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1.
Rheumatology (Oxford) ; 60(10): 4717-4727, 2021 10 02.
Artículo en Inglés | MEDLINE | ID: mdl-33521812

RESUMEN

BACKGROUND: Patients' objectives and experiences must be core to the study and management of chronic diseases, such as SSc. Although patient-reported outcomes are attracting increasing attention, evaluation of the impact of disease on the overall subjective well-being, equivalent to 'happiness', is remarkably lacking. OBJECTIVES: To examine the determinants of happiness and quality of life in patients with SSc, with emphasis on disease features and personality traits. METHODS: Observational, cross-sectional multicentre study, including 142 patients, with complete data regarding disease activity, disease impact, personality, health-related quality of life (HR-QoL) and happiness. Structural equation modelling was used to evaluate the association between the variables. RESULTS: The results indicated an acceptable fit of the model to the data. Perceived disease impact had a significant negative direct relation with HR-QoL (ß = -0.79, P < 0.001) and with happiness (ß = -0.52, P < 0.001). Positive personality traits had a positive relation with happiness (ß = 0.36, P = 0.002) and an important indirect association upon QoL (ß = 0.43) and happiness (ß = 0.23). Perceived disease impact is influenced by body image, fatigue and SSc-related disability to a higher degree (ß = 0.6-0.7) than by disease activity (ß = 0.28) or form (ß = 0.17). Impact of disease had a much stronger relation with HR-QoL than with happiness. CONCLUSIONS: The results suggest that treatment strategies targeting not only disease control but also the mitigation of relevant domains of disease impact (body image, fatigue, global disability) may be important to improve patients' experience of the disease. The reinforcement of resilience factors, such as positive psychological traits, may also play a contributory role towards better patient outcomes.


Asunto(s)
Felicidad , Calidad de Vida/psicología , Esclerodermia Sistémica/psicología , Anciano , Estudios Transversales , Femenino , Humanos , Análisis de Clases Latentes , Masculino , Persona de Mediana Edad , Personalidad , Resiliencia Psicológica , Índice de Severidad de la Enfermedad
2.
J Neurovirol ; 27(2): 364-366, 2021 04.
Artículo en Inglés | MEDLINE | ID: mdl-33721197

RESUMEN

Cytomegalovirus (CMV) infection is common worldwide, but is usually a subclinical or self-limited infection in immunocompetent patients. On the contrary, most of the ocular and central nervous system involvement occurs in immunosuppressed patient, and usually has severe consequences. Ocular manifestations of CMV infection are frequent in immunosuppressed patients (notably keratouveitis, retinitis and retinal branch angiitis), but a few cases of optic neuropathy (mostly papillitis) have been reported in the literature in immunocompetent patients. We report a case of a young and previously healthy female patient who developed a CMV optic neuropathy after a presumed CMV reinfection. Viral copies were detected in both blood and cerebrospinal fluid, as well as a high IgG titre and no evidence of immunosuppression was found. Clinical improvement was seen after high-dose corticosteroids and ganciclovir.


Asunto(s)
Infecciones por Citomegalovirus/complicaciones , Enfermedades del Nervio Óptico/virología , Femenino , Humanos , Inmunocompetencia
3.
Mol Phylogenet Evol ; 145: 106738, 2020 04.
Artículo en Inglés | MEDLINE | ID: mdl-32001365

RESUMEN

We investigated species relationships in Astraea, a primarily Neotropical genus of tribe Crotoneae centered in Brazil, using data from the nuclear ribosomal ITS, and the plastid trnL-trnF and psbA-trnH spacers. With all species of Astraea sampled, along with representatives from across Crotoneae, the evolutionary history of Astraea was interpreted in a broader framework, as well as divergence time estimates and reconstructions of ancestral areas and morphological character states for Crotoneae. Our results show that Astraea is monophyletic, consisting of three main clades, and that most of its diversification took place from the Oligocene to the Pliocene, coincident with the formation of the South American "dry diagonal". As for Crotoneae, our data show incongruent phylogenetic positions between the nuclear and chloroplast data for most of its genera, and that the ancestor of the tribe was probably arborescent and might have occupied the Amazon Basin, most likely in moist forest, from which it spread throughout South America in the early Eocene. Ancestral state reconstruction recovered deeply lobed leaves and staminate petals bearing moniliform trichomes as putative synapomorphies for Astraea, whereas the absence or strong reduction of pistillate petals is widespread in Crotoneae and may be a synapomorphy for the tribe.


