Alpha thalassemia-myelodysplastic syndrome (ATMDS) is one of the possible complications related to the genetic instability typical of clonal hemopoietic disorders such as myelodysplastic syndromes ...(MDS). Hemoglobin H acquisition, which is hemoglobin without alpha chains and with 4 beta chains is the hallmark of this disease.
An 86-year-old male with chronic, microcytic anemia was referred due to a fall in his hemoglobin level. The blood smear was remarkable for intense anisocytoses and poikilocytosis. Bone marrow analysis was followed by a diagnosis of MDS with a good prognostic score. Peripheral blood coloration with brilliant cresyl blue showed "golf ball-like" erythrocytes. Hemoglobin electrophoresis is notable for the presence of H hemoglobin. The new generation sequencing confirmed the diagnosis of ATMDS showing a non-sense mutation in the gene ATRX.
The diagnosis of ATMDS should be considered in the presence of the association of MDS, microcytic anemia and marked blood smear abnormalities such as anisocytosis and poikilocytosis. A little less than 10% of all MDS are complicated by ATMDS.
L’alpha thalassémie-syndrome myélodysplasique (ATSMD) est une complication possible associée à l’instabilité génétique des hémopathies clonales telles que les syndromes myélodysplasiques (SMD). Cette ...pathologie est caractérisée par la production d’une hémoglobine dite H constituée d’un tétramère de chaînes bêta du fait de l’incapacité à synthétiser des globines alpha.
Un patient de 86 ans avec une anémie chronique et microcytaire non carentielle, aggravait soudainement son anémie avec une anisocytose et une poïkilocytose marquées. L’examen cytologique de la moelle osseuse et le caryotype étaient en faveur d’un SMD avec dysplasie multilignée de bon pronostic. La coloration du sang périphérique au Bleu de Crésyl montrait des hématies « en balle de golf ». L’électrophorèse de l’hémoglobine montrait une hémoglobine H. Le diagnostic d’ATSMD était porté du fait d’une mutation non-sens du gène ATRX mise en évidence au séquençage de nouvelle génération.
L’association SMD, anémie microcytaire avec anisocytose et poïkilocytose doit faire évoquer le diagnostic de ATSMD, qui complique moins de 10 % des SMD.
Alpha thalassemia-myelodysplastic syndrome (ATMDS) is one of the possible complications related to the genetic instability typical of clonal hemopoietic disorders such as myelodysplastic syndromes (MDS). Hemoglobin H acquisition, which is hemoglobin without alpha chains and with 4 beta chains is the hallmark of this disease.
An 86-year-old male with chronic, microcytic anemia was referred due to a fall in his hemoglobin level. The blood smear was remarkable for intense anisocytoses and poikilocytosis. Bone marrow analysis was followed by a diagnosis of MDS with a good prognostic score. Peripheral blood coloration with brilliant cresyl blue showed “golf ball-like” erythrocytes. Hemoglobin electrophoresis is notable for the presence of H hemoglobin. The new generation sequencing confirmed the diagnosis of ATMDS showing a non-sense mutation in the gene ATRX.
The diagnosis of ATMDS should be considered in the presence of the association of MDS, microcytic anemia and marked blood smear abnormalities such as anisocytosis and poikilocytosis. A little less than 10% of all MDS are complicated by ATMDS.
This essay, based on a qualitative research project undertaken by the author while training at Roehampton University, explores the profound impact motherhood can have on the work of a music ...therapist. Motivated by the close parallels between the roles of mother and therapist as described in psychodynamic theory, the study involved three interviews with music therapist-mothers, and used Interpretative Phenomenological Analysis to analyse the data. Three superordinate themes emerged: Conflict and Growth; Drawing from Motherhood ‘Toolkit’; and Therapist Boundaries. Findings highlighted the multifaceted nature of the participants’ experiences, revealing both the positive and negative impact being a mother has on the work of a music therapist and the complex ways in which these roles intertwined with each other. During the research process, a broader picture emerged, placing the participants’ experiences within the context of Western culture’s idealised expectations of motherhood, which appeared to exert a powerful influence. It also drew attention to the limitations inherent in the ‘maternal metaphor’ which parallels the roles of mother and therapist, questioning its gender-specificity and the impact this has on music therapist-mothers. This small study provides a starting point for discussion regarding the challenges music therapist-mothers – as well as music therapists who are not mothers – face in a profession in which women make up the majority of the workforce.
