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1.
Issues Ment Health Nurs ; : 1-10, 2024 May 22.
Artículo en Inglés | MEDLINE | ID: mdl-38776548

RESUMEN

The undergraduate mental health nursing course is an optimal time to address stigma and prejudice, while developing positive student attitudes toward those who live with mental health conditions. A quasi-experimental, pretest-posttest, nonequivalent-group study with a sample of undergraduate nursing students in New York City (N = 126) was conducted to determine the impact of an undergraduate mental health nursing course on attitudes toward people living with a general mental illness, depression, or schizophrenia. The intervention resulted in a significant reduction in total prejudice scores toward those with a general mental illness when compared to the control (p = 0.033, partial η2 = 0.062). The intervention had no significant impact on total prejudice scores regarding those with depression, or schizophrenia. Subscale analysis revealed the intervention significantly reduced attitudes of fear/avoidance regarding general mental illness (p = 0.040, partial η2 = 0.058) and schizophrenia (p < 0.001, partial η2 = 0.164). There was no impact on authoritarian or malevolent attitudes. Though some attitudes were not amenable to change, this study provides evidence that positive attitudes can be cultivated through undergraduate nursing education. Curricular reform is needed to reduce all facets of prejudice and best prepare future nurses to care for those with mental health conditions.

2.
Issues Ment Health Nurs ; 45(4): 391-398, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38241519

RESUMEN

INTRODUCTION: Mental health recovery is a critical concept that needs to be thoroughly understood and supported by nurses. Undergraduate nurse educators have the opportunity to clarify misconceptions and cultivate positive recovery attitudes. AIM: To assess the impact of an undergraduate nursing course on attitudes toward mental health recovery and the relationship between recovery attitudes and prejudice toward those who experience a mental illness. METHODS: A quasi-experimental pretest-posttest, nonequivalent-control group study was conducted using a sample of undergraduate nursing students in New York City (N = 126). The intervention group was assigned to an undergraduate mental health nursing course and the control group to a pediatric/maternal health nursing course. Attitudes toward mental health recovery and prejudice were measured at the beginning and end of the semester. Two-way mixed analyses of variance were used to determine the differences in students' attitudes. Pearson product-moment correlation analyses were used to assess the relationship between prejudice toward people who experience a mental illness and attitudes toward recovery. RESULTS: The mental health nursing course had no measurable impact on students' recovery attitudes. However, there was a moderate-to-strong inverse relationship between recovery attitudes and prejudice toward those who experience a general mental illness (r = -0.54), depression (r = -0.60), or schizophrenia (r = -0.43). CONCLUSIONS: Curriculum reform is needed to optimize the impact of undergraduate education on students' attitudes. Possible changes include a more holistic approach to mental health that does not over accentuate the biomedical model, the use of nontraditional clinical sites that provide students an opportunity to interact with those further along in their recovery, and the inclusion of those in recovery in curriculum development. As there was a moderate-to-strong inverse relationship between recovery attitudes and prejudice, educational interventions that positively impact one may also impact the other. Further research is needed to investigate if the relationship is causal.


Asunto(s)
Bachillerato en Enfermería , Recuperación de la Salud Mental , Estudiantes de Enfermería , Niño , Humanos , Actitud del Personal de Salud , Estudiantes de Enfermería/psicología , Optimismo , Encuestas y Cuestionarios
3.
Issues Ment Health Nurs ; 44(8): 778-786, 2023 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-37459622

RESUMEN

INTRODUCTION: The undergraduate mental health nursing course may be an optimal time to cultivate students' positive attitudes toward people living with a mental illness. AIM: To determine the impact of an undergraduate mental health nursing course on students' attitudes toward people living with a mental illness, depression, and schizophrenia. METHOD: A quasi-experimental single-group pretest posttest study was conducted using a sample of undergraduate nursing students in New York City (N = 44). Self-reported measures of prejudice toward those living with a mental illness were collected at the beginning of a mental health nursing course and again at its conclusion. RESULTS: A statistically significant decrease in prejudice scores was found concerning mental illness (p = .03, d = 0.23), depression (p = .01, d = 0.31), and schizophrenia (p = .013, d = 0.34). Subscale analysis revealed significant decreases in the fear/avoidance and unpredictability subscales. Yet no significant change was found in the subscales of authoritarianism and malevolence for any of the three conditions. DISCUSSION: A mental health course led to a modest decrease in prejudice. However, certain facets of prejudice remain unchanged. IMPLICATIONS FOR PRACTICE: Major curricular reform is needed to optimize the impact of undergraduate nursing education.


