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1.
Clin Exp Rheumatol ; 42(1): 174-177, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38179711

RESUMEN

OBJECTIVES: To estimate digit circumference and the impact of sex and body mass index (BMI) for the calculation of the Leeds Dactylitis Index (LDI) in psoriatic arthritis (PsA) patients with bilateral dactylitis. METHODS: Digit circumference of the hands and the foot were measured with a dactylometer and were studied according to sex and BMI (divided in 4 weight categories) in healthy Portuguese subjects, using Student's t-test and One-way ANOVA, respectively. The effect size of sex and BMI were calculated using Cohen's d test and Eta squared, respectively. Multiple linear regression was used to calculate the effect of sex and BMI, as well as their interaction, to create a formula to predict digit circumference. RESULTS: Fifty-nine participants (33 women, 26 men) with a mean BMI of 24.8 were included. Men's mean digit circumferences were statistically higher than those of women (p<0.001), with a large sex effect size in most of the digits. Differences in the mean circumference between the four BMI categories were statistically significant (p<0.05) for all digits, with a large BMI effect size. Sex and BMI were independent variables to predict mean digit circumference (p<0.001). A new tool (based on regression analysis) allowing to estimate the circumference of digits for males and females of different BMIs is presented. CONCLUSIONS: Our data allows the calculation of digit circumference for males and females of different BMIs in the Portuguese population; and shows that BMI influences digital circumference supporting BMI inclusion in LDI references tables.


Asunto(s)
Artritis Psoriásica , Masculino , Humanos , Femenino , Índice de Masa Corporal , Artritis Psoriásica/diagnóstico , Mano , Análisis de Regresión , Circunferencia de la Cintura
2.
Rheumatol Int ; 42(7): 1265-1269, 2022 07.
Artículo en Inglés | MEDLINE | ID: mdl-34086073

RESUMEN

The authors present the case of a 76-year-old female patient with progressive decrease in proximal muscle strength, fatigue, dyspnea, diffuse hand edema and painful triphasic Raynaud's phenomenon. Anti-SRP and anti-SSA antibodies were detected, muscle biopsy revealed changes consistent with necrotizing myopathy and capillaroscopy had findings compatible with systemic sclerosis. High-resolution chest computed tomography revealed interstitial lung disease with a non-specific interstitial pneumonia pattern. Lung function tests demonstrated a forced vital capacity 93% and a diffusing capacity for carbon monoxide of 65% predicted. After multidisciplinary discussion, she was diagnosed with immune-mediated necrotizing myopathy/systemic sclerosis overlap syndrome with pulmonary involvement. Initially, dual immunomodulation therapy with high-dose steroids and intravenous immunoglobulin was started, but after 4 weeks, the patient had clinical and analytical deterioration. At this time, she was started on rituximab, with an excellent and sustained response at both muscle and lung, sustained after 12 months.


Asunto(s)
Enfermedades Autoinmunes , Enfermedades Musculares , Miositis , Esclerodermia Sistémica , Anciano , Autoanticuerpos , Enfermedades Autoinmunes/complicaciones , Femenino , Humanos , Enfermedades Musculares/complicaciones , Rituximab/uso terapéutico , Esclerodermia Sistémica/complicaciones , Partícula de Reconocimiento de Señal
3.
J Dairy Sci ; 105(7): 5685-5699, 2022 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-35636996

RESUMEN

More than 30 types of artisanal cheeses are known in Brazil; however, microorganisms, such as Staphylococcus spp., can contaminate raw milk cheeses through different sources, from milking to processing. Staphylococcal food poisoning results from the consumption of food in which coagulase-positive staphylococci, mostly Staphylococcus aureus, have developed and produced enterotoxins. In addition, an emerging public health concern is the increasing antimicrobial resistance of some Staphylococcus strains. Furthermore, the ability of Staphylococcus spp. in sharing antibiotic resistance-related genes with other bacteria increases this problem. In light of these observations, this review aims to discuss the presence of, enterotoxins of, and antibiotic-resistant of Staphylococcus spp. in Brazilian artisanal cheese produced with raw milk.


