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Ross I Baker, Philip Choi, Nicola Curry, Johanna Gebhart, Keith Gomez, Yvonne Henskens, Floor Heubel-Moenen, Paula James, Rezan Abdul Kadir, Peter Kouides, Michelle Lavin, Marie Lordkipanidze, Gillian Lowe, Andrew Mumford, Nicola Mutch, Michael Nagler, Maha Othman, Ingrid Pabinger, Robert Sidonio, Will Thomas, James S O'Donnell
In many patients referred with significant bleeding phenotype, laboratory testing fails to define any hemostatic abnormalities. Clinical practise with respect to diagnosis and management of this patient cohort poses significant clinical challenges. We recommend that bleeding history in these patients should be objectively assessed using the ISTH bleeding assessment tool (BAT). Patients with increased BAT scores should progress to hemostasis laboratory testing. To diagnose BDUC, normal complete blood count, prothrombin time, activated partial thromboplastin time, thrombin time, von Willebrand factor antigen, von Willebrand factor function; coagulation factors VIII, IX and XI and platelet light transmission aggregometry (LTA) should be the minimum laboratory assessment...
March 20, 2024: Journal of Thrombosis and Haemostasis: JTH
#2
REVIEW
Stella Stabouli, Anna Sommer, Stefanie Kraft, Katharina Schweer, Dirk Bethe, Aurelia Bertholet-Thomas, Suzanne Batte, Gema Ariceta, Sandra Brengmann, Justine Bacchetta, Francesco Emma, Elena Levtchenko, Rezan Topaloglu, Lore Willem, Dieter Haffner, Jun Oh
Cystinosis is a rare autosomal-recessive lysosomal storage disease that progressively affects multiple organs beginning with the kidneys. Patients require lifelong multidisciplinary care for the management of kidney disease and progressive extra-renal manifestations, and thus, they are especially fragile and vulnerable during transition from pediatric to adult care. Previous documents have provided guidance to help the medical transition of these highly burdened patients. Patients and their families often experience great psychological distress and face significant social challenges; for these reasons, they often need help from psychologists, social workers, and other psychosocial professionals...
March 22, 2024: Pediatric Nephrology
#3
JOURNAL ARTICLE
Bahar Özdemir Ulusoy, Serdar Can Güven, Hakan Babaoğlu, Can Ozan Ulusoy, Berkan Armağan, Esra Kayacan Erdoğan, Yüksel Maraş, İsmail Doğan, Kevser Orhan, Rezan Koçak Ulucaköy, Ebru Atalar, Ahmet Omma, Orhan Küçükşahin, Şükran Erten
AIM: Familial Mediterranean fever (FMF) is the most common inherited autoinflammatory disease in the world. There are known triggers to initiate an FMF attack, yet potential effects of intrauterine devices (IUD) in women of reproductive age have not been evaluated before. METHOD: Consecutive female patients with FMF who ever used IUD over the age of 18 were enrolled. Female patients with FMF were sub grouped according to the type of IUD they use. FMF attack frequency, severity, duration, presence of dysmenorrhea, severity of dysmenorrhea, having attacks during menstruation before and after IUD use were questioned...
March 13, 2024: Archives of Gynecology and Obstetrics
#4
JOURNAL ARTICLE
Maha Othman, Georgiana Nemeti, Marissa Solow, Gheorghe Cruciat, Daniel Muresan, Mariam Suzana Chaikh Sulaiman, Shivani Thaker, Rezan Abdul-Kadir, A Kinga Malinowski
BACKGROUND: Early reports have demonstrated an association of COVID-19 infection during pregnancy and postpartum period with coagulopathy and bleeding complications and indicated that pregnant people with COVID-19 are more likely to experience coagulopathy and venous thromboembolism. A recent report concerning such complications during the first wave of the pandemic was reassuring; however, no publications have evaluated these issues in the context of increased illness severity with the emergence of SARS-CoV-2 variants of concern...
