Elderly-onset rheumatoid arthritis receives less aggressive therapies than young-onset rheumatoid arthritis in an Argentinian cohort.

OBJECTIVES: When rheumatoid arthritis (RA) starts after the age of 60 it is called elderly-onset rheumatoid arthritis (EORA) and when it starts earlier, young-onset rheumatoid arthritis. (YORA). There are few Latin American studies that compared both groups. The objective of the study was to evaluate differences in the clinical characteristics, evolution and treatment among patients with RA with onset before or after 60 years of age. MATERIALS AND METHODS: Observational study of patients with RA attended consecutively in four centers in Argentina. Sociodemographic data, comorbidities, clinical manifestations at diagnosis, presence of rheumatoid factor and/or anti-CCP (cyclic citrullinated peptide) and treatments received were collected. At the last visit, swollen and tender joints, assessment of disease activity by the patient and physician, the presence of radiographic erosions, and functional status using the HAQ-DI were recorded. RESULTS: 51 patients from each group were analyzed. The EORA group had a significantly higher proportion of smokers (58.8% vs. 35.3%, p = 0.029), cardiovascular history (54.9% vs. 21.6%, p = 0.001), abrupt onset (49% vs. 29.4%, p = 0.034) or with symptoms similar to PMR (19.6% vs. 0%, p = 0.001). Lower methotrexate doses were used in the EORA group: 19 mg (15-25) vs. 21.9 mg (20-25) (p = 0.0036) and more frequently did not receive bDMARDs or tsDMARDs. DISCUSSION AND CONCLUSIONS: The benefits of intensive treatment in patients with RA have been described. In this study, the use of DMARDs in the EORA group was less intensive, suggesting that advanced age constitutes a barrier in the therapeutic choice.

La artritis reumatoide de inicio en el anciano recibe terapias menos agresivas que la artritis reumatoide de inicio en el adulto en una cohorte argentina / Elderly-onset rheumatoid arthritis receives less aggressive therapies than young-onset rheumatoid arthritis in an Argentinian cohort

Antecedentes y objetivo: Cuando la artritis reumatoide (AR) comienza después de los 60años se denomina artritis reumatoide de inicio en el anciano, y cuando se inicia antes, artritis reumatoide de inicio en el adulto. Son escasos los estudios latinoamericanos que compararon ambos grupos. El objetivo del estudio fue evaluar diferencias en las características clínicas, en la evolución y en la elección terapéutica entre los pacientes con AR de inicio antes o después de los 60años. Materiales y métodos: Estudio observacional de pacientes con AR atendidos en forma consecutiva en cuatro centros de Argentina. Se recolectaron datos sociodemográficos, comorbilidades, manifestaciones clínicas al diagnóstico, presencia de factor reumatoide y/o anti-proteínas cíclicas citrulinadas (PCC) y tratamientos recibidos. En la última visita se registraron las articulaciones tumefactas o dolorosas, la evaluación de la actividad de la enfermedad por el paciente y por el médico, la presencia de erosiones radiográficas y el estado funcional mediante el HAQ-DI. Resultados: Se analizaron 51 pacientes de cada grupo. El grupo de AR del anciano tuvo significativamente mayor proporción de fumadores (58,8% vs 35,3%, p=0,029), de antecedentes cardiovasculares (54,9% vs 21,6%, p=0,001), de inicio abrupto (49% vs 29,4%, p=0,034) o con síntomas similares a la PMR (19,6% vs 0%, p=0,001), menores dosis de metotrexato: 19mg (15-25) vs 21,9mg (20-25) (p=0,0036) y con mayor frecuencia no recibieron FAMEb o FAMEsd. Discusión y conclusiones: Se han descrito los beneficios del tratamiento intensivo en pacientes con AR. En este trabajo, el empleo de FAME en el grupo de AR de inicio en el anciano fue menos intensivo, sugiriendo que la edad avanzada constituye una barrera en la elección terapéutica.(AU)

Objectives: When rheumatoid arthritis (RA) starts after the age of 60 it is called elderly-onset rheumatoid arthritis (EORA) and when it starts earlier, young-onset rheumatoid arthritis (YORA). There are few Latin American studies that compared both groups. The objective of the study was to evaluate differences in the clinical characteristics, evolution and treatment among patients with RA with onset before or after 60years of age. Materials and methods: Observational study of patients with RA attended consecutively in four centers in Argentina. Sociodemographic data, comorbidities, clinical manifestations at diagnosis, presence of rheumatoid factor and/or anti-CCP (cyclic citrullinated peptide) and treatments received were collected. At the last visit, swollen and tender joints, assessment of disease activity by the patient and physician, the presence of radiographic erosions, and functional status using the HAQ-DI were recorded. Results: Fifty-one patients from each group were analyzed. The EORA group had a significantly higher proportion of smokers (58.8% vs. 35.3%, P=.029), cardiovascular history (54.9% vs. 21.6%, P=.001), abrupt onset (49% vs. 29.4%, P=.034) or with symptoms similar to PMR (19.6% vs. 0%, P=.001). Lower methotrexate doses were used in the EORA group: 19mg (15-25) vs. 21.9mg (20-25) (P=.0036) and more frequently did not receive bDMARDs or tsDMARDs. Discussion and conclusions: The benefits of intensive treatment in patients with RA have been described. In this study, the use of DMARDs in the EORA group was less intensive, suggesting that advanced age constitutes a barrier in the therapeutic choice.(AU)

Diferencias neuropsicológicas en la enfermedad de Alzheimer en función de la edad de presentación / Neuropsychological differences in Alzheimer's disease depending on the age of onset

