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Congenital hepatic fibrosis

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https://www.readbyqxmd.com/read/28302006/transplantation-for-large-liver-tumors-in-the-setting-of-abernethy-malformation
#1
İlgin Özden, Ayşen Yavru, Mine Güllüoğlu, Aydin Alper, Orhan Bilge, Serdar Cantez, Özlem Durmaz
In this study, we report our experiences on the role of transplantation in 2 patients with large liver tumors in the setting of Abernethy malformation. Patient 1 was a 17-year-old boy who was referred for hepatic masses and recurrent hepatic encephalopathy episodes. Computed tomography and magnetic resonance imaging showed 2 large tumors (4 and 8 cm) in the liver. The portal vein drained directly into the vena cava. Core biopsy of the larger mass revealed fibrosis and regenerative hyperplasia. There were hyperintense signals in the T1-weighted images in the globus pallidus...
March 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28260452/unusual-indications-for-a-liver-transplant-a-single-center-experience
#2
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/28253873/extended-clinical-features-associated-with-novel-glis3-mutation-a-case-report
#3
K A Alghamdi, A B Alsaedi, A Aljasser, A Altawil, Naglaa M Kamal
BACKGROUND: Mutations in the GLI-similar 3 (GLIS3) gene encoding the transcription factor GLIS3 are a rare cause of neonatal diabetes and congenital hypothyroidism with 12 reported patients to date. Additional features, previously described, include congenital glaucoma, hepatic fibrosis, polycystic kidneys, developmental delay, facial dysmorphism, osteopenia, sensorineural deafness, choanal atresia, craniosynostosis and pancreatic exocrine insufficiency. CASE PRESENTATION: We report a new case for consanguineous parents with homozygous novel mutation in GLIS3 gene who presented with neonatal diabetes mellitus, severe resistant congenital hypothyroidism, cholestatic liver disease, bilateral congenital glaucoma and facial dysmorphism...
March 2, 2017: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/28247771/poems-syndrome-and-idiopathic-portal-hypertension-a-possible-association
#4
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/28099341/laparoscopic-distal-splenoadrenal-shunt-for-the-treatment-of-portal-hypertension-in-children-with-congenital-hepatic-fibrosis-a-case-report
#5
Jin-Shan Zhang, Wei Cheng, Long Li
BACKGROUND: The distal splenorenal shunt is an effective procedure for the treatment of portal hypertension in children. However, there has been no report about laparoscopic distal splenorenal shunt in the treatment of portal hypertension in children. METHODS: From December 2015 to August 2016, 4 children with upper gastrointestinal bleeding underwent laparoscopic distal splenoadrenal shunt. Portal hypertension and splenomegaly were demonstrated on the preoperative computed tomography (CT) and sonography...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27956812/pediatric-living-donor-liver-transplantation-for-congenital-hepatic-fibrosis-using-a-mother-s-graft-with-von-meyenburg-complex-a-case-report
#6
Naoya Yamada, Yukihiro Sanada, Takumi Katano, Masahisa Tashiro, Yuta Hirata, Noriki Okada, Yoshiyuki Ihara, Atsushi Miki, Hideki Sasanuma, Taizen Urahashi, Yasunaru Sakuma, Koichi Mizuta
This is the first report of living donor liver transplantation (LDLT) for congenital hepatic fibrosis (CHF) using a mother's graft with von Meyenburg complex. A 6-year-old girl with CHF, who suffered from recurrent gastrointestinal bleeding, was referred to our hospital for liver transplantation. Her 38-year-old mother was investigated as a living donor and multiple biliary hamartoma were seen on her computed tomography and magnetic resonance imaging scan. The mother's liver function tests were normal and she did not have any organ abnormality, including polycystic kidney disease...
November 28, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27955914/liver-health-in-adults-with-fontan-circulation-a-multicenter-cross-sectional-study
#7
Fred M Wu, Brian Kogon, Michael G Earing, Jamil A Aboulhosn, Craig S Broberg, Anitha S John, Amy Harmon, Nisha I Sainani, Andrew J Hill, Robert D Odze, Melanie E Johncilla, Chinweike Ukomadu, Kimberlee Gauvreau, Anne Marie Valente, Michael J Landzberg
OBJECTIVES: Liver disease is an important contributor to morbidity and mortality in patients after Fontan surgery. There has been no large-scale survey of liver health in this population. We sought to explore the prevalence and predictors of liver disease in a multicenter cohort of adults with Fontan physiology. METHODS: Subjects were recruited from 6 adult congenital heart centers. Demographics; clinical history; and laboratory, imaging, and histopathology data were obtained...
November 12, 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27938556/-hereditary-polycystic-kidney-disease-a-neglected-etiology-of-liver-cirrhosis
#8
Y J Wu, H G Ding
There are two common types of hereditary polycystic kidney diseases, autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease. Congenital hepatic fibrosis is an autosomal recessive disorder and can occur in hereditary polycystic kidney disease. Therefore, hereditary polycystic kidney disease is one of the causes of unexplained liver fibrosis and liver cirrhosis.
