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hypersplenism review

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https://www.readbyqxmd.com/read/28585786/-results-of-the-mesoportal-bypass-rex-shunt-in-the-treatment-of-idiopathic-extrahepatic-portal-vein-obstruction-in-children
#1
E Domínguez Amillo, C De la Torre Ramos, A Andrés Moreno, J L Encinas Hernández, F Hernández Oliveros, M López Santamaría
BACKGROUND: Extrahepatic portal vein obstruction (EPVO) is the principal cause of portal hypertension in children. The objective of this study was to analyze the capacity of the surgical technique that creates a mesoportal shunt to treat changes caused by EPVO. METHODS: Retrospective review of patients with idiopathic EPVO who underwent a mesoportal shunt and analysis of the changes in the number of leucocytes, platelets, prothrombin time and spleen size one year after the surgery...
January 25, 2017: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/28535799/the-spleen-in-liver-cirrhosis-revisiting-an-old-enemy-with-novel-targets
#2
REVIEW
Liang Li, Mubing Duan, Weisan Chen, An Jiang, Xiaoming Li, Jun Yang, Zongfang Li
The spleen is a secondary lymphoid organ which can influence the progression of multiple diseases, notably liver cirrhosis. In chronic liver diseases, splenomegaly and hypersplenism can manifest following the development of portal hypertension. These splenic abnormalities correlate with and have been postulated to facilitate the progression of liver fibrosis to cirrhosis, although precise mechanisms remain poorly understood. In this review, we summarize the literature to highlight the mechanistic contributions of splenomegaly and hypersplenism to the development of liver cirrhosis, focusing on three key aspects: hepatic fibrogenesis, hepatic immune microenvironment dysregulation and liver regeneration...
May 23, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28486007/concomitant-laparoscopic-perisplenic-artery-ligation-facilitates-surgery-for-hepatobiliary-disease-coexisting-with-portal-hypertension
#3
Youlong Wang, Fei Chen, Yajuan Chu, Xiaofang Tan, Jingwang Tan, Wenbin Ji
BACKGROUND: Although liver cirrhosis with portal hypertension (PH) contributes significantly to morbidity and mortality in abdominal surgery, many authors still consider this disease as an indication for surgery. In many reports, however, numerous treatment modalities focus on hypersplenism secondary to PH, irrespective of splenomegaly and PH. The proven benefits of laparoscopy seem especially applicable to patients with this complex disease. This study evaluates the safety and efficacy of laparoscopic perisplenic artery ligation (SAL) in patients with hepatobiliary disease and PH...
May 9, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/28437324/noncirrhotic-portal-fibrosis-in-pediatric-population
#4
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/28191814/the-pathophysiologic-basis-of-anaemia-in-patients-with-malignant-diseases
#5
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/28127481/severe-thrombocytopenic-purpura-in-a-child-with-brucellosis-case-presentation-and-review-of-the-literature
#6
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/27931577/liver-transplantation-in-polish-children-with-%C3%AE-1-antitrypsin-deficiency-a-single-center-experience
#7
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/27906797/non-cirrhotic-portal-fibrosis-in-pediatric-population
#8
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/27896406/evaluation-of-rex-shunt-on-cavernous-transformation-of-the-portal-vein-in-children
#9
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/27720430/the-role-of-splenectomy-before-liver-transplantation-in-biliary-atresia-patients
#10
Yoshiaki Takahashi, Toshiharu Matsuura, Yusuke Yanagi, Koichiro Yoshimaru, Tomoaki Taguchi
BACKGROUND/PURPOSE: There is currently no unified view regarding whether liver transplantation or splenectomy should be performed for hypersplenism before liver transplantation in biliary atresia (BA) patients. We herein describe the efficacy of splenectomy before liver transplantation. METHODS: Splenectomy was performed in ten patients with hypersplenism associated with BA. We retrospectively reviewed their perioperative and postoperative courses, the number of leukocytes and thrombocytes, and the MELD score...