Coral reefs play a critical role in the socio-economic development of oceanic islands, besides offering coastal protection against the destructive forces of the sea under storm conditions. A ...Multi-Criteria Decision Making-based geospatial model is used which combine highly influential climatic, ecological, and anthropogenic reef degradation factors in view of revealing regions of high coral reef vulnerabilities to inform ecosystems conservation and management. Further investigation of the coastal seawater temperature trend revealed a rise in sea surface temperature approximating 0.66 °C over the 2003–2020 period as compared to the 1985–2003 interval, with a decadal temperature rise of 0.16 °C reported to be higher than the global average. The bleaching threshold in the region is frequently exceeded during the postmillennial period, further reducing coral fitness. Finally, management strategies are proposed here, which include the adequate design of Marine Protected Area networks, and the implementation of policy strategies for fertilizer use, sustainable coastal development projects, and control of reef predator population. The insights in this paper are expected to be applicable in the reef management of other oceanic islands.
Display omitted
•The vulnerabilities of coral reefs are determined for an oceanic island.•Climatic, ecological, and socio-economic criteria are considered.•Legal and policy strategies are proposed for effective conservation management.•The changing oceanic climate is quantified from 2003 to 2020.
Small islands in the active tropical cyclone basin of the South West Indian Ocean are among the most exposed to climate-induced coastal inundations due to their geographical positions and low ...elevations above mean sea level. Yet, these associated disproportionate impacts are still largely understudied. A geospatial-based approach is used in the current study to identify highly vulnerable areas likely to be exposed to coastal inundation over the next century in Mauritius, Comoros, and Seychelles. This study reveals that Mauritius experiences the maximum inundation of 54.1 km2 while Seychelles encounters the highest risk to coastal settlements equivalent to 9.92% of the total population under the worst-case scenario of peak surge, maximum sea level rise, and high tide. An assessment of the economic ramifications stemming from storm surges is also presented and the analysis shows that a 100-year return period storm tide event could inflict maximum direct losses attaining 30.9% of Mauritius’, 34.2% of Seychelles’, and 6.9% of Comoros’ Gross Domestic Product. These findings underscore the urgent need for strategic interventions to adapt to the far-reaching impacts of coastal inundations. In response to these challenges, a multifaceted coastal storm management strategy that encompasses technological innovation, social inclusivity measures, adaptation actions, reinforced governance structures, and pivotal legal and economic reforms is put forward. This approach holds promise for replication in other similarly vulnerable regions worldwide, thereby shaping the trajectory of climate adaptation, especially small islands.
•Vulnerable communities in Mauritius, Comoros, and Seychelles are revealed.•The combined effect of sea level rise, storm surge, and tide is determined.•Quantification of the extent and cost of damage of coastal amenities is performed.•A coastal storm management strategy for small islands is proposed.
Mauritius, a small island developing state (SIDS), has an approved government-issued smart city framework geared at facilitating the development of smart cities through an application of Internet of ...things. In an attempt to move away from privately-operated urban developments in the form of newly built smart cities, an alternate framework has been proposed by Allam and Newman to redefine this timely concept for existing cities with the main dimensions being metabolism, culture, and governance. This new framework focuses on liveability and sustainability that builds on the cultural and historical dimensions of existing cities. This study adds to the redefined smart city paradigm by proposing a new pillar in the form of smart tourism for the city of Port Louis, Mauritius. This paper reviews models of smart tourism and explores how this can be grafted to the Allam and Newman’s smart city model. The findings of this study seek to inform policy makers on alternate and the more relevant economic potential of smart tourism for the city of Port Louis.
Purpose:
To segment the fetal heart in order to facilitate the 3D assessment of the cardiac function and structure.
Methods:
Ultrasound acquisition typically results in drop-out artifacts of the ...chamber walls. The authors outline a level set deformable model to automatically delineate the small fetal cardiac chambers. The level set is penalized from growing into an adjacent cardiac compartment using a novel collision detection term. The region based model allows simultaneous segmentation of all four cardiac chambers from a user defined seed point placed in each chamber.
Results:
The segmented boundaries are automatically penalized from intersecting at walls with signal dropout. Root mean square errors of the perpendicular distances between the algorithm’s delineation and manual tracings are within 2 mm which is less than 10% of the length of a typical fetal heart. The ejection fractions were determined from the 3D datasets. We validate the algorithm using a physical phantom and obtain volumes that are comparable to those from physically determined means. The algorithm segments volumes with an error of within 13% as determined using a physical phantom.
