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hypersplenism

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https://www.readbyqxmd.com/read/28402911/hassab-s-operation-for-joubert-syndrome-with-congenital-hepatic-fibrosis-a-case-report
#1
Koji Miyazawa, Yasuyuki Hara, Kenji Shimizu, Wataru Nakanishi, Kazuaki Tokodai, Chikashi Nakanishi, Shigehito Miyagi, Naoki Kawagishi, Noriaki Ohuchi
INTRODUCTION: Joubert syndrome is characterized by psychomotor developmental delay, hypotonia, oculomotor abnormalities, occasional retinal dystrophy and cystic kidneys, and frequent and often, striking breathing abnormalities, especially in the neonatal period, with panting tachypnea followed by apnea. We report a case of Joubert syndrome with hepatic fibrosis, portal hypertension, and pancytopenia treated by Hassab's operation. PRESENTATION OF CASE: Our patient was a 27-year-old woman with a history of tachypnea, muscle hypotonia, and psychomotor retardation shortly after birth and a diagnosis of Joubert syndrome at 2 years of age...
March 28, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28357095/hepatectomy-combined-with-microwave-ablation-of-the-spleen-for-treatment-of-hepatocellular-carcinoma-complicated-with-splenomegaly-a-retrospective-study
#2
Jian-Bo Han, Feng-Wei Kong, Hai Ding, Yu-Feng Zhang, Jun-Mao Liu, Qiang Wei, Liang Hu, Liang Zhao, Chuan-Jun Xu, Yong-Xiang Yi
The present retrospective study aimed to investigate the mid-term safety and efficacy of hepatectomy combined with microwave ablation of the partial spleen for treatment of liver cancer complicated with hypersplenism. A retrospective analysis was performed on 23 patients who underwent hepatectomy combined with microwave ablation of the partial spleen for liver cancer, complicated with hypersplenism that was secondary to cirrhosis. The splenic and ablated volumes were calculated according to a contrast-enhanced computed tomography scan prior to and 2 weeks after the operation...
February 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28352148/laparoscopic-and-open-splenectomy-and-hepatectomy
#3
Jing-Feng Li, Dou-Sheng Bai, Guo-Qing Jiang, Ping Chen, Sheng-Jie Jin, Zhi-Xian Zhu
BACKGROUND AND OBJECTIVES: Patients undergoing synchronous open splenectomy and hepatectomy (OSH) for concurrent hepatocellular carcinoma (HCC) and hypersplenism usually have major surgical trauma caused by the long abdominal incision. Surgical procedures that contribute to rapid recovery with the least possible impairment are desired by both surgeons and patients. The objective of this study was to explore outcomes in patients treated with simultaneous laparoscopic or open splenectomy and hepatectomy for hepatocellular carcinoma (HCC) with hypersplenism...
January 2017: JSLS: Journal of the Society of Laparoendoscopic Surgeons
https://www.readbyqxmd.com/read/28316312/partial-splenic-artery-embolization-to-treat-hypersplenism-secondary-to-hepatic-cirrhosis-a-meta-analysis
#4
Yun-Bing Wang, Jian-Ying Zhang, Fan Zhang, Yong Zhao, Jian-Ping Gong
This meta-analysis aimed to explore the effectiveness of partial spleen arterial embolization (PSAE) in the treatment of hypersplenism secondary to hepatic cirrhosis. PubMed, Embase, Cochrane Library, Wan Fang, CNKI, Vip, and CBM databases were searched for randomized controlled trials, cohort studies, and case-control studies that compared PSAE with splenectomy in the treatment of hypersplenism secondary to hepatic cirrhosis from their inception to July 25, 2015. Statistical analysis was conducted in Cochrane Network RevMan v5...