Asunto(s)
Queso , Animales , Antibacterianos/farmacología , Brasil , Queso/microbiología , Farmacorresistencia Bacteriana , Enterotoxinas/genética , Microbiología de Alimentos , Humanos , Leche/química , Staphylococcus , Estudiantes
4.
Ann Rheum Dis ; 79(4): 490-498, 2020 04.
Artículo en Inglés | MEDLINE | ID: mdl-32193187

RESUMEN

OBJECTIVES: To assess the efficacy of golimumab in combination with methotrexate (MTX) versus MTX monotherapy in psoriatic arthritis (PsA) dactylitis. METHODS: Multicentre, investigator-initiated, randomised, double-blind, placebo-controlled, parallel-design phase 3b trial in 11 Portuguese rheumatology centres. Patients with PsA along with active dactylitis and naive to MTX and biologic disease-modifying antirheumatic drugs (bDMARDs) were randomly assigned to golimumab or placebo, both in combination with MTX. The primary endpoint was Dactylitis Severity Score (DSS) change from baseline to week 24. Key secondary endpoints included DSS and Leeds Dactylitis Index (LDI) response, and changes from baseline in the LDI and MRI dactylitis score. Analysis was by intention-to-treat for the primary endpoint. RESULTS: Twenty-one patients received golimumab plus MTX and 23 MTX monotherapy for 24 weeks. One patient from each arm discontinued. Patient inclusion was halted at 50% planned recruitment due to a favourable interim analysis. Median baseline DSS was 6 in both arms. By week 24, patients treated with golimumab plus MTX exhibited significantly greater improvements in DSS relative to MTX monotherapy (median change of 5 vs 2 points, respectively; p=0.026). In the golimumab plus MTX arm, significantly higher proportions of patients achieved at least 50% or 70% improvement in DSS and 20%, 50% or 70% improvement in LDI in comparison to MTX monotherapy. CONCLUSIONS: The combination of golimumab and MTX as first-line bDMARD therapy is superior to MTX monotherapy for the treatment of PsA dactylitis. TRIAL REGISTRATION NUMBER: NCT02065713.


Asunto(s)
Anticuerpos Monoclonales/uso terapéutico , Antirreumáticos/uso terapéutico , Artritis Psoriásica/tratamiento farmacológico , Articulaciones del Pie/fisiopatología , Articulaciones de la Mano/fisiopatología , Metotrexato/uso terapéutico , Inhibidores del Factor de Necrosis Tumoral/uso terapéutico , Adulto , Anciano , Artritis Psoriásica/diagnóstico por imagen , Artritis Psoriásica/fisiopatología , Método Doble Ciego , Quimioterapia Combinada , Femenino , Articulaciones del Pie/diagnóstico por imagen , Articulaciones de la Mano/diagnóstico por imagen , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Resultado del Tratamiento
5.
Org Biomol Chem ; 17(6): 1552-1557, 2019 02 06.
Artículo en Inglés | MEDLINE | ID: mdl-30693360

RESUMEN

Continuous-flow production of chiral intermediates plays an important role in the development of building blocks for Active Pharmaceutical Ingredients (APIs), being α-amino acids and their derivatives widely applied as building blocks. In this work we developed two different strategies for the synthesis of intermediates used on the synthesis of levetiracetam/brivaracetam and ethambutol. The results obtained show that methionine methyl ester can be continuously converted to the desired ethambutol intermediate by RANEY® Nickel dessulfurization/reduction strategy whereas levetiracetam/brivaracetam intermediates could be synthesized by both RANEY® Nickel (without H2) and Pd/C-H2 approach or by photochemical desulfurization.


Asunto(s)
Anticonvulsivantes/síntesis química , Anticonvulsivantes/farmacología , Antituberculosos/síntesis química , Antituberculosos/farmacología , Anticonvulsivantes/química , Antituberculosos/química , Técnicas de Química Sintética , Etambutol/síntesis química , Etambutol/química , Etambutol/farmacología , Levetiracetam/síntesis química , Levetiracetam/química , Levetiracetam/farmacología , Pirrolidinonas/síntesis química , Pirrolidinonas/química , Pirrolidinonas/farmacología , Estereoisomerismo , Azufre/química
6.
J Food Prot ; 87(6): 100285, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38697483

