Dermatomiositis juvenil: experiencia de 13 años en un hospital de atención terciaria. Análisis de 17 casos clínicos / Juvenile dermatomyositis: 13 years of experience in a tertiary care hospital. Analysis of 17 clinical cases / Dermatomiosite juvenil: 13 anos de experiência em hospital de nível terciário de atenção. Analise de 17 casos clinicos

La dermatomiositis juvenil (DMJ) es una miopatía inflamatoria adquirida de base inmunológica acompañada por alteraciones cutáneas características. El objetivo de este artículo es describir las características clínicas y exámenes complementarios de un grupo de 17 pacientes con diagnóstico de DMJ, su evolución y tratamiento. Material y método: se estudiaron los pacientes que se asistieron en la Policlínica de enfermedades de tejido conectivo del Centro Hospitalario Pereira Rossell (CHPR) en el período del 1 de octubre de 2003 al 1 de abril de 2017. Resultados: los rasgos clínicos más frecuentes de presentación fueron las manifestaciones cutáneas características, debilidad muscular, síntomas constitucionales, manifestaciones gastrointestinales y respiratorias. Las enzimas musculares estuvieron aumentadas en todos los casos. La resonancia nuclear magnética, electromiograma y la biopsia muscular fueron patológicos en todos los casos realizados. El tratamiento se realizó fundamentalmente en base a corticoides y fármacos inmunosupresores, siendo el metotrexate la droga de elección. En los casos graves o refractarios se asoció gamaglobulina, ciclofosfamida o ciclosporina. La duración del tratamiento tuvo una mediana de 3 años 10 meses. Se logró remisión en 47% de los pacientes. La evolución fue monofásica en 15,4%, de los casos, polifásica en 8% y crónica en 77%. No hubo fallecimientos registrados, ni enfermedad maligna asociada. Conclusiones: la DMJ es una enfermedad de baja incidencia. La mayoría de los pacientes tuvieron una evolución crónica. Esto determina la necesidad de un tratamiento inmunosupresor prolongado con los efectos adversos de la misma. Se logró la remisión en 47% de los pacientes. No se registraron fallecimientos en la serie estudiada.

Juvenile dermatomyositis (JDM) is an acquired inflammatory myopathy with an immunologic basis and characteristic cutaneous rash. The aim of this article is to describe clinical features and most important exams of a group of 17 patients with JDM their evolution and treatment. Methods: children with JDM recruited from Connective tissue Diseases Office of Pereira Rossell Hospital from 1/10/2003 through 1/4/2017 were studied. Results: the most frequent features were: characteristic cutaneous rash, muscle weakness, systemic symptoms, gastrointestinal and respiratory manifestations. The diagnostic investigations showed an increase serum muscle enzymes in all patients. The nuclear magnetic resonance, electromyogram and muscle biopsy resulted abnormal in all the investigated cases. Treatment was based on corticosteroids and immunosuppressive drugs being methotrexate the preferred drug. In severe or refractory cases cyclophosphamide, human gammaglobulin or cyclosporine were associated. Median treatment length was 3 years 10 months Remission was achieved in 47 percent. The evolution was monophasic in 15.4 percent, polyphasic in 7.7 and chronic in 77 percent. No deaths were registered neither malignant associated diseases Conclusions: JDM is an infrequent illness. Most of the patients had chronic evolution. This obliges to prolonged immunosuppression with its adverse effects. Remission was achieved in 47% of the cases. No deaths were registered in this population.

A dermatomiosite juvenil (DMJ) é uma miopatia inflamatória imunológica adquirida acompanhada de alterações cutâneas características. O objetivo deste artigo é descrever as características clínicas e os exames complementares de um grupo de 17 pacientes diagnosticados com DMJ, sua evolução e tratamento. Materiais e métodos: foram estudados os pacientes que compareceram à Policlínica das Doenças do Tecido Conjuntivo no Centro Hospitalar Pereira Rossell (CHPR) no período de 01/10/2003 a 04/01/2017. Resultados: as características clínicas mais frequentes foram manifestações cutâneas características, fraqueza muscular, sintomas constitucionais, manifestações gastrointestinais e respiratórias. As enzimas musculares estiveram aumentadas em todos os casos. A Ressonância nuclear magnética, o eletromiograma e a biópsia muscular foram patológicos em todos os casos. O tratamento foi baseado principalmente em corticosteroides e drogas imunossupressoras, e o metotrexato foi a droga de escolha. Em casos graves ou refratários, também se administrou gamaglobulina, ciclofosfamida ou ciclosporina. A duração do tratamento teve uma mediana de 3 anos e 10 meses. A remissão foi alcançada em 47% dos pacientes. A evolução foi monofásica em 15,4% dos casos, polifásica em 8% e crônica em 77%. Não houve mortes registradas, nem doença maligna associada. Conclusões: a DMJ é uma doença de baixa incidência. A maioria dos pacientes teve evolução crônica. Isso determina a necessidade de um tratamento imunossupressor prolongado com os seus conseguintes efeitos adversos. A remissão foi alcançada em 47% dos pacientes. Nenhuma morte foi registrada na série estudada.