January 2024: Research and Practice in Thrombosis and Haemostasis
#5
JOURNAL ARTICLE
Velibor Tasic, Vidar O Edvardsson, Evgenia Preka, Larisa Prikhodina, Constantinos J Stefanidis, Rezan Topaloglu, Diamant Shtiza, Ashot Sarkissian, Thomas Mueller-Sacherer, Rena Fataliyeva, Ina Kazyra, Elena Levtchenko, Danka Pokrajac, Dimitar Roussinov, Danko Milošević, Avraam Elia, Tomas Seeman, Mia Faerch, Inga Vainumae, Janne Kataja, Michel Tsimaratos, Irakli Rtskhiladze, Peter F Hoyer, George Reusz, Atif Awan, Danny Lotan, Licia Peruzzi, Nazim Nigmatullina, Nasira Beishebaeva, Edite Jeruma, Augustina Jankauskiene, Olivier Niel, Valerie Said-Conti, Angela Ciuntu, Snežana Pavićević, Michiel Oosterveld, Anna Bjerre, Marcin Tkaczyk, Ana Teixeira, Adrian C Lungu, Alexey Tsygin, Vesna Stojanović, Ludmila Podracka, Tanja Kersnik Levart, Mar Espino-Hernández, Per Brandström, Giuseppina Sparta, Harika Alpay, Dmytro Ivanov, Jan Dudley, Komiljon Khamzaev, Dieter Haffner, Jochen Ehrich
BACKGROUND: Primary, secondary and tertiary healthcare services in Europe create complex networks covering pediatric subspecialties, sociology, economics and politics. Two surveys of the European Society for Paediatric Nephrology (ESPN) in 1998 and 2017 revealed substantial disparities of kidney care among European countries. The purpose of the third ESPN survey is to further identify national differences in the conceptualization and organization of European pediatric kidney health care pathways during and outside normal working hours...
2024: Frontiers in Pediatrics
#6
Alessandro Casini, Rezan Abdul Kadir, Magy Abdelwahab, Marilyn J Manco-Johnson, Sanj Raut, Cecil Ross, Philippe de Moerloose, Cristina Santoro, Suchitra Acharya
Congenital fibrinogen disorders (CFDs) are a heterogeneous group of rare congenital quantitative and / or qualitative fibrinogen deficiency. The spectrum of molecular anomalies is broad, leading to several subtypes of fibrinogen disorders (i.e., afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, hypodysfibrinogenemia). Pregnancy in women with CFDs is a high-risk clinical situation, with an increased tendency to miscarriages, bleeding, and thrombosis. Even though it is well established that management of such pregnancies requires a multidisciplinary approach involving specialists (hematologists and maternal / fetal medicine experts with expertise in the management of inherited bleeding disorders), specific guidelines are lacking...
January 22, 2024: Journal of Thrombosis and Haemostasis: JTH
#7
JOURNAL ARTICLE
Demet Baltu, Eda Didem Kurt-Sukur, Tugba Tastemel Ozturk, Bora Gulhan, Fatih Ozaltin, Ali Duzova, Rezan Topaloglu
BACKGROUND: COVID-19 is known to have a mild course in children, however more data on pediatric chronic kidney disease (CKD) is needed. We aimed to assess the incidence and severity of COVID-19 in pediatric CKD patients. METHODS: A questionnaire including demographics, COVID-19 history, symptoms, and vaccination status was applied to patients with CKD. We also retrospectively reviewed the presentation and outcomes of SARS-CoV-2 infection in this patient group from March 2020 to December 2021...
January 15, 2024: Klinische Pädiatrie
#8
JOURNAL ARTICLE
Şükrü Burak Tönük, Zeynep Rezan Yorgancıoğlu, Selma Uysal Ramadan, Seher Kocaoğlu
BACKGROUND: Individuals with high systemic bone mineral density (BMD) may have an increased risk of incident knee osteoarthritis (OA). Besides that, radiographic osteophytes are strongly associated with BMD. Because of these reasons, the aim of the study was to investigate the possible association between radiological subchondral bone cyst (SBC) grade and systemic BMD and vitamin D status in the postmenopausal female patients with knee OA in a crosss-sectional study. METHODS: This study included of 48 osteoporosis treatment-free postmenopausal patients diagnosed with symptomatic medial compartment knee OA...