RESUMEN

Staphylococcus aureus is one of the primary pathogenic agents found in cheeses produced with raw milk. Some strains of S. aureus are enterotoxigenic, possessing the ability to produce toxins responsible for staphylococcal food poisoning when present in contaminated foods. This study aimed to genotypically characterize, assess the antimicrobial resistance profile, and examine the enterotoxigenic potential of strains of S. aureus isolated from artisanal colonial cheese. Additionally, a bacterial diversity assessment in the cheeses was conducted by sequencing the 16S rRNA gene. The metataxomic profile revealed the presence of 68 distinct species in the cheese samples. Fifty-seven isolates of S. aureus were identified, with highlighted resistance to penicillin in 33% of the isolates, followed by clindamycin (28%), erythromycin (26%), and tetracycline (23%). The evaluated strains also exhibited inducible resistance to clindamycin, with nine isolates considered multidrug-resistant (MDR). The agr type I was the most prevalent (62%) among the isolates, followed by agr type II (24%). Additionally, ten spa types were identified. Although no enterotoxins and their associated genes were detected in the samples and isolates, respectively, the Panton-Valentine leukocidin gene (lukS-lukF) was found in 39% of the isolates. The presence of MDR pathogens in the artisanal raw milk cheese production chain underscores the need for quality management to prevent the contamination and dissemination of S. aureus strains.


Asunto(s)
Antibacterianos , Queso , Leche , Staphylococcus aureus , Queso/microbiología , Brasil , Leche/microbiología , Animales , Staphylococcus aureus/efectos de los fármacos , Antibacterianos/farmacología , Pruebas de Sensibilidad Microbiana , Virulencia , Microbiología de Alimentos , Humanos , Farmacorresistencia Bacteriana , Contaminación de Alimentos/análisis , Enterotoxinas/genética
7.
J Med Genet ; 49(2): 119-25, 2012 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-22147889

RESUMEN

BACKGROUND: Split-hand/foot malformation (SHFM)-also known as ectrodactyly-is a congenital disorder characterised by severe malformations of the distal limbs affecting the central rays of hands and/or feet. A distinct entity termed SHFLD presents with SHFM and long bone deficiency. Mouse models suggest that a defect of the central apical ectodermal ridge leads to the phenotype. Although six different loci/mutations (SHFM1-6) have been associated with SHFM, the underlying cause in a large number of cases is still unresolved. METHODS: High resolution array comparative genomic hybridisation (CGH) was performed in patients with SHFLD to detect copy number changes. Candidate genes were further evaluated for expression and function during limb development by whole mount in situ hybridisation and morpholino knock-down experiments. RESULTS: Array CGH showed microduplications on chromosome 17p13.3, a locus previously associated with SHFLD. Detailed analysis of 17 families revealed that this copy number variation serves as a susceptibility factor for a highly variable phenotype with reduced penetrance, particularly in females. Compared to other known causes for SHFLD 17p duplications appear to be the most frequent cause of SHFLD. A ~11.8 kb minimal critical region was identified encompassing a single gene, BHLHA9, a putative basic loop helix transcription factor. Whole mount in situ hybridisation showed expression restricted to the limb bud mesenchyme underlying the apical ectodermal ridge in mouse and zebrafish embryos. Knock down of bhlha9 in zebrafish resulted in shortening of the pectoral fins. CONCLUSIONS: Genomic duplications encompassing BHLHA9 are associated with SHFLD and non-Mendelian inheritance characterised by a high degree of non-penetrance with sex bias. Knock-down of bhlha9 in zebrafish causes severe reduction defects of the pectoral fin, indicating a role for this gene in limb development.


Asunto(s)
Factores de Transcripción con Motivo Hélice-Asa-Hélice Básico/genética , Ectromelia/genética , Duplicación de Gen , Deformidades Congénitas de la Mano/genética , Patrón de Herencia , Tibia/anomalías , Animales , Femenino , Dedos/anomalías , Técnicas de Silenciamiento del Gen , Estudios de Asociación Genética , Genotipo , Humanos , Deformidades Congénitas de las Extremidades/genética , Masculino , Linaje , Fenotipo , Pez Cebra/embriología , Pez Cebra/genética
8.
ARP Rheumatol ; 1(3): 260-261, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36242593

RESUMEN

Sjogren's syndrome (SS) is an autoimmune pathology which mainly affects salivary and lacrimal glands. Cerebellar degeneration association with SS is very rare, with only a few cases described. The treatment of SS with central nervous system involvement is not consensual. We present a 48-year-old woman with dysarthria, diplopia and ataxia associated with xerostomia. The brain magnetic imaging revealed bilateral cerebellar atrophy. She had antibody positivity for anti-SSA and anti-SSB and minor salivary glands biopsy revealed lymphocytic infiltration. Methylprednisolone, cyclophosphamide and intravenous immunoglobulin yielded no results. Rituximab was initiated with improvement in dysarthria and coordination.