Introducción La enfermedad de Alzheimer (EA) es la forma más común de demencia entre las personas mayores. La enfermedad de Alzheimer de inicio precoz (EAIP) se ha definido como una demencia debido a EA que se presenta antes de la edad arbitrariamente establecida de 65 años. De los pacientes con EA precoz, 50% debutan con síntomas atípicos y muestran alteraciones neuropsicológicas diferentes de aquellos pacientes que debutan más tarde. Estas atipias conllevan un retraso en el diagnóstico y en el inicio del tratamiento. Métodos Seleccionamos retrospectivamente 359 pacientes con diagnóstico de probable demencia por EA. Subdividimos a los pacientes en tres grupos atendiendo a la edad de aparición de la enfermedad: EAIP, menores de 65 años; EA de inicio tardío (EAIT; entre 65 y 80); y EA de inicio muy tardío (EAIMT; definido como edad de inicio mayor de 80 años) y comparamos sus resultados neuropsicológicos. Resultados Los pacientes de EA con una edad de inicio más joven puntuaron peor en atención, función ejecutiva y habilidades visuoespaciales, mientras que los pacientes de mayor edad puntuaron peor en tareas de memoria y lenguaje. Los pacientes de inicio muy tardío se diferenciaron de los de inicio tardío en un mayor deterioro de la fluidez semántica y la denominación. Conclusión Aunque la edad de 65 años podría corresponder a un punto de separación arbitrario entre la forma precoz y la forma de inicio más tardío de la EA, nuestro estudio demuestra que existen diferencias significativas entre estos grupos desde un punto de vista neuropsicológico. Sin embargo, estas diferencias parecen seguir una tendencia lineal con la edad, en lugar de representar cuadros clínicos fundamentalmente distintos. (AU)

Introduction Early-onset Alzheimer's disease (EOAD) has been defined as a dementia due to AD presenting before the arbitrarily established age of 65 (as opposed to late-onset Alzheimer's disease or LOAD). There is still little research about other age sub-groups, the use of so-called senile dementia has been banished, usually including it within the late-onset Alzheimer's dementia. To the extent of our knowledge, there are no studies comparing the neuropsychological features of very-late-onset patients with early and late-onset ones. Methods We retrospectively selected 359 patients with a diagnosis of probable AD dementia. We subdivided patients into three groups attending to the age of onset of the disease: early-onset AD (EOAD; younger than 65 years old), late-onset AD (LOAD; between 65 and 80) and very-late-onset AD (VLOAD; defined here as onset age older than 80), and then we compared their neuropsychological results. Results AD patients with a younger age at onset scored worse on attention, executive function and visuospatial skills, while older-onset patients scored worse in memory tasks and language. Patients with a very-late-onset differed from the late-onset ones in a greater impairment of semantic fluency and naming. Conclusion Although the point of separation between EOAD and later-onset forms of EA at the age of 65 is an arbitrary one, our study shows that there are significant differences between these groups from a neuropsychological point of view. However, these differences do seem to follow a linear trend with age, rather than representing fundamentally distinct clinical pictures. (AU)

Diferencias neuropsicológicas en la enfermedad de Alzheimer en función de la edad de presentación / Neuropsychological differences in Alzheimer's disease depending on the age of onset

Introducción La enfermedad de Alzheimer (EA) es la forma más común de demencia entre las personas mayores. La enfermedad de Alzheimer de inicio precoz (EAIP) se ha definido como una demencia debido a EA que se presenta antes de la edad arbitrariamente establecida de 65 años. De los pacientes con EA precoz, 50% debutan con síntomas atípicos y muestran alteraciones neuropsicológicas diferentes de aquellos pacientes que debutan más tarde. Estas atipias conllevan un retraso en el diagnóstico y en el inicio del tratamiento. Métodos Seleccionamos retrospectivamente 359 pacientes con diagnóstico de probable demencia por EA. Subdividimos a los pacientes en tres grupos atendiendo a la edad de aparición de la enfermedad: EAIP, menores de 65 años; EA de inicio tardío (EAIT; entre 65 y 80); y EA de inicio muy tardío (EAIMT; definido como edad de inicio mayor de 80 años) y comparamos sus resultados neuropsicológicos. Resultados Los pacientes de EA con una edad de inicio más joven puntuaron peor en atención, función ejecutiva y habilidades visuoespaciales, mientras que los pacientes de mayor edad puntuaron peor en tareas de memoria y lenguaje. Los pacientes de inicio muy tardío se diferenciaron de los de inicio tardío en un mayor deterioro de la fluidez semántica y la denominación. Conclusión Aunque la edad de 65 años podría corresponder a un punto de separación arbitrario entre la forma precoz y la forma de inicio más tardío de la EA, nuestro estudio demuestra que existen diferencias significativas entre estos grupos desde un punto de vista neuropsicológico. Sin embargo, estas diferencias parecen seguir una tendencia lineal con la edad, en lugar de representar cuadros clínicos fundamentalmente distintos. (AU)

Introduction Early-onset Alzheimer's disease (EOAD) has been defined as a dementia due to AD presenting before the arbitrarily established age of 65 (as opposed to late-onset Alzheimer's disease or LOAD). There is still little research about other age sub-groups, the use of so-called senile dementia has been banished, usually including it within the late-onset Alzheimer's dementia. To the extent of our knowledge, there are no studies comparing the neuropsychological features of very-late-onset patients with early and late-onset ones. Methods We retrospectively selected 359 patients with a diagnosis of probable AD dementia. We subdivided patients into three groups attending to the age of onset of the disease: early-onset AD (EOAD; younger than 65 years old), late-onset AD (LOAD; between 65 and 80) and very-late-onset AD (VLOAD; defined here as onset age older than 80), and then we compared their neuropsychological results. Results AD patients with a younger age at onset scored worse on attention, executive function and visuospatial skills, while older-onset patients scored worse in memory tasks and language. Patients with a very-late-onset differed from the late-onset ones in a greater impairment of semantic fluency and naming. Conclusion Although the point of separation between EOAD and later-onset forms of EA at the age of 65 is an arbitrary one, our study shows that there are significant differences between these groups from a neuropsychological point of view. However, these differences do seem to follow a linear trend with age, rather than representing fundamentally distinct clinical pictures. (AU)

[Neuropsychological differences in Alzheimer's disease depending on the age of onset]. / Diferencias neuropsicológicas en la enfermedad de Alzheimer en función de la edad de presentación.

