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https://www.readbyqxmd.com/read/29350663/effectiveness-of-a-tailored-medical-support-to-overcome-the-barriers-to-education-treatment-and-good-metabolic-control-in-children-with-type-1-diabetes-from-ethnic-minorities
#1
Brunella Iovane, Antonina Marta Cangelosi, Ilaria Bonaccini, Carla Mastrorilli, Dora Di Mauro, Valentina Fainardi, Giovanni Chiari, Marilena Maltese, Carlo Caffarelli, Maurizio Vanelli
AIM: To analyze the effectiveness of a tailored medical support to help children from ethnic minorities to achieve the same good metabolic control of autochthonous peers with type-1 diabetes (T1D). METHODS: Children <10 years of age belonging to ethnic minority (EM) families (Group 1) were compared with autochthonous peers (Group 2) who received the diagnosis of T1D in 2014-2016. The Protocol for minorities included other than the standard protocol: booklets translated in ethnic minority languages; weekly visits at home or at school; family-guides; clinic visits supported by professional interpreters...
January 16, 2018: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/29348288/budd-chiari-syndrome-a-rare-and-life-threatening-complication-of-crohn-s-disease
#2
Camila C Simoes, Yezaz A Ghouri, Shehzad N Merwat, Heather L Stevenson
Budd-Chiari syndrome (BCS) is characterised by obstruction of hepatic venous outflow and may be triggered by the prothrombotic state associated with inflammatory bowel disease (IBD). We reported a case of Crohn's disease (CD) that presented with anasarca, ascites, symptomatic hepatomegaly, elevated liver enzymes, increased prothrombin time and low albumin. Oesophagogastroduodenoscopy and colonoscopy confirmed active CD. Abdominal CT showed hepatic vein thrombosis. Liver biopsy revealed severe perivenular sinusoidal dilation with areas of hepatocyte dropout, bands of hepatocyte atrophy and centrizonal fibrosis, suggestive of BCS...
January 17, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29345006/embryology-of-the-craniocervical-junction-and-posterior-cranial-fossa-part-i-development-of-the-upper-vertebrae-and-skull
#3
Mohammadali M Shoja, Rebecca Ramdhan, Chad J Jensen, Joshua Chern, W Jerry Oakes, R Shane Tubbs
Although the embryology of the posterior cranial fossa can have life altering effects on a patient, a comprehensive review on this topic is difficult to find in the peer-reviewed medical literature. Therefore, this review paper, using standard search engines, seemed timely. The embryology of the posterior cranial fossa is complex and relies on a unique timing of various neurovascular and bony elements. Derailment of these developmental processes can lead to a wide range of malformations such as the Chiari malformations...
January 18, 2018: Clinical Anatomy
https://www.readbyqxmd.com/read/29344999/the-newer-classifications-of-the-chiari-malformations-with-clarifications-an-anatomical-review
#4
Fatima Azahraa Haddad, Ibraheem Qaisi, Nagham Joudeh, Hamada Dajani, Fareed Jumah, Amjad Elmashala, Nimer Adeeb, Joshua J Chern, R Shane Tubbs
In 1891 Hans Chiari described a group of congenital hindbrain anomalies, which were eventually named after him. He classified these malformations into three types (Chiari malformations I, II, and III), and four years later added the Chiari IV malformation. However, numerous reports across the literature do not seem to fit Chiari's original descriptions of these malformations, so researchers have been encouraged to propose new classifications to encompass these variants (e.g., Chiari 0, Chiari1.5, and Chiari 3...
January 18, 2018: Clinical Anatomy
https://www.readbyqxmd.com/read/29344994/embryology-of-the-craniocervical-junction-and-posterior-cranial-fossa-part-ii-embryogenesis-of-the-hindbrain
#5
Mohammadali M Shoja, Chad J Jensen, Rebecca Ramdhan, Joshua Chern, W Jerry Oakes, R Shane Tubbs
Although pathology of the hindbrain and its derivatives can have life altering effects on a patient, a comprehensive review on its embryology is difficult to find in the peer-reviewed medical literature. Therefore, this review paper, using standard search engines, seemed timely. The embryology of the hindbrain is complex and relies on a unique timing of various neurovascular and bony elements. Derailment of these developmental processes can lead to a wide range of malformations such as the Chiari malformations...