Asunto(s)
Evolución Biológica , Euphorbiaceae/clasificación , Teorema de Bayes , Brasil , Euphorbiaceae/anatomía & histología , Euphorbiaceae/genética , Bosques , Intrones , Filogenia , Filogeografía , Plastidios/genética
6.
Epilepsy Behav ; 55: 75-8, 2016 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-26773673

RESUMEN

The occurrence of seizures in specific types of epilepsies can follow a 24-hour nonuniform or nonrandom pattern. We described the 24-hour pattern of clinical seizures in patients with focal refractory epilepsy who underwent video-electroencephalography monitoring. Only patients who were candidates for epilepsy surgery with an unequivocal seizure focus were included in the study. A total of 544 seizures from 123 consecutive patients were analyzed. Specific time of seizures were distributed along 3- or 4-hour time blocks or bins throughout the 24-hour period. The mean age of the subjects was 37.7 years, with standard deviation of 11.5 years, median of 37. The majority were females (70/56%). The majority of patients had a seizure focus located in the mesial temporal lobe (102/83%) and in the neocortical temporal lobe (13/11%). The remaining patients had a seizure focus located in the extratemporal lobe (8/6%). The most common etiology was mesial temporal sclerosis (86/69.9%). Nonuniform seizure distribution was observed in seizures arising from the temporal lobe (mesial temporal lobe and neocortical temporal lobe), with two peaks found in both 3- and 4-hour bins: 10:00-13:00/16:00-19:00 and 08:00-12:00/16:00-20:00 respectively (p=0.004). No specific 24-hour pattern was identified in seizures from extratemporal location. The 24-hour rhythmicity of seizure distribution is recognized in certain types of epilepsy, but studies on the topic are scarce. Their replication and validation is therefore needed. Our study confirms the bimodal pattern of temporal lobe epilepsy independently of the nature of the lesion. However, peak times differ between different studies, suggesting that the ambient, rhythmic exogenous factors or environmental/social zeitgebers, may modulate the 24-hour rhythmicity of seizures. Characterization of these 24-hour patterns of seizure occurrence can influence diagnosis and treatment in selected types of epilepsy, such as the case of temporal lobe epilepsy, the most common drug-resistant epilepsy.


Asunto(s)
Ritmo Circadiano/fisiología , Epilepsia del Lóbulo Temporal/diagnóstico , Epilepsia del Lóbulo Temporal/fisiopatología , Convulsiones/diagnóstico , Convulsiones/fisiopatología , Adolescente , Adulto , Electroencefalografía/métodos , Epilepsias Parciales/diagnóstico , Epilepsias Parciales/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Lóbulo Temporal/fisiopatología , Adulto Joven
7.
Stroke ; 46(4): 1110-2, 2015 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-25712943

RESUMEN

BACKGROUND AND PURPOSE: Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is a rare form of nonhypertensive cerebral small-vessel disease caused by mutations in the HTRA1 gene. CARASIL is characterized by early adulthood onset of subcortical infarcts, cognitive impairment, alopecia, and spondylosis. Until recently, this disorder was almost exclusively reported in the Asian population. METHODS: Description of the clinical, imaging, and genetic study of 2 siblings with CARASIL, with a brief comparative review of published non-Asian cases of the disease. RESULTS: Both patients exhibited the typical phenotype: cerebral small-vessel disease, spondylosis, and abnormal hair lost. Mutation screening was performed for NOTCH3 and HTRA1 genes. No mutations were found in NOTCH3. The study revealed the presence of a homozygous c.496C>T substitution in HTRA1 in both siblings. CONCLUSION: This report highlights the need of considering this entity in the differential diagnosis of cerebral small-vessel disease in young patients, even in the non-Asian populations.