INTRODUCTION: Early-onset Alzheimer's disease (EOAD) has been defined as a dementia due to AD presenting before the arbitrarily established age of 65 (as opposed to late-onset Alzheimer's disease or LOAD). There is still little research about other age sub-groups, the use of so-called senile dementia has been banished, usually including it within the late-onset Alzheimer's dementia. To the extent of our knowledge, there are no studies comparing the neuropsychological features of very-late-onset patients with early and late-onset ones. METHODS: We retrospectively selected 359 patients with a diagnosis of probable AD dementia. We subdivided patients into three groups attending to the age of onset of the disease: early-onset AD (EOAD; younger than 65 years old), late-onset AD (LOAD; between 65 and 80) and very-late-onset AD (VLOAD; defined here as onset age older than 80), and then we compared their neuropsychological results. RESULTS: AD patients with a younger age at onset scored worse on attention, executive function and visuospatial skills, while older-onset patients scored worse in memory tasks and language. Patients with a very-late-onset differed from the late-onset ones in a greater impairment of semantic fluency and naming. CONCLUSION: Although the point of separation between EOAD and later-onset forms of EA at the age of 65 is an arbitrary one, our study shows that there are significant differences between these groups from a neuropsychological point of view. However, these differences do seem to follow a linear trend with age, rather than representing fundamentally distinct clinical pictures.

Pain and late-onset of hidradenitis suppurativa can have a negative influence on occupational status and educational level. A cross-sectional study. / [Artículo traducido] El dolor y la aparición tardía de la hidrosadenitis supurativa pueden tener una influencia negativa en el estatus ocupacional y el nivel educativo. Estudio transversal.

BACKGROUND AND OBJECTIVES: Hidradenitis suppurativa (HS) is a chronic and painful condition with negative impact on daily activity. Little information on the impact of disease-specific factors on educational level and occupational status in hidradenitis suppurativa patients has been reported. We sought to identify how disease-specific factors could influence occupational status and educational level in patients with HS. METHODS: Cross-sectional study of patients with HS seen between September 2017 and September 2018. Disease-specific variables were analyzed to find associations in patients with different educational levels and occupational status. RESULTS: Ninety-eight patients were included. Patients with non-university studies had more frequently ≥ 3 affected areas (22.5% [16/73] vs. 4.8% [1/22], p = 0.049), a higher number of painful days (8.5 [SD 8.8] vs. 4.6 [SD 4.8], p = 0.048) and a higher score on the VAS scale (6.7 [SD 2.8] vs. 5.0 [3.3], p = 0.031). Patients from the inactive group had a significantly increased number of painful days (11.2 [SD 10.4] vs. 5.7 [SD 6.2], p = 0.004). This group had a greater number of patients with a history of depression (61.3% [19/31] vs. 27.4% [17/62], p = 0.002) and a higher mean BMI (32.3 [9.1] vs. 28.4 [6.4], p = 0.016). Late disease onset was significantly associated with being «inactive¼ (26.7% [8/31] vs. 6.5% [4/62], p = 0.026). No significant differences between severity scales of HS and educational level or occupational status were found. LIMITATIONS: cross-sectional and single center study. CONCLUSIONS: Pain, ≥ 3 affected areas, history of depression, higher mean BMI, and late onset of HS, are associated with low education level and inactive occupational status.

Pain and Late-Onset of Hidradenitis Suppurativa Can Have a Negative Influence on Occupational Status and Educational Level. A Cross-Sectional Study.

BACKGROUND AND OBJECTIVES: Hidradenitis suppurativa is a chronic and painful condition with negative impact on daily activity. Little information on the impact of disease-specific factors on educational level and occupational status in hidradenitis suppurativa patients has been reported. We sought to identify how disease-specific factors could influence occupational status and educational level in patients with hidradenitis suppurativa. METHODS: Cross-sectional study of patients with hidradenitis suppurativa seen between September 2017 and September 2018. Disease-specific variables were analyzed to find associations in patients with different educational levels and occupational status. RESULTS: Ninety-eight patients were included. Patients with non-university studies had more frequently≥3 affected areas (22.5% [16/73] vs 4.8% [1/22], p=0.049), a higher number of painful days (8.5 [SD 8.8] vs 4.6 [SD 4.8], p=0.048) and a higher score on the VAS scale (6.7 [SD 2.8] vs 5.0 [3.3], p=0.031). Patients from the inactive group had a significantly increased number of painful days (11.2 [SD 10.4] vs 5.7 [SD 6.2], p=0.004). This group had a greater number of patients with a history of depression (61.3% [19/31] vs 27.4% [17/62], p=0.002) and a higher mean BMI (32.3 [9.1] vs 28.4 [6.4], p=0.016). Late disease onset was significantly associated with being "inactive" (26.7% [8/31] vs 6.5% [4/62], p=0.026). No significant differences between severity scales of hidradenitis suppurativa and educational level or occupational status were found. LIMITATIONS: cross-sectional and single center study. CONCLUSIONS: Pain, ≥3 affected areas, history of depression, higher mean BMI, and late onset of hidradenitis suppurativa, are associated with low education level and inactive occupational status.

Enfermedad de Pompe de inicio tardío: análisis de una casuística de 19 pacientes mexicanos / Late onset Pompe disease: an analysis of 19 patients from Mexico

Introducción: La enfermedad de Pompe es una miopatía metabólica rara con espectro clínico heterogéneo, especialmente la de inicio tardío, cuya sintomatología es de progresión más lenta y representa un gran reto diagnóstico. Objetivo: Describir el genotipo y las características clínicas de pacientes mexicanos con Pompe de inicio tardío (LOPD). Material y métodos. Se incluyó a 19 pacientes mexicanos con LOPD confirmada mediante actividad enzimática y estudio molecular del gen GAA. Se evaluaron datos clínicos y se revisaron las mutaciones en bases de datos genómicas. Resultados: La mediana de edad de inicio de los síntomas fue de 19 años (rango: 2-43 años), y la edad de diagnóstico, de 36 años (rango: 9-52 años). Los síntomas más frecuentes fueron debilidad axial y proximal (n = 17; 89,5%), marcha basculante (n = 17; 89,5%) e hiperlordosis (n = 7; 36,8%). A 16 pacientes (84,2%) se les realizó electromiografía; 11 (57,8%) describieron patrón miopático y sólo en cinco pacientes (26%) se incluyó la valoración de los músculos paraespinales. Las variantes patogénicas más frecuentes en nuestra casuística fueron c.-32-13T>G, c.1799G>A y c.1082C>T. Conclusiones: Parecido a lo comunicado en publicaciones internacionales, la LOPD en México es clínicamente heterogénea; los pacientes pueden tardar años en llegar al diagnóstico. La debilidad muscular axial y proximal es el dato clínico más frecuente, por lo que la electromiografía debe incluir valoración de los músculos paraespinales. A excepción de una, las mutaciones encontradas en nuestra serie de casos se encuentran previamente descritas en las bases de datos de enfermedad de Pompe.(AU)

