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https://www.readbyqxmd.com/read/28723802/tocilizumab-for-uncontrollable-systemic-inflammatory-response-syndrome-complicating-adult-onset-still-disease-case-report-and-review-of-literature
#1
Asami Masui-Ito, Ryuji Okamoto, Kaoru Ikejiri, Mika Fujimoto, Muneyoshi Tanimura, Shiro Nakamori, Tomohiro Murata, Eiji Ishikawa, Norikazu Yamada, Hiroshi Imai, Masaaki Ito
RATIONALE: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, fever spikes, arthralgia, and lymphadenopathy. AOSD usually has a good prognosis, but it can sometimes be fatal, especially when it is complicated by systemic inflammatory response syndrome (SIRS) and multiple organ failure. PATIENT CONCERNS: A previously healthy 26-year-old woman was referred to our hospital for persistent high fever and mild systemic edema...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28722461/-disorders-of-water-and-electrolyte-metabolism-and-changes-in-acid-base-balance-in-patients-with-ascitic-liver-cirrhosis
#2
Halima Gottfriedová, Miroslava Horáčková, Milena Čáslavská, Julius Špičák, Otto Schück
In patients with advanced cirrhosis with ascites disorders of water and electrolyte metabolism are often present and they are associated with changes in acid-base balance. These changes can be very complicated, their diagnosis and treatment difficult. Dilutional hyponatremia is the most common disorder. Hyponatremia in these patients is associated with increased morbidity and mortality before and after liver transplantation. Other common disorders include hyperchloremic acidosis, hypokalemia, metabolic alkalosis, lactic acidosis, respiratory alkalosis...
2017: Casopís Lékar̆ů C̆eských
https://www.readbyqxmd.com/read/28717121/impact-of-doppler-ultrasound-on-diagnosis-and-therapy-control-of-lienalis-steal-syndrome-after-liver-transplantation
#3
Eva M Teegen, Timm Denecke, Rosa B Schmuck, Robert Öllinger, Dominik Geisel, Johann Pratschke, Sascha S Chopra
BACKGROUND Lienalis steal syndrome is a rare complication after orthotopic liver transplantation leading to severe complications. Routine duplex sonography allows early and safe detection of lienalis steal syndrome and secondarily helps to monitor the outcome by evaluating the hemodynamics. MATERIAL AND METHODS This analysis included eight patients who after orthotopic liver transplantation needed splenic artery embolization due to lienalis steal syndrome. Lienalis steal syndrome was assumed in case of elevated transaminases, bilirubinemia or persistent ascites, and the absence of further pathologies...
July 18, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/28710306/ruxolitinib-treatment-in-an-infant-with-jak2-polycythaemia-vera-associated-budd-chiari-syndrome
#4
Mehmet Enes Coskun, Sue Height, Anil Dhawan, Nedim Hadzic
Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction commonly seen with myeloproliferative neoplasms (MPNs). Polycythaemia vera (PV) is a very rare MPN in childhood. This is the youngest reported patient diagnosed with PV and BCS secondary to JAK V617F mutation.A 26-month-old girl was admitted with a 5-month history of abdominal distension, hepatosplenomegaly and ascites. Imaging studies revealed occlusion of the right hepatic vein and marked attenuation of the middle and left hepatic veins...
July 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28690899/struma-ovarii-with-pseudo-meigs-syndrome-and-raised-cancer-antigen-125-levels-masquerading-as-an-ovarian-carcinoma-case-report-and-literature-review
#5
Surekha Yadav, Reena Tomar, Nidhi Verma, Nita Khurana, Rewa Triathi
The monodermal teratoma struma ovarii is a rare ovarian tumour; however, struma ovarii presenting with pseudo-Meigs' syndrome and raised cancer antigen (CA)-125 levels is even rarer. In elderly patients, this presentation can potentially lead to a misdiagnosis of a malignant ovarian carcinoma, resulting in unnecessary extensive surgery. We report a 55-year-old female who presented to the Lok Nayak Jai Prakash Narayan Hospital, New Delhi, India, in 2016 with progressive abdominal discomfort, fatigue and abdominal distention...