Asunto(s)
Bachillerato en Enfermería , Trastornos Mentales , Estudiantes de Enfermería , Humanos , Estudiantes de Enfermería/psicología , Actitud del Personal de Salud , Trastornos Mentales/psicología , Prejuicio
4.
Zootaxa ; 5271(3): 477-502, 2023 Apr 27.
Artículo en Inglés | MEDLINE | ID: mdl-37518115

RESUMEN

We describe two new species of moderate-sized (male body length 26.5-29.8 mm and 41.0 mm), predominantly green treefrogs in the genus Litoria from hill forest on the northern slopes of Papua New Guinea's Central Cordillera. Phylogenetic analysis of mitochondrial ND4 nucleotide sequences shows that the first species is related to Litoria iris (Tyler) and its allies. It is morphologically most similar to Litoria mystax, a small green treefrog known only from the holotype that was described more than 100 years ago from the north coast of western New Guinea but differs from that species in having longer legs and a broader head. The second species is closest to Litoria gasconi, a species known only from the foothills of the Foja Mountains in Papua Province, Indonesian New Guinea, and the Prince Alexander Mountains in northern Papua New Guinea. It has a net average sequence divergence of 10% from L. gasconi and can be distinguished morphologically from it and from other pelodryadids by the presence of a striking pattern of spots and blotches on the ventral surfaces and on the hidden surfaces of the limbs. These descriptions add to the rapidly increasing known diversity of frogs in hill and lower montane forest, habitats that support the most diverse frog communities on mainland New Guinea.


Asunto(s)
Anuros , Ecosistema , Masculino , Animales , Papúa Nueva Guinea , Filogenia
5.
Zootaxa ; 5296(2): 101-146, 2023 May 26.
Artículo en Inglés | MEDLINE | ID: mdl-37518451

RESUMEN

We examined the morphology, colour patterns and genetic relationships of Nososticta populations allied to N. salomonis (Selys) from across Melanesia. Seven species-level taxa are recognised in the N. salomonis 'complex': N. africana (Schmidt), N. boonei sp. nov., N. chrismulleri Theischinger & Richards, N. hedigeri sp. nov., N. salomonis (Selys), N. stueberi sp. nov., and N. tagula sp. nov. All of these species are black damselflies with blue markings, and they differ from all other Nososticta by having: 1) a prominent spike on the male superior appendage, 2) a prominent angular base of the male inferior appendage, and 3) a complex posterior lobe on the female pronotum bearing two pairs of processes in the rough shape of a chair when viewed laterally. A molecular phylogeny based on the DNA barcode fragment of the COI gene plus two nuclear genes indicates that these seven species are closely related, but more extensive sampling of Nososticta species is required to confirm that they form a monophyletic group.

6.
Zootaxa ; 5263(2): 151-190, 2023 Apr 05.
Artículo en Inglés | MEDLINE | ID: mdl-37044991

RESUMEN

New Guinea has the most diverse insular frog fauna in the world, and rates of species discovery and description have increased rapidly in the last two decades. Pelodryadid treefrogs are the second most diverse family of anurans on the island but their taxonomy, relationships, and especially ecology remain poorly documented. Based on differences in morphology, advertisement calls (where available) and phylogenetic analyses of a 787 base pair alignment from the mitochondrial ND4 gene and flanking tRNA, we describe five new species of small treefrogs from hill and lower montane forests in the high rainfall belt that straddles the southern versant of Papua New Guinea's Central Cordillera. Three of these species are known only from forest growing on karst substrates, adding to the growing number of herpetofauna species currently known only from the extensive karst habitats of Papua New Guinea's South-fold Mountains. We also describe the arboreal breeding strategies of two of the new species, and report obligate treehole (phytotelm) breeding in New Guinean frogs for the first time. The new phytotelm-breeding species has juveniles with colour and patterning that closely resemble bird droppings, suggesting defensive mimicry or masquerade. A preliminary phylogeny suggests that arboreal-breeding frogs do not form a monophyletic group and that arboreal breeding has evolved multiple times within the New Guinean pelodryadid radiation. A further striking feature of the phylogeny is poor support for most basal nodes in the most diverse radiation of Melanesian Pelodryadidae, suggesting rapid ecological diversification and speciation, potentially following colonisation from Australia and/or mountain uplift. These new taxa and observations highlight previously unrecognised ecological and reproductive diversity in the Melanesian Pelodryadidae.