March 1, 2017: American Surgeon
https://www.readbyqxmd.com/read/28299974/causes-of-peripheral-cytopenia-in-hepatitic-cirrhosis-and-portal-hypertensive-splenomegaly
#5
Yunfu Lv, Wan Yee Lau, Hongfei Wu, XiaoYu Han, Xiaoguang Gong, Ning Liu, Jie Yue, Qingqing Li, YeJuan Li, Jie Deng
The clinical data of 183 patients with hepatitic cirrhosis and portal hypertensive splenomegaly complicated by peripheral cytopenia were retrospectively analyzed to investigate the causes of peripheral cytopenia, as well as the proportion of the causes in these patients. All patients underwent splenectomy. Before operation, these patients had one or more types of peripheral cytopenia (cumulative cytopenia: 390 patient-times). After splenectomy, blood counts in 79.2% (309/390) returned to normal, while in 15...
April 2017: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28293407/thrombocytopenia-in-patients-with-chronic-hepatitis-c-virus-infection
#6
REVIEW
Sumit Dahal, Smrity Upadhyay, Rashmi Banjade, Prajwal Dhakal, Nabin Khanal, Vijaya Raj Bhatt
Thrombocytopenia in patients with chronic hepatitis C virus (HCV) infection is a major problem. The pathophysiology is multifactorial, with auto-immunogenicity, direct bone marrow suppression, hypersplenism, decreased production of thrombopoietin and therapeutic adverse effect all contributing to thrombocytopenia in different measures. The greatest challenge in the care of chronic HCV patients with thrombocytopenia is the difficulty in initiating or maintaining IFN containing anti-viral therapy. Although at present, it is possible to avoid this challenge with the use of the sole Direct Antiviral Agents (DAAs) as the primary treatment modality, thrombocytopenia remains of particular interest, especially in cases of advanced liver disease...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28256406/microwave-ablation-in-the-spleen-versus-partial-splenic-artery-embolisation-a-new-technique-for-hypersplenism-in-cirrhosis
#7
Fathia Assal, Mohamed El Kassas, Eslam Esmail, Amr A Elbadry, Sabry Abousaif, Reem Mahdy, Asem Elfert
BACKGROUND AND STUDY AIMS: Splenectomy has been associated with high perioperative morbidity and mortality. Partial splenic artery embolisation (PSE) was found to be an effective and safer method but with numerous complications. Local thermal ablative techniques such as microwave ablation (MWA) have been tried and were found to be safe and effective alternatives. This randomised controlled study aimed to compare the efficacy and safety of MWA versus PSE in the treatment of hypersplenism in patients with post-hepatitis C cirrhosis...
February 28, 2017: Arab Journal of Gastroenterology: the Official Publication of the Pan-Arab Association of Gastroenterology
https://www.readbyqxmd.com/read/28191814/the-pathophysiologic-basis-of-anaemia-in-patients-with-malignant-diseases
#8
Umma A Ibrahim, Aminu A Yusuf, Sagir G Ahmed
Cancer patients frequently present with anaemia that may result from the direct or indirect effects of the tumor or its treatment. Anaemia is an independent adverse prognostic factor that exerts negative influence on quality of life and survival of cancer patients. Anaemia in malignant disorders often arises from an interplay of multiple aetiological and pathophysiologic mechanisms. Understanding these mechanisms will help the oncologist identify and treat specific causes of the anaemia thereby minimizing the use of blood transfusion, which is associated with many adverse effects...
September 2016: Gulf Journal of Oncology
https://www.readbyqxmd.com/read/28135891/successful-treatment-of-hypersplenism-in-wilson-s-disease-by-partial-splenic-embolization
#9
Liang-Yong Li, Huai-Zhen Chen, Yuan-Cheng Bao, Qing-Sheng Yu, Wen-Ming Yang
AIM: Hypersplenism can occur in patients with Wilson's disease (WD). Surgical splenectomy is a conventional treatment for this condition; however, emotional and neurological deterioration may follow splenectomy. In recent years, partial splenic embolization (PSE) has been increasingly performed as a nonsurgical alternative treatment for hypersplenism. The aim of this study was to evaluate the effectiveness and safety of PSE compared with splenectomy in the treatment of hypersplenism in WD patients...