May 2017: Sultan Qaboos University Medical Journal
https://www.readbyqxmd.com/read/28678688/flood-syndrome-spontaneous-umbilical-hernia-rupture-leaking-ascitic-fluid-a-case-report
#6
Emilie T Nguyen, Leah A Tudtud-Hans
INTRODUCTION: We report a rare case of Flood syndrome, which is a spontaneous rupture of an umbilical hernia. CASE PRESENTATION: A 42-year-old man with decompensated hepatitis C and alcoholic cirrhosis complicated by ascites and esophageal varices presented with 1 day of ascitic fluid drainage after rupture of a preexisting umbilical hernia associated with diffuse abdominal pain and tenderness. A pigtail drain was placed in the right upper abdominal quadrant to decrease fluid drainage from the abdominal wall defect, allowing it to heal naturally...
2017: Permanente Journal
https://www.readbyqxmd.com/read/28660225/incidental-unilateral-tuberculous-sacroiliitis-detected-by-18-f-fdg-pet-ct-in-a-patient-with-abdominal-tuberculosis
#7
Domenico Albano, Giorgio Treglia, Paolo Desenzani, Francesco Bertagna
Tuberculosis is a systemic disease which involves skeletal and articular system very rarely. Osteoarticular tuberculosis commonly occurs in the vertebral column and more rarely in the sacroiliac joints. In this study, we report a 44-years-old male patient with low-grade fever, malabsorption syndrome, abdominal and pelvic ascites and low-back pain, that underwent (18)F-FDG PET/CT for identifying the cause of signs and symptoms after a negative abdominal CT and negative thorax radiography. The study revealed increased tracer uptake at the peritoneal ascites and at the right sacroiliac joint in absence of bone alteration suggesting a sacroiliitis...
2017: Asia Oceania Journal of Nuclear Medicine & Biology
https://www.readbyqxmd.com/read/28643816/-budd-chiari-syndrome-and-ulcerative-colitis-in-an-adolescent
#8
Federico Marchetti, Martina Mainetti, Michela Giovannini, Cristina Morelli
In patients with inflammatory bowel disease (IBD) there is an increased incidence of thromboembolic events. We report a case of a female, age 14, with a 6 months history of diarrhea with occasional presence of red blood and with a very distended abdomen with evident ascites. The diagnosis was Budd-Chiari syndrome (BCS) in ulcerative colitis (UC). Therapy with subcutaneous low molecular weight heparin, methylprednisolone, mesalazine was started. Clinical and radiological features quickly improved. No thrombophilia abnormality nor other risk factor for thrombosis were detected...
May 2017: Recenti Progressi in Medicina
https://www.readbyqxmd.com/read/28638005/sclerosing-mesenteritis-causing-chylous-ascites-and-small-bowel-perforation
#9
Huei-Wen Lim, Keith S Sultan
BACKGROUND Sclerosing mesenteritis (SM) is a rare idiopathic inflammation and fibrosis of the mesentery. Its etiology and pathogenesis remain unclear. It has been linked to abdominal trauma related to peritoneal dialysis, recent abdominal surgery, idiopathic inflammatory disorder, paraneoplastic syndrome, ischemia/infection, and autoimmunity. Abdominal CT is the most sensitive imaging modality, and diagnosis is usually confirmed by surgical biopsy. Patients most often present with abdominal pain, nausea, vomiting, diarrhea, and weight loss, and less commonly with chylous ascites and small bowel obstruction...
June 22, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28637843/poems-syndrome-a-rare-cause-of-exudative-ascites-and-chronic-peripheral-neuropathy
#10
Omar Al-Mayoof, Hana Al Sughaiyer, Wuroud Abuomar, Maria Khan
POEMS syndrome (Peripheral neuropathy, Organomegaly, Endocrinopathy, M protein, Skin changes) is a rare plasma cell disorder with multisystem involvementA 40-year-old man with a chronic history of unexplained peripheral neuropathy , presented with exudative ascites.He was found to have an incidental osteolytic lesion of the sacrum proven to be a plasmacytoma. Immunoelectrophoresis for monoclonal protein was negative.In addition, the patient was found to have hepatosplenomegaly, erectile dysfunction, hyperprolactinaemia and hypothyroidism...