Asunto(s)
Alopecia , Infarto Cerebral , Leucoencefalopatías , Enfermedades de la Columna Vertebral , Adulto , Alopecia/genética , Alopecia/patología , Alopecia/fisiopatología , Infarto Cerebral/genética , Infarto Cerebral/patología , Infarto Cerebral/fisiopatología , Femenino , Humanos , Leucoencefalopatías/genética , Leucoencefalopatías/patología , Leucoencefalopatías/fisiopatología , Masculino , Persona de Mediana Edad , Portugal , Hermanos , Enfermedades de la Columna Vertebral/genética , Enfermedades de la Columna Vertebral/patología , Enfermedades de la Columna Vertebral/fisiopatología
8.
Rheumatology (Oxford) ; 54(6): 981-93, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-25398382

RESUMEN

OBJECTIVE: The aim of this study was to systematically review outcome domains and measurement tools used in gout trials and their accordance with the preliminary OMERACT gout recommendations published in 2005. METHODS: Randomized controlled trials (RCTs) and quasi-RCTs investigating any intervention for gout published up to February 2013 were included. Recruitment start dates and all measured outcomes were extracted. Risk of bias (RoB) was assessed with the Cochrane Collaboration tool. Numbers of OMERACT domains were compared for trials at low vs unclear/high RoB and for recruitment start date before 2005 or 2005 and later. RESULTS: Of 9784 articles screened, 38 acute and 30 chronic gout trials were included. Mean (s.d.) number of OMERACT outcomes was 2.9 (1.1) (out of 5) and 2.5 (1.2) (out of 9) for acute and chronic gout trials, respectively. Health-related quality of life, participation and joint damage imaging were not assessed in any trial. Tools used to measure individual domains varied widely. There were no differences in the number of OMERACT outcomes reported in acute or chronic gout trials recruiting before 2005 vs 2005 or later [mean (s.d.): 3.0 (1.1) vs 3.5 (1.3), P = 0.859 and 2.7 (1.1) vs 2.8 (1.4), P = 0.960, respectively]. While both acute and chronic trials at low RoB reported more OMERACT domains than trials at unclear/high RoB, these differences were not significant. Industry-funded trials and trials performed by OMERACT investigators reported more OMERACT outcome domains. CONCLUSION: We found no appreciable impact of the OMERACT recommendations for gout trials to date.


Asunto(s)
Gota/tratamiento farmacológico , Guías de Práctica Clínica como Asunto , Ensayos Clínicos Controlados Aleatorios como Asunto , Enfermedad Aguda , Enfermedad Crónica , Humanos , Resultado del Tratamiento
9.
Am J Bot ; 102(6): 833-47, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-26101410

RESUMEN

PREMISE OF THE STUDY: Phylogenetic and morphological studies have helped clarify the systematics of large and complex groups such as the tribe Crotoneae (Euphorbiaceae). However, very little is known about the diversity, structure, and function of anatomical features in this tribe. Crotoneae comprises the species-rich pantropical genus Croton and six small neotropical genera. Here we characterized the anatomy of leaf secretory structures in members of this tribe and explored their function and evolutionary significance. METHODS: Young and mature leaves of 26 species were studied using standard anatomical light microscopy techniques. Three sections of Croton and one representative of Brasiliocroton and Astraea were sampled. KEY RESULTS: We identified five types of secretory structures: laticifers, colleters, extrafloral nectaries, idioblasts, and secretory trichomes. Laticifers were present in all species studied except Croton alabamensis, which instead presented secretory parenchyma cells. Articulated laticifers are reported in Crotoneae for the first time. Colleters of the standard type were observed in the majority of the sampled taxa. Extrafloral nectaries were present in section Cleodora and in B. mamoninha, but absent in section Lamprocroton and Astraea lobata. Idioblasts were spread throughout the palisade and/or spongy parenchyma in most of the studied species. Secretory trichomes were restricted to Lamprocroton except for C. imbricatus. CONCLUSIONS: This study revealed a high diversity of secretory structures, including novel ones, in one of the largest clades of Euphorbiaceae. Our results are promising for investigations on the anatomical and ecophysiological bases of species diversification within Euphorbiaceae.