Introduction: Pompe disease (PD) is a rare metabolic myopathy with an ample and heterogeneous clinical spectrum, particularly late onset PD (LOPD), which is characterized by appearance at older age and slower disease progression, leading to diagnostic confirmation difficulty and delay. Aim: To describe the genotype and clinical characteristics of Mexican patients with LOPD. Material and methods: Clinical information from 19 Mexican patients with LOPD confirmed with enzyme activity and GAA gene analysis was reviewed. Genetic information of our population was crossed with international genetic databases. Results: Median age between onset of symptoms and diagnosis was 19 years (range 2-43) and diagnostic confirmation 36 years (range 9-52). Most frequently referred symptoms were proximal axial weakness (n = 17; 89.5%), waddling gait (n = 17; 89.5%) and hyperlordosis (n = 7; 36.8%). Sixteen patients (84.2%) were evaluated with electromyography; a myopathic pattern was reported in 11 (57.8%), but only in 5 patients (26%) paraspinal muscle evaluation was included. The most pathogenic mutations in our group were c.-32-13T>G, c.1799G>A and c.1082C>T. Conclusions: Similar to other international publications, LOPD in Mexico is clinically heterogeneous; patients may delay years before diagnosis is established. Axial and proximal weakness is the most frequent clinical feature; thus, electromyography with paraspinal muscle evaluation is essential. Except for one, the mutations found in our patients have been previously reported in PD genetic databases.(AU)

Rituximab para el tratamiento de la miastenia grave generalizada: experiencia en la práctica clínica / Rituximab for the treatment of generalised myasthenia gravis: experience in clinical practice

Introducción y objetivos: El rituximab (RTX) es un anticuerpo monoclonal anti-CD20 que se ha utilizado en casos de miastenia grave (MG) refractaria. El objetivo de este trabajo es analizar la eficacia y la seguridad del RTX en la MG en la práctica clínica real en un hospital terciario. Pacientes y métodos:Se realiza un estudio retrospectivo de pacientes con MG tratados con RTX en nuestro centro de marzo de 2014 a septiembre de 2020. Se recogen datos demográficos, serológicos, tratamiento inmunomodulador previo, respuesta clínica y efectos adversos. Resultados: Veinte pacientes con MG –el 100%, generalizada: el 70%, MG de inicio tardío (LOMG), y el 30%, MG de inicio temprano (EOMG)– han recibido RTX (edad media: 66,8 años; 70%, varones). El 90% son seropositivos –16 con anticuerpos antirreceptor de la acetilcolina positivos y dos con anticuerpos antitirosincinasa muscular específica (anti-MuSK) positivos–. Todos habían fracasado con tratamientos previos: el 100% con esteroides, el 100% con inmunoglobulinas intravenosas y/o con plasmaféresis, el 55% con otros inmunosupresores (25%, un inmunosupresor previo; 10%, dos; 15%, tres; y 5%, cuatro) y el 35% con timectomía. Tras el RTX, presentó respuesta clínica el 75% de los pacientes (12 pacientes, remisión completa con posibilidad de la retirada de los esteroides sin recurrencia; y tres parcial, con posibilidad de la reducción de la dosis de esteroides) y fracaso terapéutico el 25%; en todos estos casos se retiró el RTX. El 100% de los pacientes anti-MuSK+ y el 92,8% de los de LOMG presentaron respuesta al RTX, mientras que el 66% de los pacientes con EOMG fracasaron. Sólo tres pacientes presentaron efectos adversos, todos leves, que no requirieron la retirada del RTX. Conclusión: En nuestra experiencia, el rituximab es un tratamiento seguro y eficaz en la MG generalizada agresiva con anticuerpos anti-MuSK o de inicio tardío (LOMG).(AU)

Introduction and aims: Rituximab (RTX) is an anti-CD20 monoclonal antibody that has been used in cases of refractory myasthenia gravis (MG). The aim of this work is to analyse the efficacy and safety of RTX in MG in real clinical practice in a tertiary hospital. Patients and methods: A retrospective study was conducted with patients with MG treated with RTX in our centre from March 2014 to September 2020. Demographic and serological data, together with information about previous immunomodulatory treatment, clinical response and adverse effects are collected. Results: Twenty patients with MG – 100% generalised: 70% late-onset MG (LOMG) and 30% early-onset MG (EOMG) – were given RTX (mean age: 66.8 years; 70% male). A total of 90% are seropositive, 16 of them with positive anti-acetylcholine receptor antibodies and two with positive muscle-specific tyrosine kinase (anti-MuSK) antibodies. All had failed previous treatments: 100% with steroids, 100% with intravenous immunoglobulins and/or plasmapheresis, 55% with other immunosuppressants (25% with one previous immunosuppressant, 10% with two, 15% with three and 5% with four) and 35% with thymectomy. After RTX, 75% of patients showed a clinical response (12 patients with complete remission and the possibility of steroid withdrawal without recurrence; and three patients with partial remission and the possible reduction of steroid dosage) and 25% therapeutic failure; in all these cases RTX was withdrawn. All the anti-MuSK+ patients (100%) and 92.8% of the LOMG patients responded to RTX, while 66% of EOMG patients failed. Only three patients reported adverse effects, all of which were mild and did not require RTX withdrawal. Conclusion: In our experience, rituximab is a safe and effective treatment in aggressive generalised MG with anti-MuSK or late-onset MG (LOMG).(AU)

Placental Findings in Preterm and Term Preeclampsia: An Integrative Review of the Literature / Achados placentários na pré-eclâmpsia pré-termo e a termo: Uma revisão integrativa da literatura