Asunto(s)
Enfermedades Neurodegenerativas , Síndrome de Sjögren , Ciclofosfamida/uso terapéutico , Disartria/complicaciones , Femenino , Humanos , Inmunoglobulinas Intravenosas , Metilprednisolona , Persona de Mediana Edad , Enfermedades Neurodegenerativas/complicaciones , Rituximab/uso terapéutico , Síndrome de Sjögren/complicaciones
9.
Pest Manag Sci ; 78(6): 2492-2501, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35334151

RESUMEN

BACKGROUND: Knowledge of the spatio-temporal distribution of pests is important for the development of accurate management approaches. The boll weevil, Anthonomus grandis grandis Boh., is a deleterious cotton, Gossypium hirsutum L., pest in the western hemisphere. The spread of boll weevils across cotton fields remains poorly understood. We assessed the dispersal pattern of adult weevils through cotton fields cultivated in a tropical area during dry and wet seasons using geostatistics for the number of adults and infested reproductive structures (buds, bolls and total). RESULTS: Adult weevils and infested reproductive structures increased across both seasons despite the prevailing climatic variables. In both seasons, boll weevil adults and infested reproductive structures followed an aggregated distribution. The distances over which samples maintained spatial dependence varied from 0.7 to 43.4 m in the dry season and from 6.0 to 614.4 m in the wet season. Boll weevil infestations started at field borders and the infested reproductive structures (oviposition and/or feeding punctured) were greater than the adults regardless of cotton growth stage. CONCLUSION: Sampling for boll weevils in cotton fields should start at the field borders and focus on total infested reproductive structures (buds + bolls) and as cotton plants develop, sampling should focus on the field as a whole. Distances among samples will vary from 6 to 470 m. Thus, despite the cotton phenological stage or growing season, monitoring of boll weevil should be done by sampling total infested reproductive structures with a minimum distance of 6 m among samples. © 2022 Society of Chemical Industry.


Asunto(s)
Gorgojos , Animales , Femenino , Gossypium , Oviposición , Estaciones del Año
10.
Neuroimage Clin ; 36: 103202, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36126514

RESUMEN

CONTEXT: The neural regulation of appetite and energy homeostasis significantly overlaps with the neurobiology of stress. Frequent exposure to repeated acute stressors may cause increased allostatic load and subsequent dysregulation of the cortico-limbic striatal system leading to inefficient integration of postprandial homeostatic and hedonic signals. It is therefore important to understand the neural mechanisms by which stress generates alterations in appetite that may drive weight gain. OBJECTIVE: To determine glucocorticoid effects on metabolic, neural and behavioral factors that may underlie the association between glucocorticoids, appetite and obesity risk. METHODS: A randomized double-blind cross-over design of overnight infusion of hydrocortisone or saline followed by a fasting morning perfusion magnetic resonance imaging to assess regional cerebral blood flow (CBF) was completed. Visual Analog Scale (VAS) hunger, cortisol and metabolic hormones were also measured. RESULTS: Hydrocortisone relative to saline significantly decreased whole brain voxel based CBF responses in the hypothalamus and related cortico-striatal-limbic regions. Hydrocortisone significantly increased hunger VAS pre-scan, insulin, glucose and leptin, but not other metabolic hormones versus saline CBF groups. Hydrocortisone related increases in hunger were predicted by less reduction of CBF (hydrocortisone minus saline) in the medial OFC, medial brainstem and thalamus, left primary sensory cortex and right superior and medial temporal gyrus. Hunger ratings were also positively associated with plasma insulin on hydrocortisone but not saline day. CONCLUSIONS: Increased glucocorticoids at levels akin to those experienced during psychological stress, result in increased fasting hunger and decreased regional cerebral blood flow in a distinct brain network of prefrontal, emotional, reward, motivation, sensory and homeostatic regions that underlie control of food intake.


Asunto(s)
Glucocorticoides , Hambre , Humanos , Glucocorticoides/farmacología , Hambre/fisiología , Apetito/fisiología , Circulación Cerebrovascular , Insulina/metabolismo , Hidrocortisona , Imagen por Resonancia Magnética
11.
ARP Rheumatol ; 1(1): 42-48, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35633576