Asunto(s)
Biodiversidad , Evolución Biológica , Euphorbiaceae/anatomía & histología , Hojas de la Planta/anatomía & histología , Euphorbiaceae/citología , Filogenia , Hojas de la Planta/citología , Especificidad de la Especie
11.
Biology (Basel) ; 12(2)2023 Jan 21.
Artículo en Inglés | MEDLINE | ID: mdl-36829452

RESUMEN

The subtribe Ditaxinae in the plant family Euphorbiaceae is composed of five genera (Argythamnia, Caperonia, Chiropetalum, Ditaxis and Philyra) and approximately 120 species of perennial herbs (rarely annual) to treelets. The subtribe is distributed throughout the Americas, with the exception of Caperonia, which also occurs in tropical Africa and Madagascar. Under the current classification, Ditaxinae includes genera with a questionable morphology-based taxonomy, especially Argythamnia, Chiropetalum and Ditaxis. Moreover, phylogenetic relationships among genera are largely unexplored, with previous works sampling <10% of taxa, showing Ditaxinae as paraphyletic. In this study, we inferred the phylogenetic relationships within Ditaxinae and related taxa using a dataset of nuclear (ETS, ITS) and plastid (petD, trnLF, trnTL) DNA sequences and a wide taxon sampling (60%). We confirmed the paraphyly of Ditaxinae and Ditaxis, both with high support. Following our phylogenetic results, we combined Ditaxis in Argythamnia and upgraded Ditaxinae to the tribe level (Ditaxeae). We also established and described the tribe Caperonieae based on Caperonia, and transferred Philyra to the tribe Adelieae, along with Adelia, Garciadelia, Lasiocroton and Leucocroton. Finally, we discuss the main morphological synapomorphies for the genera and tribes and provide a taxonomic treatment, including all species recognized under each genus.

12.
J Multidiscip Healthc ; 16: 1127-1139, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37131932

RESUMEN

Background: Idiopathic inflammatory myopathies (IIM) are a rare heterogeneous group of diseases characterised by chronic skeletal muscle inflammation, but other organs are also frequently involved. IMM represent a diagnostic challenge and a multidisciplinary approach is important to ensure successful diagnosis and adequate follow-up of these patients. Objective: To describe the general functioning of our multidisciplinary myositis clinic, highlighting the benefits of multidisciplinary team management in patients with confirmed or suspected IIM and to characterise our clinical experience. Methods: Description of the organization of a dedicated multidisciplinary myositis outpatient clinic, supported by IMM specific electronic assessment tools and protocols based on our Portuguese Register - Reuma.pt. In addition, an overview of our activity between 2017 and 2022 is provided. Results: An IIM multidisciplinary care clinic, based on a close collaboration between Rheumatologists, Dermatologists and Physiatrist is detailed in this paper. One hundred and eighty-five patients were assessed in our myositis clinic; 138 (75%) of those were female, with a median age of 58 [45-70] years. At the last appointment, 130 patients had a confirmed IIM diagnosis, and the mean disease duration was 4 [2-6] years. The most frequent diagnosis was dermatomyositis (n = 34, 26.2%), followed by antisynthetase syndrome (n = 27, 20.8%) and clinically amyopathic/paucimyopathic dermatomyositis (n = 18, 13.8%). Twenty-four patients (18.5%) were on monotherapy and 94 (72.3%) were on combination therapy. Conclusion: A multidisciplinary approach is important to ensure the correct diagnosis and follow-up of these patients. A myositis clinic, with a standardised practice at a tertiary hospital level, contributes to a standardization of care and opens research opportunities.