January 18, 2018: Clinical Anatomy
https://www.readbyqxmd.com/read/29340812/partial-amniotic-carbon-dioxide-insufflation-paci-during-minimally-invasive-fetoscopic-interventions-on-fetuses-with-spina-bifida-aperta
#6
Miriam Ziemann, Rolf Fimmers, Anastasiia Khaleeva, Rainer Schürg, Markus A Weigand, Thomas Kohl
BACKGROUND: Percutaneous partial amniotic carbon dioxide insufflation (PACI) is one of the most important means for improving visualization during minimally invasive fetoscopic surgery of fetal spina bifida. The purpose of the present study was to analyze maternal and fetal safety aspects of PACI in a recent patient cohort and to present management improvements. METHODS: PACI under general materno-fetal anesthesia was performed during 65 interventions for fetoscopic patch coverage of fetal spina bifida aperta between 21 + 0 and 29 + 1 weeks of gestation...
January 16, 2018: Surgical Endoscopy
https://www.readbyqxmd.com/read/29340275/timing-of-transjugular-intrahepatic-portosystemic-shunt-for-budd-chiari-syndrome-an-italian-hepatologist-s-perspective
#7
Andrea Mancuso
Budd-Chiari syndrome (BCS) management flow-chart is derived from experts' opinion and is not evidence-based. Guidelines suggest BCS management should follow a stepwise strategy: medical therapy as first-line treatment, revascularization or transjugular intrahepatic portosystemic shunt (TIPS) if no response to medical therapy, and liver transplant as rescue therapy. Recent evidence suggests that only medical therapy results in a bad long-term outcome. The biggest criticism of guidelines is the indication that BCS should receive further treatment only when hemodynamic consequences of portal hypertension become clinically evident...
December 2017: Journal of Translational Internal Medicine
https://www.readbyqxmd.com/read/29327862/endoscopic-fenestration-of-a-posterior-fossa-arachnoid-cyst-to-treat-a-chiari-like-headache
#8
Matteo Vitali, Andrea Cattalani, Vincenzo M Grasso, Alessandro Bertuccio, Andrea Barbanera
No abstract text is available yet for this article.
January 10, 2018: Journal of Neurosurgical Sciences
https://www.readbyqxmd.com/read/29325958/novel-assessment-of-cerebrospinal-fluid-dynamics-by-time-spatial-labeling-inversion-pulse-magnetic-resonance-imaging-in-patients-with-chiari-malformation-type-i
#9
Tatsuya Ohtonari, Nobuharu Nishihara, Shinzo Ota, Akio Tanaka
OBJECTIVE: We investigated CSF dynamics at CCJ using Time-SLIP MRI to demonstrate the significance of ventral and dorsal combined CSF dynamics in assessing CSF flow disturbance in patients with CM-I. METHODS: Fifteen examinations were performed in nine cases of CM-I (three females patients; mean age, 24.7 years; age range, 11-46 years) before or after craniocervical decompression. The longitudinal maximum movement of the caudal edge of tagged mid-sagittal CSF at CCJ was measured as a LOM, and total on the ventral and dorsal sides was defined as total LOM...
January 8, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29316565/transjugular-intrahepatic-portosystemic-shunt-in-patients-with-portal-hypertension-patency-depends-on-coverage-and-interventionalist-s-experience
#10
Matthias Buechter, Paul Manka, Guido Gerken, Ali Canbay, Sandra Blomeyer, Axel Wetter, Jens Altenbernd, Alisan Kahraman, Jens M Theysohn
BACKGROUND AND AIMS: Transjugular intrahepatic portosystemic shunt (TIPS) is the treatment of choice in decompensated portal hypertension. TIPS revision due to thrombosis or stenosis increases morbidity and mortality. Our aim was to investigate patient- and procedure-associated risk factors for TIPS-revision. PATIENTS AND METHODS: We retrospectively evaluated 189 patients who underwent the TIPS procedure. Only patients who required TIPS revision within 1 year (Group I, 34 patients) and patients who did not require re-intervention within the first year (Group II [control group], 54 patients) were included...
