How I treat dysfibrinogenemia

Blood ◽  
2021 ◽  
Author(s):  
Alessandro Casini ◽  
Philippe de Moerloose
Keyword(s):  
At Risk ◽  
Genetic Testing ◽  
Structural Changes ◽  
Clinical Course ◽  
Clinical Settings ◽  
Antifibrinolytic Agents ◽  
Clinical Scenarios

Congenital dysfibrinogenemia (CD) is caused by structural changes in fibrinogen that modify its function. Diagnosis is based on discrepancy between decreased fibrinogen activity and normal fibrinogen antigen levels and is confirmed by genetic testing. CD results from monoallelic mutations in fibrinogen genes leading to clinically heterogenous disorders. Most patients with CD are asymptomatic at time of diagnosis but the clinical course may be complicated by a tendency to bleeding and/or thrombosis. Patients with a thrombotic-related fibrinogen variant are particularly at risk and in such patients long-term anticoagulation should be considered. Management of surgery and pregnancy raise important and difficult issues. The mainstay of CD treatment remains fibrinogen supplementation. Antifibrinolytic agents are part of the treatment in some specific clinical settings. In this article, we discuss five clinical scenarios to highlight common clinical challenges. We detail our approach to establish a diagnosis of CD and discuss strategies for the management of bleeding, thrombosis, surgery and pregnancy.

Introduction

2017 ◽  
Author(s):  
Igor Galynker
Keyword(s):  
Risk Assessment ◽  
At Risk ◽  
Suicide Risk ◽  
Assessment Methods ◽  
Suicidal Patient ◽  
Clinical Settings ◽  
Short Term ◽  
Imminent Risk ◽  
Intended Use

One of the most difficult determinations a psychiatrist makes is whether the chronically suicidal patient is at risk for imminent suicide. The Introduction presents the challenges of the imminent risk assessment and explains the purpose of The Suicidal Narrative—the first clinical guide to such an assessment. It introduces the distinction between long-term and short-term suicide risk, highlights the lack of structured instruments for imminent risk assessment, and discusses the advantages of multimodal and multi-informant assessment methods. It then describes intended uses of the guide, lists its inherent limitations, and identifies possible areas of unintended misuse. The Introduction concludes by naming the main intended use of The Suicidal Narrative: as a roadmap for a comprehensive assessment of imminent suicide risk in diverse clinical settings.

Coming Together Through Falling Apart

2019 ◽  
Vol 40 (2) ◽  
pp. 151-168
Author(s):  
Heather Churchill ◽  
Jeremy M. Ridenour
Keyword(s):  
Data Analysis ◽  
Qualitative Data ◽  
Structural Changes ◽  
Systematic Approach ◽  
Qualitative Approach ◽  
Psychological Assessments ◽  
Transference And Countertransference ◽  
Testing Data ◽  
Psychoanalytically Oriented Psychotherapy

Abstract. Assessing change during long-term psychotherapy can be a challenging and uncertain task. Psychological assessments can be a valuable tool and can offer a perspective from outside the therapy dyad, independent of the powerful and distorting influences of transference and countertransference. Subtle structural changes that may not yet have manifested behaviorally can also be assessed. However, it can be difficult to find a balance between a rigorous, systematic approach to data, while also allowing for the richness of the patient’s internal world to emerge. In this article, the authors discuss a primarily qualitative approach to the data and demonstrate the ways in which this kind of approach can deepen the understanding of the more subtle or complex changes a particular patient is undergoing while in treatment, as well as provide more detail about the nature of an individual’s internal world. The authors also outline several developmental frameworks that focus on the ways a patient constructs their reality and can guide the interpretation of qualitative data. The authors then analyze testing data from a patient in long-term psychoanalytically oriented psychotherapy in order to demonstrate an approach to data analysis and to show an example of how change can unfold over long-term treatments.

Parents' long-term adjustment to the genetic test results of minors at risk for Long QT Syndrome

2004 ◽  
Author(s):  
K. S. W. H. Hendriks ◽  
F. J. M. Grosfeld ◽  
A. A. M. Wilde ◽  
J. van den Bout ◽  
I. M. van Langen ◽  
...  
Keyword(s):  
At Risk ◽  
Long Qt Syndrome ◽  
Genetic Test ◽  
Test Results ◽  
Long Qt ◽  
Genetic Test Results ◽  
Qt Syndrome

Economic Institutions and Growth Nexus: The Role of Governance and Democratic Institutions—Evidence from Time Varying Parameters’ (TVPs) Models