Abstract Introduction Preeclampsia (PE) is a pregnancy complication associated with increased maternal and perinatal morbidity and mortality. The disease presents with recent onset hypertension (after 20 weeks of gestation) and proteinuria, and can progress to multiple organ dysfunction, with worse outcomes among early onset preeclampsia (EOP) cases (<34 weeks). The placenta is considered the root cause of PE; it represents the interface between the mother and the fetus, and acts as a macromembrane between the two circulations, due to its villous and vascular structures. Therefore, in pathological conditions, macroscopic and microscopic evaluation can provide clinically useful information that can confirm diagnosis and enlighten about outcomes and future therapeutic benefit. Objective To perform an integrative review of the literature on pathological placental findings associated to preeclampsia (comparing EOP and late onset preeclampsia [LOP]) and its impacts on clinical manifestations. Results: Cases of EOP presented worse maternal and perinatal outcomes, and pathophysiological and anatomopathological findings were different between EOP and LOP placentas, with less placental perfusion, greater placental pathological changes with less villous volume (villous hypoplasia), greater amount of trophoblastic debris, syncytial nodules, microcalcification, villous infarcts, decidual arteriolopathy in EOP placentas when compared with LOP placentas. Clinically, the use of low doses of aspirin has been shown to be effective in preventing PE, as well asmagnesium sulfate in preventing seizures in cases of severe features. Conclusion The anatomopathological characteristics between EOP and LOP are significantly different, with large morphological changes in cases of EOP, such as

Resumo Introdução A pré-eclâmpsia (PE) é uma complicação da gravidez associada ao aumento da morbidade e mortalidade materna e perinatal. A doença se apresenta com hipertensão de início recente (após 20 semanas de gestação) e proteinúria, que pode progredir para disfunção de múltiplos órgãos, com resultados piores entre os casos de início precoce (<34 semanas). A placenta é considerada a principal causa da PE, representando a interface entre a mãe e o feto, e atuando como uma macromembrana entre as duas circulações, devido às suas estruturas vilosas e vasculares, demodo que, em condições patológicas, avaliações macroscópicas e microscópicas podem fornecer informações clinicamente úteis, que podem fornecer diagnóstico, prognóstico e benefício terapêutico. Objetivo Realizar uma revisão integrativa da literatura para compreender e descrever os achados placentários patológicos associados à pré-eclâmpsia e seus impactos nas manifestações clínicas. Resultados Os casos de início precoce apresentaram piores desfechos maternos e perinatais, e os achados fisiopatológicos e anatomopatológicos foram diferentes entre as placentas de início precoce e início tardio, commenor perfusão placentária, maiores alterações patológicas placentárias commenor volume viloso (hipoplasia vilosa), maior quantidade de debris trofoblásticos, nódulos sinciciais, microcalcificação, infartos vilosos, arteriolopatia decidual em placentas de início precoce quando comparadas com placentas de início tardio. Clinicamente, o uso de baixas doses de aspirina tem se mostrado significativo na prevenção da PE, assim como o sulfato de magnésio na prevenção de convulsões na doença com manifestações de gravidade. Conclusão As características anatomopatológicas entre a pré-eclâmpsia precoce e tardia são significativamente diferentes, com grandes alterações morfológicas nos casos de início precoce, como hipóxia, infartos vilosos e hipoplasia, entre outros, na tentativa de estabilizar o fluxo sanguíneo para o feto. Portanto, um entendimento comum do exame macroscópico básico e dos padrões histológicos da lesão é importante para maximizar o benefício diagnóstico, prognóstico e terapêutico do exame da placenta e, consequentemente, reduzir os riscos para a mãe e o feto.

Tratamiento con testosterona en el hipogonadismo de inicio tardío / Late-Onset Hypogonadism Treatment with Testosterone

RESUMEN Introducción: El uso de la terapia de reemplazo con testosterona en hombres mayores se ha incrementado en los últimos años, lo que ha generado múltiples controversias aún no resueltas acerca de sus beneficios y riesgos potenciales, sobre todo los relacionados con el desarrollo o agravamiento de la enfermedad prostática o cardiovascular. Métodos: Se realizó una revisión bibliográfica con el objetivo de ofrecer un estado de la cuestión que ayude a los médicos a tomar decisiones al considerar el tratamiento con testosterona en pacientes con hipogonadismo de inicio tardío. La búsqueda de información se realizó en las bases de datos Google Académico, Medline y Pubmed. Conclusiones: El tratamiento con testosterona en el hipogonadismo de inicio tardío es seguro, racional y basado en evidencia, pero no se recomienda ofrecerlo a todos los hombres mayores con niveles bajos de testosterona sérica. Se aconseja en aquellos con síntomas manifiestos de deficiencia androgénica, sin cáncer de próstata activo, de mama o hígado, hematocrito elevado, hiperplasia prostática benigna con síntomas obstructivos graves, nódulo o induración prostática no evaluada, antígeno prostático específico > 4 ng/mL (o > 3 ng/mL en pacientes con alto riesgo), apnea obstructiva del sueño severa no tratada, deseos de fertilidad a corto plazo, insuficiencia cardiaca no controlada, infarto agudo de miocardio o accidente cerebrovascular en los últimos SEIS meses o trombofilia. Se recomienda realizar monitoreo trimestral durante el primer año y luego según cada caso, que incluya evaluación de la respuesta clínica, de condiciones que pueden agravarse con el tratamiento y de parámetros de laboratorio(AU)

ABSTRACT Introduction: The use of testosterone replacement therapy in older men has increased in recent years, which has generated multiple controversies not yet resolved about its benefits and potential risks, especially those related to the development or worsening of the prostate or cardiovascular disease. Methods: A literature review was conducted with the aim of offering a state of the art that helps clinicians make decisions when considering testosterone treatment in patients with late-onset hypogonadism. The information search was carried out with the Google Scholar, Medline and Pubmed search engines. Conclusions: Testosterone treatment in late-onset hypogonadism is safe, rational, and evidence-based, but it is not recommended to offer it to all older men with low serum testosterone levels. It is advised in those with overt symptoms of androgen deficiency, without active prostate, breast or liver cancer, elevated hematocrit, benign prostatic hyperplasia with severe obstructive symptoms, untested prostate nodule or induration, prostate specific antigen > 4 ng / mL (or > 3 ng / mL in high-risk patients), severe untreated obstructive sleep apnea, short-term fertility wishes, uncontrolled heart failure, acute myocardial infarction or stroke in the last SIX months, or thrombophilia. It is recommended to carry out quarterly monitoring during the first year and then according to each case, which includes evaluation of the clinical response, of conditions that can be aggravated by treatment, and of laboratory parameters(AU)

[The cognitive-behavioural impact of hypertension]. / El impacto cognitivo-conductual de la hipertensión.