January 9, 2018: Digestive Diseases
https://www.readbyqxmd.com/read/29316476/numerical-simulation-of-hemodynamics-in-membranous-obstruction-of-the-suprahepatic-inferior-vena-cava-based-on-a-subject-specific-budd-chiari-syndrome-model
#11
Deqiang Cheng, Yinping Zhuang, Qiqi Kou, Min Zhang, Yinghong Zhao, Cuiping Han, Jingjing Li, Yong Wang, Kai Xu, Fei Mo, Jiawei Zhang
BACKGROUND: This study was performed to determine the hemodynamic changes of Budd-Chiari syndrome when the inferior vena vein membrane is developing. METHODS: A patient-specific Budd-Chiari syndrome vascular model was reconstructed based on magnetic resonance images using Mimics software and different degrees (16%, 37%, and 54%) of idealized membrane were built based on the Budd-Chiari syndrome vascular model using Geomagic software. Three membrane obstruction Budd-Chiari syndrome vascular models were established successfully and fluent software was used to simulate hemodynamic parameters, including blood velocity and wall shear stress...
January 2, 2018: Clinical Biomechanics
https://www.readbyqxmd.com/read/29305620/-correction-to-therapeutic-use-of-stem-cells-in-orthopedics
#12
C Chiari, S Walzer, D Stelzeneder, M Schreiner, R Windhager
No abstract text is available yet for this article.
January 5, 2018: Der Orthopäde
https://www.readbyqxmd.com/read/29303466/delayed-neurological-deterioration-following-atlantoaxial-facet-joint-distraction-and-fixation-in-a-patient-with-chiari-malformation-type-i
#13
Tatsushi Inoue, Natsuki Hattori, Tsukasa Ganaha, Tadashi Kumai, Shinichiro Tateyama, Yuichi Hirose
Chiari malformation type I (CM-I) is typically treated with foramen magnum decompression. However, a recent study proposed a new technique for patients with CM-I, wherein only short atlantoaxial joint fusion and distraction is applied. Posterior fusion with or without atlantoaxial distraction is a potential option for patients with CM-I associated with basilar invagination or complex anomalous bony craniovertebral junction pathology, since this procedure allows clinicians to avoid using the technically demanding transoral approach in which some or all of the odontoid tip is invisible...
January 5, 2018: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/29302380/a-case-of-fatal-pulmonary-hypoplasia-with-congenital-diaphragmatic-hernia-thoracic-myelomeningocele-and-thoracic-dysplasia
#14
Ai Ito, Hideshi Fujinaga, Sachiko Matsui, Kumiko Tago, Yuka Iwasaki, Shuhei Fujino, Junko Nagasawa, Shoichiro Amari, Masao Kaneshige, Yuka Wada, Shigehiro Takahashi, Keiko Tsukamoto, Osamu Miyazaki, Takako Yoshioka, Akira Ishiguro, Yushi Ito
Background  Congenital diaphragmatic hernia (CDH) is fatal in severe cases of pulmonary hypoplasia. We experienced a fatal case of pulmonary hypoplasia due to CDH, thoracic myelomeningocele (MMC), and thoracic dysplasia. This constellation of anomalies has not been previously reported. Case Report  A male infant with a prenatal diagnosis of thoracic MMC with severe hydrocephalus and scoliosis was born at 36 weeks of gestation. CDH was found after birth and the patient died of respiratory failure due to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn at 30 hours of age despite neonatal intensive care...
