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portal hypertension syndrome

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https://www.readbyqxmd.com/read/29609450/hepatopulmonary-syndrome-caused-by-hypothalamic-obesity-and-nonalcoholic-fatty-liver-disease-after-surgery-for-craniopharyngioma-a-case-report
#1
Dai Jung, Go Hun Seo, Yoon-Myung Kim, Jin-Ho Choi, Han-Wook Yoo
Hypothalamic obesity is often complicated in patients with craniopharyngioma due to hypothalamic damage by the tumor itself, treatment modalities, and associated multiple pituitary hormone deficiency. Hypothalamic obesity causes secondary diseases such as nonalcoholic fatty liver disease (NAFLD) and diabetes mellitus (DM). We report a 19-year-old female who was diagnosed with craniopharyngioma, developed hypothalamic obesity after tumor resection, and progressed to hepatopulmonary syndrome. She manifested NAFLD 1 year after tumor resection...
March 2018: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/29573542/pulmonary-transit-time-derived-from-pulmonary-angiography-for-the-diagnosis-of-hepatopulmonary-syndrome
#2
He Zhao, Jiaywei Tsauo, Xiaowu Zhang, Huaiyuan Ma, Ningna Weng, Luhua Wang, Xiao Li
BACKGROUND & AIMS: Pulmonary transit time (PTT) is the transit time of blood from the right side of the heart to the left side of the heart. The aim of the present study was to evaluate the role of the PTT derived from pulmonary angiography in the diagnosis of hepatopulmonary syndrome (HPS). METHODS: From December 2014 to September 2015, all patients with chronic liver disease and/or portal hypertension undergoing a venous interventional radiologic procedure at our institution were eligible for inclusion in this prospective study...
March 24, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29552242/congestive-cirrhosis-in-osler-weber-rendu-syndrome-a-rare-case-report
#3
Patrícia Leitão, André Carvalho, Conceição Guerra, José Gonçalves, Isabel Ramos
Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu syndrome is a rare autosomal dominant vascular disorder characterized by epistaxis, mucocutaneous telangiectasias, and arteriovenous malformations affecting various organs and systems. The liver is a commonly involved organ (74% of patients with hereditary hemorrhagic telangiectasia), although symptomatic liver disease is quite infrequent. In symptomatic cases, clinical manifestations relate most commonly to the predominant type of vascular shunting present (arteriovenous, arterioportal, or portovenous)...
February 2018: Radiology Case Reports
https://www.readbyqxmd.com/read/29528017/budd-chiari-syndrome-diagnosed-in-a-patient-listed-for-liver-transplant-and-considered-to-be-contraindicated-for-the-operation
#4
Kakharman Yesmembetov, Zhansaya Muratova, Sergey Borovskiy, Irina Ten, Kulpash Kaliaskarova
We report the clinical case of 23-year-old patient with liver cirrhosis of unknown genesis, significant resistant ascites, and 2 episodes of bleeding from esophageal varices. Evaluation did not find any cause of liver disease, and the patient was placed on the transplant wait list due to subcompensated liver function (Model for End-Stage Liver Disease score of 16, Child-Pugh class B) and poorly controlled severe portal hypertension. After treatment with diuretics, largevolume paracentesis, antibiotics, and vasoconstrictors, hepatorenal syndrome and spontaneous bacterial peritonitis resolved and liver function improved significantly...
March 2018: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/29520672/features-associated-with-pulmonary-arterial-hypertension-in-chinese-hospitalized-systemic-lupus-erythematosus-patients
#5
Shu-Zhen Xu, Yan Liang, Xiang-Pei Li, Xiao-Mei Li, Zong-Wen Shuai, Rui-Xue Leng, Hai-Feng Pan, Dong-Qing Ye
Pulmonary arterial hypertension (PAH) is an increasingly recognized complication of systemic lupus erythematosus (SLE). This study aims to estimate the point prevalence of PAH and identify risk factors for PAH in a large cohort of hospitalized SLE patients. We have collected the medical records of patients hospitalized with SLE at the First Affiliated Hospital of Anhui Medical University and Anhui Provincial Hospital. Resting transthoracic echocardiography (TTE) was used to estimate pulmonary artery pressure (PAP) and PAH was defined as systolic PAP (PASP) > 30 mmHg...
March 8, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29472420/budd-chiari-syndrome-in-a-patient-with-ulcerative-colitis
#6
Gopal Krishana Bohra, Vivek Chhabra, Naresh Midha, Binit Sureka
We report a case of 26-year-old man who was admitted on our ward for the evaluation of ascites. He was a known case of inflammatory bowel disease(ulcerative colitis) and was on regular mesalamine therapy. On evaluation, he was having high serum ascites albumin gradient. CT scan of the abdomen revealed features of portal hypertension and non-visualisation of right and middle hepatic veins along with thrombus in inferior vena cava, suggesting a diagnosis of Budd-Chiari syndrome (BCS). There are only few case reports available showing association of BCS with UC...