Asunto(s)
Anuros , Animales , Papúa Nueva Guinea , Filogenia
7.
Commun Biol ; 5(1): 1182, 2022 11 04.
Artículo en Inglés | MEDLINE | ID: mdl-36333588

RESUMEN

Identifying hotspots of biological diversity is a key step in conservation prioritisation. Melanesia-centred on the vast island of New Guinea-is increasingly recognised for its exceptionally species-rich and endemic biota. Here we show that Melanesia has the world's most diverse insular amphibian fauna, with over 7% of recognised global frog species in less than 0.7% of the world's land area, and over 97% of species endemic. We further estimate that nearly 200 additional candidate species have been discovered but remain unnamed, pointing to a total fauna in excess of 700 species. Nearly 60% of the Melanesian frog fauna is in a lineage of direct-developing microhylids characterised by smaller distributions than co-occurring frog families, suggesting lineage-specific high beta diversity is a key driver of Melanesian anuran megadiversity. A comprehensive conservation status assessment further highlights geographic concentrations of recently described range-restricted threatened taxa that warrant urgent conservation actions. Nonetheless, by world standards, the Melanesian frog fauna is relatively intact, with 6% of assessed species listed as threatened and no documented extinctions; and thus it provides an unparalleled opportunity to understand and conserve a megadiverse and relatively intact insular biota.


Asunto(s)
Biodiversidad , Biota , Animales , Melanesia , Anuros
8.
Sci Rep ; 12(1): 16806, 2022 10 07.
Artículo en Inglés | MEDLINE | ID: mdl-36207453

RESUMEN

Humans and Acanthamoeba polyphaga mimivirus share numerous homologous genes, including collagens and collagen-modifying enzymes. To explore this homology, we performed a genome-wide comparison between human and mimivirus using DELTA-BLAST (Domain Enhanced Lookup Time Accelerated BLAST) and identified 52 new putative mimiviral proteins that are homologous with human proteins. To gain functional insights into mimiviral proteins, their human protein homologs were organized into Gene Ontology (GO) and REACTOME pathways to build a functional network. Collagen and collagen-modifying enzymes form the largest subnetwork with most nodes. Further analysis of this subnetwork identified a putative collagen glycosyltransferase R699. Protein expression test suggested that R699 is highly expressed in Escherichia coli, unlike the human collagen-modifying enzymes. Enzymatic activity assay and mass spectrometric analyses showed that R699 catalyzes the glucosylation of galactosylhydroxylysine to glucosylgalactosylhydroxylysine on collagen using uridine diphosphate glucose (UDP-glucose) but no other UDP-sugars as a sugar donor, suggesting R699 is a mimiviral collagen galactosylhydroxylysyl glucosyltransferase (GGT). To facilitate further analysis of human and mimiviral homologous proteins, we presented an interactive and searchable genome-wide comparison website for quickly browsing human and Acanthamoeba polyphaga mimivirus homologs, which is available at RRID Resource ID: SCR_022140 or https://guolab.shinyapps.io/app-mimivirus-publication/ .


Asunto(s)
Acanthamoeba , Mimiviridae , Acanthamoeba/genética , Acanthamoeba/metabolismo , Colágeno/metabolismo , Genómica , Glucosa/metabolismo , Glucosiltransferasas , Glicosiltransferasas/genética , Glicosiltransferasas/metabolismo , Humanos , Mimiviridae/genética , Azúcares/metabolismo , Uridina Difosfato Glucosa/metabolismo , Proteínas Virales/genética
9.
Cytometry B Clin Cytom ; 102(6): 487-497, 2022 11.
Artículo en Inglés | MEDLINE | ID: mdl-36134740