Arterial hypertension is considered the main modifiable vascular risk factor that causes silent damage to brain vessels. This vascular brain injury could be the common nucleus that justifies the cognitive (cognitive impairment, dementia and Alzheimer's disease) and behavioural symptoms (late-life depression) of target organ damage mediated-hypertension. Incomplete knowledge about the complex pathophysiology that links hypertension with cognitive-behavioural changes is overlooking brain involvement and underestimating cardio and cerebrovascular risk. The confluence of cognitive impairment, depression and arterial hypertension in elderly adults, warns of the need for a comprehensive evaluation to plan treatment, improve prognosis and contribute to reducing the risk of dementia and its incidence.

Doença de Still do adulto: um desafio diagnóstico / Adult-onset Still's disease: a diagnostic challenge

A doença de Still do adulto é uma rara condição inflamatória, cujo diagnóstico é um desafio, por se tratar de diagnóstico de exclusão, após vasta investigação. Manifesta-se com febre alta diária, amigdalite não supurativa, artrite, rash evanescente, leucocitose e hiperferritinemia. O presente caso demonstra a doença de Still do adulto e sua vasta investigação, motivando a realização de revisão bibliográfica sobre inovações na fisiopatologia, no diagnóstico e no tratamento.

Adult onset Still's disease is a rare inflammatory condition, the diagnosis of which is a challenge, because it is a diagnosis of exclusion, and demands extensive investigation. It manifests with high daily fever, nonsuppurative tonsillitis, arthritis, evanescent rash, leukocytosis, and hyperferritinemia. The present case de­monstrates adult-onset Still's disease and its extensive inves­tigation, motivating literature review on innovations of its pathophysiology, diagnosis, and treatment.

Complement factor H levels are decreased and correlated with serum C-reactive protein in late-onset Alzheimer's disease / Os níveis de fator H do complemento estão diminuídos e correlacionados com a proteína C-reativa sérica na doença de Alzheimer de início tardio

Abstract Alzheimer's disease (AD) is the most common cause of dementia. Despite numerous studies on the subject, the pathologies for AD are still unclear and there is still no ideal biomarker for diagnosis. The present study aimed to investigate clinical significance of human complement factor H (CFH) in patients with late-onset AD. Methods: The present prospective study included 187 late-onset AD patients who went to our hospital from January 2015 to December 2017. One hundred patients with mild cognitive impairment (MCI) and 80 healthy individuals who were age and gender matched to AD patients were enrolled as controls. Demographic data such as age, gender, and education duration were recorded. Blood samples were collected and serum levels of C-reactive protein (CRP), CFH, and brain-derived neurotrophic factor (BDNF) were determined by Enzyme-linked immunosorbent assay (ELISA). The mini-mental state examination (MMSE) score was measured for all patients. Results: No significant difference was found in age, gender, and education duration for all participants. The MMSE scores showed AD patients had lower MMES scores than the other two groups. All factors of CFH, CRP, and BDNF were dramatically decreased in AD patients compared with the MCI and the ealthy control. Levels of CFH were found to be positively correlated with levels of CRP; however, no significant correlation was found between CFH and BDNF, nor CFH and MMSE. Conclusion: CFH was decreased in late-onset AD patients, and serum levels of CFH was correlated with serum levels of CRP, but not MMSE and BDNF. These results may provide more clinical evidences for the role of CFH in AD patients.

Resumo A doença de Alzheimer (DA) é a causa mais comum de demência. Apesar de inúmeros estudos sobre DA, suas patologias ainda não são claras e ainda não existe um biomarcador ideal para o diagnóstico da condição. O presente estudo teve como objetivo investigar a significância clínica do fator H do complemento humano (CFH) em pacientes com DA de início tardio. Métodos: O presente estudo prospectivo incluiu um total de 187 pacientes com DA de início tardio que foram ao nosso hospital entre janeiro de 2015 e dezembro de 2017. Cem pacientes com comprometimento cognitivo leve (CCL) e 80 indivíduos saudáveis com idade e sexo pareados com pacientes com DA foram incluídos como controle. Dados demográficos como idade, sexo e duração da educação foram registrados. As amostras de sangue foram coletadas e os níveis séricos de proteína C-reativa (PCR), CFH e fator neurotrófico derivado do cérebro (BDNF) foram determinados pelo ensaio imunoabsorvente ligado à enzima (ELISA). O escore do miniexame do estado mental (MEEM) foi medido para todos os pacientes. Resultados: Não foram encontradas diferenças significativas em idade, sexo e duração da educação para todos os participantes. Pacientes com DA tinham os menores escores de MEEM em relação aos outros dois grupos. Todos os fatores de CFH, PCR e BDNF diminuíram drasticamente em pacientes com DA em comparação com o CCL e o controle saudável. Os níveis de CFH mostraram correlação positiva com os níveis de PCR; no entanto, não foi encontrada correlação significativa entre CFH e BDNF, nem CFH e MEEM. Conclusão: A CFH diminuiu nos pacientes com DA de início tardio e os níveis séricos de CFH foram correlacionados com os níveis séricos de PCR, mas não o MEEM e o BDNF. Esses resultados podem fornecer mais evidências clínicas do papel da CFH em pacientes com DA.