Emotional and psychopathological disorders in laryngectomized oncological patients.

INTRODUCTION AND OBJECTIVES: It is unknown if patients who suffer from laryngeal cancer and undergo total laryngectomy experience as much emotional shock and psychological distress as patients with cancers in other locations do. The aim of the study was to identify the incidence of emotional and psychological disorders in laryngectomized patients and describe their symptomatological nuances. METHODS: A descriptive cross-sectional study of emotional and psychopathological response of 100 cancer patients undergoing total laryngectomy was performed. The patients were evaluated immediately after surgery (n=35), when initiating communicative rehabilitation (n=23) and 5 years after diagnosis (n=42), versus a control of 55 healthy subjects. Psychopathological assessment battery for anxiety, depression, intrusion, avoidance, arousal and posttraumatic stress disorder consisted of a specific interview, the Hospital Anxiety and Depression Scale (HADS) and the Impact of Event Scale-Revised (IES-R). RESULTS: Laryngectomized patients had low incidence of emotional and psychological disorders such as anxiety (6.9%), depression (5.9%) and/or posttraumatic stress disorder (28.4%), but with sufficient intensity to constitute a psychopathological diagnosis. A significant level of distress was found in 57.4% of patients, with clear diffuse traumatic nature in 52.6% of them, which was more prevalent and intense in the rehabilitation group. CONCLUSIONS: Symptoms of anxiety, depression and posttraumatic stress disorder during different stages after total laryngectomy are not of sufficient intensity and quality to be diagnosable clinical entities; they can be considered an adaptive disorder. The symptoms are less than those of patients with cancer in other locations and appear mostly in the rehabilitation stage, where preventive performances and psychological support should be focused.

Hipertensión portal en niños / Portal hypertension in children

Introducción: la hipertensión portal (HTP) es unaentidad poco frecuente en pediatría, peropotencialmente grave cuando ocurre el sangradodigestivo debido a várices esofágicas, con unamortalidad de hasta el 25%. Las enfermedades que ladeterminan evolucionan a menudo de forma silente,entorpeciendo el diagnóstico oportuno.Objetivo: conocer la presentación y causas quedeterminaron la HTP en los pacientes admitidos en unservicio de pediatría polivalente.Metodología: estudio descriptivo retrospectivoanalizando las historias clínicas de los pacientesingresados en el Departamento de Pediatría delSanatorio Americano entre enero de 2007 y diciembrede 2010, con diagnóstico de hipertensión portal,hepatopatía, hepatitis o patología digestiva.Resultados: ingresaron 3.581 niños, 173 presentaronlos diagnósticos mencionados. Se confirmó HTP en 6pacientes. Las patologías que provocaron la HTP.

[Validation of the Questionnaire of Emotional Maladjustment and Adaptive Resources in Infertility (DERA)]. / Validación del Cuestionario de Desajuste Emocional y Recursos Adaptativos en Infertilidad (DERA).

Validation of the Questionnaire of Emotional Maladjustment and Adaptive Resources in Infertility (DERA). Given the absence of measures to help psychologists working with infertile couples, this paper presents the process of developing a standardized measure to assess emotional maladjustment and adaptive resources in this population. A cross-sectional design was utilized to gather data from the assisted reproduction units of two public hospitals. Preliminary analyses were performed with a sample of 85 infertile patients. Psychometric properties of the measure were tested with a second sample of 490 infertile patients. Concerning reliability analyses, alpha indexes were adequate both for the measure and its factors. Concerning validity, second-order factor analysis yielded a four-factor solution that conjointly explains 56% of the total variance. Additional analyses with a third sample of 50 participants from the general population matched with a sample of 50 infertile participants were performed. In sum, this measure seems to be a useful psychological assessment tool to determine emotional adjustment, and individual, and interpersonal resources, for coping with infertility diagnosis and treatment.

Acupuncture as a complementary therapy to the pharmacological treatment of osteoarthritis of the knee: randomised controlled trial.