13.
ARP Rheumatol ; 1(2): 168-173, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35810375

RESUMEN

Interstitial lung disease (ILD) occurs with Idiopathic Inflammatory Myopathy (IIM) as a life-threating complication and is considered the most important prognostic determinant in this disease group. The antibody anti-melanoma differentiation-associated gene 5 (anti-MDA5) is associated to rapidly progressive ILD and poor overall survival. Rituximab (RTX) is becoming a drug of choice in management of refractory IIM-ILDs and rapidly progressive ILDs, despite its low level of evidence. We report the case of a 49-year-old man with new-onset clinically amyopathic dermatomyositis (CADM) with severe respiratory symptoms and mixed radiologic pattern of non-specific interstitial and organizing pneumonia, refractory to high dose corticosteroids and intravenous immunoglobulin and oxygen dependent. He was started on RTX 375mg/m2/week of which he completed 4 perfusions, with significant clinical improvement, and has been on maintenance to date with the rheumatology RTX standard protocol with no need for oxygen supplementation. RTX may represent a rescue therapy for patients with severe anti-MDA5-related CADM-ILD refractory to conventional immunotherapies. We identified reports of a total of 12 patients treated with RTX. Infection was the only reported adverse event (25%). Respiratory improvement (defined by symptoms, imaging or PFTs) was observed in 75% of patients, with 2 (17%) having achieved clinical remission. A total of three deaths occurred (25%), all resulting from ILD progression despite treatment. No therapeutic protocol with RTX seems to be more efficient nor associated with more adverse events than the others. Comparative studies are necessary.


Asunto(s)
Dermatomiositis , Enfermedades Pulmonares Intersticiales , Miositis , Autoanticuerpos/uso terapéutico , Dermatomiositis/complicaciones , Humanos , Helicasa Inducida por Interferón IFIH1 , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Miositis/complicaciones , Rituximab/uso terapéutico
14.
Plants (Basel) ; 11(8)2022 Apr 15.
Artículo en Inglés | MEDLINE | ID: mdl-35448804

RESUMEN

Stem succulence evolved independently in many plant lineages as an adaptation to arid environments. One of the most interesting cases is the convergence between Cactaceae and Euphorbia, which have anatomical adaptations mostly to increase photosynthetic capability and water storage. Our goal was to describe the shoot development in two succulent species of Euphorbia using light microscopy coupled with high-resolution X-ray-computed tomography. Collateral cortical bundles were observed associated with the stem ribs in both species. The analysis of vasculature demonstrated that these bundles are, in fact, leaf traces that run axially along a portion of the internode. That structural pattern is due to an ontogenetic alteration. During shoot development, the leaf-bases remain adnate to the stem near the SAM, forming an axial component. When the internode elongates, the leaf bundles stretch as cortical bundles. The meristematic activity associated with the bundles forms the stem ribs, as leaf veins near the node, and induce rib formation along the entire internode even in the portion where the leaf traces join the stele. In addition, heterochronic shifts are also involved in the evolution of the shoot system in these Euphorbia, being related to early deciduous reduced leaves and the transference of the main photosynthetic function to the stem. This study demonstrates for the first time the influence of leaf developmental shifts and stem rib formation in Euphorbia and sheds new light on the evolution of stem succulence.