Conclusions:
Our original work in fetal cardiac segmentation compares automatic and manual tracings to a physical phantom and also measures inter observer variation.
Psychic transparency is described as a psychic crisis occurring during pregnancy. The objective was to test if it was clinically detectable.
Seven primiparous and seven nulliparous subjects were ...recorded during 5 min of spontaneous speech about their dreams. 25 raters from five groups (psychoanalysts, psychiatrists, general practitioners, pregnant women and medical students) listened to the audiotapes. They were asked to rate the probability of the women being pregnant or not. Their ability to discriminate the primiparous women was tested. The probability of being identified correctly or not was calculated for each woman. A qualitative analysis of the speech samples was performed.
No group of rater was able to correctly classify pregnant and non-pregnant women. However, the raters' choices were not completely random. The wish to be pregnant or to have a baby could be linked to a primiparous classification whereas job priorities could be linked to a nulliparous classification.
It was not possible to detect Psychic transparency in this study. The wish for a child might be easier to identify. In addition, the raters' choices seemed to be connected to social representations of motherhood.
Congenital heart disease affects just under one percentage of all live births 1. Those defects that manifest themselves as changes to the cardiac chamber volumes are the motivation for the research ...presented in this thesis. Blood volume measurements in vivo require delineation of the cardiac chambers and manual tracing of foetal cardiac chambers is very time consuming and operator dependent. This thesis presents a multi region based level set snake deformable model applied in both 2D and 3D which can automatically adapt to some extent towards ultrasound noise such as attenuation, speckle and partial occlusion artefacts. The algorithm presented is named Mumford Shah Sarti Collision Detection (MSSCD). The level set methods presented in this thesis have an optional shape prior term for constraining the segmentation by a template registered to the image in the presence of shadowing and heavy noise. When applied to real data in the absence of the template the MSSCD algorithm is initialised from seed primitives placed at the centre of each cardiac chamber. The voxel statistics inside the chamber is determined before evolution. The MSSCD stops at open boundaries between two chambers as the two approaching level set fronts meet. This has significance when determining volumes for all cardiac compartments since cardiac indices assume that each chamber is treated in isolation. Comparison of the segmentation results from the implemented snakes including a previous level set method in the foetal cardiac literature show that in both 2D and 3D on both real and synthetic data, the MSSCD formulation is better suited to these types of data. All the algorithms tested in this thesis are within 2mm error to manually traced segmentation of the foetal cardiac datasets. This corresponds to less than 10% of the length of a foetal heart. In addition to comparison with manual tracings all the amorphous deformable model segmentations in this thesis are validated using a physical phantom. The volume estimation of the phantom by the MSSCD segmentation is to within 13% of the physically determined volume.
Congenital heart disease affects just under one percentage of all live births 1. Those defects that manifest themselves as changes to the cardiac chamber volumes are the motivation for the research ...presented in this thesis. Blood volume measurements in vivo require delineation of the cardiac chambers and manual tracing of foetal cardiac chambers is very time consuming and operator dependent. This thesis presents a multi region based level set snake deformable model applied in both 2D and 3D which can automatically adapt to some extent towards ultrasound noise such as attenuation, speckle and partial occlusion artefacts. The algorithm presented is named Mumford Shah Sarti Collision Detection (MSSCD). The level set methods presented in this thesis have an optional shape prior term for constraining the segmentation by a template registered to the image in the presence of shadowing and heavy noise. When applied to real data in the absence of the template the MSSCD algorithm is initialised from seed primitives placed at the centre of each cardiac chamber. The voxel statistics inside the chamber is determined before evolution. The MSSCD stops at open boundaries between two chambers as the two approaching level set fronts meet. This has significance when determining volumes for all cardiac compartments since cardiac indices assume that each chamber is treated in isolation. Comparison of the segmentation results from the implemented snakes including a previous level set method in the foetal cardiac literature show that in both 2D and 3D on both real and synthetic data, the MSSCD formulation is better suited to these types of data. All the algorithms tested in this thesis are within 2mm error to manually traced segmentation of the foetal cardiac datasets. This corresponds to less than 10% of the length of a foetal heart. In addition to comparison with manual tracings all the amorphous deformable model segmentations in this thesis are validated using a physical phantom. The volume estimation of the phantom by the MSSCD segmentation is to within 13% of the physically determined volume.
PDF
