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https://www.readbyqxmd.com/read/28433104/wilson-disease-symptomatic-liver-therapy
#1
Jan Pfeiffenberger, Karl-Heinz Weiss, Wolfgang Stremmel
Wilson disease leads to symptomatic impairment of liver function or liver cirrhosis. Strict adherence to decoppering agents is essential in these patients. Secondary prevention of additional hepatic damage by avoidance of other toxic substances (e.g., alcohol, drugs) and sufficient calorie intake is recommended. Routine examinations in cirrhotic patients include screening for signs of portal hypertension (esophagus varices), development of ascites, and hepatic encephalopathy. Where varices are present, primary or secondary preventive interventions may include treatment with nonselective beta-blockers or variceal ligation, similar to the approach in patients with liver cirrhosis due to other etiologies...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28402911/hassab-s-operation-for-joubert-syndrome-with-congenital-hepatic-fibrosis-a-case-report
#2
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...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28358421/extravasated-platelet-aggregation-in-the-livers-of-rats-with-drug%C3%A2-induced-hepatic-sinusoidal-obstruction-syndrome
#3
Miki Hirata, Hidehiro Tajima, Tomoharu Miyashita, Takashi Miyata, Shinichi Nakanuma, Isamu Makino, Hironori Hayashi, Katsunobu Oyama, Hiroyuki Takamura, Itasu Ninomiya, Sachio Fushida, Hiroki Nakata, Shoichi Iseki, Shinichi Harada, Tomohiko Wakayama, Tetsuo Ohta
Oxaliplatin-based chemotherapy plays an important role in the treatment of colorectal liver metastases. Oxaliplatin, however, causes sinusoidal obstruction syndrome (SOS), which is characterized by portal hypertension, splenomegaly, thrombocytopenia, and liver dysfunction. SOS is diagnosed histopathologically by disruption of the sinusoidal endothelium, collagen deposition, fibrosis especially around zone 3, dilatation of the sinusoidal space and congestion. This study assessed the characteristics of a rat model of SOS...
May 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28355401/schistosoma-mansoni-associated-glomerulopathy-with-iga-mesangial-deposits-case-report
#4
Fabiana Oliveira Gonçalves, Tânia Maria de Souza Fontes, Ana Paula Pereira Santana Lemes Canuto
INTRODUCTION: Renal involvement is a severe form of schistosomiasis and occurs in 10% to 15% of patients with the hepatosplenic form of the disease. Nephrotic syndrome is the most common clinical presentation. It is a complication caused by immune complexes (IC), it is rare to appear in the Brazilian context with a immunoglobulin A (IgA) deposits. When installed the renal injury by Schistosoma mansoni, classically presents as membranoproliferative glomerulonephritis (mesangiocapillary) with lobular accentuation...
March 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/28353569/case-report-and-systematic-literature-review-of-a-novel-etiology-of-sinistral-portal-hypertension-presenting-with-ugi-bleeding-left-gastric-artery-pseudoaneurysm-compressing-the-splenic-vein-treated-by-embolization-of-the-pseudoaneurysm
#5
REVIEW
Seifeldin Hakim, Jared Bortman, Molly Orosey, Mitchell S Cappell
INTRODUCTION: A novel case is reported of upper gastrointestinal (UGI) bleeding from sinistral portal hypertension, caused by a left gastric artery (LGA) pseudoaneurysm (PA) compressing the splenic vein (SV) that was successfully treated with PA embolization. CASE REPORT: A 41-year-old man with previous medical history of recurrent, alcoholic pancreatitis presented with several episodes of hematemesis and abdominal pain for 48 hours. Physical examination revealed a soft abdomen, with no abdominal bruit, no pulsatile abdominal mass, and no stigmata of chronic liver disease...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28348478/portal-hypertension-imaging-of-portosystemic-collateral-pathways-and-associated-image-guided-therapy
#6
REVIEW
Murad Feroz Bandali, Anirudh Mirakhur, Edward Wolfgang Lee, Mollie Clarke Ferris, David James Sadler, Robin Ritchie Gray, Jason Kam Wong
Portal hypertension is a common clinical syndrome, defined by a pathologic increase in the portal venous pressure. Increased resistance to portal blood flow, the primary factor in the pathophysiology of portal hypertension, is in part due to morphological changes occurring in chronic liver diseases. This results in rerouting of blood flow away from the liver through collateral pathways to low-pressure systemic veins. Through a variety of computed tomographic, sonographic, magnetic resonance imaging and angiographic examples, this article discusses the appearances and prevalence of both common and less common portosystemic collateral channels in the thorax and abdomen...