RESUMEN

BACKGROUND: Flow cytometric immunophenotyping is essential for the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH). Most cases have easy to interpret flow cytometry profiles with red cells, neutrophils and monocytes showing complete deficiency of glycophosphatidylinositol (GPI) linked antigen expression. Some cases are more challenging to interpret due to the presence of multiple populations of PNH cells and variable levels of GPI antigen expression. METHODS: We studied 46 known PNH patients, many with complex immunophenotypic profiles using a novel, single tube, multi-parameter 7-color immunophenotyping assay that allowed simultaneous detection and assessment of PNH clones within multiple lineages of peripheral blood leucocytes. Red cell PNH clones were also assessed in total and immature (CD71+) components by CD59 expression. RESULTS: For individual patients, total PNH clones in each cell lineage were highly correlated. Monocytes, eosinophils and basophils showed the highest proportions of PNH cells. Red cell PNH clones were typically smaller than monocyte and neutrophil PNH clones. In most cases, PNH clones were detectable in minor leucocyte populations where multiple populations of PNH cells were present, variability in the proportions of type II and type III cells was seen across different cell lineages, even though total PNH clones remained similar. CONCLUSIONS: This study shows that PNH patients with multiple PNH clones do not always display the same abnormality across all cell lineages routinely tested. There is no simple explanation for this but is likely due to a combination of complex molecular, genetic and biochemical dysfunction in different blood cell types.


Asunto(s)
Hemoglobinuria Paroxística , Humanos , Inmunofenotipificación , Linaje de la Célula , Citometría de Flujo , Antígenos CD59/metabolismo , Células Clonales
10.
Biodivers Conserv ; 31(8-9): 2045-2062, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35633848

RESUMEN

Cryptic ecologies, the Wallacean Shortfall of undocumented species' geographical ranges and the Linnaean Shortfall of undescribed diversity, are all major barriers to conservation assessment. When these factors overlap with drivers of extinction risk, such as insular distributions, the number of threatened species in a region or clade may be underestimated, a situation we term 'cryptic extinction risk'. The genus Lepidodactylus is a diverse radiation of insular and arboreal geckos that occurs across the western Pacific. Previous work on Lepidodactylus showed evidence of evolutionary displacement around continental fringes, suggesting an inherent vulnerability to extinction from factors such as competition and predation. We sought to (1) comprehensively review status and threats, (2) estimate the number of undescribed species, and (3) estimate extinction risk in data deficient and candidate species, in Lepidodactylus. From our updated IUCN Red List assessment, 60% of the 58 recognized species are threatened (n = 15) or Data Deficient (n = 21), which is higher than reported for most other lizard groups. Species from the smaller and isolated Pacific islands are of greatest conservation concern, with most either threatened or Data Deficient, and all particularly vulnerable to invasive species. We estimated 32 undescribed candidate species and linear modelling predicted that an additional 18 species, among these and the data deficient species, are threatened with extinction. Focusing efforts to resolve the taxonomy and conservation status of key taxa, especially on small islands in the Pacific, is a high priority for conserving this remarkably diverse, yet poorly understood, lizard fauna. Our data highlight how cryptic ecologies and cryptic diversity combine and lead to significant underestimation of extinction risk. Supplementary Information: The online version contains supplementary material available at 10.1007/s10531-022-02412-x.

11.
Int J Lab Hematol ; 44(5): 868-874, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-35614534

RESUMEN

INTRODUCTION: The reliable diagnosis of paroxysmal nocturnal haemoglobinuria (PNH) by flow cytometry is based on mandatory analysis of the erythroid, neutrophilic and monocytic lineages. In this study, we have evaluated the performance characteristics of a recently published immature red blood cell (iRBC) assay as a potential screening test for PNH by flow cytometry. METHODS: Intra- and inter-assay imprecision were determined in five replicates of small, medium and large PNH iRBC clones. Analytical and functional sensitivity was assessed by performing spiking tests for five replicates. Thirty healthy donors and 441 PNH patients were tested for evaluation of clinical specificity, sensitivity, positive and negative predictive values. RESULTS: Coefficients of variation (CV) for intra-/inter-assay imprecision analyses were 1.31/1.50, 3.19/2.61 and 3.99/1.58 for the big, medium and small clone sizes, respectively. Absolute values (100%) were found for both clinical specificity and sensitivity as well as for both positive and negative predictive values. The CV from 5 replicate results for 10 clustered events was 15.7%. The coefficient of determination (r2 ), Pearson's correlation coefficient (r) and Bland-Altman mean bias were 0.9436/0.9234/1.7 for PNH iRBC compared to PNH neutrophils and 0.9553/0.9387/2.1 for PNH iRBCs compared to PNH monocytes. CONCLUSION: Our results confirm very good performance characteristics, high analytical and functional sensitivity, absolute clinical specificity and sensitivity as well as favourable correlation between PNH iRBCs and both PNH neutrophils and monocytes, suggesting that this cost-effective 3-colour iRBC assay can be used as a reliable screening test for evaluation of small, medium and large PNH clones by flow cytometry.


