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https://www.readbyqxmd.com/read/28086841/good-outcome-following-liver-transplantation-using-pericardial-peritoneum-window-for-hepato-atrial-anastomosis-to-overcome-advanced-hepatic-alveolar-echinococcosis-and-secondary-budd-chiari-syndrome-a-case-report
#1
Konrad Kobryń, Rafał Paluszkiewicz, Krzysztof Dudek, Urszula Ołdakowska-Jedynak, Michał Korba, Joanna Raszeja-Wyszomirska, Piotr Remiszewski, Michał Grąt, Piotr Milkiewicz, Waldemar Patkowski, Marek Krawczyk
BACKGROUND: This report presents a case of a 57- year old female with advanced Hepatic Alveolar Echinococcosis causing a secondary Budd-Chiari Syndrome due to infiltration of the suprahepatic inferior vena cava treated successfully by liver transplantation. CASE PRESENTATION: A temporary veno-venous bypass was introduced, but a typical end to end cavo-caval anastomosis wasn't possible in this case. In order to access a disease free part of the inferior vena cava, an oval window of the diaphragm was excised, providing communication between the peritoneum and pericardium...
January 13, 2017: BMC Surgery
https://www.readbyqxmd.com/read/28073417/-a-case-of-budd-chiari-syndrome-misdiagnosed-as-liver-cancer
#2
J J Wang, X Ren, H Wang
No abstract text is available yet for this article.
December 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/28068425/predictive-factors-for-pressure-ulcers-in-an-older-adult-population-hospitalized-for-hip-fractures-a-prognostic-cohort-study
#3
Paolo Chiari, Cristiana Forni, Monica Guberti, Domenica Gazineo, Sabrina Ronzoni, Fabio D'Alessandro
BACKGROUND: Older adult patients with fragility hip fractures constitute a population at high risk for complications, in particular pressure ulcers. The aim was to evaluate the incidence of pressure ulcers and potential predictive factors. METHODS AND FINDINGS: A prospective multicentric prognostic cohort study in orthopedic wards in three Italian public hospitals. Participants were all consecutive patients 65 years of age or older diagnosed with a fragility hip fracture...
2017: PloS One
https://www.readbyqxmd.com/read/28058764/diagnosis-and-treatment-guidelines-for-aberrant-portal-hemodynamics
#4
Fuminori Moriyasu, Yoshihiro Furuichi, Atsushi Tanaka, Hajime Takikawa, Hiroshi Yoshida, Isao Sakaida, Katsutoshi Obara, Makoto Hashizume, Masayoshi Kage, Satoko Ohfuji, Seigo Kitano, Seiji Kawasaki, Shigehiro Kokubu, Shoichi Matsutani, Susumu Eguchi, Susumu Shiomi, Tetsuhito Kojima, Yoshihiko Maehara, Yukio Kuniyoshi
Idiopathic portal hypertension (IPH), causing aberrant portal hemodynamics, is a disease with an as yet unidentified cause and no treatment has been established so far. The Japanese research group on IPH in Japan was set up in 1975 by the Ministry of Health, Labour and Welfare, furthermore extra-hepatic portal obstruction (EHO) and Budd-Chiari syndrome (BCS) have been added to the research subjects and further work has continued. The aims of the research group are to accurately evaluate the current status of the three diseases in Japan, elucidate the disease aetiology and pathogenesis, and develop new treatments...
January 6, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28058703/fatal-outcome-of-haemodialysis-catheter-malposition-into-the-hepatic-vein-causing-budd-chiari-syndrome
#5
Mohammad Ali Husainy, Rajdeep Chhina, Riad Alchanan, Praveen Peddu
No abstract text is available yet for this article.
January 3, 2017: Journal of Vascular Access
https://www.readbyqxmd.com/read/28052881/headache-outcomes-in-children-undergoing-foramen-magnum-decompression-for-chiari-i-malformation
#6
Saba Raza-Knight, Kshitij Mankad, Prab Prabhakar, Dominic Thompson
OBJECTIVE: A common symptom of Chiari I malformation (CIM) is headache, which is diagnosed using non-validated criteria from the International Headache Society (IHS). CIM-associated headaches should resolve following neurosurgical treatment of the malformation by foramen magnum decompression (FMD). We aimed to validate the IHS criteria and determine (1) the efficacy of FMD in treating headache and (2) whether duraplasty confers an advantage over simple bony decompression in the treatment of this symptom...