December 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27703501/hypersplenism-history-and-current-status
#11
Yunfu Lv, Wan Yee Lau, Yejuan Li, Jie Deng, Xiaoyu Han, Xiaoguang Gong, Ning Liu, Hongfei Wu
Hypersplenism is a common disorder characterized by an enlarged spleen which causes rapid and premature destruction of blood cells. This review summarizes the history of hypersplenism, discuss its classification and pathogenesis, and examines its diagnosis and treatment options. We performed a comprehensive literature search using PubMed, Web of Knowledge and the China National Knowledge Infrastructure (CNKI) database, reviewed hypersplenism-related articles and summarized the major findings. According to its etiological causes, hypersplenism is characterized by splenomegaly and peripheral cytopenias...
October 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/27465326/laparoscopic-versus-traditional-open-splenectomy-for-hepatocellular-carcinoma-with-hypersplenism
#12
Han-Hua Dong, Bin Mei, Fei-Long Liu, Zhi-Wei Zhang, Bi-Xiang Zhang, Zhi-Yong Huang, Xiao-Ping Chen, Wan-Guang Zhang
This study aimed to examine the efficacy of the laparoscopic vs. traditional open splenectomy for hepatocellular carcinoma (HCC) with hypersplenism. Between 2002 and 2013, 51 Chinese HCC patients with hypersplenism underwent either simultaneous laparoscopic splenectomy plus anticancer therapies (Lap-S&A) (n=25) or traditional open splenectomy plus anti-cancer therapies (TOS&A) (n=26). The outcomes were reviewed during and after the operation. Anti-cancer therapies for HCC included laparoscopic hepatectomy (LH) and laparoscopic microwave ablation (LMA)...
August 2016: Journal of Huazhong University of Science and Technology. Medical Sciences
https://www.readbyqxmd.com/read/27464949/laparoscopic-rfa-with-splenectomy-for-hepatocellular-carcinoma
#13
Kunpeng Hu, Purun Lei, Zhicheng Yao, Chenhu Wang, Qingliang Wang, Shilei Xu, Zhiyong Xiong, He Huang, Ruiyun Xu, Meihai Deng, Bo Liu
BACKGROUND: The treatment of hepatocellular carcinoma (HCC) is complicated and challenging because of the frequent presence of cirrhosis. Therefore, we propose a novel surgical approach to minimize the invasiveness and risk in patients with HCC, hypersplenism, and esophagogastric varices. METHODS: This was a retrospective study carried out in 25 patients with HCC and hypersplenism and who underwent simultaneous laparoscopic-guided radio-frequency ablation and laparoscopic splenectomy with endoscopic variceal ligation...
July 27, 2016: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/26911860/recurrent-variceal-haemorrhage-managed-with-splenic-vein-stenting
#14
REVIEW
W El Kininy, L Kearney, N Hosam, P Broe, A Keeling
INTRODUCTION: Extrahepatic Portal Hypertension (EPH) is defined as extrahepatic hypertension of the portal venous system in the absence of liver cirrhosis. Isolated splenic vein stenosis/occlusion as one of the causes of extrahepatic portal hypertension is uncommon, comprising less than 5 % of all cases of portal hypertension. However, it is an increasingly recognised complication of both acute and chronic pancreatitis, and with the advent of more effective diagnostic methods, interventional radiological methods for its management are also becoming more effective...
May 2017: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/26887878/-analysis-of-risk-factors-for-adverse-perinatal-outcomes-in-women-with-pancytopenia
#15
Xueyan Wang, Chao Zhang, Meiying Liang, Xiaohong Zhang, Jianliu Wang, Xia Zhang
OBJECTIVE: To explore the pregnancy outcomes of women with pancytopenia and the risk factors for the adverse perinatal outcomes. METHODS: A total of 106 pregnant women with pancytopenia were admitted to Peking University People's Hospital from Jan. 2005 to Sep. 2014. The clinical data and the pregnancy outcomes were reviewed retrospectively to investigate the risk factors for the adverse perinatal outcomes. RESULTS: (1 ) Eighty-four patients were found pancytopenia before pregnancy while 22 were found for the first time during pregnancy...