Asunto(s)
Hemoglobinuria Paroxística , Células Clonales , Color , Eritrocitos , Citometría de Flujo/métodos , Hemoglobinuria Paroxística/diagnóstico , Humanos
12.
Zootaxa ; 5057(2): 260-270, 2021 Oct 21.
Artículo en Inglés | MEDLINE | ID: mdl-34811210

RESUMEN

We describe a new species in the Cyrtodactylus loriae group from the northern versant of New Guineas Central Cordillera in far western Papua New Guinea. Cyrtodactylus hutchinsoni sp. nov. can be distinguished from related taxa by its large size, absence of serrate tubercles on the tail and ventrolateral fold, and further aspects of scalation and colouration. Genetic data suggest the new species is closely allied to C. serratus from the southern versant of the Central Cordillera of New Guinea, providing a potential example of a species pair isolated by Pliocene uplift of these mountains. The recognised diversity of Cyrtodactylus from New Guinea and other islands east of Lydekkers Line now stands at 30 species, of which 22 have been described in the last 20 years.


Asunto(s)
Lagartos , Animales , Ecosistema , Lagartos/genética , Nueva Guinea , Filogenia
13.
Eur J Haematol ; 107(2): 211-218, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-34060690

RESUMEN

OBJECTIVES: A retrospective population-based study to determine the incidence and prevalence of patients with the rare blood disease paroxysmal nocturnal haemoglobinuria (PNH). METHODS: All patients were identified by flow cytometric detection of blood cells deficient in glycosylphosphatidylinositol (GPI) linked proteins at a single diagnostic reference laboratory that serves the Yorkshire based, Haematological Malignancy Research Network (HMRN) with a population of 3.8 million. RESULTS: One hundred and ninety-seven patients with detectable PNH clones at a level of >0.01% in at least two lineages of cells (neutrophils, monocytes and/or red cells) were identified over a 15-year period (2004-2018). Of these, 88% had aplastic anaemia (AA), 8% classical PNH and 3% myelodysplastic syndrome. The overall incidence rate was estimated at 0.35 cases per 100 000 people per year. This equates to 220 cases newly diagnosed in the United Kingdom each year. The overall prevalence rate was 3.81 per 100 000, this equates to an estimated 2400 prevalent cases in the UK. The overall and relative 5-year survival rates were 72% and 82.7%, respectively. CONCLUSIONS: This study showed that classical haemolytic PNH is a rare disease and represents only a small proportion overall of patients with detectable PNH cells, the majority of which have aplastic anaemia.


Asunto(s)
Anemia Aplásica/complicaciones , Anemia Aplásica/epidemiología , Hemoglobinuria Paroxística/complicaciones , Hemoglobinuria Paroxística/epidemiología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Anemia Aplásica/diagnóstico , Anemia Aplásica/historia , Biomarcadores , Niño , Preescolar , Femenino , Hemoglobinuria Paroxística/diagnóstico , Hemoglobinuria Paroxística/historia , Historia del Siglo XXI , Humanos , Inmunofenotipificación , Incidencia , Masculino , Persona de Mediana Edad , Vigilancia de la Población , Prevalencia , Estudios Retrospectivos , Síndrome , Reino Unido/epidemiología , Adulto Joven
14.
Zootaxa ; 4926(1): zootaxa.4926.1.2, 2021 Feb 04.
Artículo en Inglés | MEDLINE | ID: mdl-33756757

RESUMEN

We describe a new species of groundsnake of the genus Stegonotus (Colubridae) from the Purari River basin in Gulf Province, Papua New Guinea. The new species can be most readily distinguished from all other New Guinean Stegonotus by its unique dorsal colour pattern which consists of a dark head and creamy-white anterior one third to two thirds of the body, grading into increasingly dense dark pigmentation on the posterior of the body and tail. It is most similar to S. iridis from the Raja Ampat Archipelago off western New Guinea, but that species has a different pattern of pigmentation dorsally, has a lower ventral scale count (198-211 vs. 229-239), and exhibits a different temporal scale arrangement. The description of S. aplini sp. nov. brings to fourteen the number of Stegonotus species described from New Guinea. A dichotomous key to described species in the New Guinea region is provided.