RESUMEN

BACKGROUND: Axial spondyloarthritis (axSpA), particularly ankylosing spondylitis was historically considered a male's disease and has been under-recognized in women. Emerging evidence reveals sex differences in pathophysiology, disease presentation and therapeutic efficacy. OBJECTIVE: To identify differences between sexes in a Portuguese cohort of patients with axSpA regarding clinical manifestations, disease activity, functional capacity, patient related outcomes and presence of sacroiliitis on x-ray or magnetic resonance imaging. METHODS: Patients with ≥18 years fulfilling the ASAS- Assessment of Spondyloarthritis International Society classification criteria for axSpA registered in the electronic Rheumatic Diseases Portuguese Register (Reuma.pt) were included in this multicentric cross-sectional study. Sociodemographic data, clinical features and imaging were collected from the first record in Reuma.pt. These variables were compared between sexes using Mann-Whitney test and Chi-Square test. Variables with a significant association with variable sex were considered in the multiple variable analysis to adjust the sex effect on the outcome variables. Statistical analysis was performed with R version 4.0.2 and p <0.05 was considered statistically significant. RESULTS: A total of 1995 patients were included, 1114 (55.9%) men and 881 (44.1%) women. Men had an earlier disease onset (25.1 vs 28.4, p <0.001), were younger at diagnosis (26.9 vs 30.4, p<0.001) and were more frequently smokers (32.1% vs 15.7%, p <0.001). Comparing to women, men had worse Bath Ankylosing Spondylitis Metrological Index scores (4.0 vs 3.4, p<0.001), higher levels of C-Reactive Protein (10.5 vs 6.9 mg/L, p <0.001) and were more often Human Leukocyte Antigen-B27 positive (67.8% vs 54%, p <0.001). In contrast, women more frequently had inflammatory bowel disease (8.8% vs 4.9%, p =0.004), higher levels of erythrocyte sedimentation rate (25.0 vs 21.0mm/h, p=0.003) and worse patient-related outcomes- Bath Ankylosing Spondylitis Disease Activity Index (5.7 vs 4.5, p<0.001), Patient Global Assessment (60.0 vs 50.0, p <0.001) and fatigue (6.2 vs 5.0, p <0.001). DISCUSSION: In this large multicentric study from a Portuguese axSpA cohort, we confirmed sex differences in patients with axSpA. This work brings awareness to these differences, resulting in less underdiagnosis and misdiagnosis, optimizing treatment strategies, and improving outcomes in axSpA.


Asunto(s)
Espondiloartritis Axial , Espondiloartritis , Espondilitis Anquilosante , Estudios Transversales , Femenino , Humanos , Masculino , Portugal/epidemiología , Caracteres Sexuales , Espondiloartritis/diagnóstico , Espondilitis Anquilosante/diagnóstico
12.
Acta Reumatol Port ; 46(4): 365-367, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34962252

RESUMEN

Systemic lupus erythematosus (SLE) is an autoimmune disease with potential multisystemic involvement. Mesenteric panniculitis (MP) has been described as a rare feature in patients with SLE. The authors present a case of a 26 years old patient with previous diagnosis of SLE presenting with abdominal pain and distension and a peri-umbilical mass. Imagological findings were compatible with MP and ganglion biopsy revealed inflammatory pattern. Corticosteroid therapy was initiated with a resolution of pain after 6 months of treatment, with image reevaluation showing improvement of previous findings.


Asunto(s)
Lupus Eritematoso Sistémico , Dolor Abdominal/etiología , Adulto , Biopsia , Humanos , Lupus Eritematoso Sistémico/complicaciones
13.
J Am Geriatr Soc ; 69(5): 1116-1127, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33818759

RESUMEN

BACKGROUND: Frailty screening using the Clinical Frailty Scale (CFS) has been proposed to guide resource allocation in acute care settings during the pandemic. However, the association between frailty and coronavirus disease 2019 (COVID-19) prognosis remains unclear. OBJECTIVES: To investigate the association between frailty and mortality over 6 months in middle-aged and older patients hospitalized with COVID-19 and the association between acute morbidity severity and mortality across frailty strata. DESIGN: Observational cohort study. SETTING: Large academic medical center in Brazil. PARTICIPANTS: A total of 1830 patients aged ≥50 years hospitalized with COVID-19 (March-July 2020). MEASUREMENTS: We screened baseline frailty using the CFS (1-9) and classified patients as fit to managing well (1-3), vulnerable (4), mildly (5), moderately (6), or severely frail to terminally ill (7-9). We also computed a frailty index (0-1; frail >0.25), a well-known frailty measure. We used Cox proportional hazards models to estimate the association between frailty and time to death within 30 days and 6 months of admission. We also examined whether frailty identified different mortality risk levels within strata of similar age and acute morbidity as measured by the Sequential Organ Failure Assessment (SOFA) score. RESULTS: Median age was 66 years, 58% were male, and 27% were frail to some degree. Compared with fit-to-managing-well patients, the adjusted hazard ratios (95% confidence interval [CI]) for 30-day and 6-month mortality were, respectively, 1.4 (1.1-1.7) and 1.4 (1.1-1.7) for vulnerable patients; 1.5 (1.1-1.9) and 1.5 (1.1-1.8) for mild frailty; 1.8 (1.4-2.3) and 1.9 (1.5-2.4) for moderate frailty; and 2.1 (1.6-2.7) and 2.3 (1.8-2.9) for severe frailty to terminally ill. The CFS achieved outstanding accuracy to identify frailty compared with the Frailty Index (area under the curve = 0.94; 95% CI = 0.93-0.95) and predicted different mortality risks within age and acute morbidity groups. CONCLUSIONS: Our results encourage the use of frailty, alongside measures of acute morbidity, to guide clinicians in prognostication and resource allocation in hospitalized patients with COVID-19.