15.
ARP Rheumatol ; 1(ARP Rheumatology, nº3 2022): 190-196, 2022 11 01.
Artículo en Inglés | MEDLINE | ID: mdl-35891592

RESUMEN

BACKGROUND: Antisynthetase syndrome (ASyS) is characterised by the association of inflammatory myopathy, interstitial lung disease (ILD), arthritis, Raynaud's phenomenon (RP) or mechanic's hands (MH), with the presence of anti-aminoacyl-tRNA-synthetase antibodies (anti-ARS). It has been suggested that different anti-ARS may be associated with distinct clinical pictures. OBJECTIVE: To characterise the clinical and immunological features of a multicentric nationwide cohort of ASyS patients. METHODS: This is a multicentre retrospective cohort study including patients with ASyS from nine Portuguese rheumatology centres. Data on patients' demographics, signs and symptoms, laboratory results, pulmonary imaging findings and treatment with immunomodulators were collected. Comparison between patients with different anti-ARS antibodies was made using the Chi-square test for categorical variables and Student's t-test or Man-Whitney test for continuous variables, considering anti-Jo1 positive patients as the reference group. RESULTS: Seventy patients were included (70% female) with a median age in years at disease onset of 52 (15-75) years and median follow-up time of 3 years (range 0-32). The three most common clinical manifestations were ILD (n=53, 75.7%), followed by arthritis (n=43, 61.4%) and myositis (n=37, 52.9%). Forty-three patients were positive for anti-Jo1 (61.4%), 11 for anti-PL12 (15.7%), 10 for anti-PL7 (14.3%), 4 for anti-EJ (5.7%), and 2 for anti-OJ (2.9%) antibodies. Antibody co-positivity with anti-Ro52 antibodies was found in 15 patients (21.4%) and was more prevalent in anti-Jo1 patients. ILD prevalence was similar in the different anti-ARS subgroups, without statistically significant differences. Patients positive for anti-PL7 antibodies had significantly lower risk of presenting arthritis (p =< 0.05) and those positive for anti-PL-12 antibodies had a significantly lower risk of presenting myositis than the reference group of anti-Jo1 positive patients (p =< 0.05). RP was more frequently found in patients positive for anti-PL-12 than in anti-Jo1-positive patients (p =< 0.05). Malignancies were reported in four (5.7%) patients, none of whom were anti-Ro52-positive, and one of such patients had a double malignancy. Only three deaths were reported. Corticosteroids were the most frequently prescribed therapy and the use of immunosuppressive drugs was decided according to the type of predominant clinical manifestation. CONCLUSION: The three most common clinical manifestations were ILD, followed by arthritis and myositis. Patients positive for anti-PL7 antibodies had significantly lower risk of presenting arthritis and those positive for anti-PL-12 antibodies had a significantly lower risk of presenting myositis than the reference group of anti-Jo1 positive patients. RP was more frequently found in patients positive for anti-PL-12 than in anti-Jo1-positive patients. Corticosteroids were the most frequently prescribed therapy. These results are generally concordant with data retrieved from international cohorts.


Asunto(s)
Artritis , Enfermedades Pulmonares Intersticiales , Miositis , Humanos , Femenino , Masculino , Estudios Retrospectivos , Autoanticuerpos , Miositis/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/diagnóstico , Estudios de Cohortes , Anticuerpos Antinucleares/uso terapéutico , Artritis/diagnóstico
16.
ARP Rheumatol ; 1(1): 21-29, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35633574

RESUMEN

BACKGROUND: Systemic sclerosis (SSc) is a rare connective tissue disorder with heterogeneous manifestations and outcomes. Besides differences in disease characteristics among distinct ethnic groups and geographical regions, several questions regarding the impact of the disease and the effectiveness of treatments remain unanswered. To address these questions, the Rheumatic Diseases Portuguese Register (Reuma.pt) launched a specific protocol for the prospective follow-up of SSc patients. OBJECTIVES: To describe the baseline characteristics, disease subsets, treatments used and survival of SSc patients registered in Reuma.pt/SSc. METHODS: Data from adult patients with SSc included in Reuma.pt up to November 2020 were analysed. Demographic features, SSc subsets, fulfilment of classification criteria, main clinical and immunological features, comorbidities, treatments used and survival data were described and compared between diffuse cutaneous (dc) and limited cutaneous (lc) disease subsets. Survival was calculated for patients included in Reuma.pt within the first two years of diagnosis. RESULTS: In total, 1054 patients were included, 87.5% female, with a mean age at diagnosis of 52.7 +/- 14.8 years. The most common subset was lcSSc (56.3%), followed by dcSSc (17.5%), preclinical SSc (13%), overlap syndrome (9.8%) and SSc sine scleroderma (3.3%). Raynaud's phenomenon (93.4%) and skin thickening (76.9%) were the most frequently observed clinical manifestations. Gastrointestinal (62.8% versus 47.8%), pulmonary (59.5% versus 23%) and cardiac (12.8% versus 6.9%) involvements were significantly more prevalent in dcSSc than lcSSc. Ninety per-cent of patients were Antinuclear antibody positive, 52.5% were Anti-centromere antibody positive and 21% anti-topoisomerase positive, with significant differences between lcSSc and dcSSc. One-third of patients were treated with immunomodulators, 53.6% with vasodilators, 23% with glucocorticoids and 2.3% with biologics. During follow-up, 83 deaths (7.9%) were reported. The overall 1-, 2- and 5-year survivals were 98.0%, 96.8% and 92.6%, respectively, without significant differences between lcSSc and dcSSc. CONCLUSION: Reuma.pt/SSc data highlights the importance of registries in improving knowledge about rare and complex diseases, such as SSc. Clinical features of Portuguese SSc patients are similar to those of other populations. In recently diagnosed patients, 5-year survival is over 92%. To the best of our knowledge, this is the first study showing that clinical features of Portuguese SSc are similar to those of other cohorts.