January 31, 2017: Journal of Investigative Surgery: the Official Journal of the Academy of Surgical Research
https://www.readbyqxmd.com/read/28127481/severe-thrombocytopenic-purpura-in-a-child-with-brucellosis-case-presentation-and-review-of-the-literature
#10
Alexandros Makis, Aikaterini Perogiannaki, Nikolaos Chaliasos
Brucellosis is still endemic and a significant public health problem in many Mediterranean countries, including Greece. It is a multisystemic disease with a broad spectrum of clinical manifestations including hematological disorders, such as anemia, pancytopenia, leucopenia, and thrombocytopenia. Thrombocytopenia is usually moderate and attributed to bone marrow suppression or hypersplenism. Rarely, autoimmune stimulation can cause severe thrombocytopenia with clinically significant hemorrhagic manifestations...
2017: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/28035311/primary-splenic-lymphoma-current-diagnostic-trends
#11
EDITORIAL
Sachin B Ingle, Chitra R Hinge Ingle
The primary splenic lymphoma is extremely uncommon, can present with grave complications like hypersplenism and splenic rupture. In view of vague clinical presentation, it is difficult to arrive at the diagnosis. In such circumstances, histopathological diagnosis is very important. A precise diagnosis can only be made on histopathology and confirmed on immunohistochemistry.Emergency splenectomy is preferred as an effective therapeutic and diagnostic tool in cases with giant splenomegaly. Core biopsy is usually not advised due to a high risk of post-core biopsy complications in view of its high vascularity and fragility...
December 16, 2016: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/28031104/hepatocellular-carcinoma-with-hypersplenic-thrombocytopenia-and-situs-inversus-totalis-a-case-report
#12
Jian-Bo Xu, Gang Xu, Guo-Feng Chen, Dian-Hua Gu, Jian-Huai Zhang, Fu-Zhen Qi
No abstract text is available yet for this article.
June 20, 2016: Chinese Medical Sciences Journal, Chung-kuo i Hsüeh K'o Hsüeh Tsa Chih
https://www.readbyqxmd.com/read/27931577/liver-transplantation-in-polish-children-with-%C3%AE-1-antitrypsin-deficiency-a-single-center-experience
#13
A Bakula, J Pawlowska, O Niewiadomska, I Jankowska, M Teisseyre, P Kaliciński, P Socha
BACKGROUND: α1-Antitrypsin deficiency (ATD) is the most common genetic cause of liver injury in young children. Asymptomatic hepatitis is observed in most patients. However, the course of liver disease due to ATD is unpredictable, and some children develop liver cirrhosis. Liver transplantation (Ltx) dramatically improves their outcome and in some cases is required in the first years of life. The aim of the study was to evaluate the course of the disease in children with ATD treated with Ltx in a single center...
December 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27920483/outcomes-of-partial-splenic-embolization-in-patients-with-massive-splenomegaly-due-to-idiopathic-portal-hypertension
#14
Omer Ozturk, Gonca Eldem, Bora Peynircioglu, Taylan Kav, Aysegul Görmez, Barbaros Erhan Cil, Ferhun Balkancı, Cenk Sokmensuer, Yusuf Bayraktar
AIM: To determine the outcomes of partial splenic embolization (PSE) for massive splenomegaly due to idiopathic portal hypertension (IPH). METHODS: In this prospective study, we evaluated the characteristics and prognosis of consecutive patients with IPH who underwent PSE for all indications at a single medical center between June 2009 and January 2015. The inclusion criteria were: presence of hypersplenism, massive splenomegaly, and resultant pancytopenia. The exclusion criteria were: presence of other diseases causing portal hypertension...
November 21, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27906797/non-cirrhotic-portal-fibrosis-in-pediatric-population
#15
Vikrant Sood, Bikrant Bihari Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari Sharma, Seema Alam
BACKGROUND: Non-cirrhotic 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 was evaluated...