January 4, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28049537/imperforated-cor-triatriatum-dexter-in-a-dog-with-concurrent-caudal-vena-cava-wall-mineralization
#7
Tetyda Paulina Dobak, Gregory Starrak, Kathleen Linn, Elisabeth Christine Roberston Snead
BACKGROUND: Cor triatriatum dexter (CTD) is a rare congenital cardiac malformation with various manifestations and has been sporadically described in dogs. Clinically the dogs present with nonspecific signs of right heart failure or Budd-Chiari-like syndrome. Other associated concurrent cardiovascular anomalies are commonly reported. Diagnosis and full characterization of this complex malformation requires careful investigation and often a multimodal imaging approach. CASE PRESENTATION: A 10-week-old, male intact, Golden Retriever was presented with clinical signs of stunted growth, anorexia, and progressive ascites...
January 3, 2017: Acta Veterinaria Scandinavica
https://www.readbyqxmd.com/read/28036062/an-unusual-case-of-post-traumatic-headache-complicated-by-intracranial-hypotension
#8
Sara Siavoshi, Carrie Dougherty, Jessica Ailani, Kaustubh Yadwadkar, Frank Berkowitz
We present a case of post-traumatic headache complicated by intracranial hypotension resulting in an acquired Chiari malformation and myelopathy with syringomyelia. This constellation of findings suggest a possible series of events that started with a traumatic cerebral spinal fluid (CSF) leak, followed by descent of the cerebellar tonsils and disruption of CSF circulation that caused spinal cord swelling and syrinx. This unusual presentation of post-traumatic headache highlights the varying presentations and the potential sequelae of intracranial hypotension...
December 29, 2016: Brain Sciences
https://www.readbyqxmd.com/read/28029524/-external-lumbar-drainage-with-volumetric-continuing-infusion-pump-in-patients-with-cerebrospinal-fluid-leak-a-case-series
#9
Ana Belén Manso Melgosa, Helena García Gutiérrez, Mónica Fernández Porras, Ana Berta Castrillo Manero, Belén Pérez Marijuán
OBJECTIVE: To describe the incidence and complications arising in a number of cases of patients with cerebrospinal fluid leak treated by external lumbar drainage with infusion pump (IP) volumetric continuous from 2001 to 2014. Quantify cerebrospinal fluid leak closed by lumbar drainage with IP. METHODS: Retrospective descriptive case series study. POPULATION: patients undergoing transsphenoidal pituitary surgery, Chiari surgery and laminectomy, that developed postoperative cerebrospinal fluid leak treated with continuous external lumbar drainage by IP...
October 28, 2016: Enfermería Clínica
https://www.readbyqxmd.com/read/28018608/digenic-mutations-of-human-ocrl-paralogs-in-dent-s-disease-type-2-associated-with-chiari-i-malformation
#10
Daniel Duran, Sheng Chih Jin, Tyrone DeSpenza, Carol Nelson-Williams, Andrea G Cogal, Elizabeth W Abrash, Peter C Harris, John C Lieske, Serena Je Shimshak, Shrikant Mane, Kaya Bilguvar, Michael L DiLuna, Murat Günel, Richard P Lifton, Kristopher T Kahle
OCRL1 and its paralog INPP5B encode phosphatidylinositol 5-phosphatases that localize to the primary cilium and have roles in ciliogenesis. Mutations in OCRL1 cause the X-linked Dent disease type 2 (DD2; OMIM# 300555), characterized by low-molecular weight proteinuria, hypercalciuria, and the variable presence of cataracts, glaucoma and intellectual disability without structural brain anomalies. Disease-causing mutations in INPP5B have not been described in humans. Here, we report the case of an 11-year-old boy with short stature and an above-average IQ; severe proteinuria, hypercalciuria and osteopenia resulting in a vertebral compression fracture; and Chiari I malformation with cervico-thoracic syringohydromyelia requiring suboccipital decompression...
