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Hepatic cardiac syndrome

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https://www.readbyqxmd.com/read/29625787/tropical-diseases-in-the-icu-a-syndromic-approach-to-diagnosis-and-treatment
#1
Dilip R Karnad, Guy A Richards, Gisele Sampaio Silva, Pravin Amin
Tropical infections form 20-30% of ICU admissions in tropical countries. Diarrheal diseases, malaria, dengue, typhoid, rickettsial diseases and leptospirosis are common causes of critical illness. Overlapping clinical features makes initial diagnosis challenging. A systematic approach involving (1) history of specific continent or country of travel, (2) exposure to specific environments (forests or farms, water sports, consumption of exotic foods), (3) incubation period, and (4) pattern of organ involvement and subtle differences in manifestations help in differential diagnosis and choice of initial empiric therapy...
March 27, 2018: Journal of Critical Care
https://www.readbyqxmd.com/read/29624870/characteristics-and-risk-factors-of-pulmonary-arterial-hypertension-in-patients-with-primary-sj%C3%A3-gren-s-syndrome
#2
Shumin Yan, Mengtao Li, Hui Wang, Xiaoxi Yang, Jiuliang Zhao, Qian Wang, Yongtai Liu, Jinzhi Lai, Zhuang Tian, Hui Song, Yan Zhao, Xiaofeng Zeng
AIM: To describe baseline characteristics of patients with primary Sjögren's syndrome (pSS) with right heart catheterization (RHC)-confirmed pulmonary arterial hypertension (PAH) and explore risk factors for PAH in pSS. METHODS: This case-control study included consecutive patients hospitalized with pSS-PAH from 2007 to 2015, and pSS patients without PAH (in a 4 : 1 ratio) as controls. All patients fulfilled the 2002 American-European Consensus Group classification criteria for pSS-PAH was defined according to RHC-based European Society of Cardiology/European Respiratory Society guidelines...
April 6, 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29604340/a-case-of-pitt-hopkins-syndrome-with-de-novo-mutation-in-tcf4-clinical-features-and-treatment-for-epilepsy
#3
Yedan Liu, Ya Guo, Peipei Liu, Fei Li, Chengqing Yang, Jie Song, Jingfei Hu, Dandan Xin, Zongbo Chen
Pitt-Hopkins syndrome (PTHS), belonging to the group of 18q-syndromes, is a rare genetic disorder caused by mutations in TCF4. PTHS is characterized by distinctive facial appearance, intermittent hyperventilation, intellectual disability and developmental delay. Although patients with PTHS generally have various systemic symptoms, most of themwith a TCF4 mutation manifest the central nervous system (CNS) disorders. We described the first Chinese case with Pitt-Hopkins syndrome based on clinical presentations and genetic findings...
March 28, 2018: International Journal of Developmental Neuroscience
https://www.readbyqxmd.com/read/29600900/bilirubin-acts-as-a-multi-potent-guardian-of-cardiovascular-integrity-more-than-just-a-radical-idea
#4
Andrew Cameron Bulmer, Bhavisha A Bakrania, Eugene Francois Du Toit, Ai-Ching Boon, Paul J Clark, Lawrie W Powell, Karl-Heinz Wagner, John P Headrick
Bilirubin, a potentially toxic catabolite of heme and indicator of hepato-biliary insufficiency, exhibits potent cardiac and vascular protective properties. Individuals with Gilbert's syndrome (GS) may experience hyperbilirubinemia in response to stressors including reduced hepatic bilirubin excretion/increased red blood cell breakdown, with individuals usually informed by their clinician that their condition is of little consequence. However, GS appears to protect from all-cause mortality, with progressively elevated total bilirubin associated with protection from ischemic heart and chronic obstructive pulmonary diseases...