Asunto(s)
Síndrome CREST , Enfermedades del Tejido Conjuntivo , Esclerodermia Difusa , Esclerodermia Sistémica , Enfermedades de la Piel , Adulto , Anticuerpos Antinucleares , Femenino , Humanos , Masculino , Estudios Prospectivos , Sistema de Registros , Esclerodermia Difusa/diagnóstico , Esclerodermia Sistémica/diagnóstico
17.
Clin Rheumatol ; 41(4): 1139-1144, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-34799767

RESUMEN

Evidence for the role of sex in the clinical manifestations of systemic sclerosis (SSc) patients is emerging. Some multicenter cohorts have shown that male SSc patients have more severe disease and worse survival. To assess the differences in clinical manifestations and survival in Portuguese SSc patients according to gender. Data from male and female adult SSc patients included in the Rheumatic Diseases Portuguese Register (Reuma.pt) were analysed and compared. Survival was calculated for patients included in Reuma.pt. within the first two years of diagnosis (inception cohort). In total, 1054 adult patients with SSc were included, 12.5% males. No differences in demographic features and comorbidities were found between the sexes, except for a higher rate of cigarette smokers among men. Diffuse cutaneous SSc and anti-topoisomerase antibodies were more prevalent in males than females. Additionally, male patients presented significantly more myositis, interstitial lung disease and gastric involvement. There were no differences in the patterns of drug use between the sexes. During follow-up, more deaths were reported in men than women (12.1% vs 7.3%, p = 0.04). The overall 1-, 3-, and 5-year survivals from diagnosis of the inception cohort (N = 469) for men vs women were 96.4% vs 98.2%, 93% vs 95.9%, and 75.8% vs 93.2%, respectively, with statistically significant differences (p < 0.01). This study confirms the existence of gender differences in clinical and immunological SSc features. Although SSc is less common in men than women, men have a more severe expression of skin and internal organ involvement and worse survival. Key Points • There are differences in SSc disease manifestations between sexes. • Males more commonly have diffuse cutaneous SSc, anti-topoisomerase antibodies, pulmonary and musculoskeletal involvement. • In the inception cohort, men had worse survival rates than women.


Asunto(s)
Esclerodermia Difusa , Esclerodermia Sistémica , Adulto , Estudios de Cohortes , Femenino , Humanos , Masculino , Portugal/epidemiología , Esclerodermia Difusa/diagnóstico , Esclerodermia Sistémica/diagnóstico , Factores Sexuales
18.
Mol Phylogenet Evol ; 60(2): 193-206, 2011 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-21554970

