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syndrome hepatopulmonary

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https://www.readbyqxmd.com/read/28078819/live-donor-liver-transplantation-for-a-child-presented-with-severe-hepatopulmonary-syndrome-and-nodular-liver-lesions-due-to-abernethy-malformation
#1
Cahit Yilmaz, Zafer Onen, Rasim Farajov, Onur S Duygu, Latife Doganay, Alper Yuksel, Mehmet Alper, Murat Zeytunlu, Cigdem Arikan, Murat Kilic
A 15-year-old boy first presented with severe lung lesions and hypoxia and he was considered as a lung transplant candidate. Upon evaluation, hepatopulmonary syndrome, multiple nodular liver lesions, and Abernethy type 1b malformation were diagnosed. The patient underwent successful right lobe live donor liver transplantation, and all of the symptoms disappeared soon after the transplant. He is currently alive and well with excellent liver and lung functions 4 years after surgery.
January 12, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28042141/prolonged-unexplained-hypoxemia-as-initial-presentation-of-cirrhosis-a-case-report
#2
Anand Puttappa, Kumaraswamy Sheshadri, Aurelie Fabre, Georgina Imberger, John Boylan, Silke Ryan, Masood Iqbal, Niamh Conlon
BACKGROUND Hepatopulmonary syndrome (HPS) is a pulmonary complication of advanced liver disease with dyspnea as the predominant presenting symptom. The diagnosis of HPS can often be missed due to its nonspecific presentation and the presence of other comorbidities. CASE REPORT We present an interesting case of an obese 43-year-old man who presented with progressive, unexplained hypoxemia and shortness of breath in the absence of any symptoms or signs of chronic liver disease. After extensive cardiopulmonary investigations, he was diagnosed with severe HPS as a result of non-alcoholic steatohepatitis (NASH) leading to cirrhosis...
January 2, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28039901/living-donor-liver-transplantation-in-a-pediatric-patient-with-preexisting-yolk-sac-tumor
#3
Taiichi Wakiya, Yoshikazu Toyoki, Keinosuke Ishido, Daisuke Kudo, Norihisa Kimura, Shinji Tsutsumi, Tadashi Odagiri, Akiko Suto, Chiaki Uchida, Kenichi Hakamada
There is ongoing discussion regarding the indications and timing of LT for patients with a preexisting extrahepatic malignancy. We herein report a pediatric case that underwent LDLT after therapy for YST. The patient, a 13-year-old female with biliary atresia, had undergone portoenterostomy at 2 months of age. She developed a left ovarian tumor with a high serum alpha-fetoprotein concentration at 10 years of age. She underwent left oophorectomy and was diagnosed with ovarian YST (Stage I). After surgery, hepatopulmonary syndrome progressed gradually...
December 30, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/28009313/character-and-frequency-of-the-variations-of-co-and-polymorbid-syntropic-extrahepatic-lesions-and-their-dependence-on-the-hepatopulmonary-syndrome-severity-degree-in-cirrhotic-patients
#4
O Abrahamovych, M Abrahamovych, S Tolopko, O Fayura, M Ferko
In this study, we comprehensively examined 93 patients with liver cirrhosis, selected in a randomized manner, with the preliminary stratification by the presence of hepatopulmonary syndrome with the aim to study the nature and frequency of extrahepatic syntropic lesions of organs and systems and their dependence on the severity of lung injury that is necessary for the appointment of the most effective individualized comprehensive treatment. The results of our calculations showed that with the increasing of the hepatopulmonary syndromeseverity degree, the nature and the frequency of the syntropic co- and polymorbid functional and organic extrahepatic lesions of the organs and body systems was significantly (p<0...
November 2016: Georgian Medical News
https://www.readbyqxmd.com/read/28009008/down-regulation-of-common-nf%C3%AE%C2%BAb-inos-pathway-by-chronic-thalidomide-treatment-improves-hepatopulmonary-syndrome-and-muscle-wasting-in-rats-with-biliary-cirrhosis
#5
Tzu-Hao Li, Pei-Chang Lee, Kuei-Chuan Lee, Yun-Cheng Hsieh, Chang-Youh Tsai, Ying-Ying Yang, Shiang-Fen Huang, Tung-Hu Tsai, Shie-Liang Hsieh, Ming-Chih Hou, Han-Chieh Lin, Shou-Dong Lee
Thalidomide can modulate the TNFα-NFκB and iNOS pathway, which involve in the pathogenesis of hepatopulmonary syndrome (HPS) and muscle wasting in cirrhosis. In bile duct ligated-cirrhotic rats, the increased circulating CD16(+) (inflammatory) monocytes and its intracellular TNFα, NFκB, monocyte chemotactic protein (MCP-1) and iNOS levels were associated with increased circulating MCP-1/soluable intercellular cell adehesion molecule-1 (sICAM-1), pulmonary TNFα/NOx, up-regulated M1 polarization, exacerbated angiogenesis and hypoxemia (increased AaPO2) in bronchoalveolar lavage (BAL) fluid and pulmonary homogenates...