2016: Human Genome Variation
https://www.readbyqxmd.com/read/28018469/type-i-chiari-malformation-presenting-orthostatic-syncope-who-treated-with-decompressive-surgery
#11
Hyun-Seung Shin, Jeong A Kim, Dong-Seok Kim, Joon Soo Lee
Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical symptoms associated with CM-I, including suboccipital headache, weakness in the upper extremities, facial numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28006871/budd-chiari-syndrome-and-acute-liver-failure-a-complex-condition-requiring-a-rapid-response
#12
EDITORIAL
John G O'Grady
No abstract text is available yet for this article.
December 22, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/27991732/acute-myeloid-leukemia-associated-dnmt3a-p-arg882his-mutation-in-a-patient-with-tatton-brown-rahman-overgrowth-syndrome-as-a-constitutional-mutation
#13
Rika Kosaki, Hiroshi Terashima, Masaya Kubota, Kenjiro Kosaki
DNA methylation plays a critical role in both embryonic development and tumorigenesis and is mediated through various DNA methyltransferases. Constitutional mutations in the de novo DNA methyltransferase DNMT3A cause a recently identified Tatton-Brown-Rahman overgrowth syndrome (TBRS). Somatically acquired mutations in DNMT3A are causally associated with acute myeloid leukemia (AML), and p.Arg882His represents the most prevalent hotspot. So far, no patients with TBRS have been reported to have subsequently developed AML...
January 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/27991500/high-frequency-of-diabetic-ketoacidosis-at-diagnosis-of-type-1-diabetes-in-italian-children-a-nationwide-longitudinal-study-2004-2013
#14
Valentino Cherubini, Edlira Skrami, Lucia Ferrito, Stefano Zucchini, Andrea Scaramuzza, Riccardo Bonfanti, Pietro Buono, Francesca Cardella, Vittoria Cauvin, Giovanni Chiari, Giuseppe D Annunzio, Anna Paola Frongia, Dario Iafusco, Ippolita Patrizia Patera, Sonia Toni, Stefano Tumini, Ivana Rabbone, Fortunato Lombardo, Flavia Carle, Rosaria Gesuita
This longitudinal population-based study analyses the frequency of diabetic ketoacidosis (DKA) at type 1 diabetes diagnosis in Italian children under 15 years of age, during 2004-2013. DKA was defined as absent (pH ≥ 7.30), mild/moderate (7.1 ≤ pH < 7.30) and severe (pH < 7.1). Two multiple logistic regression models were used to evaluate the time trend of DKA frequency considered as present versus absent and severe versus absent, adjusted for gender, age group and geographical area of residence at diagnosis...
December 19, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27989412/a-retrospective-analysis-of-the-utility-of-head-computed-tomography-and-or-magnetic-resonance-imaging-in-the-management-of-benign-macrocrania
#15
M Edward Haws, Luke Linscott, Cameron Thomas, Emily Orscheln, Rupa Radhakrishnan, Beth Kline-Fath
OBJECTIVE: To assess whether computed tomography (CT), magnetic resonance imaging (MRI), and neurosurgical evaluations altered the diagnosis or management of children diagnosed with benign macrocrania of infancy by ultrasonography (US). STUDY DESIGN: We queried our radiology database to identify patients diagnosed with benign macrocrania of infancy by US between 2006 and 2013. Medical records of those with follow-up CT/MRI were reviewed to determine clinical/neurologic status and whether or not CT/MRI imaging resulted in diagnosis of communicating hydrocephalus or required neurosurgical intervention...
December 15, 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27982292/2-nd-brazilian-consensus-on-chagas-disease-2015
#16
João Carlos Pinto Dias, Alberto Novaes Ramos, Eliane Dias Gontijo, Alejandro Luquetti, Maria Aparecida Shikanai-Yasuda, José Rodrigues Coura, Rosália Morais Torres, José Renan da Cunha Melo, Eros Antonio de Almeida, Wilson de Oliveira, Antônio Carlos Silveira, Joffre Marcondes de Rezende, Fabiane Scalabrini Pinto, Antonio Walter Ferreira, Anis Rassi, Abílio Augusto Fragata, Andréa Silvestre de Sousa, Dalmo Correia, Ana Maria Jansen, Glaucia Manzan Queiroz Andrade, Constança Felícia De Paoli de Carvalho Britto, Ana Yecê das Neves Pinto, Anis Rassi, Dayse Elisabeth Campos, Fernando Abad-Franch, Silvana Eloi Santos, Egler Chiari, Alejandro Marcel Hasslocher-Moreno, Eliane Furtado Moreira, Divina Seila de Oliveira Marques, Eliane Lages Silva, José Antonio Marin-Neto, Lúcia Maria da Cunha Galvão, Sergio Salles Xavier, Sebastião Aldo da Silva Valente, Noêmia Barbosa Carvalho, Alessandra Viana Cardoso, Rafaella Albuquerque E Silva, Veruska Maia da Costa, Simone Monzani Vivaldini, Suelene Mamede Oliveira, Vera da Costa Valente, Mayara Maia Lima, Renato Vieira Alves
Chagas disease is a neglected chronic condition with a high burden of morbidity and mortality. It has considerable psychological, social, and economic impacts. The disease represents a significant public health issue in Brazil, with different regional patterns. This document presents the evidence that resulted in the Brazilian Consensus on Chagas Disease. The objective was to review and standardize strategies for diagnosis, treatment, prevention, and control of Chagas disease in the country, based on the available scientific evidence...