OBJECTIVES: To analyse the efficacy of acupuncture as a complementary therapy to the pharmacological treatment of osteoarthritis of the knee, with respect to pain relief, reduction of stiffness, and increased physical function during treatment; modifications in the consumption of diclofenac during treatment; and changes in the patient's quality of life. DESIGN: Randomised, controlled, single blind trial, with blinded evaluation and statistical analysis of results. SETTING: Pain management unit in a public primary care centre in southern Spain, over a period of two years. PARTICIPANTS: 97 outpatients presenting with osteoarthritis of the knee. INTERVENTIONS: Patients were randomly separated into two groups, one receiving acupuncture plus diclofenac (n = 48) and the other placebo acupuncture plus diclofenac (n = 49). MAIN OUTCOME MEASURES: The clinical variables examined included intensity of pain as measured by a visual analogue scale; pain, stiffness, and physical function subscales of the Western Ontario and McMaster Universities (WOMAC) osteoarthritis index; dosage of diclofenac taken during treatment; and the profile of quality of life in the chronically ill (PQLC) instrument, evaluated before and after the treatment programme. RESULTS: 88 patients completed the trial. In the intention to treat analysis, the WOMAC index presented a greater reduction in the intervention group than in the control group (mean difference 23.9, 95% confidence interval 15.0 to 32.8) The reduction was greater in the subscale of functional activity. The same result was observed in the pain visual analogue scale, with a reduction of 26.6 (18.5 to 34.8). The PQLC results indicate that acupuncture treatment produces significant changes in physical capability (P = 0.021) and psychological functioning (P = 0.046). Three patients reported bruising after the acupuncture sessions. CONCLUSIONS: Acupuncture plus diclofenac is more effective than placebo acupuncture plus diclofenac for the symptomatic treatment of osteoarthritis of the knee.

La codependencia en el actual sistema sanitario / Codependence in health system

En las últimas décadas asistimos a un incremento de investigaciones sobre aquellas familias que tienen que asumir el cuidado de sujetos con enfermedades crónicas, cuya evolución puede implicar una falta de autonomía y una incapacidad para realizar las tareas de autocuidado. En general, estos cuidadores, generalmente mujeres, han de afrontar no sólo los cambios conductuales que presentan estos enfermos sino también asumir el cuidado y la atención de sus necesidades (incluso de las más básicas o de autocuidado) y, además compatibilizarlo bien con otras responsabilidades familiares o bien con su actividad laboral. Entre el numeroso cuerpo de investigaciones se destacan, desde sus inicios, aquellas relacionadas con el estrés que se deriva de la situación de cuidador. Sin embargo, en pocas ocasiones se ha analizado la importancia que las variables de personalidad (o incluso los trastornos de personalidad) pueden ejercer la calidad diaria cuidador- cuidado. Es este el propósito del presente trabajo, en el que analizaremos el papel que la codependencia juega en dicha relación.

In the last decade, numerous studies report the challenges of family caregiving, particularly as experienced by the female caregiven who must contend with a complex seto f behavioral changes evidence by their care receiver, the provision of activities of daily living and, reside, accommodate caregiving responsibilities and employment or others household tasks, informal caregiving has moved from descriptive beginnings to a number of theoretically driven approaches, one of the most influential being stress theory and these is a substantial literature documenting the stressfulness of caregiving for a family member witch chronic illness. However, one personal resource of caregivers which has been identified as potentially important, yet which has not received much attention, is personality and / or personality disorders. The aim of this article is to examine the role of codependency in...

Influencia de las galletas fortificadas en los níveles basales de hemoglobina en adolescentes varones de 12 a 16 años: Barrios Altos en los meses de febrero-marzo 1997 / Influence of cakes fortified in the basal levels of hemoglobin in adolescent men of 12 to 16 years: Barrios Altos in the months of February - March 1997

De acuerdo a los resultados obtenidos en el presente estudio de investigación se llegó a las siguientes conclusiones: La experiencia descrita demuestra que la fortificación con hierro heme, durante un mes incrementó los niveles basales de hemoglobina en un 76.5 por ciento de la población sujeto de estudio. La mayor proporción de la muestra (35.3 por ciento) presentó un incremento de 0.33 gr/dl de hemoglobina. Las galletas fortificadas con hierro hemínico son ampliamente aceptadas y toleradas por los adolescentes que integran la población muestral. Aceptabilidad calificada como buena, con observación en la consistencia que alcanzó la calificación de regular. El patrón dietético de las unidades muestrales, queda constituída en base a verduras, cereales y menestras.