March 30, 2018: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/29595666/components-of-metabolic-syndrome-increase-the-risk-of-mortality-in-nonalcoholic-fatty-liver-disease-nafld
#5
Pegah Golabi, Munkhzul Otgonsuren, Leyla de Avila, Mehmet Sayiner, Nila Rafiq, Zobair M Younossi
Nonalcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in the United States. Metabolic syndrome (MS) components are highly prevalent in NAFLD. Our aim is to assess the relationship of NAFLD and MS with long-term outcome of mortality.The Third National Health and Nutrition Examination Survey (NHANES) was utilized. NAFLD was diagnosed by ultrasound in the presence of hepatic steatosis and no other causes of chronic liver disease. History of MS and its components were obtained from self-reported NHANES questionnaires...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29589604/nephrotic-syndrome-in-adults
#6
Viyaasan Mahalingasivam, John Booth, Michael Sheaff, Magdi Yaqoob
Nephrotic syndrome is an important presentation of glomerular disease characterised by heavy proteinuria, hypoalbuminaemia and oedema. The differential diagnosis of the underlying condition is wide including primary renal disorders and secondary diseases such as malignancy, infection, diabetes and amyloid. Presentations to acute medicine may be with hypervolaemia, complications of the nephrotic state (such as venous thromboembolism), or complications of therapy (such as infection). Early recognition of nephrotic syndrome is possible through simple urinalysis for protein and testing serum albumin, although a high index of suspicion is sometimes required in patients with comorbidities including potentially distracting cardiac or hepatic diseases...
2018: Acute Medicine
https://www.readbyqxmd.com/read/29559876/dietary-glycine-is-rate-limiting-for-glutathione-synthesis-and-may-have-broad-potential-for-health-protection
#7
REVIEW
Mark F McCarty, James H O'Keefe, James J DiNicolantonio
Background: Glutathione is a key scavenging antioxidant that opposes the proinflammatory signaling of hydrogen peroxide. Boosting cellular glutathione levels may have broad utility in the prevention and treatment of disorders driven by oxidative stress. Supplemental N-acetylcysteine has been employed for this purpose. Could supplemental glycine likewise promote glutathione synthesis? Methods: We conducted a review of the pertinent literature using PubMed. Results: Tissue glycine levels are lower than the glutathione synthase Michaelis constant (Km ) for glycine...
2018: Ochsner Journal
https://www.readbyqxmd.com/read/29537380/resolution-of-consumptive-hypothyroidism-secondary-to-infantile-hepatic-hemangiomatosis-with-combination-propranolol-and-levothyroxine
#8
V Campbell, R Beckett, N Abid, S Hoey
Infantile hepatic hemangiomas (IHH), particularly of the diffuse subtype, can in severe cases be associated with hepatic and cardiac failure, compartment syndrome, and consumptive hypothyroidism. Early recognition and treatment of these pathologies is paramount in order to minimise the risk of longterm sequelae. We report an interesting case of a female infant who presented with systemic compromise, in the absence of large or obvious cutaneous infantile hemangiomas (IH). Imaging identified innumerable hepatic hemangiomas, consistent with diffuse infantile hepatic hemangiomatosis...
February 28, 2018: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/29536209/right-trisectionectomy-and-caval-reconstruction-with-peritoneal-patch-under-short-total-vascular-exclusion-for-hepatocellular-carcinoma-with-tumoral-thrombus-in-suprahepatic-vena-cava
#9
Safi Dokmak, Béatrice Aussilhou, Tiziana Marchese, Nizar Kardoun, François Cauchy, Anne Sophie Schneck, Olivier Soubrane
BACKGROUND: Hepatocellular carcinoma (HCC) can be complicated by major vascular invasion, and resection can be beneficial in some patients.1 Some of these patients are traditionally operated under total vascular exclusion (TVE), refrigeration, extracorporeal circulatory bypass, and cardiac surgery, with high morbidity and mortality.2 However, HCC thrombi are not adherent to the venous wall, and with advances in surgical techniques, resection can be simplified and performed during short-duration TVE alone...
March 13, 2018: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/29516774/hepatic-adenoma-arising-in-a-patient-with-alagille-syndrome-a-case-report
#10
M Cristina Pacheco, Eric J Monroe, Simon P Horslen
Alagille syndrome is associated with decreased bile ducts, cardiac abnormalities, vertebral body fusion defects, and a typical facies. While regenerative nodules and hepatocellular carcinoma have been described in these patients, hepatic adenoma has not. Herein, we present a patient with Alagille syndrome caused by a mutation in NOTCH2 with a hepatic adenoma. The clinical, imaging, and histologic features are discussed.