October 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/27891514/evidence-for-a-pathogenic-triumvirate-in-congenital-hepatic-fibrosis-in-autosomal-recessive-polycystic-kidney-disease
#9
REVIEW
Lu Jiang, Pingping Fang, James L Weemhoff, Udayan Apte, Michele T Pritchard
Autosomal recessive polycystic kidney disease (ARPKD) is a severe monogenic disorder that occurs due to mutations in the PKHD1 gene. Congenital hepatic fibrosis (CHF) associated with ARPKD is characterized by the presence of hepatic cysts derived from dilated bile ducts and a robust, pericystic fibrosis. Cyst growth, due to cyst wall epithelial cell hyperproliferation and fluid secretion, is thought to be the driving force behind disease progression. Liver fibrosis is a wound healing response in which collagen accumulates in the liver due to an imbalance between extracellular matrix synthesis and degradation...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27789099/1-carbon-cycle-metabolites-methylate-their-way-to-fatty-liver
#10
REVIEW
Amy Karol Walker
Fatty liver is a complex disease often accompanying metabolic syndrome and Type 2 diabetes mellitus (T2DM). Hepatosteatosis may have roots in multiple metabolic abnormalities. However, metabolic dysfunction in the 1-carbon cycle (1CC), which produces the methyl donor S-adenosylmethionine (SAM) and phosphatidylcholine (PC), induces hepatic lipogenesis in model systems. Human diseases where 1CC or PC synthesis is disrupted, such as alcoholism, congenital lipodystrophy, or cystic fibrosis, often present with fatty liver...
January 2017: Trends in Endocrinology and Metabolism: TEM
https://www.readbyqxmd.com/read/27785575/diagnosis-of-joubert-syndrome-via-ultrasonography
#11
Baris Buke, Emre Canverenler, Göksun İpek, Semiha Canverenler, Hatice Akkaya
Joubert syndrome (JS) and related disorders (JSRD) are a group of multiple congenital anomaly syndromes in which the diagnostic hallmark is the molar tooth sign (MTS), a complex midbrain malformation visible on brain imaging. Detection of the MTS should be followed by a diagnostic protocol to assess multi-organ involvement. The incidence of JSRD ranges between 1/80,000 and 1/100,000 live births, although these values may represent an underestimate. The neurological components of JSRD include hypotonia, ataxia, intellectual disability, abnormal eye movements, and neonatal breathing problems...
October 26, 2016: Journal of Medical Ultrasonics
https://www.readbyqxmd.com/read/27592026/predictive-value-of-biomarkers-of-hepatic-fibrosis-in-adult-fontan-patients
#12
Fred M Wu, Michael G Earing, Jamil A Aboulhosn, Melanie E Johncilla, Michael N Singh, Robert D Odze, Chinweike Ukomadu, Kimberlee Gauvreau, Michael J Landzberg, Anne Marie Valente
BACKGROUND: Hepatic fibrosis is highly prevalent in individuals with Fontan circulation. FibroSure (LabCorp, Burlington, NC) and hyaluronic acid (HA) have been validated for assessment of hepatic fibrosis in several forms of liver disease. We sought to determine whether these tests could identify Fontan patients with advanced hepatic fibrosis or cirrhosis. METHODS: Subjects who had liver biopsy and FibroSure or HA testing within 6 months of biopsy were identified from the Alliance for Adult Research in Congenital Cardiology Fontan Liver Health study...
July 27, 2016: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/27553859/outcomes-associated-with-fetal-hepatobiliary-cysts-a-systematic-review-and-meta-analysis
#13
Martina Leombroni, Danilo Buca, Claudio Celentano, Marco Liberati, Francesca Bascietto, Sarah Gustapane, Luisa Marrone, Lamberto Manzoli, Giuseppe Rizzo, Francesco D'Antonio
OBJECTIVES: To explore the outcome of fetal hepatobiliary cysts. METHODS: Medline and databases were searched. The outcomes observed were: resolution/reduction and increase in cyst size, associated congenital anomalies of the biliary tract and liver, abnormal post-natal liver function tests, clinical symptoms, need for surgery, post-surgical complications and predictive accuracy. Meta-analyses of proportions were used to analyse data. RESULTS: 1483 articles were identified and 22 studies (252 fetuses) included in the systematic review...
August 23, 2016: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27524533/congenital-hepatic-fibrosis-clinical-presentation-laboratory-features-and-management-at-a-tertiary-care-hospital-of-lahore
#14
Arit Parkash, Huma Arshad Cheema, Hassan Suleman Malik, Zafar Fayyaz
OBJECTIVE: To describe the clinical presentations, laboratory features and management of congenital hepatic fibrosis patients at a tertiary care hospital. METHODS: The case series was conducted at The Children Hospital and Institute of Child Health, Lahore, Pakistan, from July 2013 to June 2015, and comprised patients of congenital hepatic fibrosis diagnosed on the basis of liver biopsy. SPSS 20 was used for statistical analysis. RESULTS: The mean age of 25 patients in the study was 8...