October 2017: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/29298440/effect-of-posterior-fossa-decompression-for-chiari-malformation-i-on-scoliosis
#15
Silky Chotai, Jade Basem, Stephen Gannon, Michael Dewan, Chevis N Shannon, John C Wellons, Christopher M Bonfield
BACKGROUND/AIMS: Scoliosis is common in patients with Chiari malformation-I (CM-I). This study examined the change in scoliosis severity after posterior fossa decompression (PFD) for CM-I. METHODS: We conducted a retrospective review at a single tertiary center for children undergoing PFD with untreated scoliosis, and identified 17 patients with complete follow-up data and imaging. RESULTS: Overall, scoliosis improved in 7 (41.2%) patients, worsened in 9 (52...
January 4, 2018: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29297972/prognostic-scoring-systems-and-outcome-of-endovascular-radiological-intervention-of-chronic-budd-chiari-syndrome-in-children
#16
Sumit Kumar Singh, Moinak Sen Sarma, Rajanikant Yadav, Sheo Kumar, Raghunandan Prasad, Surender Kumar Yachha, Anshu Srivastava, Ujjal Poddar
BACKGROUND AND AIM: Prognostic scoring systems (PSS) have not been validated in children with chronic Budd-Chiari syndrome (BCS). We aimed to analyse the long-term outcome of radiological intervention (RI) and validate the PSS in children. METHODS: Chronic BCS children were analysed in four subgroups: SI: successful intervention (primary or secondary stent patency) b) PO: poor outcome (refractory stent block or requirement of liver transplantation), c) NU: naïve unintervened (awaiting RI) and d) DBI: died before intervention...
January 3, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29296024/beh%C3%A3-et-syndrome-a-contemporary-view
#17
REVIEW
Hasan Yazici, Emire Seyahi, Gulen Hatemi, Yusuf Yazici
The presence of symptom clusters, regional differences in disease expression and similarities with, for example, Crohn's disease suggest multiple pathological pathways are involved in Behçet syndrome. These features also make formulating disease criteria difficult. Genetic studies have identified HLA-B*51 to be the important genetic risk factor. However, the low prevalence of HLA-B*51 in many patients with bone fide disease, especially in non-endemic regions, suggests other factors must also be operative in Behçet syndrome...
January 3, 2018: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/29288861/resolution-of-tachyarrhythmia-following-posterior-fossa-decompression-surgery-for-chiari-malformation-type-i-a-case-report
#18
Christopher Elia, James Brazdzionis, Vartan Tashjian
INTRODUCTION: Chiari malformation type I (CM) commonly present with symptoms such as tussive headaches, paresthesias and in severe cases, corticobulbar dysfunction.3 However, patients may present with atypical symptoms lending to the complexity in this patient population. We present a case of a CM patient presenting with atypical cardiac symptoms and arrhythmias, all of which resolved after surgical decompression. CASE DESCRIPTION: A 31-year-old female presented with atypical chest pain, palpitations, tachycardia, headaches, and dizziness for 2 years...
December 27, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29285598/diagnosis-of-budd-chiari-syndrome
#19
Morgane Van Wettere, Onorina Bruno, Pierre-Emmanuel Rautou, Valérie Vilgrain, Maxime Ronot
Budd-Chiari syndrome (BCS) is defined by clinical and laboratory signs associated with partial or complete impairment of hepatic venous drainage in the absence of right heart failure or constrictive pericarditis. Primary BCS is the most frequent type and is a complication of hypercoagulable states, in particular myeloproliferative neoplasms. Secondary BCS involves tumor invasion or extrinsic compression. Most patients present with chronic BCS including a non-cirrhotic, dysmorphic, chronic liver disease with various degrees of fibrosis deposition...
December 28, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/29285404/cervicothoracic-syringomyelia-caused-by-cervical-spinal-stenosis-case-report-and-literature-review
#20
Mohamed Badri, Ghassen Gader, Kamel Bahri, Ihsen Zammel
Background: Syringomyelia is commonly associated with Chiari malformations, spinal trauma, arachnoiditis, or tumors. However, rarely, cervical canal stenosis is implicated in intramedullary cavitations. Case Discription: Here, we report the case of a 60-year-old male patient who presented with loss of pain and temperature sensation in upper extremities associated with a spastic tetraparesis. On magnetic resonance imaging, the patient was found to have syringomyelia extending from C1 to Th3...
2017: Surgical Neurology International
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