December 2016: Revista da Sociedade Brasileira de Medicina Tropical
https://www.readbyqxmd.com/read/27981932/g6pd-deficiency-with-arnold-chiari-malformation
#17
Shilpi Verma, Pradeep Kumar Bhatia, Vandana Sharma, Priyanka Sethi, Yogendra Raj Singh
A neonate with glucose-6-phosphate dehydrogenase (G6PD) deficiency and Arnold-Chiari Malformation (ACM) type 2 underwent lumbar meningomyelocele (MMC) repair. Patients with G6PD deficiency are prone to develop haemolysis following any kind of oxidative stress and in ACM, there is a disturbed cranio-spinal pressure relationship. The neonate was managed under general anaesthesia with propofol for induction as well as for maintenance along with fentanyl and oxygen-nitrous mixture.
November 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/27973434/performance-evaluation-of-state-of-the-art-systems-for-physical-activity-classification-of-older-subjects-using-inertial-sensors-in-a-real-life-scenario-a-benchmark-study
#18
Muhammad Awais, Luca Palmerini, Alan K Bourke, Espen A F Ihlen, Jorunn L Helbostad, Lorenzo Chiari
The popularity of using wearable inertial sensors for physical activity classification has dramatically increased in the last decade due to their versatility, low form factor, and low power requirements. Consequently, various systems have been developed to automatically classify daily life activities. However, the scope and implementation of such systems is limited to laboratory-based investigations. Furthermore, these systems are not directly comparable, due to the large diversity in their design (e.g., number of sensors, placement of sensors, data collection environments, data processing techniques, features set, classifiers, cross-validation methods)...
December 11, 2016: Sensors
https://www.readbyqxmd.com/read/27966293/beh%C3%A3-et-syndrome-the-vascular-cluster
#19
Hasan Yazıcı, Emire Seyahi
Although skin-mucosa lesions are common in almost all patients with Behçet syndrome (BS), clinical properties may differ from one patient to another. Within BS, there are subsets with different organ involvement and hence probably different pathological pathways. These subsets can be described as a) solo skin-mucosa disease with no major organ involvement, b) eye disease, c) seronegative spondyloarthropathy-like disease (arthritis, enthesopathy, and folliculitis), d) Crohn-like disease, and finally the topic of this chapter: e) vascular disease...
November 17, 2016: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/27956913/myxoma-immediately-above-the-junction-of-the-inferior-vena-cava-and-the-right-atrium-a-rare-cause-of-budd-chiari-syndrome
#20
Vahid Mohammad Karimi, Amir Anushiravani, Mohammad Hossein Dabbaghmanesh, Massood Hosseinzadeh, Ali Reza Rasekhi, Mahmoud Zamirian, Amir Anushiravani
The Budd-Chiari syndrome (BCS) is a rare disorder caused by the obstruction of the hepatic veins or the inferior vena cava (IVC) at the suprahepatic level. This syndrome is developed by either hepatic vein thrombosis or mechanical venous obstruction and leads to centrilobular hepatic congestion with the subsequent development of fibrosis and cirrhosis. Intracardiac tumors have been rarely reported as a cause of the BCS. These tumors usually originate from the atrial septum. Very rarely, they arise either from the junction of the IVC and the right atrium or from the Eustachian valve...
July 6, 2016: Journal of Tehran Heart Center
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