Disfunción Mioneural Periférica Como Único Elemento Para El Diagnostico De La Distrofia Miotónica Tipo I / Myoneural Disfunction For The Diagnosis Of Myotonic Dystrophy Type I

Resumen La distrofia miotónica tipo 1, también conocida como enfermedad de Steinert, es un trastorno mulsistémico que afecta principalmente al sistema músculo esquelético y liso, así como al ojo, corazón, sistema endócrino y sistema nervioso central. Esta patología es infrecuente y se caracteriza por miotonía generalizada y daño multiorgánico. Su expresión clínica es variable, pero en la mayoría de los casos se presenta un grado variable de debilidad muscular, arritmias cardiacas y otros trastornos de la conducción, alteraciones endócrinas, trastornos del sueño, cataratas y calvicie. Esta es una enfermedad hereditaria con tres fenotipos reconocibles: leve, clásico y congénito. Dependiendo de su presentación puede tener mal pronóstico y una progresión usualmente rápida, la misma que carece de un tratamiento efectivo. Presentación del caso: Paciente femenina de 54 años que ingresa al Servicio de Traumatología del Hospital San Vicente de Paul de Ibarra, Ecuador por presentar una fractura de fémur izquierdo resultante de una caída desde su silla de ruedas. Durante la hospitalización la paciente presenta insuficiencia respiratoria tipo II sin causa aparente por lo cual es ingresada a UCI para soporte ventilatorio. La paciente presenta dificultad para lograr el destete ventilatorio debido a la debilidad muscular distal y proximal. La electromiografía revela un patrón miopático compatible con el diagnóstico de distrofia miotónica tipo I. Se realiza traqueotomía y es dada de alta para seguimiento por el servicio de Medicina Interna. Se sugiere la realización de estudio molecular diagnóstico. Conclusiones: El estudio molecular es la opción diagnóstica indicada para determinar con certeza la presencia de la distrofia miotónica tipo I, además de permitir determinar su severidad dependiendo del número de repetidos. Sin embargo, las limitaciones de recursos en el presente caso forzaron a que se busquen evidencias para el diagnóstico a través de la electromiografía. Hasta le alta, el tratamiento sigue siendo sintomático. Debido a que su modo de herencia es autosómico dominante, por expansión de trinucléotidos, se debe buscar familiares que pueden encontrarse asintomáticos y podrían tener esta patología.

Abstract Myotonic dystrophy type 1, also known as Steinert's disease, is a mulsystemic disorder that primarily affects the skeletal and smooth muscle, as well as the eye, heart, endocrine system and central nervous system. This pathology is uncommon and is characterized by generalized myotonia and multiorgan damage. Its clinical expression is variable, but in most cases, there is a variable degree of muscle weakness, cardiac arrhythmias and other conduction disorders, endocrine disorders, sleep disorders, cataracts and baldness. This is a hereditary disease with three recognizable phenotypes: mild, classic and congenital. Depending on the presentation, it may show poor prognosis and a usually rapid progression, which lacks of effective treatment. Case presentation: 54-year-old female patient who enters the Traumatology service of San Vicente de Paul Hospital in Ibarra, Ecuador for presenting a left femur fracture resulting from a fall of her own height. During hospitalization, the patient presented with type II respiratory failure without apparent cause, so she was admitted to the ICU for ventilatory support. The patient had difficulty achieving ventilatory weaning due to distal and proximal muscle weakness. Electromyography reveals a myopathic pattern compatible with the diagnosis of myotonic dystrophy type I. A tracheotomy was performed, and she was discharged for follow-up by the Internal Medicine service. The performance of a molecular diagnostic study was suggested. Conclusions: The molecular study is the diagnostic gold standard to determine with certainty the presence of myotonic dystrophy type I, besides allowing to determine its severity depending on the number of repeated. However, resource limitations in the present case forced evidence to be sought for diagnosis through electromyography. The treatment remains symptomatic. Because of its inheritance pattern being autosomal dominant, due to the expansion of trinucleotides, family members must be evaluated because they may have the diagnosis even though asymptomatic.

Late-onset Cogan's syndrome associated with large-vessel vasculitis.

Cogan's syndrome is a rare autoimmune disease that usually affects young Caucasian adults and is classically defined as the combination of nonsyphilitic interstitial keratitis and audiovestibular symptoms resembling Meniere's disease, both of them developed in an interval of less than two years. Nevertheless, cases with atypical ophthalmologic and audiovestibular features, with systemic manifestations or affecting children and older patients have also been reported, expanding the clinical spectrum of Cogan's syndrome. Herein, we present the case of a late-onset Cogan's syndrome associated with a large-vessel vasculitis.

Artritis reumatóide de inicio tardío asociada a enfermedad pulmonar intersticial / Late onset rheumatoid arthritis associated with interstitial lung disease

RESUMEN La artritis reumatoide de inicio tardío es considerada en la población mayor de 65 arios, presentando diferencias a las manifestaciones clínicas y de laboratorio respecto a la artritis reumatoide en población joven, con mayor riesgo de presentar formas agresivas de la enfermedad y de comenzar con un compromiso sistémico. Se establece en este caso clínico la presencia de una probable relación entre la enfermedad pulmonar intersticial asociada con artritis reumatoide de inicio tardío. En la mayoría de casos la afectación pulmonar se presenta posterior al compromiso articular, aunque puede aparecer simultáneamente e incluso ser la primera manifestación. Los hallazgos patológicos de las manifestaciones pulmonares asociadas con enfermedades autoinmunes son similares a las neumonías intersticiales idiopáticas. Se describe el caso de una paciente que presenta compromiso pulmonar por neumonía intersticial idiopática y posteriormente presenta dolor articular, por lo que se documentó artritis reumatoide.

ABSTRACT Rheumatoid arthritis of late onset occurs in the population over 65 years of age, presenting differences in clinical and laboratory manifestations compared to rheumatoid arthritis in younger people, with a higher risk of presenting with aggressive forms of the disease, and with systemic compromise. The presence of a probable relationship between the interstitial lung disease associated with late-onset rheumatoid arthritis is established in this case. In most cases pulmonary involvement occurs after the joint problems, although they may appear simultaneously, and may even be the first manifestation. Pathological findings in interstitial pneumonias associated with collagen diseases are similar to idiopathic interstitial pneumonias. A case is presented of a woman who had pulmonary involvement due to idiopathic interstitial pneumonia, and subsequently presented with joint pain with rheumatoid arthritis being documented.