Asunto(s)
COVID-19 , Anciano Frágil/estadística & datos numéricos , Evaluación Geriátrica , Hospitalización , Pronóstico , Centros Médicos Académicos , Anciano , Brasil , COVID-19/mortalidad , COVID-19/terapia , Estudios de Cohortes , Femenino , Humanos , Masculino , Persona de Mediana Edad , Puntuaciones en la Disfunción de Órganos , Factores de Tiempo
14.
Clin J Am Soc Nephrol ; 15(5): 732-742, 2020 05 07.
Artículo en Inglés | MEDLINE | ID: mdl-32284323

RESUMEN

Metabolic disorders are highly prevalent in kidney transplant candidates and recipients and can adversely affect post-transplant graft outcomes. Management of diabetes, hyperparathyroidism, and obesity presents distinct opportunities to optimize patients both before and after transplant as well as the ability to track objective data over time to assess a patient's ability to partner effectively with the health care team and adhere to complex treatment regimens. Optimization of these particular disorders can most dramatically decrease the risk of surgical and cardiovascular complications post-transplant. Approximately 60% of nondiabetic patients experience hyperglycemia in the immediate post-transplant phase. Multiple risk factors have been identified related to development of new onset diabetes after transplant, and it is estimated that upward of 7%-30% of patients will develop new onset diabetes within the first year post-transplant. There are a number of medications studied in the kidney transplant population for diabetes management, and recent data and the risks and benefits of each regimen should be optimized. Secondary hyperparathyroidism occurs in most patients with CKD and can persist after kidney transplant in up to 66% of patients, despite an initial decrease in parathyroid hormone levels. Parathyroidectomy and medical management are the options for treatment of secondary hyperparathyroidism, but there is no randomized, controlled trial providing clear recommendations for optimal management, and patient-specific factors should be considered. Obesity is the most common metabolic disorder affecting the transplant population in both the pre- and post-transplant phases of care. Not only does obesity have associations and interactions with comorbid illnesses, such as diabetes, dyslipidemia, and cardiovascular disease, all of which increase morbidity and mortality post-transplant, but it also is intimately inter-related with access to transplantation for patients with kidney failure. We review these metabolic disorders and their management, including data in patients with kidney transplants.


Asunto(s)
Trasplante de Riñón , Enfermedades Metabólicas/terapia , Insuficiencia Renal Crónica/cirugía , Humanos , Trasplante de Riñón/efectos adversos , Trasplante de Riñón/mortalidad , Enfermedades Metabólicas/diagnóstico , Enfermedades Metabólicas/mortalidad , Enfermedades Metabólicas/fisiopatología , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/mortalidad , Insuficiencia Renal Crónica/fisiopatología , Medición de Riesgo , Factores de Riesgo , Resultado del Tratamiento
15.
Acta Reumatol Port ; 45(1): 26-33, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32572012

