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Congenital hepatic fibrosis

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
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
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
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
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
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
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
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
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
Sunil Pawar, Vinay Zanwar, Ashok Mohite, Ravindra Surude, Pravin Rathi, Meenakshi Balasubramani
Congenital hepatic fibrosis is a rare cause of portal hypertension and esophageal varices in children. We report cases of siblings with biopsy proven congenital hepatic fibrosis and with atypical retinitis pigmentosa. They presented with repeated episodes of jaundice along with progressive decrease of vision in night. They had hepatosplenomegaly and portal hypertension with esophageal varices. One of the siblings had a large regenerating nodule replacing the entire right lobe of the liver and other one developed repeated hematemesis...
November 5, 2015: Clinics and Practice
F B Li, H Zhao, K R Peng, Z G Gao, S J Huang, J F Tou, X L Shu, W Z Gu
We investigated the expression of transforming growth factor-β1 (TGF-β1) and connective tissue growth factor (CTGF) in the liver tissue of infants with congenital biliary atresia and neonatal hepatitis, as well as the relationship between the expression of the two factors and liver fibrosis. Thirty-six infants who met the cholestasis criteria were classified into congenital biliary atresia and neonatal hepatitis groups. All specimens were stained with hematoxylin and eosin and Masson's trichrome, and the degree of liver fibrosis was assessed...
2016: Genetics and Molecular Research: GMR
Hidenori Yasuda, Kohei Amakasu, Yuki Tochigi, Kentaro Katayama, Hiroetsu Suzuki
Renal hypoplasia due to a congenitally reduced number of nephrons progresses to chronic kidney disease and may cause renal anemia, given that the kidneys are a major source of erythropoietin in adults. Hypoplastic kidney (HPK) rats have only about 20% of the normal number of nephrons and develop CKD. This study assessed the renal function and hematologic changes in HPK rats from 70 to 210 d of age. HPK rats demonstrated deterioration of renal excretory function, slightly macrocytic erythropenia at all days examined, age-related increases in splenic hemosiderosis accompanied by a tendency toward increased hemolysis, normal plasma erythropoietin levels associated with increased hepatic and decreased renal erythropoietin production, and maintenance of the response for erythropoietin production to hypoxic conditions, with increased interstitial fibrosis at 140 d of age...
February 2016: Comparative Medicine
Gloria Rossi, Teresa di Chio, Silvia Nastasio, Giuseppe Maggiore
No abstract text is available yet for this article.
February 4, 2016: Journal of Pediatric Gastroenterology and Nutrition
Luigi Locatelli, Massimiliano Cadamuro, Carlo Spirlì, Romina Fiorotto, Silvia Lecchi, Carola Maria Morell, Yury Popov, Roberto Scirpo, Maria De Matteis, Mariangela Amenduni, Andrea Pietrobattista, Giuliano Torre, Detlef Schuppan, Luca Fabris, Mario Strazzabosco
UNLABELLED: Congenital hepatic fibrosis (CHF) is a disease of the biliary epithelium characterized by bile duct changes resembling ductal plate malformations and by progressive peribiliary fibrosis, in the absence of overt necroinflammation. Progressive liver fibrosis leads to portal hypertension and liver failure; however, the mechanisms leading to fibrosis in CHF remain elusive. CHF is caused by mutations in PKHD1, a gene encoding for fibrocystin, a ciliary protein expressed in cholangiocytes...
March 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Zeynep Ersoy, Coşkun Araz, Mahir Kırnap, Pınar Zeyneloğlu, Adnan Torgay, Gülnaz Arslan
Primary hyperoxaluria type 1 is an autosomal recessive disorder that is responsible for the overproduction of oxalate and has an incidence of 1 in 120 000 live births. Indications for combined liver and kidney transplant are still debated. However, combined liver and kidney transplant is preferred in various conditions, including primary hyperoxaluria, liver-based metabolic abnormalities affecting the kidney, and structural diseases affecting both the liver and the kidney, such as congenital hepatic fibrosis and polycystic kidney disease...
November 2015: Experimental and Clinical Transplantation
Canhong Yang, Zhihua Liu
No abstract text is available yet for this article.
June 2015: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
Michael Hagan, Sumeet K Asrani, Jayant Talwalkar
Over the past decade, several advances have been made in the non-invasive assessment of liver fibrosis. Both serum markers and imaging-based tissue elastography predict the presence of advanced fibrosis compared with liver biopsy. Serum markers may be indirect or direct markers of liver structure and function. Imaging-based techniques measure liver stiffness as a surrogate for fibrosis and include ultrasound and MRI-based methods. Most non-invasive techniques work well at identifying subjects at the extremes of fibrosis but may not accurately discern intermediate stages...
2015: Expert Review of Gastroenterology & Hepatology
Iolanda Ribeiro, Rolando Pinho, Adélia Rodrigues, Luís Alberto, Carlos Fernandes, João Carvalho
No abstract text is available yet for this article.
September 2015: Revista Española de Enfermedades Digestivas
Maitham A Moslim, Ganesh Gunasekaran, David Vogt, Michael Cruise, Gareth Morris-Stiff
BACKGROUND: Caroli's disease is a rare congenital condition characterized by non-obstructive dilatation of intrahepatic ducts. In Caroli's syndrome, there is additionally an associated congenital hepatic fibrosis. METHODS: With institutional review board approval, we identified all patients with Caroli's disease and syndrome. RESULTS: Nine patients were identified, seven males and two females, with a median age of 40 years. Final pathological diagnoses included Caroli's disease (n = 6) and Caroli's syndrome (n = 3)...
November 2015: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
Eujin Park, Jiwon M Lee, Yo Han Ahn, Hee Gyung Kang, I I Soo Ha, Joo Hoon Lee, Young Seo Park, Nayoung K D Kim, Woong-Yang Park, Hae Ii Cheong
BACKGROUND: Hepatorenal fibrocystic diseases (HRFCDs) are a group of monogenic disorders characterized by developmental abnormalities involving the liver and kidney. In this study, we performed genotype and phenotype analyses of children with HRFCDs to determine the distribution of underlying diseases. METHODS: A total of 36 children with HRFCDs were recruited, with genetic tests being performed in 22 patients and 14 patients diagnosed clinically as having autosomal recessive polycystic kidney disease (ARPKD)...
January 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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