RESUMEN

Phylogenetic relationships of Croton section Cleodora (Klotzsch) Baill. were evaluated using the nuclear ribosomal ITS and the chloroplast trnL-F and trnH-psbA regions. Our results show a strongly supported clade containing most previously recognized section Cleodora species, plus some other species morphologically similar to them. Two morphological synapomorphies that support section Cleodora as a clade include pistillate flowers in which the sepals overlap to some degree, and styles that are connate at the base to varying degrees. The evolution of vegetative and floral characters that have previously been relied on for taxonomic decisions within this group are evaluated in light of the phylogenetic hypotheses. Within section Cleodora there are two well-supported clades, which are proposed here as subsections (subsection Sphaerogyni and subsection Spruceani). The resulting phylogenetic hypothesis identifies the closest relatives of the medicinally important and essential oil-rich Croton cajucara Benth. as candidates for future screening in phytochemical and pharmacological studies.


Asunto(s)
Evolución Biológica , Croton/genética , Filogenia , Secuencia de Bases , Brasil , Croton/anatomía & histología , Croton/clasificación , ADN de Cloroplastos/química , ADN de Cloroplastos/genética , ADN Espaciador Ribosómico/química , ADN Espaciador Ribosómico/genética , Flores/anatomía & histología , Genes de Plantas/genética , Datos de Secuencia Molecular , Fenotipo , Plantas Medicinales/anatomía & histología , Plantas Medicinales/genética , Alineación de Secuencia , Análisis de Secuencia de ADN , Especificidad de la Especie
19.
Acta Reumatol Port ; 46(3): 257-265, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34628459

RESUMEN

Systemic sclerosis (SSc) is an uncommon condition, with a wide range of manifestations, characterized by specific antibody production, vasculopathy and fibrosis of the skin and other internal organs. It is a complex disease, which is estimated to be rare in Portugal, although specific incidence data are missing. The aetiology of SSc remains unknown, but is likely to be multifactorial, involving genetic and environmental aspects. Its management is challenging and often requires a multidisciplinary approach. In 2011, we established a dedicated outpatient clinic for patients with SSc. Clinical data of every patient with a confirmed diagnosis of SSc is prospectively registered in Reuma.pt/SSc. In this manuscript, we aim to describe the general functioning of our SSc outpatient clinic, and to characterise the population of patients with SSc who are followed herein.


Asunto(s)
Esclerodermia Sistémica , Instituciones de Atención Ambulatoria , Fibrosis , Humanos , Esclerodermia Sistémica/diagnóstico , Esclerodermia Sistémica/patología , Esclerodermia Sistémica/terapia , Piel/patología , Centros de Atención Terciaria
20.
Pharm Biol ; 48(4): 433-8, 2010 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-20645723

RESUMEN

The GC-MS analysis revealed that the leaf essential oils of Myrciaria tenella (DC.) Berg and Calycorectes sellowianus O. Berg (Myrtaceae) were composed of 34 and 37 compounds, respectively. The main constituents of M. tenella oil were beta-caryophyllene (25.1%), and spathulenol (9.7%), while for C. sellowianus were guaiol (13.1%) and beta-caryophyllene (8.6%). The anti-inflammatory effect of both essential oils was investigated in vitro and in vivo. Both oils reduced significantly (p < 0.005) the treated neutrophils chemotaxis with 93% and 91% inhibition for M. tenella and C. sellowianus, respectively. However, in the systemic treatment with the essential oils (50 mg/kg p.o.) only the M. tenella oil was able to significantly reduce the carrageenan-induced paw edema with a similar effect to that observed for indomethacin (10 mg/kg), the positive control.


Asunto(s)
Antiinflamatorios no Esteroideos/farmacología , Myrtaceae/química , Aceites Volátiles/farmacología , Animales , Antiinflamatorios no Esteroideos/aislamiento & purificación , Antiinflamatorios no Esteroideos/uso terapéutico , Quimiotaxis de Leucocito/efectos de los fármacos , Edema/tratamiento farmacológico , Cromatografía de Gases y Espectrometría de Masas , Masculino , Neutrófilos/efectos de los fármacos , Neutrófilos/fisiología , Aceites Volátiles/aislamiento & purificación , Aceites Volátiles/uso terapéutico , Hojas de la Planta/química , Ratas , Ratas Wistar
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