RESUMEN

OBJECTIVE: The Assessments of SpondyloArthritis international Society Health Index (ASAS HI), estimates the impact of Spondyloarthritis (SpA) on global functioning and health. This article assesses the construct validity, reliability and responsiveness of the Portuguese version of the ASAS HI. PATIENTS AND METHODS: Patients fulfilling ASAS classification criteria for axial (axSpA) or peripheral SpA (pSpA) were included. Construct validity was assessed through Spearman's correlation analysis with other health outcomes. Discriminant validity was tested comparing the ASAS HI across disease activity and functional states using the Kruskal-Wallis test. Internal consistency was assessed by Cronbach's α, and test-retest reliability by intraclass correlation coefficients (ICC). Responsiveness was evaluated by the standardized response mean (SRM) in patients with active disease who required therapy escalation. RESULTS: Among the 91 patients included, 67% were male, mean (SD) age 47.2 (12.9) years, 63 patients with axSpA and 28 patients with pSpA. The hypothesis defined a priori to test construct validity were confirmed. The ASAS HI showed ability to discriminate between patients with different disease activity and functional states (p<0.001). Internal consistency (Cronbach's α: 0.88) and test-retest reliability [ICC=0.76 (95%CI 0.09-0.91)] were good. Responsiveness was moderate (SRM=-0.53). The smallest detectable change was 3.0. CONCLUSIONS: The Portuguese version of the ASAS HI is a comprehensible questionnaire that is valid, reliable and responsive. It can be used to assess the impact of SpA and its treatment on functioning and health, in clinical practice and for research purposes.


Asunto(s)
Estado de Salud , Medición de Resultados Informados por el Paciente , Calidad de Vida , Espondiloartritis , Absentismo , Análisis de Varianza , Antiinflamatorios no Esteroideos/uso terapéutico , Antirreumáticos/uso terapéutico , Estudios Transversales , Recolección de Datos/métodos , Depresión/diagnóstico , Femenino , Humanos , Lenguaje , Masculino , Persona de Mediana Edad , Portugal , Presentismo , Psicometría , Reproducibilidad de los Resultados , Índice de Severidad de la Enfermedad , Espondiloartritis/complicaciones , Espondiloartritis/tratamiento farmacológico , Espondiloartritis/fisiopatología , Espondiloartritis/psicología , Estadísticas no Paramétricas , Traducciones , Inhibidores del Factor de Necrosis Tumoral/uso terapéutico
16.
Am J Med Genet A ; 146A(24): 3126-31, 2008 Dec 15.
Artículo en Inglés | MEDLINE | ID: mdl-19012338

RESUMEN

We describe an apparently new genetic syndrome in six members of a family living in a remote area in Northeastern Brazil. This syndrome comprises: short stature due to a marked decrease in the length of the lower limbs (predominantly mesomelic with fibular agenesis/marked hypoplasia), grossly malformed/deformed clubfeet with severe oligodactyly, upper limbs with acromial dimples and variable motion limitation of the forearms and/or hands, severe nail hypoplasia/anonychia sometimes associated with mild brachydactyly and occasionally with pre-axial polydactyly. This syndrome is apparently distinct from the syndrome of brachydactyly-ectrodactyly with fibular aplasia or hypoplasia (OMIM 113310), the syndrome of fibular aplasia or hypoplasia, femoral bowing and poly-, syn-, and oligodactyly (OMIM 228930), and from other previously described conditions exhibiting fibular agenesis/hypoplasia.


Asunto(s)
Pie Equinovaro/complicaciones , Anomalías Congénitas/patología , Peroné/anomalías , Uñas Malformadas/complicaciones , Anciano , Brasil , Niño , Pie Equinovaro/diagnóstico por imagen , Anomalías Congénitas/diagnóstico por imagen , Femenino , Peroné/diagnóstico por imagen , Geografía , Humanos , Masculino , Persona de Mediana Edad , Uñas Malformadas/diagnóstico por imagen , Linaje , Radiografía , Síndrome
17.
Open Access Rheumatol ; 10: 141-149, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30425593

RESUMEN

Gout is currently the most frequent cause of inflammatory arthritis worldwide and is responsible for poor health-related quality of life and loss of work productivity. It is caused by high levels of serum urate, leading to the deposition of monosodium urate crystals in joints and soft tissues. This condition is associated with acute flares and, if untreated or refractory, chronic and potentially destructive arthritis and tophi formation. Pegloticase is a recombinant, pegylated uricase used in the treatment of gout patients who fail conventional urate-lowering therapy. In this review, we discuss the impact of pegloticase on patient outcomes in refractory gout. We analyze different parameters, such as plasma uric acid concentration, frequency of flares, tophi reduction, pain, function, quality of life, and safety.