January 11, 2024: BMC Musculoskeletal Disorders
#9
JOURNAL ARTICLE
Katharina Rohner, Matko Marlais, Yo Han Ahn, Alaa Ali, Abrar Alsharief, Anja Blejc Novak, Marta Brambilla, Evrim Kargin Cakici, Cengiz Candan, Nur Canpolat, Eugene Yu-Hin Chan, Stéphane Decramer, Madeleine Didsbury, Filipa Durao, Anne M Durkan, Ali Düzova, Thomas Forbes, Valentina Gracchi, Tulin Güngör, Tomoko Horinouchi, Belde Kasap Demir, Yasuko Kobayashi, Mikael Koskela, Eda Didem Kurt-Sukur, Claudio La Scola, Dean Langan, Xiaozhong Li, Gabriele Malgieri, Antonio Mastrangelo, Jeesu Min, Malgorzata Mizerska-Wasiak, Nabila Moussaoui, Aytul Noyan, Matti Nuutinen, Jennifer O'Gormon, Takayuki Okamoto, Louise Oni, Michiel Oosterveld, Malgorzata Pańczyk-Tomaszewska, Gonul Parmaksiz, Andrea Pasini, Pornpimol Rianthavorn, Joris Roelofs, Yunyan Shen, Rajiv Sinha, Rezan Topaloglu, Diletta Domenica Torres, Tomohiro Udagawa, Martin Wennerström, Yok Chin Yap, Kjell Tullus
BACKGROUND AND HYPOTHESIS: IgA vasculitis with nephritis (IgAVN) is the most common vasculitis in children. Treatment recommendations are, due to a lack of evidence, based on expert opinion resulting in variation. The aim of this study was to describe clinical presentation, treatment and outcome of an extremely large cohort of children with biopsy proven IgAVN to identify prognostic risk factors and signals of treatment efficacy. METHODS: Retrospective data were collected on 1148 children with biopsy proven IgAVN between 2005 and 2019 from 41 international paediatric nephrology centres across 25 countries and analyzed using multivariate analysis...
January 11, 2024: Nephrology, Dialysis, Transplantation
#10
JOURNAL ARTICLE
Mohamad Hafiz Abd Rahim, Siti Hajar Mahamad Dom, Mohd Shah Rezan Hamzah, Siti Hawa Azman, Zahirah Zaharuddin, Mathumalar Loganathan Fahrni
BACKGROUND: Under-utilisation of immunisation services remains a public health challenge. Pharmacists act as facilitators and increasingly as immunisers, yet relatively little robust evidence exists of the impact elicited on patient health outcome and vaccination uptake. OBJECTIVE: To evaluate the influence of pharmacist interventions on public vaccination rate. METHODS: SCOPUS, PubMed, and Web of Science were searched from inception to April 2023 to retrieve non- and randomised controlled clinical trials (RCTs)...
2024: Journal of Pharmaceutical Policy and Practice
#11
REVIEW
Javad Momeni, Elnaz Naserzadeh, Ali Sepehrinezhad, Rezan Ashayeri Ahmadabad, Sajad Sahab Negah
Despite enormous efforts, no effective medication has been found to significantly halt or even slow the progression of neurological diseases, such as acquired (e.g., traumatic brain injury, spinal cord injury, etc.) and chronic (e.g., Parkinson's disease, Alzheimer's disease, etc.) central nervous system disorders. So, researchers are looking for alternative therapeutic modalities to manage the disease's symptoms and stop it from worsening. Concerning disease-modifying capabilities, stem cell therapy has emerged as an expanding domain...