June 20, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28634518/role-of-albumin-in-cirrhosis-from-a-hospitalist-s-perspective
#11
REVIEW
Saqib Walayat, Daniel Martin, Jaymon Patel, Umair Ahmed, Muhammad N Asghar, Aparna U Pai, Sonu Dhillon
Albumin, a negatively charged globular protein encoded on chromosome 4, is one of the most abundant proteins in the plasma and accounts for approximately 75% of plasma oncotic pressure. The role of albumin in the management of various disease states has shown to be beneficial historically. Low serum albumin is a predictor of mortality and poor outcomes. In cirrhotics undergoing paracentesis, albumin infusion prevents rapid re-accumulation of ascitic fluid while simultaneously decreasing the risk of post-paracentesis related circulatory dysfunction...
January 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28631648/leiomyosarcoma-of-inferior-vena-cava-with-intracardiac-extension-presenting-as-budd-chiari-syndrome-report-of-a-rare-case
#12
Kaniyappan Nambiyar, Arvind Ahuja, Minakshi Bhardwaj
Primary leiomyosarcoma of vascular origin is a rare malignant smooth muscle tumor. This report describes a case of primary leiomyosarcoma of the inferior vena cava (IVC) in a 52-year-old male who presented with complaints of abdominal pain, chest pain, and bilateral lower limb swelling for 4 months. Imaging of the chest and abdomen revealed a mass in the IVC extending into right atrium, heterogeneous enhancement of liver, hepatosplenomegaly, and ascites. Histopathological examination showed a malignant spindle cell tumor with cells arranged in fascicular and hemangiopericytomatous pattern...
April 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28629605/ct-findings-in-11-patients-with-tafro-syndrome-a-variant-of-multicentric-castleman-s-disease
#13
T Kiguchi, C Sato, K Takai, Y Nakai, Y Kaneko, M Matsuki
AIM: To assess detailed computed tomography (CT) findings in patients with the recently described thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome, in order to contribute to imaging interpretation in the challenging diagnosis of this disease. MATERIALS AND METHODS: The institutional review board approved this retrospective study and waived the need for informed consent. Eleven patients (six men, five women; mean age, 52.5 years) with confirmed TAFRO syndrome were included in this study...
June 16, 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28614198/the-almost-normal-liver-biopsy-presentation-clinical-associations-and-outcome
#14
Thomas W Czeczok, John S Van Arnam, Laura D Wood, Michael S Torbenson, Taofic Mounajjed
Liver biopsies obtained for abnormal liver enzymes or unexplained ascites occasionally appear histologically almost normal. The differential diagnosis for these cases is challenging because literature addressing this topic is lacking. We aimed to establish a differential diagnosis and determine clinical associations and outcomes for almost-normal liver biopsies. Ninety-seven histologically almost-normal liver biopsies were collected from 2 institutions. All cases lacked significant inflammation, fatty change, biliary tract disease, vascular disease, nodular regenerative hyperplasia, iron overload, inherited metabolic or storage disorder, viral hepatitis, or fibrosis...
June 13, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28611533/functional-budd-chiari-syndrome-associated-with-severe-polycystic-liver-disease
#15
Precil Diego Miranda de Menezes Neves, Bruno Eduardo Pedroso Balbo, Elieser Hitoshi Watanabe, Vinicius Rocha-Santos, Wellington Andraus, Luiz Augusto Carneiro D'Albuquerque, Luiz Fernando Onuchic
A 50-year-old woman with end-stage renal disease secondary to autosomal dominant polycystic kidney disease was referred to a quaternary care center due to significantly increased abdominal girth. Her physical examination revealed tense ascites and abdominal collateral veins. A 10-L paracentesis improved abdominal discomfort and disclosed a transudate, suggestive of portal hypertension. A computed tomographic scan revealed massive hepatomegaly caused by multiple cysts of variable sizes, distributed throughout all hepatic segments...