December 23, 2016: Scientific Reports
https://www.readbyqxmd.com/read/28008407/severe-hepatopulmonary-syndrome-in-an-adolescent-patient-with-non-cirrhotic-portal-fibrosis
#6
Vikrant Sood, S Rajesh, Bikrant Bihari Lal, Dinesh Rawat, Seema Alam
No abstract text is available yet for this article.
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#7
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
https://www.readbyqxmd.com/read/27981304/the-impact-of-hypoxaemia-on-the-outcome-in-liver-cirrhosis
#8
Andrei Mihai Voiosu, Theodor Alexandru Voiosu, Bianca Smarandache, Aurelia Rădoi, Radu Bogdan Mateescu, Cristian Răsvan Băicuş, Mihail Radu Voiosu, Mihai Mircea Diculescu
BACKGROUND AND AIMS: Prognostic factors for poor evolution are critical in the setting of limited access to liver transplantation for patients with cirrhosis. We aimed to investigate the impact of hypoxaemia on the outcome in cirrhosis and the evolution of arterial oxygen tension during long-term follow-up in these patients. METHODS: Consecutive cirrhotic patients were prospectively enroled and followed-up in our tertiary referral center. Clinical features, biological tests, arterial blood gases, NT-proBNP levels, pulse oximetry measurements, 12-lead ECG, and transthoracic contrast echocardiography were documented on enrolment...
December 2016: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/27951601/serum-bile-acids-in-patients-with-hepatopulmonary-syndrome
#9
Thomas Horvatits, Andreas Drolz, Karoline Rutter, Kevin Roedl, Günter Fauler, Christian Müller, Stefan Kluge, Michael Trauner, Peter Schenk, Valentin Fuhrmann
Background Hepatopulmonary syndrome (HPS) occurs in 20 - 30 % of patients with cirrhosis and is associated with increased mortality. Cholestasis and accumulation of bile acids (BAs) play a major role in chronic liver disease. Aim We aimed to evaluate the clinical role of serum BAs in patients with HPS. Methods Seventy-four patients with cirrhosis were included in this prospective study. Marker for cholestasis as total and individual serum BAs, bilirubin, alkaline phosphatase (AP), and gamma-glutamyl transpeptidase (GGT) were analyzed in patients screened for HPS...
December 12, 2016: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/27879294/cxcr2-is-involved-in-pulmonary-intravascular-macrophage-accumulation-and-angiogenesis-in-a-rat-model-of-hepatopulmonary-syndrome
#10
Xujiong Li, Yunxia Chen, Yongli Chang, Shufen Li, Zhongfu Zhao, Huiying Zhang
Hepatopulmonary syndrome (HPS) is a lung complication in various liver diseases, with high incidence, poor prognosis and no effective non-surgical treatments in patients with hepatocirrhosis. Therefore, assessing HPS pathogenesis to explore proper therapy strategies is clinically relevant. In the present study, male Sprague-Dawley rats underwent sham operation or common bile duct ligation (CBDL). Two weeks post-surgery, the following groups were set up for 2 weeks of treatment: sham + normal saline, CBDL + CXCR2 antagonist SB225002, CBDL + tumour necrosis factor α (TNF-α) antagonist PTX and CBDL + normal saline groups...
January 1, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/27851335/1700-va-ecmo-for-a-pediatric-patient-with-worsening-hepatopulmonary-syndrome-following-liver-transplant
#11
Laura Schroeder, Gregory Yurasek, Khalid Khan, David Steinhorn
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27816135/anesthesia-for-patients-with-concomitant-hepatic-and-pulmonary-dysfunction
#12
REVIEW
Geraldine C Diaz, Michael F O'Connor, John F Renz
Hepatic function and pulmonary function are interrelated with failure of one organ system affecting the other. With improved therapies, patients with concomitant hepatic and pulmonary failure increasingly enjoy a good quality of life and life expectancy. Therefore, the prevalence of such patients is increasing with more presenting for both emergent and elective surgical procedures. Hypoxemia requires a thorough evaluation in patients with end-stage liver disease. The most common etiologies respond to appropriate therapy...
December 2016: Anesthesiology Clinics
https://www.readbyqxmd.com/read/27777895/hypoxic-hepatitis-a-review-and-clinical-update
#13
REVIEW
Najeff Waseem, Po-Hung Chen
Hypoxic hepatitis (HH), also known as ischemic hepatitis or shock liver, is characterized by a massive, rapid rise in serum aminotransferases resulting from reduced oxygen delivery to the liver. The most common predisposing condition is cardiac failure, followed by circulatory failure as occurs in septic shock and respiratory failure. HH does, however, occur in the absence of a documented hypotensive event or shock state in 50% of patients. In intensive care units, the incidence of HH is near 2.5%, but has been reported as high as 10% in some studies...