Asunto(s)
Colubridae , Animales , Color , Nueva Guinea , Papúa Nueva Guinea , Ríos
15.
Zootaxa ; 4933(2): zootaxa.4933.2.3, 2021 Feb 19.
Artículo en Inglés | MEDLINE | ID: mdl-33756796

RESUMEN

We show that the Wotjulum frog, Litoria watjulumensis (Copland, 1957), comprises two deeply divergent mitochondrial DNA lineages that are also reciprocally monophyletic for a nuclear gene locus and have discrete distributions. The taxa are differentiated in multivariate analysis of shape but show no appreciable differences in colour and pattern. The two taxa differ substantially in the degree of female biased sexual size dimorphism, with the western taxon showing considerably more pronounced dimorphism. We subsequently resurrect Litoria (Hyla) spaldingi (Hosmer, 1964) for populations from east of the Daly River system in the Northern Territory through to western Queensland and restrict L. watjulumensis to populations from the Kimberley region of north-western Australia and the Victoria River system of the western Northern Territory. The complex advertisement call of L. spaldingi is described for the first time.


Asunto(s)
Anuros , ADN Mitocondrial , Animales , Anuros/genética , ADN Mitocondrial/genética , Femenino , Filogenia
17.
Zootaxa ; 4903(1): zootaxa.4903.1.7, 2021 Jan 06.
Artículo en Inglés | MEDLINE | ID: mdl-33757109

RESUMEN

We describe a new species of large green treefrog from southern Papua Province, Indonesia. Litoria lubisi sp. nov. is placed in the L. infrafrenata Group based on its size, colouration and configuration of hand webbing, but differs from other members of this group in aspects of body size and proportions, extent of hand webbing, colouration and male advertisement call. Litoria lubisi sp. nov. occurs in close proximity to two other species in the L. infrafrenata Group, emphasising an until-recently overlooked pattern that multiple species of large, arboreal Litoria have overlapping distributions across much of the lowlands of southern New Guinea.


Asunto(s)
Anuros , Árboles , Animales , Tamaño Corporal , Indonesia , Masculino
18.
Zootaxa ; 4728(3): zootaxa.4728.3.3, 2020 Jan 23.
Artículo en Inglés | MEDLINE | ID: mdl-32230562

RESUMEN

Systematic investigations of vertebrate faunas from the islands of Melanesia are revealing high levels of endemism, dynamic biogeographic histories, and in some cases surprisingly long evolutionary histories of insularity. The bent-toed geckos in the Cyrtodactylus sermowaiensis Group mainly occur in northern New Guinea and nearby islands, however a further isolated population occurs on Manus Island in the Admiralty Archipelago approximately 300 km to the north of New Guinea. Here we first present a review of the genetic diversity, morphological variation and distribution of Cyrtodactylus sermowaiensis from northern New Guinea. Genetic structure and distributional records within Cyrtodactylus sermowaiensis broadly overlap with underlying Terranes in northern New Guinea, suggesting divergence on former islands that have been obscured by the infill and uplift of sedimentary basins since the late Pleistocene. Based on a combination of genetic and morphological differentiation we then describe the population from Manus Island as a new species, Cyrtodactylus crustulus sp. nov. This new species emphasises the high biological endemism and conservation significance of the Admiralty Islands, and especially Manus Island.


Asunto(s)
Lagartos , Animales , Evolución Biológica , Color , Ecosistema , Filogenia
19.
Br J Haematol ; 189(5): 954-966, 2020 06.
Artículo en Inglés | MEDLINE | ID: mdl-32103498

RESUMEN

A retrospective analysis of presentation clinical, laboratory and immunophenotypic features of 1 081 patients with paroxysmal nocturnal haemoglobinuria (PNH) clones [glycosylphosphatidylinositol (GPI)-deficient blood cells] identified at our hospital by flow cytometry over the past 25 years was undertaken. Three distinct clusters of patients were identified and significant correlations between presentation disease type and PNH clone sizes were evident. Smaller PNH clones predominate in cytopenic and myelodysplastic subtypes; large PNH clones were associated with haemolytic, thrombotic and haemolytic/thrombotic subtypes. Rare cases with an associated chronic myeloproliferative disorder had either large or small PNH clones. Cytopenia was a frequent finding, highlighting bone marrow failure as the major underlying feature associated with the detection of PNH clones in the peripheral blood. Red cell PNH clones showed significant correlations between the presence of type II (partial GPI deficiency) red cells and thrombotic disease. Haemolytic PNH was associated with type III (complete GPI deficiency) red cell populations of >20%. Those with both haemolytic and thrombotic features had major type II and type III red cell populations. Distinct patterns of presentation age decade were evident for clinical subtypes with a peak incidence of haemolytic PNH in the 30-49 year age group and a biphasic age distribution for the cytopenia group.