December 2015: Zhonghua Fu Chan Ke za Zhi
https://www.readbyqxmd.com/read/26818539/postoperative-outcomes-after-open-splenectomy-versus-laparoscopic-splenectomy-in-cirrhotic-patients-a-meta-analysis
#16
REVIEW
Khaled Al-raimi, Shu-Sen Zheng
BACKGROUND: Laparoscopic splenectomy is considered the gold standard for resecting normal-to-moderately bigger spleens in benign conditions, and in addition could be tried for patients with malignant splenic disorders. However, the safety of laparoscopic splenectomy in patients with hypersplenism is not well-known. This study aimed to investigate the efficacy and safety of laparoscopic splenectomy for patients with hypersplenism secondary to liver cirrhosis by comparing with the open splenectomy...
February 2016: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/26588331/gaucher-disease-clinical-biological-and-therapeutic-aspects
#17
REVIEW
Azza Dandana, Souhaira Ben Khelifa, Hinda Chahed, Abdelhédi Miled, Salima Ferchichi
We present a brief review of Gaucher disease (GD), the most common lysosomal storage disease. GD is a rare autosomal recessive disorder characterized by the defective function of the catabolic enzyme β-glucocerebrosidase (GBA), leading to an accumulation of its substrate, glucocerebroside. Clinical signs and symptoms include neurological dysfunctions, bone infarcts and malformations, hepatosplenomegaly and hypersplenism leading to anemia, neutropenia and thrombocytopenia. Enzyme replacement therapy with recombinant GBA is the mainstay of treatment for GD, which became the first successfully managed lipid storage disease...
2016: Pathobiology: Journal of Immunopathology, Molecular and Cellular Biology
https://www.readbyqxmd.com/read/26397320/an-overview-of-splenic-embolization
#18
REVIEW
Chaitanya Ahuja, Khashayar Farsad, Meghna Chadha
OBJECTIVE: The purpose of this article is to define the role of splenic embolization in trauma patients and in patients presenting for treatment of thrombocytopenia and portal hypertension. This article reviews the indications, technical considerations, outcomes, and complications of splenic artery embolization. CONCLUSION: Transcatheter splenic artery embolization has a major role in the management of traumatic splenic injuries and as an adjunctive procedure in the treatment of thrombocytopenia and portal hypertension...
October 2015: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/26205377/long-term-efficacy-of-subtotal-splenectomy-due-to-portal-hypertension-in-cirrhotic-patients
#19
Haibo Chu, Wei Han, Lei Wang, Yongbo Xu, Fengguo Jian, Weihua Zhang, Tao Wang, Jianhua Zhao
BACKGROUND: Portal hypertension (PHT) requires invasive measures to prevent rupture and bleeding of esophagogastric varices; however, the long-term results of subtotal splenectomy plus fixation of the retrosternal omentum majus (SSFROM) have not been reported. Specifically, the advantages and disadvantages of surgery that preserves the spleen and the long-term hematologic effects have not been described. STUDY DESIGN: Our studies relating to SSFROM commenced in February 1999...
2015: BMC Surgery
https://www.readbyqxmd.com/read/26111208/multimodality-imaging-of-primary-extrahepatic-portal-vein-obstruction-ehpvo-what-every-radiologist-should-know
#20
A Arora, S K Sarin
Portal vein thrombosis (PVT) is a frequent complication of liver cirrhosis, but it can also occur as a primary vascular disorder amid absent liver disease. Extrahepatic portal vein obstruction (EHPVO) refers to the obstruction of the extrahepatic portal vein with or without involvement of the intrahepatic portal vein branches, splenic and/or superior mesenteric vein. It is a distinct disorder that excludes PVT occurring in concurrence with liver cirrhosis or hepatocellular carcinoma. The term "EHPVO" implies chronicity and is principally reserved for a long-standing condition characterized by cavernous transformation of the portal vein...
August 2015: British Journal of Radiology
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