February 22, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29404365/acute-right-ventricular-failure-after-orthotopic-liver-transplantation
#7
Rohan M Goswami, Rami N Khouzam
The interdependence between the heart and liver in maintaining hemodynamic stability during the perioperative period of either orthotopic heart (OHT) or liver (OLT) transplantation is important. The pre-transplant hemodynamic changes that occur in patients with end-stage liver disease (ESLD) can include decreased systemic vascular resistance, poor ventricular response to stress and increased cardiac output (CO). Concomitant pulmonary disorders are often present in ESLD. Portopulmonary hypertension (PoPHTN) is an important marker for increased mortality in liver transplant patients...
January 2018: Annals of Translational Medicine
https://www.readbyqxmd.com/read/29362613/protective-effects-of-the-third-generation-vasodilatory-%C3%AE-eta-blocker-nebivolol-against-d-galactosamine-induced-hepatorenal-syndrome-in-rats
#8
Ahmed Atwa, Rehab Hegazy, Rania Mohsen, Neamat Yassin, Sanaa Kenawy
BACKGROUND: Renal dysfunction is very common in patients with advanced liver cirrhosis and portal hypertension. The development of renal failure in the absence of clinical, anatomical or pathological causes renal of failure is termed hepatorenal syndrome (HRS). AIM: The present study was constructed to investigate the possible protective effects of nebivolol (Nebi) against D-galactosamine (Gal)-induced HRS in rats. MATERIAL AND METHODS: Rats were treated with Nebi for ten successive days...
December 15, 2017: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29340275/timing-of-transjugular-intrahepatic-portosystemic-shunt-for-budd-chiari-syndrome-an-italian-hepatologist-s-perspective
#9
Andrea Mancuso
Budd-Chiari syndrome (BCS) management flow-chart is derived from experts' opinion and is not evidence-based. Guidelines suggest BCS management should follow a stepwise strategy: medical therapy as first-line treatment, revascularization or transjugular intrahepatic portosystemic shunt (TIPS) if no response to medical therapy, and liver transplant as rescue therapy. Recent evidence suggests that only medical therapy results in a bad long-term outcome. The biggest criticism of guidelines is the indication that BCS should receive further treatment only when hemodynamic consequences of portal hypertension become clinically evident...
December 2017: Journal of Translational Internal Medicine
https://www.readbyqxmd.com/read/29319259/alstr%C3%A3-m-syndrome-with-portal-hypertension
#10
Ameya S Joshi, Ashok R Mohite, Premlata K Varthakavi, Pravin M Rathi
Alstrom syndrome is an autosomal recessive multisystem disorder caused by mutation in ALMS1 (2p13). Very few cases of same are reported so far of same. We report a case of Alstrom syndrome (AS) who presented with type II diabetes mellitus and portal hypertension. Unilateral anorchia with hypergonadotropic hypogonadism is another unique feature of our case.
October 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29318565/a-case-of-severe-stenosis-of-hepatic-veins-and-inferior-vena-cava-with-stomal-variceal-bleeding-induced-by-oxaliplatin-based-chemotherapy
#11
Hayato Yamaguchi, Yoshihiro Furuichi, Yoshitaka Kasai, Hirohito Takeuchi, Yuu Yoshimasu, Katsutoshi Sugimoto, Ikuo Nakamura, Takao Itoi
A 27-year-old woman with colon cancer and liver metastasis was referred to our hospital. Colectomy and colostomy were performed to improve her ileus. Following 13 sessions of oxaliplatin-based chemotherapy (OC) with mFOLFOX6 + bevacizumab, thrombocytopenia and frequent peristomal bleeding occurred. Computed tomography showed severe ascites, splenomegaly, significant collateral veins around the stoma, and severe stenosis of the hepatic veins (HV) and inferior vena cava (IVC). Ultrasound elastography showed high liver (and spleen) stiffness values...
April 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29316565/transjugular-intrahepatic-portosystemic-shunt-in-patients-with-portal-hypertension-patency-depends-on-coverage-and-interventionalist-s-experience
#12
Matthias Buechter, Paul Manka, Guido Gerken, Ali Canbay, Sandra Blomeyer, Axel Wetter, Jens Altenbernd, Alisan Kahraman, Jens M Theysohn
BACKGROUND AND AIMS: Transjugular intrahepatic portosystemic shunt (TIPS) is the treatment of choice in decompensated portal hypertension. TIPS revision due to thrombosis or stenosis increases morbidity and mortality. Our aim was to investigate patient- and procedure-associated risk factors for TIPS-revision. PATIENTS AND METHODS: We retrospectively evaluated 189 patients who underwent the TIPS procedure. Only patients who required TIPS revision within 1 year (Group I, 34 patients) and patients who did not require re-intervention within the first year (Group II [control group], 54 patients) were included...