Recognizing Late Onset Frontotemporal Dementia with the DAPHNE scale: A case report / Reconhecendo a demência frontotemporal de início tardio com a escala daphne: um relato de caso

ABSTRACT Frontotemporal dementias are classically described as early onset dementias with personality and behavioral changes, however, late onset forms can also be found. Considering the paucity of information about late onset behavioral variant frontotemporal dementia and its challenging diagnosis, we present a case report of an 85-year-old woman with behavioral changes and slow progression to dementia who was first diagnosed as having bipolar disorder and then Alzheimer's disease. The Daphne scale provided a structured means to improve clinical diagnosis, also supported by characteristic features on MRI and SPECT, while CSF biomarkers ruled out atypical Alzheimer's disease.

RESUMO As demências frontotemporais são classicamente descritas como demências de início precoce com mudanças de personalidade e comportamento, porém as formas de início tardio também podem ser encontradas. Considerando a escassez de informações sobre a demência frontotemporal - variante comportamental de início tardio e o diagnóstico desafiador, apresentamos um relato de caso de uma mulher de 85 anos com alterações comportamentais e progressão lenta para demência que foi diagnosticada pela primeira vez com transtorno bipolar e, em seguida, doença de Alzheimer. A escala DAPHNE foi utilizada permitindo a estruturação das características clínicas, aumentando a precisão do diagnóstico clínico, apoiado por características em RM e SPECT, enquanto os biomarcadores no líquor descartaram a doença de Alzheimer.

Early- and late-onset Alzheimer disease: Are they the same entity? / Enfermedad de Alzheimer de inicio precoz y de inicio tardío: ¿son la misma entidad?

Early-onset Alzheimer disease (EOAD), which presents in patients younger than 65 years, has frequently been described as having different features from those of late-onset Alzheimer disease (LOAD). This review analyses the most recent studies comparing the clinical presentation and neuropsychological, neuropathological, genetic, and neuroimaging findings of both types in order to determine whether EOAD and LOAD are different entities or distinct forms of the same entity. We observed consistent differences between clinical findings in EOAD and in LOAD. Fundamentally, the onset of EOAD is more likely to be marked by atypical symptoms, and cognitive assessments point to poorer executive and visuospatial functioning and praxis with less marked memory impairment. Alzheimer-type features will be more dense and widespread in neuropathology studies, with structural and functional neuroimaging showing greater and more diffuse atrophy extending to neocortical areas (especially the precuneus). In conclusion, available evidence suggests that EOAD and LOAD are 2 different forms of a single entity. LOAD is likely to be influenced by ageing-related processes.

Nivel de información sobre el síndrome de declinación de la función testicular en el envejecimiento masculino / Level of information on the declining testicular function syndrome in male aging

Introducción: el síndrome de declinación de la función testicular del hombre que envejece ha cobrado relevancia reciente, pero se asume que se conoce poco. Objetivo: identificar el nivel de información, en población y proveedores de salud, sobre este síndrome. Métodos: estudio descriptivo transversal, que involucró a 452 personas de población general, 109 médicos especialistas afines al tema y 406 de atención primaria. Se emplearon cuestionarios autoadministrados, estadísticas descriptivas y prueba chi2. Resultados: de la muestra poblacional 70,30 por ciento de las mujeres y 56,0 por ciento de los hombres reconocieron que el hombre experimenta un proceso equivalente al climaterio femenino; 64,04 por ciento no conocía los síntomas y 47,12 por ciento de los hombres mayores de 40 años señalaron edad de comienzo superior a la suya. De los especialistas afines, solo 10 habían oído hablar de todos los términos que se emplean para referirse al síndrome, 77,06 por ciento habían escuchado frecuentemente andropausia y 70,65 por ciento climaterio masculino; 27,52 por ciento dio definiciones incorrectas. De atención primaria, 28,57 por ciento no reconoció ningún término, 21,18 por ciento había escuchado frecuentemente andropausia y 19,95 por ciento climaterio masculino; 51,7 por ciento no definió correctamente el síndrome. El 74,14 por ciento no mencionó síntomas, 76,85 por ciento señaló contraindicaciones excesivas al tratamiento y 85,22 por ciento valoró su conocimiento como insuficiente. El nivel de información no se relacionó con edad, sexo o tiempo de graduado (p> 0,05). Conclusiones: la población, principalmente las mujeres, reconoce el síndrome, pero no domina sus manifestaciones. En médicos, con independencia de la edad, sexo o tiempo de graduado, la información se limita mayoritariamente a términos como andropausia y climaterio masculino; el dominio conceptual, del cuadro clínico y tratamiento, es insuficiente(AU)

Introduction: declining testicular function syndrome of the aging man has gained recent relevance but it is accepted that little is known about it. Objective: to find out the level of information of the population and of the health providers on this syndrome. Methods: cross-sectional and descriptive study involving 452 people from the general population, 109 medical specialists related to this topic and 406 primary care physicians. Self-administered questionnaires, summary statistics and chi-square test were all used. Results: in the population sample, 70.30 percent of women and 56 percent of men admitted that man experiences a process similar to the female climaterium; 64.04 percent did not know the symptoms and 47.12 percent of men older than 40 years stated that this process occurred at an age above that of theirs. As to the related specialists, just 10 had heard about all the terms used to mention this syndrome, 77.06 percent had often heard the term andropause and 70.65 percent the term male climaterium, and 27.52 percent gave incorrect definitions. In the primary health care physician group, 28.57 percent did not recognize any term, 21.18 percent had frequently heard about andropause and 19.95 percent about male climaterium, and 51.7 percent did not give a correct definition of the syndrome. In the sample 74.14 percent did not mention any symptom, 76.85 percent pointed out excessive treatment contraindications and 85.22 percent assessed their knowledge as poor. The level of information was not associated to age, sex or time of graduation (p> 0.05). Conclusions: the population, mainly women, recognizes the syndrome but did not know well the symptoms. Regardless of age, sex or time of graduation, the physicians' information about the syndrome is mostly limited to terms such as andropause and male climaterium but they did not master the concept, the clinical picture or the treatment(AU)