18.
Rev Paul Pediatr ; 36(1): 7, 2018.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-29091129

RESUMEN

OBJECTIVE: To identify the prevalence of feeding difficulties in preschoolers, its association with epidemiological factors and previous eating habits, and repercussion on nutritional status. METHODS: Cross-sectional study with a questionnaire given to the mothers of 301 children aged 2-6 years enrolled in public and private kindergartens in Natal, Northeast Brazil, conducted in 2014-2015. Feeding difficulty was assessed according to Kerzner's criteria, resulting in the profiles "highly selective intake", "active child with small appetite", "fear of feeding", and "child with psychological disorder or neglected". Association with the following independent variables was analyzed by logistic regression: breastfeeding time, age of cows' milk and complementary feeding introduction, age range, family income, type of school, mothers' profile (responsive or nonresponsive), and body mass index (BMI). RESULTS: Feeding difficulty was found in 37.2% of cases, with predominance of "highly selective intake" (25.4%). It was not associated with infancy feeding practices, family income or type of school. There were no differences between the BMI Z score means for the groups with and without feeding difficulty (1.0±1.5 SD and 1.1±1.4 SD, respectively). The five-to-six age range had more occurrences (OR 1.8; 95%CI 1.1-2.9). Children of responsive mothers were less likely to have feeding difficulties (OR 0.4; 95%CI 0.2-0.8). CONCLUSIONS: Feeding difficulties were very frequent. Nutritional status was not impacted by it, and infancy eating habits were not associated with it. Responsive mothers' profile is a protective factor against eating difficulties and reinforces the importance of behavioral factors and mother-child interaction.


OBJETIVO: Identificar a prevalência de dificuldade alimentar (DA) em pré-escolares, sua associação com fatores epidemiológicos e práticas alimentares pregressas, bem como sua repercussão sobre o estado nutricional. MÉTODOS: Estudo transversal com aplicação de questionário às mães de 301 crianças de dois a seis anos de creches públicas e privadas em Natal, Rio Grande do Norte, em 2014 e 2015. Identificou-se DA segundo critérios de Kerzner, incluindo os perfis de "ingestão altamente seletiva", "criança agitada com baixo apetite", "fobia alimentar" e "criança com distúrbio psicológico ou negligenciada". As variáveis de associação analisadas por regressão logística foram: tempo de aleitamento materno, idade de introdução de leite de vaca e da alimentação complementar, faixa etária, renda familiar, tipo de escola, perfil das mães (responsivas ou não responsivas) e índice de massa corpórea (IMC). RESULTADOS: DA foi encontrada em 37,2% dos casos analisados, com predomínio de "ingestão altamente seletiva" (25,4%). Não houve associação entre DA e práticas alimentares na fase de lactente, renda familiar e tipo de escola. Não houve diferença entre as médias de escore Z IMC para os grupos com e sem DA (1,0±1,5DP e 1,1±1,4DP, respectivamente). A faixa etária de cinco a seis anos apresentou maior ocorrência de DA (OR 1,8; IC95% 1,1-2,9) e filhos de mães com perfil responsivo tiveram menores chances de apresentar DAs (OR 0,4; IC95% 0,2-0,8). CONCLUSÕES: DA foi de alta prevalência. Não houve repercussão sobre o estado nutricional nem associação às práticas alimentares pregressas. O perfil responsivo das mães é fator protetor para as DAs e reforça a importância da natureza comportamental e da interação mãe-filho.


Asunto(s)
Conducta Alimentaria , Trastornos de Alimentación y de la Ingestión de Alimentos/epidemiología , Estado Nutricional , Niño , Preescolar , Estudios Transversales , Humanos
19.
Acta Reumatol Port ; 43(2): 80-92, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30091952

RESUMEN

The GO-DACT is an investigator-initiated, national, multicentric randomized placebo-controlled double-blinded trial, that assesses dactylitis as primary endpoint. Psoriatic arthritis patients naïve to methotrexate and biologic disease modifying anti-rheumatic drugs, with at least one active dactylitis, were assigned to golimumab in combination with methotrexate or placebo in combination with methotrexate, for 24 weeks. Both clinical (dactylitis severity score and the Leeds dactylitis index) and imaging (high resolution magnetic resonance imaging), among others, were assessed as outcomes. The main objective of GO-DACT is to provide evidence to improve the treatment algorithm and care of psoriatic arthritis patients with active dactylitis. In this manuscript we describe the GO-DACT protocol and general concepts of the methodology of this trial.


Asunto(s)
Anticuerpos Monoclonales/administración & dosificación , Artritis Psoriásica/tratamiento farmacológico , Entesopatía/tratamiento farmacológico , Articulaciones de los Dedos , Inmunosupresores/administración & dosificación , Metotrexato/administración & dosificación , Articulación del Dedo del Pie , Método Doble Ciego , Quimioterapia Combinada , Femenino , Humanos , Masculino , Resultado del Tratamiento
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