January 1, 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29471569/does-transjugular-intrahepatic-portosystemic-shunt-stent-differentially-improve-survival-in-a-subset-of-cirrhotic-patients
#11
Jonel Trebicka
Does transjugular intrahepatic portosystemic shunt stent (TIPS) improve survival in a subgroup of patients? Yes. TIPS nearly halves portal pressure and increases the effective blood volume. In cases of acute variceal hemorrhage and with a high risk of treatment failure, defined as either hepatic venous pressure gradient higher than 20 mm Hg, Child B with active bleeding at the endoscopy, or Child C with less than 14 points, early or preemptive placement of TIPS (within 72 hours) improves survival. Also, in suitable patients with intractable or refractory ascites, TIPS improves survival if placed early in the course of treatment...
February 2018: Seminars in Liver Disease
https://www.readbyqxmd.com/read/29388442/liver-function-assessment-by-indocyanine-green-plasma-disappearance-rate-in-patients-with-intra-abdominal-hypertension-after-non-hepatic-abdominal-surgery
#12
Natasa Kovac, Mladen Peric
BACKGROUND AND OBJECTIVE: Liver function assessment in patients with intra-abdominal hypertension (IAH) after major abdominal surgery is complex and often confounding. Elevated intra-abdominal pressure (IAP) often occurs after major abdominal surgery, and is associated with decreased abdominal blood flow and organ dysfunction, and it could cause abdominal compartment syndrome (ACS), which is a life-threatening condition. Plasma disappearance rate (PDR) of indocyanine green (ICG) and ICG retention rate after 15 min (R15) were used to evaluate liver function and as a prognostic parameter after major abdominal surgery...
February 28, 2018: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/29355456/icariin-improves-fanconi-anemia-hematopoietic-stem-cell-function-through-sirt6-mediated-nf-kappa-b-inhibition
#13
Yibo Li, Xue Li, Allison Cole, Sarah McLaughlin, Wei Du
Icariin (ICA) is a flavonoid glucoside derived from the Epimedium plant genus, which has potent regenerative properties and is used in western medicine to treat impotence. Recently, ICA has generated great interest in improving hepatic stellate cell function and cardiac rejuvenation. However, how this natural component functions in hematopoiesis remains unexplored. Here we have examined the role of ICA on hematopoietic stem cells (HSCs) using the cancer-prone disease model of Fanconi anemia (FA), an inherited bone marrow failure syndrome with extremely high risk of leukemic predisposition...
2018: Cell Cycle
https://www.readbyqxmd.com/read/29288403/the-impact-of-non-alcoholic-fatty-liver-disease-fibrosis-score-on-cardiac-prognosis-in-patients-with-chronic-heart-failure
#14
Tetsuya Takahashi, Tetsu Watanabe, Tetsuro Shishido, Ken Watanabe, Takayuki Sugai, Taku Toshima, Daisuke Kinoshita, Miyuki Yokoyama, Harutoshi Tamura, Satoshi Nishiyama, Takanori Arimoto, Hiroki Takahashi, Tamon Yamanaka, Takuya Miyamoto, Isao Kubota
Liver abnormalities have a strong impact on clinical outcomes in patients with heart failure (HF), and are known as cardio-hepatic syndrome. The non-alcoholic fatty liver disease (NAFLD) fibrosis score (NFS) has been developed to identify liver fibrosis in patients with NAFLD. It remains to be determined whether NFS is associated with cardiovascular prognosis in patients with chronic heart failure (CHF). We calculated NFS in 516 patients with CHF admitted to our hospital. The clinical endpoints were deaths due to progressive HF, myocardial infarction, stroke, and sudden cardiac death, and rehospitalization for worsening HF...
December 29, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/29163953/hypokalemia-a-potentially-life-threatening-complication-of-tenofovir-therapy
#15
Abhilash Koratala, Rupam Ruchi
Tenofovir is a nucleotide analog reverse transcriptase inhibitor approved for the treatment of HIV and hepatitis B infections. It is widely prescribed and an integral part of the recommended regimens for the treatment of HIV infection in antiretroviral-naive patients. Tenofovir is implicated in renal proximal tubular dysfunction, which can be associated with Fanconi syndrome and hypokalemia. When the hypokalemia is severe, it can lead to life-threatening complications. We describe the case of a 59-year-old woman who suffered a cardiac arrest secondary to severe hypokalemia from tenofovir use...