2018: Digestive Diseases
https://www.readbyqxmd.com/read/29290508/the-emergency-medicine-evaluation-and-management-of-the-patient-with-cirrhosis
#13
REVIEW
Brit Long, Alex Koyfman
BACKGROUND: Cirrhosis is a significant cause of death in the U.S. and has a variety of causes, most commonly Hepatitis C and alcohol. Liver fibrosis and nodule formation result in significant complications due to portal system hypertension. There are several deadly complications emergency physicians must consider. OBJECTIVE OF THE REVIEW: Provide an evidence-based update for the resuscitation of decompensating cirrhotic patients and an overview of cirrhosis complications...
December 23, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29229261/preliminary-experience-in-combined-somatic-and-cerebral-oximetry-monitoring-in-liver-transplantation
#14
Tina Hu, Yves Collin, Réal Lapointe, François Martin Carrier, Luc Massicotte, Annik Fortier, Jean Lambert, Franck Vandenbroucke-Menu, André Y Denault
OBJECTIVE: The use of cerebral near-infrared spectroscopy (NIRS) has become widespread in cardiac surgery after research demonstrated an association between perioperative cerebral desaturations and postoperative complications. Somatic NIRS desaturation also is associated with an increased risk of postoperative complications and mortality. The objective of this study was to explore the trends of both somatic and cerebral NIRS during liver transplantation. DESIGN: A prospective, single-site, observational case series...
February 2018: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29211374/-splanchnic-vein-thrombosis
#15
Bobby Gouin, Helia Robert-Ebadi, Alessandro Casini, Yan Beauverd, Pierre Fontana, Marc Righini, Laure Elkrief
Splanchnic vein thromboses include thrombosis of the portal venous system (including the portal, mesenteric and splenic vein) and hepatic vein thrombosis (also called Budd-Chiari syndrome). They are rare manifestations of venous thromboembolism. These thromboses are frequently associated with local or systemic factors. The therapeutic approach is often complex due to heterogeneity of patients and limited available data in the literature. The cornerstone of treatment is anticoagulation. However, the bleeding risk, related to portal hypertension, should be accurately assessed to individualize the treatment...
December 6, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/29209118/chronic-liver-disease-is-universal-in-children-with-biliary-atresia-living-with-native-liver
#16
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/29176474/hepatic-parenchymal-injury-in-crigler-najjar-type-i
#17
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/29124669/shunt-occlusion-for-portosystemic-shunt-syndrome-related-refractory-hepatic-encephalopathy-a-single-center-experience-in-21-patients-from-kerala
#18
Cyriac Abby Philips, Lijesh Kumar, Philip Augustine
INTRODUCTION: Large spontaneous portosystemic shunts (SPSS) are seen in a subset of patients with liver disease and medically refractory recurrent/persistent hepatic encephalopathy (MRHE). Shunt occlusion has been shown to improve clinical outcomes. METHODS: We retrospectively analyzed patient characteristics, SPSS attributes, procedural features, baseline clinical and investigational parameters, neurological outcomes, adverse effects (procedure and portal hypertension related), and risk factors predicting outcomes in liver disease patients undergoing shunt occlusion procedure for MRHE...
September 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/29112083/characteristics-of-liver-disease-in-100-individuals-with-joubert-syndrome-prospectively-evaluated-at-a-single-center
#19
Anna Strongin, Theo Heller, Dan Doherty, Ian A Glass, Melissa A Parisi, Joy Bryant, Peter Choyke, Baris Turkbey, Kailash Daryanani, Deniz Yildirimli, Meghana Vemulapalli, Jim C Mullikin, May C Malicdan, Thierry Vilboux, William A Gahl, Meral Gunay-Aygun
BACKGROUND AND AIMS: Joubert Syndrome (JS) is a rare, inherited, ciliopathy defined by cerebellar and brainstem malformations and is variably associated with liver, kidney, and ocular dysfunction. This study characterizes the hepatic findings in JS and identifies factors associated with probable portal hypertension. METHODS: Hundred individuals with JS were prospectively evaluated at the National Institutes of Health Clinical Center. Laboratory tests, imaging, and DNA sequencing were performed...
March 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29076289/orthotopic-liver-transplantation-for-sensenbrenner-syndrome
#20
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...
October 26, 2017: Pediatric Transplantation
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