March 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28260452/unusual-indications-for-a-liver-transplant-a-single-center-experience
#7
Aydincan Akdur, Mahir Kirnap, Ebru H Ayvazoglu Soy, Figen Ozcay, Gokhan Moray, Gulnaz Arslan, Mehmet Haberal
OBJECTIVES: This study sought to evaluate the efficacy of liver transplant for unusual liver diseases. MATERIALS AND METHODS: The results of 476 patients who underwent liver transplant from 1988 to January 2015 were retrospectively analyzed. Two hundred forty-five of them were adult patients and 231 of them were pediatric. Thirty-one patients had unusual liver disease. RESULTS: Of the 31 patients with unusual liver disease, 9 (29%) were adult and 22 (71%) were pediatric patients...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28254530/current-understanding-and-management-of-splenic-steal-syndrome-after-liver-transplant-a-systematic-review
#8
REVIEW
Chaolun Li, Baljendra Kapoor, Eunice Moon, Cristiano Quintini, Weiping Wang
BACKGROUND: Splenic steal syndrome (SSS) is a condition that can occur after orthotopic liver transplant (OLT). However, limited information is available about this condition. METHODS: A systematic literature search of studies performed through May 2016 was conducted to identify reports of angiographically confirmed SSS and its variants. All of the factors relevant to this disorder were collected and analyzed. RESULTS: A total of 219 cases of SSS and its variants were identified...
February 17, 2017: Transplantation Reviews
https://www.readbyqxmd.com/read/28247771/poems-syndrome-and-idiopathic-portal-hypertension-a-possible-association
#9
Sara Campos, Cláudia Agostinho, Maria Augusta Cipriano
A 48-year old female patient was admitted to the emergency department with upper gastrointestinal bleeding. Endoscopy showed large esophageal varices that were treated with band ligation. She had been treated with cyclophosphamide, melphalan, lenalidomide and corticosteroids for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy or edema, M protein, skin changes). She had no other risk factors for chronic liver disease. Laboratory and radiological examinations could not confirm the etiology of portal hypertension...
March 1, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28245243/serelaxin-as-a-potential-treatment-for-renal-dysfunction-in-cirrhosis-preclinical-evaluation-and-results-of-a-randomized-phase-2-trial
#10
Victoria K Snowdon, Neil J Lachlan, Anna M Hoy, Patrick W F Hadoke, Scott I Semple, Dilip Patel, Will Mungall, Timothy J Kendall, Adrian Thomson, Ross J Lennen, Maurits A Jansen, Carmel M Moran, Antonella Pellicoro, Prakash Ramachandran, Isaac Shaw, Rebecca L Aucott, Thomas Severin, Rajnish Saini, Judy Pak, Denise Yates, Neelesh Dongre, Jeremy S Duffield, David J Webb, John P Iredale, Peter C Hayes, Jonathan A Fallowfield
BACKGROUND: Chronic liver scarring from any cause leads to cirrhosis, portal hypertension, and a progressive decline in renal blood flow and renal function. Extreme renal vasoconstriction characterizes hepatorenal syndrome, a functional and potentially reversible form of acute kidney injury in patients with advanced cirrhosis, but current therapy with systemic vasoconstrictors is ineffective in a substantial proportion of patients and is limited by ischemic adverse events. Serelaxin (recombinant human relaxin-2) is a peptide molecule with anti-fibrotic and vasoprotective properties that binds to relaxin family peptide receptor-1 (RXFP1) and has been shown to increase renal perfusion in healthy human volunteers...