2016 ◽  
Vol 55 (4I-II) ◽  
pp. 675-688
Author(s):  
Ghulam Murtaza ◽  
Muhammad Zahir Faridi
Keyword(s):  
Structural Changes ◽  
Jel Classification ◽  
Economic Institutions ◽  
Time Varying ◽  
Democratic Institutions ◽  
Main Hypothesis ◽  
Dynamic Relationship ◽  
Rolling Window

The present study has investigated the channels through which the linkage between economic institutions and growth is gauged, by addressing the main hypothesis of the study that whether quality of governance and democratic institutions set a stage for economic institutions to promote the long-term growth process in Pakistan. To test the hypothesis empirically, our study models the dynamic relationship between growth and economic institutions in a time varying framework in order to capture institutional developments and structural changes occurred in the economy of Pakistan over the years. Study articulates that, along with some customary specifics, the quality of government and democracy are the substantial factors that affect institutional quality and ultimately cause to promote growth in Pakistan. JEL Classification: O40; P16; C14; H10 Keywords: Economic Institutions, Growth, Governance and Democracy, Rolling Window Two-stage Least Squares, Pakistan

Individual differences in white and grey matter structure associated with verbal habits of thought

2019 ◽  
Author(s):  
Justin C. Hayes ◽  
Katherine L Alfred ◽  
Rachel Pizzie ◽  
Joshua S. Cetron ◽  
David J. M. Kraemer
Keyword(s):  
Individual Differences ◽  
White Matter ◽  
Cognitive Processing ◽  
Grey Matter ◽  
Structural Changes ◽  
Structural Integrity ◽  
Diffusion Tensor ◽  
Self Report ◽  
White Matter Tracts

Modality specific encoding habits account for a significant portion of individual differences reflected in functional activation during cognitive processing. Yet, little is known about how these habits of thought influence long-term structural changes in the brain. Traditionally, habits of thought have been assessed using self-report questionnaires such as the visualizer-verbalizer questionnaire. Here, rather than relying on subjective reports, we measured habits of thought using a novel behavioral task assessing attentional biases toward picture and word stimuli. Hypothesizing that verbal habits of thought are reflected in the structural integrity of white matter tracts and cortical regions of interest, we used diffusion tensor imaging and volumetric analyses to assess this prediction. Using a whole-brain approach, we show that word bias is associated with increased volume in several bilateral language regions, in both white and grey matter parcels. Additionally, connectivity within white matter tracts within an a priori speech production network increased as a function of word bias. These results demonstrate long-term structural and morphological differences associated with verbal habits of thought.

Salivary C-reactive protein among at-risk adolescents: A methods investigation of out of range immunoassay data

2018 ◽  
Author(s):  
Elizabeth Landau ◽  
John Arthur Trinder ◽  
Julian Simmons ◽  
Monika Raniti ◽  
Matthew Blake ◽  
...  
Keyword(s):  
At Risk ◽  
Temporal Stability ◽  
Management Technique ◽  
Clinical Settings ◽  
Diurnal Pattern ◽  
C Reactive Protein ◽  
Reactive Protein ◽  
Treatment Techniques ◽  
Salivary Biomarker ◽  
At Risk Adolescents

Inflammatory markers including C-Reactive Protein (CRP) are increasingly used within research and clinical settings. Yet, varying methodologies for cleaning immunoassay data with out of range (OOR) samples may alter characteristic levels of CRP, thereby obscuring interpretation and reliability. This study investigated the influence of eight immunoassay OOR data treatment techniques on salivary CRP (sCRP) samples from at-risk adolescents. Participants from the ‘Sleep and Education: learning New Skills Early’ (SENSE) Study were 86 adolescents at-risk for depression (50 female), aged 14.29 years (SD=1.04). ANOVA results showed no statistically significant differences in average morning (F(7, 590)=1.24, p=.28) and evening (F(7, 599)=1.29, p=.25) values produced by each OOR data cleaning technique. However, varying techniques produced differences in the magnitude of Pearson’s correlations between consecutive saliva samples (r’s between .27 – .78), and influenced the significance of a sCRP diurnal pattern; two techniques produced statistically higher morning than evening sCRP levels (t(85)=2.70, p=.01 and t(85)=2.67, p=.01), whereas six techniques failed to find statistical differences between morning and evening sCRP levels (p’s >.05). Varying techniques also produced statistically divergent associations between sCRP and age and depressive symptoms. Results from this study provide evidence for the temporal stability of sCRP among adolescents, show winsorization as an effective OOR data management technique, and highlight the influence of methodological decisions in cleaning salivary biomarker data and the need for consistency within the field.

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