Asunto(s)
Glicosilfosfatidilinositoles/deficiencia , Hemoglobinuria Paroxística/sangre , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Anemia Aplásica/etiología , Anemia Hemolítica/etiología , Antígenos CD55/deficiencia , Antígenos CD59/deficiencia , Niño , Preescolar , Evolución Clonal , Células Clonales/patología , Progresión de la Enfermedad , Femenino , Citometría de Flujo , Hemoglobinuria Paroxística/complicaciones , Hemoglobinuria Paroxística/genética , Hemoglobinuria Paroxística/patología , Humanos , Inmunofenotipificación , Lactante , Linfocitos/patología , Masculino , Persona de Mediana Edad , Trastornos Mieloproliferativos/etiología , Neutrófilos/patología , Receptores de Transferrina/sangre , Estudios Retrospectivos , Trombosis/etiología , Adulto Joven
20.
Cytometry B Clin Cytom ; 98(2): 179-192, 2020 03.
Artículo en Inglés | MEDLINE | ID: mdl-31705743

RESUMEN

BACKGROUND: The diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) relies on flow cytometric demonstration of loss of glycosyl-phosphatidyl inositol (GPI)-anchored proteins from red blood cells (RBC) and white blood cells (WBC). High-sensitivity multiparameter assays have been developed to detect loss of GPI-linked structures on PNH neutrophils and monocytes. High-sensitivity assays to detect PNH phenotypes in RBCs have also been developed that rely on the loss of GPI-linked CD59 on CD235a-gated mature RBCs. The latter is used to delineate PNH Type III (total loss of CD59) and PNH Type II RBCs (partial loss of CD59) from normal (Type I) RBCs. However, it is often very difficult to delineate these subsets, especially in patients with large PNH clones who continue to receive RBC transfusions, even while on eculizumab therapy. METHODS: We have added allophycocyanin (APC)-conjugated CD71 to the existing CD235aFITC/CD59PE RBC assay allowing simultaneous delineation and quantification of PNH Type III and Type II immature RBCs (iRBCs). RESULTS: We analyzed 24 medium to large-clone PNH samples (>10% PNH WBC clone size) for PNH Neutrophil, PNH Monocyte, Type III and Type II PNH iRBCs, and where possible, Type III and Type II PNH RBCs. The ability to delineate PNH Type III, Type II, and Type I iRBCs was more objective compared to that in mature RBCs. Additionally, total PNH iRBC clone sizes were very similar to PNH WBC clone sizes. CONCLUSIONS: Addition of CD71 significantly improves the ability to analyze PNH clone sizes in the RBC lineage, regardless of patient hemolytic and/or transfusion status.


Asunto(s)
Antígenos CD/fisiología , Eritrocitos/metabolismo , Citometría de Flujo/métodos , Hemoglobinuria Paroxística/diagnóstico , Receptores de Transferrina/fisiología , Antígenos CD/sangre , Antígenos CD59/metabolismo , Diferenciación Celular , Estudios de Cohortes , Diagnóstico Diferencial , Eritrocitos/patología , Citometría de Flujo/instrumentación , Citometría de Flujo/normas , Glicoforinas/metabolismo , Hemoglobinuria Paroxística/sangre , Hemoglobinuria Paroxística/clasificación , Hemoglobinuria Paroxística/patología , Humanos , Inmunofenotipificación/instrumentación , Inmunofenotipificación/métodos , Inmunofenotipificación/normas , Recuento de Leucocitos/instrumentación , Recuento de Leucocitos/métodos , Leucocitos/patología , Monocitos/metabolismo , Monocitos/patología , Neutrófilos/metabolismo , Neutrófilos/patología , Receptores de Transferrina/sangre
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