January 8, 2024: International Journal of Stem Cells
#12
REVIEW
Rezan Topaloglu
Cystinosis is a rare autosomal recessive disease with an incidence 1 per 100,000-200,000 live births. It is caused by pathogenic variants of the cystinosin (CTNS) gene that lead to impaired cystine transport from lysosomes to cystosol, resulting in cystine accumulation in lysosomes and subsequent cellular dysfunction. The initial manifestation, cystine accumulation in proximal tubular cells (PTCs), causes renal Fanconi syndrome, which presents with proximal renal tubular acidosis and generalized dysfunction of the proximal tubule, including the presence of polyuria, glycosuria, phosphaturia, aminoaciduria, tubular proteinuria, growth retardation, and rickets...
December 21, 2023: Pediatric Nephrology
#13
JOURNAL ARTICLE
Hasan Ersöz, Rezan Karaali, Vladimir Kuzmichev, Winfried Rebhandl, Firas Abu-Akar, Marouane Lakranbi, Mustafa Yüksel
Parallel- and cross-bar techniques are surgical methods used in the pectus excavatum. While the parallel bar is used in many centers, the cross bar is a new technique. The aim of the study is to evaluate the data of centers using cross bar and parallel bar. The aim of this multicenter study is to retrospectively evaluate the data of centers have been using both cross-bar and parallel-bar techniques. 213 parallel bars and 205 cross bars were used. Parallel-bar advantages: the mean patient satisfaction score was 9...
December 14, 2023: Updates in Surgery
#14
JOURNAL ARTICLE
Fu Liu, Wenqing Lu, Jiaqiang Huang, Vanessa Pimenta, Steven Boles, Rezan Demir-Cakan, Jean-Marie Tarascon
Challenges in enabling next-generation rechargeable batteries with lower cost, higher energy density, and longer cycling life stem not only from combining appropriate materials, but from optimally using cell components. One-size-fits-all approaches to operational cycling and monitoring are limited in improving sustainability if they cannot utilize and capture essential chemical dynamics and states of electrodes and electrolytes. Herein we describe and show how the use of tilted fiber Bragg grating (TFBG) sensors to track, via the monitoring of both temperature and refractive index metrics, electrolyte-electrode coupled changes that fundamentally control lithium sulfur batteries...
November 14, 2023: Nature Communications
#15
JOURNAL ARTICLE
İpek Baysal, Samiye Yabanoglu-Ciftci, Emirhan Nemutlu, Cemil Can Eylem, Elif Damla Gök-Topak, Kezban Ulubayram, Sedef Kır, Bora Gulhan, Gülberk Uçar, Fatih Ozaltin, Rezan Topaloglu
Cystinosis is an autosomal recessive disease caused by mutations in the CTNS gene encoding a protein called cystinosine, which is a lysosomal cystine transporter. Disease causing mutations lead accumulation of cystine crystals in the lysosomes and thereby causing dysfunction of vital organs. Determination of the increased leukocyte cystine level is one of the most used methods for diagnosis. However, this method is expensive, difficult to perform, and may yield different results in different laboratories. In this study, a disease model was created with CTNS gene-silenced HK2 cells, which can mimic cystinosis in cell culture, and multi-omics methods (i...
November 8, 2023: Laboratory Investigation; a Journal of Technical Methods and Pathology
#16
JOURNAL ARTICLE
Rezan Karaali, Ejder Saylav Bora, Firdevs Topal
INTRODUCTION: Cirrhosis is a common liver disease, which is characterized by life-limiting complications. In cirrhosis, liver ACE2 mRNA levels were 34-times upregulated, ACE2 protein 97-times upregulated, and ACE2 receptors increased in 80% of hepatocytes. Increased ACE2 receptor sensitizes hepatocytes to COVID-19. AIM: To evaluate the applications of cirrhosis patients to the Emergency Department before and after the pandemic. MATERIAL AND METHODS: The study was conducted retrospectively in a single centre on cirrhotic patients who applied to the Emergency Department in a 2-year period...