February 2017: PLoS Medicine
https://www.readbyqxmd.com/read/28223722/to-screen-or-not-to-screen-celiac-antibodies-in-liver-diseases
#11
REVIEW
Janaína Luz Narciso-Schiavon, Leonardo Lucca Schiavon
Celiac disease (CD) is a systemic immune-mediated disorder triggered by dietary gluten in genetically predisposed individuals. The typical symptoms are anemia, diarrhea, fatigue, weight loss, and abdominal pain. CD has been reported in patients with primary sclerosing cholangitis, primary biliary cholangitis, autoimmune hepatitis, aminotransferase elevations, nonalcoholic fatty liver disease, hepatitis B, hepatitis C, portal hypertension and liver cirrhosis. We evaluate recommendations for active screening for CD in patients with liver diseases, and the effect of a gluten-free diet in these different settings...
February 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28166812/efficacy-of-high-intensity-focused-ultrasound-assisted-hepatic-resection-hifu-ar-on-blood-loss-reduction-in-patients-with-liver-metastases-requiring-hepatectomy-study-protocol-for-a-randomized-controlled-trial
#12
Aurélien Dupré, David Pérol, Ellen Blanc, Patrice Peyrat, Valéria Basso, Yao Chen, Jérémy Vincenot, Anthony Kocot, David Melodelima, Michel Rivoire
BACKGROUND: Liver resection is the only potentially curative treatment for colorectal liver metastases (LM). It is considered a safe procedure, but is often associated with blood loss during liver transection. Blood transfusions are frequently needed, but they are associated with increased morbidity and risk of recurrence. Many surgical devices have been developed to decrease blood loss. However, none of them has proven superior to the standard crushing technique. We developed a new, powerful intra-operative high-intensity focused ultrasound (HIFU) transducer which destroys tissue by coagulative necrosis...
February 6, 2017: Trials
https://www.readbyqxmd.com/read/28160419/adams-oliver-syndrome-review-of-the-literature-refining-the-diagnostic-phenotype
#13
REVIEW
Susan Hassed, Shibo Li, John Mulvihill, Christopher Aston, Susan Palmer
The Adams-Oliver syndrome (AOS) is defined as aplasia cutis congenita (ACC) with transverse terminal limb defects (TTLD). Frequencies of associated anomalies are not well characterized. Six causative genes have been identified: ARHGAP31, DOCK6, EOGT, RBPJ, NOTCH1, and DLL4. We review 385 previously described individuals (139 non-familial and 246 familial probands and family members) and add clinical data on 13 previously unreported individuals with AOS. In addition to ACC and TTLD, the most commonly associated anomalies included a wide variety of central nervous system (CNS) anomalies and congenital heart defects each seen in 23%...
March 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28153558/middle-east-respiratory-syndrome-coronavirus-mers-cov-outbreak-in-south-korea-2015-epidemiology-characteristics-and-public-health-implications
#14
K H Kim, T E Tandi, J W Choi, J M Moon, M S Kim
BACKGROUND: Since the first case of Middle East respiratory syndrome coronavirus (MERS-CoV) in South Korea was reported on 20(th) May 2015, there have been 186 confirmed cases, 38 deaths and 16,752 suspected cases. Previously published research on South Korea's MERS outbreak was limited to the early stages, when few data were available. Now that the outbreak has ended, albeit unofficially, a more comprehensive review is appropriate. METHODS: Data were obtained through the MERS portal by the Ministry for Health and Welfare (MOHW) and Korea Centres for Disease Control and Prevention, press releases by MOHW, and reports by the MERS Policy Committee of the Korean Medical Association...
February 2017: Journal of Hospital Infection
https://www.readbyqxmd.com/read/28150309/portal-systemic-shunt-between-the-hepatic-portal-vein-and-right-renal-vein-in-a-patient-with-multifocal-hepatocellular-carcinoma-case-report
#15
Mateja Sabol Pušić, Ivan Budimir, Zdravko Dorosulić, Branko Ostrički, Marko Nikolić, Gordana Lovrenčić Prpić, Katherina B Sreter
Portal hypertension is a clinical syndrome characterized by the development of collateral circulation and portosystemic shunts, as well as ascites and hepatic encephalopathy. We present the case of a large portosystemic shunt between the hepatic portal vein and aneurysmal right renal vein in a cirrhotic 64-year-old man with thrombosis of the portal vein and hepatocellular carcinoma. This is a very rare clinical manifestation which, to our knowledge, has been described only once previously in the literature...