2017: Clinical Medicine Insights. Gastroenterology
https://www.readbyqxmd.com/read/28602971/new-developments-in-hepatorenal-syndrome
#16
Ayse L Mindikoglu, Stephen C Pappas
Hepatorenal Syndrome (HRS) continues to be one of the major complications of decompensated cirrhosis leading to death in the absence of liver transplantation. Challenges in precisely evaluating renal function in the patient with cirrhosis remain due to the limitations of serum creatinine (Cr) alone in estimating glomerular filtration rate (GFR); current GFR estimating models appear to underestimate renal function. Newer models incorporating renal biomarkers, such as the Cr-Cystatin C GFR Equation for Cirrhosis appear to more accurately estimate true GFR...
June 7, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28599693/clinical-characteristics-and-outcome-of-budd-chiari-syndrome-at-a-tertiary-care-hospital-in-pakistan
#17
Om Parkash, Aysha Khan, Saeed Hamid
OBJECTIVE: To determine the clinical characteristics of Budd-Chiari syndrome (BCS), its causes and outcome at a tertiary care hospital. STUDY DESIGN: An observational study. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital,Karachi, from 2004 to 2014. METHODOLOGY: Aretrospective analysis of data was conducted. Apredesigned questionnaire was filled from medical records of patients with BCS. Clinical features, etiology, management and outcome was noted from 2004 to 2014...
May 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28596302/carcinoid-heart-disease
#18
REVIEW
Saamir A Hassan, Jose Banchs, Cezar Iliescu, Arvind Dasari, Juan Lopez-Mattei, Syed Wamique Yusuf
Rare neuroendocrine tumours (NETs) that most commonly arise in the gastrointestinal tract can lead to carcinoid syndrome and carcinoid heart disease. Patients with carcinoid syndrome present with vasomotor changes, hypermotility of the gastrointestinal system, hypotension and bronchospasm. Medical therapy for carcinoid syndrome, typically with somatostatin analogues, can help control symptoms, inhibit tumour progression and prolong survival. Carcinoid heart disease occurs in more than 50% of these patients and is the initial presentation of carcinoid syndrome in up to 20% of patients...
June 8, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28585310/direct-acting-antivirals-for-chronic-hepatitis-c
#19
REVIEW
Janus C Jakobsen, Emil Eik Nielsen, Joshua Feinberg, Kiran Kumar Katakam, Kristina Fobian, Goran Hauser, Goran Poropat, Snezana Djurisic, Karl Heinz Weiss, Milica Bjelakovic, Goran Bjelakovic, Sarah Louise Klingenberg, Jian Ping Liu, Dimitrinka Nikolova, Ronald L Koretz, Christian Gluud
BACKGROUND: Millions of people worldwide suffer from hepatitis C, which can lead to severe liver disease, liver cancer, and death. Direct-acting antivirals (DAAs) are relatively new and expensive interventions for chronic hepatitis C, and preliminary results suggest that DAAs may eradicate hepatitis C virus (HCV) from the blood (sustained virological response). However, it is still questionable if eradication of hepatitis C virus in the blood eliminates hepatitis C in the body, and improves survival and leads to fewer complications...
June 6, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28580156/castleman-s-disease-with-tafro-syndrome-a-case-report-from-syria
#20
Sami Alhoulaiby, Basel Ahmad, Ali Alrstom, Mayssoun Kudsi
Castleman's disease is a rare disorder, yet a rarer newly described syndrome called TAFRO syndrome was discovered to accompany it. TAFRO represents the constellation of symptoms (Thrombocytopenia, Anasarca, MyeloFibrosis, Renal failure, Organomegaly). Most cases were described in Japan. We present the first case of TAFRO syndrome in Syria. A 58-year-old Caucasian male with no relevant history presented with fatigue, oliguria, decreased platelets, increased creatinine level, hepatosplenomegaly, ascites, pitting edema and lymph node enlargement...
June 2017: Oxford Medical Case Reports
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