in different subsets of portal hypertension. We prospectively studied hepatopulmonary syndrome in children with cirrhosis and extrahepatic portal venous obstruction. Their comparison might shed light on the pathogenesis of hepatopulmonary syndrome. METHODS: Between 1 to 18 years of age, 135 children with portal hypertension (cirrhosis 35, extrahepatic portal venous obstruction 100) were studied...
June 2015: Liver International: Official Journal of the International Association for the Study of the Liver
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