2017: SAGE Open Medical Case Reports
https://www.readbyqxmd.com/read/29161691/failure-to-thrive-in-the-context-of-carney-complex
#16
Amit Tirosh, Adi Auerbach, Belen Bonella, Phaedon D Zavras, Elena Belyavskaya, Charalampos Lyssikatos, Karen Meir, Ram Weiss, Hagit Daum, Maya B Lodish, David Gillis, Constantine A Stratakis
BACKGROUND/AIMS: Carney complex (CNC) is a rare syndrome associated with multiple tumors and several other unique manifestations. We describe the clinical, genetic, and laboratory findings in a cohort of patients with CNC and failure to thrive (FTT). METHODS: A retrospective case series of pediatric patients with CNC presenting with FTT. RESULTS: We describe a patient with infantile Cushing syndrome (CS) who presented with severe FTT and liver disease; the patient was subsequently diagnosed with CNC...
2018: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29120023/role-of-liver-magnetic-resonance-imaging-in-hyperferritinaemia-and-the-diagnosis-of-iron-overload
#17
Axel Ruefer, Christine Bapst, Rudolf Benz, Jens Bremerich, Nathan Cantoni, Laura Infanti, Kaveh Samii, Mathias Schmid, Jean-Paul Vallée
Hyperferritinaemia is a frequent clinical problem. Elevated serum ferritin levels can be detected in different genetic and acquired diseases and can occur with or without anaemia. It is therefore important to determine whether hyperferritinaemia is due to iron overload or due to a secondary cause. The main causes of iron overload are intestinal iron hyperabsorption disorders and transfusion-dependent disorders. Iron homeostasis and iron overload are quantified by different diagnostic approaches. The evaluation of serum ferritin and transferrin saturation is the first diagnostic step to identify the cause of hyperferritinaemia...
November 9, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/29110789/long-term-management-of-end-stage-heart-failure
#18
REVIEW
Marlena V Habal, A Reshad Garan
End-stage heart failure manifests as severe and often relentless symptoms that define the clinical syndrome of heart failure, namely congestion and hypoperfusion. These patients suffer from dyspnea, fatigue, abdominal discomfort, and ultimately cardiac cachexia. Renal and hepatic dysfunction frequently further complicates the process. Recurrent hospitalizations, cardiac arrhythmias, and intolerance to standard heart failure therapies are common as the disease progresses. Management focuses on controlling symptoms, correcting precipitants, avoiding triggers, and maximizing therapies with demonstrable survival benefit...
June 2017: Best Practice & Research. Clinical Anaesthesiology
https://www.readbyqxmd.com/read/28866669/observational-monitoring-of-patients-with-aplastic-anemia-and-low-intermediate-1-risk-of-myelodysplastic-syndromes-complicated-with-iron-overload
#19
Yali Du, Zhangbiao Long, Miao Chen, Bing Han, Bo Hou, Feng Feng
BACKGROUND: This study focuses on the iron overload (IOL) of patients with transfused aplastic anemia (AA) or a low/intermediate-1 risk of myelodysplastic syndrome (MDS). METHODS: Ninety-two AA or MDS patients with IOL were prospectively recruited. Clinical data were collected every 6 months, and organ magnetic resonance imaging T2* values were collected annually. Patients with IOL were chelated. RESULTS: Serum ferritin was correlated with liver T2* and pancreatic T2* in the AA and MDS groups...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28836926/platypnoea-orthodeoxia-syndrome-in-the-elderly-a-difficult-to-make-diagnosis-of-intracardiac-right-to-left-shunt
#20
Enrico M Zardi, Silvia Spoto, Luciana Locorriere, Giulio Cacioli, Silvia Mazzaroppi, Bruno B Zobel, Germano Di Sciascio, Sebastiano Costantino
Introduction Platypnea-orthodeoxia syndrome is a combination of positional dyspnoea and hypoxemia; it is caused by several cardiac, pulmonary and hepatic diseases. Case presentation In this study, we describe a 77-year-old female affected by unexplained dizziness and hypoxia that exacerbated in upright position. After diagnosing platypnea-orthodeoxia syndrome and excluding all possible causes (liver cirrhosis, acute and chronic pulmonary diseases and arteriovenous malformations), the origin of the syndrome was individuated in the presence of a patent foramen ovale with right-to-left shunt...
August 2017: Scottish Medical Journal
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