September 28, 2016: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/27721918/implication-of-the-intestinal-microbiome-in-complications-of-cirrhosis
#14
REVIEW
Mamatha Bhat, Bianca M Arendt, Venkat Bhat, Eberhard L Renner, Atul Humar, Johane P Allard
The intestinal microbiome (IM) is altered in patients with cirrhosis, and emerging literature suggests that this impacts on the development of complications. The PubMed database was searched from January 2000 to May 2015 for studies and review articles on the composition, pathophysiologic effects and therapeutic modulation of the IM in cirrhosis. The following combination of relevant text words and MeSH terms were used, namely intestinal microbiome, microbiota, or dysbiosis, and cirrhosis, encephalopathy, spontaneous bacterial peritonitis, hepatorenal syndrome, variceal bleeding, hepatopulmonary syndrome, portopulmonary hypertension and hepatocellular carcinoma...
September 28, 2016: World Journal of Hepatology
https://www.readbyqxmd.com/read/27720430/the-role-of-splenectomy-before-liver-transplantation-in-biliary-atresia-patients
#15
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/27650268/biliary-atresia-indications-and-timing-of-liver-transplantation-and-optimization-of-pretransplant-care
#16
REVIEW
Shikha S Sundaram, Cara L Mack, Amy G Feldman, Ronald J Sokol
Biliary atresia (BA) is a progressive, fibro-obliterative disorder of the intrahepatic and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation (LT). Indications for LT in BA include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extrahepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for LT...
January 2017: Liver Transplantation
https://www.readbyqxmd.com/read/27580545/pulmonary-arterial-hypertension-associated-with-congenital-portosystemic-shunts-treated-with-transcatheter-embolization-and-pulmonary-vasodilators
#17
Haruka Sato, Masanobu Miura, Nobuhiro Yaoita, Saori Yamamoto, Shunsuke Tatebe, Tatsuo Aoki, Kimio Satoh, Hideki Ota, Kei Takase, Koichiro Sugimura, Hiroaki Shimokawa
Cardiopulmonary abnormalities are often present in patients with liver diseases. We herein report a case of congenital portosystemic shunts complicated by hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH). A 57-year-old woman complained of dyspnea and was subsequently diagnosed with HPS and PoPH caused by congenital portosystemic shunts. Although shunt closure by transcatheter embolization was successfully performed, her dyspnea worsened and pulmonary artery pressure and pulmonary vascular resistance elevated...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27509319/microrna-196a2-rs11614913-c%C3%A2-%C3%A2-t-polymorphism-correlates-with-an-increased-risk-of-hepatopulmonary-syndrome-in-liver-cirrhosis-a-case-control-study-in-china
#18
Hai-Yong Chen, Yao-Min Chen, Jian Wu, Fu-Chun Yang, Zhen Lv, Xiao-Feng Xu, Shu-Sen Zheng, Sang-Sang Liao, Yi-Hui Luo
OBJECTIVE: This case-control study is designed to explore the relationship between microRNA-196a2 (miR-196a2) rs11614913 C > T polymorphism and the risk of hepatopulmonary syndrome (HPS) in liver cirrhosis METHODS: From January 2013 to January 2015, 163 liver cirrhosis patients with HPS (case group), 264 liver cirrhosis patients without HPS (control group) and 195 healthy people (normal group) were selected. DNA extraction Kit was used to extract plasma DNA from peripheral blood...
August 10, 2016: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/27504083/very-early-presentation-of-extrahepatic-portal-vein-obstruction-causing-portal-hypertension-in-an-infant-uncertainties-in-the-management-and-therapeutic-limitations
#19
Parisá Khodayar-Pardo, Andrés Peña Aldea, Ana Ramírez González, Adela Meseguer Carrascosa, Cristina Calabuig Bayo
Extrahepatic portal vein obstruction, although rare in children, is a significant cause of portal hypertension (PHT) leading to life-threatening gastrointestinal bleeding in the pediatric age group. PHT may also lead to other complications such as hyperesplenism, cholangyopathy, ascites, and even hepatopulmonary syndrome and portopulmonary hypertension that may require organ transplantation. Herein we report the case of an asymptomatic 11-month-old infant wherein a hepatomegaly and cavernous transformation of the portal vein was detected by liver ultrasound...
May 2016: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/27484427/-a-man-with-slowly-progressive-dyspnea-on-exertion
#20
S T Laurens, S O Simons, A I J Arens
A 42-year-old man with a medical history of liver cirrhosis and portal hypertension was admitted to the hospital because of slowly progressive shortness of breath and hypoxemia. The diagnosis hepatopulmonary syndrome was confirmed by a pulmonary perfusion scan with 99m Tc-albumin. The scan showed a right-left shunt, because the 99m Tc-albumin transited the lungs and appeared in the brain, the thyroid gland, the kidneys and the spleen. The patient received a liver transplantation, which is considered the only definitive treatment...
2016: Nederlands Tijdschrift Voor Geneeskunde
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