February 2, 2017: Journal of Clinical Ultrasound: JCU
https://www.readbyqxmd.com/read/28115278/the-role-of-thromboelastography-in-pediatric-patients-with-sinusoidal-obstructive-syndrome-receiving-defibrotide
#16
Joanna L Gendreau, Christine Knoll, Roberta H Adams, Leon L Su
Sinusoidal obstructive syndrome (SOS) is a potentially fatal form of hepatic injury after hematopoietic stem cell transplantation. Patients can develop liver dysfunction, portal hypertension, ascites, coagulopathies, and multisystem organ failure. The mortality rate of severe SOS has been reported as high as 98% by day 100 after transplantation. Defibrotide, which is now approved for the treatment of SOS, has significantly decreased mortality. Defibrotide is a polynucleotide with profibrinolytic, anti-ischemic, and anti-inflammatory activity...
April 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28115008/hereditary-hemorrhagic-telangiectasia-liver-disease-and-elevated-serum-testosterone-osler-weber-rendu-syndrome-a-case-report
#17
R Dissanayake, K P K Y M D S Wickramarathne, S N Seneviratne, S N Perera, M U J Fernando, V P Wickramasinghe
BACKGROUND: A Sri Lankan girl with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is described. CASE PRESENTATION: She presented with recurrent spontaneous epistaxis, pulmonary arterio venous malformation and oral telangiectasia. A diagnosis of Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) was made based on the presence of three Curacao criteria (out of four). Evaluations of her jaundice revealed chronic parenchymal liver disease with multiple nodules in the liver with early portal hypertension...
January 23, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28091982/death-from-pulmonary-embolism-of-cyanoacrylate-glue-following-gastric-varix-endoscopic-injection
#18
Michael Philip Burke, Chris O'Donnell, Yeliena Baber
We present the case of a 25 year old woman with a complex past medical history including Crigler-Najjar syndrome (Type 1) with a liver transplant in 1993 and subsequent development of cirrhosis with portal hypertension in the transplanted liver. The deceased presented to hospital with hematemesis and investigations showed a large gastric varix. The varix was injected with cyanoacrylate glue. Within 30 min of injection the patient became acutely hypoxic. Urgent chest X-ray demonstrated radio opaque glue within the pulmonary arteries...
March 2017: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/28058764/diagnosis-and-treatment-guidelines-for-aberrant-portal-hemodynamics-the-aberrant-portal-hemodynamics-study-group-supported-by-the-ministry-of-health-labor-and-welfare-of-japan
#19
(no author information available yet)
Idiopathic portal hypertension (IPH), causing aberrant portal hemodynamics, is a disease with an as yet unidentified cause and no established treatment protocol. The Japanese research group on IPH in Japan was set up in 1975 by the Ministry of Health, Labor and Welfare. Extrahepatic portal obstruction and Budd-Chiari syndrome (BCS) have since been added to the group's research subjects. The aims of the research group are to accurately evaluate the current status of the three diseases in Japan, elucidate their etiology and pathogenesis, and develop new treatments...
April 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#20
Güzide Doğan, Fatih Düzgün, Serdar Tarhan, Yeliz Çağan Appak, Erhun Kasırga
Congenital intrahepatic portosystemic shunts are rare in children. Portosystemic venous malformations are characterized by extreme clinical variability. We report a full-term 33-day-old male infant presenting with neonatal jaundice. On physical examination, he had generalized icterus and the liver was palpable 3.5 cm below the right costal margin. He had no other symptoms. Laboratory tests showed AST 632 U/L, ALT 198 U/L, total bilirubin 12.1 mg/dL, conjugated bilirubin 10.2 mg/dL, alkaline phosphatase 753 U/L, GGT 47 U/L and glucose 67 U/L...
December 2016: Journal of Clinical and Experimental Hepatology
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