August 2016: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/27512163/anaesthesia-and-intensive-care-for-simultaneous-liver-kidney-transplantation-a-single-centre-experience-with-12-recipients
#15
Akila Rajakumar, Shiwalika Gupta, Selvakumar Malleeswaran, Joy Varghese, Ilankumaran Kaliamoorthy, Mohamed Rela
BACKGROUND AND AIMS: The perioperative management of patients presenting for simultaneous liver and kidney transplantation (SLKT) is a complex process. We analysed SLKTs performed in our institution to identify preoperative, intraoperative and post-operative challenges encountered in the management. METHODS: We retrospectively studied the case records of 12 patients who underwent SLKT between 2009 and 2014 and analysed details of pre-operative evaluation and optimisation, intraoperative anaesthetic management and the implications of use of perioperative continuous renal replacement therapy (CRRT) and the post-operative course of these patients...
July 2016: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/27177892/a-glasgow-tipple-transjugular-intrahepatic-portosystemic-shunt-insertion-prior-to-whipple-resection
#16
Salman A A Jabbar, Nigel B Jamieson, Andrew J Morris, Karin A Oien, Fraser Duthie, Colin J McKay, Christopher R Carter, Euan J Dickson
Abdominal surgery performed in patients with significant liver disease and portal hypertension is associated with high mortality rates, with even poorer outcomes associated with complex pancreaticobiliary operations. We report on a patient requiring portal decompression via transjugular intrahepatic portosystemic shunt (TIPS) prior to a pancreaticoduodenectomy. The 49-year-old patient presented with pain, jaundice and weight loss. At ERCP an edematous ampulla was biopsied, revealing high-grade dysplasia within a distal bile duct adenoma...
2016: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/27148679/an-emerging-recognizable-facial-phenotype-in-association-with-mutations-in-gli-similar-3-glis3
#17
Paul Dimitri, Elisa De Franco, Abdelhadi M Habeb, Fatih Gurbuz, Khairya Moussa, Doris Taha, Jerry K H Wales, Jacob Hogue, Anne Slavotinek, Ambika Shetty, Meena Balasubramanian
Neonatal diabetes and hypothyroidism (NDH) syndrome was first described in 2003 in a consanguineous Saudi Arabian family where two out of four siblings were reported to have presented with proportionate IUGR, neonatal non-autoimmune diabetes mellitus, severe congenital hypothyroidism, cholestasis, congenital glaucoma, and polycystic kidneys. Liver disease progressed to hepatic fibrosis. The renal disease was characterized by enlarged kidneys and multiple small cysts with deficient cortico-medullary junction differentiation and normal kidney function...
July 2016: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/27097579/-clinical-features-of-different-clinical-forms-of-childhood-congenital-hepatic-fibrosis
#18
Xin Wu, Xiao-Rang DU, Jin-Fang Ding, Meng-Jin Wu, Sheng-Qiang Luo, Xing-Zhong Feng
OBJECTIVE: To compare the clinical features of children with different clinical forms of congenital hepatic fibrosis (CHF), and provides a description of the characteristics of childhood CHF. METHODS: Sixty children with CHF between January 2002 and June 2015 were enrolled, including 26 children with portal hypertensive CHF (PH CHF), 3 children with cholangitic CHF, 30 children with combined portal hypertensive and cholangitic CHF (mixed CHF), and 1 child with latent forms of CHF...
April 2016: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/27008370/right-and-left-ventricular-assist-devices-support-and-liver-dysfunction-prognostic-and-therapeutic-implications
#19
REVIEW
Simon Maltais, John M Stulak
PURPOSE OF REVIEW: Advanced heart failure is a chronic, systemic disease, which affects other organs in ways that are incompletely understood. Patients with advanced heart failure commonly develop congestive hepatopathy, which may lead to progressive hepatic injury and fibrosis. Because the independent adverse effects of hepatic dysfunction on early and late outcomes after continuous flow left ventricular assist devices have also not been entirely demonstrated, we review its impact on this patient population...
May 2016: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/26968005/a-safety-evaluation-of-ivacaftor-for-the-treatment-of-cystic-fibrosis
#20
REVIEW
Susanna A McColley
INTRODUCTION: Ivacaftor is indicated for treatment of cystic fibrosis (CF) mediated by 10 mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene that causes gating or partial function abnormalities. In placebo-controlled and open-label studies, ivacaftor-treated subjects showed improved pulmonary function, nutrition and quality of life measures. This article reviews ivacaftor safety. AREAS COVERED: Safety findings in ivacaftor clinical trials, and reported subsequently, were accessed by a PubMed search using key words "VX-770" or "ivacaftor"...
May 2016: Expert Opinion on Drug Safety
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