November 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27898169/cross-sectional-analysis-of-progressive-familial-intrahepatic-cholestasis-in-puerto-rican-children
#16
Dellys M Soler, Antonio I Del Valle, David Fernandez-Lube, Benjamin L Shneider
OBJECTIVE: Specific inherited disorders may be more common in island communities. Prior case reports suggest that cholestatic liver diseases may constitute a group of these inherited disorders in Puerto Rico. A cross-sectional survey of liver diseases in children was conducted to assess this hypothesis. METHODS: A cross-sectional analysis was performed in patients with chronic cholestasis at "Hospital Pediátrico Universitario" in San Juan, Puerto Rico. Ten potential participants with high gamma-glutamyl transpeptidase (GGTP) cholestasis were identified...
December 2016: Puerto Rico Health Sciences Journal
https://www.readbyqxmd.com/read/27896406/evaluation-of-rex-shunt-on-cavernous-transformation-of-the-portal-vein-in-children
#17
Ruo-Yi Wang, Jun-Feng Wang, Xiao-Gang Sun, Qian Liu, Jia-Long Xu, Qi-Gang Lv, Wei-Xiu Chen, Jin-Liang Li
BACKGROUND: Children with cavernous transformation of the portal vein (CTPV) develop severe complications from prehepatic portal hypertension, such as recurrent variceal bleeding and thrombocytopenia. In this study, we reported the results of 30 children with symptomatic CTPV that were treated by a Rex shunt. The effectiveness of this surgical approach was evaluated. METHODS: A retrospective review was performed of 30 children aged between 3 and 18 years with CTPV, who underwent a Rex shunt between 2008 and 2015...
April 2017: World Journal of Surgery
https://www.readbyqxmd.com/read/27860293/thrombocytopenia-in-chronic-liver-disease
#18
REVIEW
Markus Peck-Radosavljevic
Thrombocytopenia is a common haematological disorder in patients with chronic liver disease. It is multifactorial and severity of liver disease is the most influential factor. As a result of the increased risk of bleeding, thrombocytopenia may impact upon medical procedures, such as surgery or liver biopsy. The pathophysiology of thrombocytopenia in chronic liver disease has long been associated with the hypothesis of hypersplenism, where portal hypertension causes pooling and sequestration of all corpuscular elements of the blood, predominantly thrombocytes, in the enlarged and congested spleen...
November 17, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27803822/pancytopenia-in-a-patient-with-rendu-osler-weber-syndrome-and-uncommon-vascular-abnormalities
#19
Nicolò Binello, Antonio Gasbarrini, Eleonora Gaetani
Rendu-Osler-Weber syndrome, or hereditary hemorrhagic teleangiectasia (HHT), is a rare autosomal dominant vascular disorder, characterized by multiple mucocutaneous teleangiectases with recurrent nasal and gastrointestinal bleedings and/or solid-organ arteriovenous shunts. We describe the first case to our knowledge of pancytopenia in a 53-year-old patient, with a known history of HHT and recurrent nasal and gastrointestinal bleedings, who was found to have a major splenic artery aneurysm and other uncommon vascular abnormalities...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27757214/positive-impact-of-eculizumab-therapy-on-surgery-for-budd-chiari-syndrome-in-a-patient-with-paroxysmal-nocturnal-hemoglobinuria-and-a-long-term-history-of-thrombosis
#20
Silvia De-la-Iglesia, Hugo Luzardo, Angelina Lemes, Melissa Torres, Maria Teresa Gómez-Casares, Naylen Cruz, Teresa Molero
Paroxysmal nocturnal hemoglobinuria (PNH) is associated with severe end-organ damage and a high risk of thrombosis. Budd-Chiari syndrome, which develops after thrombotic occlusion of major hepatic blood vessels, is relatively common in PNH and has been associated with increased mortality. We report the case of a 46-year-old male with PNH who presented with Budd-Chiari syndrome associated with portal cavernoma, portal hypertension and hypersplenism. In September 2010, the patient suffered gastrointestinal bleeding, hematuria, and elevated plasma lactate dehydrogenase; he started eculizumab therapy with a good response...
September 28, 2016: Hematology Reports
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