2023: Przegla̜d Gastroenterologiczny
#17
JOURNAL ARTICLE
Elif Ciftcioglu, Rezan Sungur Guzel, Gozde Akbal Dincer, Gunes Karakaya, Enver Sedat Kucukay
PURPOSE: This in vitro study aimed to assess the performance of ProTaper Universal Retreatment (PTUR), Reciproc Blue (RB), and XP-endo Shaper (XPS) system in the removal of bioceramic root canal filling. MATERIALS AND METHODS: Forty-five human single-rooted mandibular premolars were prepared up to 30/.04 and filled with Endosequence BC sealer and BC points before being assigned into three groups (n=15). The root canal fillings were removed until reaching predetermined working length (WL) with PTUR in group 1, RB in group 2, and XPS in group 3...
September 6, 2023: European Oral Research
#18
Demet Baltu, Nihan Avcu Oral, Selman Kesici, Rezan Topaloğlu, Osman İlhami Özcebe, Tekin Aksu, Diclehan Orhan, Fatih Özaltın
BACKGROUND: Intravascular hemolysis is a serious and rare condition in children and causes the release of hemoglobin and heme into circulation, which have proinflammatory properties. These substances lead to inflammation, oxidative stress, apoptosis, and organelle dysfunction that lead to acute kidney injury (AKI). We report a pediatric case diagnosed with hemolysis-associated hemoglobin cast nephropathy due to autoimmune hemolytic anemia. CASE: A 4-year-old boy, who was admitted to another hospital with complaints of fever and dark urine for one day, developed anemia and kidney failure in the follow-up, was referred to our hospital...
2023: Turkish Journal of Pediatrics
#19
JOURNAL ARTICLE
Elisabeth L Metry, Sander F Garrelfs, Lisa J Deesker, Cecile Acquaviva, Viola D'Ambrosio, Justine Bacchetta, Bodo B Beck, Pierre Cochat, Laure Collard, Julien Hogan, Pietro Manuel Ferraro, Casper F M Franssen, Jérôme Harambat, Sally-Anne Hulton, Graham W Lipkin, Giorgia Mandrile, Cristina Martin-Higueras, Nilufar Mohebbi, Shabbir H Moochhala, Thomas J Neuhaus, Larisa Prikhodina, Eduardo Salido, Rezan Topaloglu, Michiel J S Oosterveld, Jaap W Groothoff, Hessel Peters-Sengers
INTRODUCTION: Primary hyperoxaluria type 1 (PH1) has a highly heterogeneous disease course. Apart from the c.508G>A (p.Gly170Arg) AGXT variant, which imparts a relatively favorable outcome, little is known about determinants of kidney failure. Identifying these is crucial for disease management, especially in this era of new therapies. METHODS: In this retrospective study of 932 patients with PH1 included in the OxalEurope registry, we analyzed genotype-phenotype correlations as well as the impact of nephrocalcinosis, urolithiasis, and urinary oxalate and glycolate excretion on the development of kidney failure, using survival and mixed model analyses...
October 2023: KI Reports
#20
JOURNAL ARTICLE
Wala Al Arashi, Lorenzo G R Romano, Frank W G Leebeek, Marieke J H A Kruip, Karin P M van Galen, Ozlem Turan, Rezan Abdul Kadir, Marjon H Cnossen
BACKGROUND: Although desmopressin (DDAVP) is an accessible and inexpensive hemostatic drug, its use in pregnancy is still debated due to safety uncertainties. OBJECTIVES: We aimed to review the safety and effectiveness of DDAVP in women with an inherited bleeding disorder during pregnancy and delivery. METHODS: Databases were searched for articles up to July 25, 2022, reporting maternal and/or neonatal outcomes. PRISMA methodology for systematic reviews and meta-analyses was followed (PROSPERO CRD42022316490)...
September 30, 2023: Journal of Thrombosis and Haemostasis: JTH
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