Enfermedad de Dupuytren en múltiples sitios / Multiple Site Dupuytren Disease

RESUMEN Introducción: La enfermedad de Dupuytren es una contractura de la fascia palmar debido a una proliferación fibrosa que provoca deformidades en flexión y pérdida de la función de los dedos de la mano. Puede también localizarse en las plantas de los pies, en el pene y otras localizaciones. La causa es desconocida. Objetivo: Describir la enfermedad en dos pacientes con características peculiares en múltiples sitios. Conclusiones: La enfermedad es más frecuente en el sexo masculino y en el grupo de edad de mayores de 40 años. El tratamiento utilizado fue la cirugía mediante fasciectomia más zetaplastia y la amputación(AU)

ABSTRACT Introduction: Dupuytren's disease is a contracture of the palmar fascia due to a fibrous proliferation that causes flexion deformities and loss of function of fingers. It can also be located on the soles of the feet, on the penis or other locations. The cause is unknown. Objectives: To describe the disease in two patients with peculiar characteristics at multiple sites. Conclusions: The disease is more frequent in males and older than 40 years. The treatment used was fasciectomy surgery plus zetaplasty and amputation(AU)

Clinical Profile and Outcome of Pediatric Sarcoidosis

Objectives: To document clinical features and outcome of children with sarcoidosis.Methods: Case records of 18 children (mean (SD) age 9 (2.2) years) diagnosed withsarcoidosis between 2006 and 2016 were reviewed. All children were followed up every 2-3months and monitored for clinical and laboratory parameters. Their treatment and outcomewere recorded. Results: Clinical features at the time of diagnosis were fever (83%), uveitis(50%), difficulty in breathing (44%), hepatosplenomegaly, weight loss, arthritis and peripheraladenopathy. Imaging findings included: hilar adenopathy (94%), abdominal nodes (50%) andpulmonary infiltrates (44%). All children were treated with steroids (range 6-12 months) andweekly low dose oral methotrexate. All patients showed significant improvement over a mean(SD) duration of follow-up of 3.1 (0.9) years, as assessed by resolution of clinical symptoms,and improvement in spirometry parameters, erythrocyte sedimentation rate, and serumangiotensin converting enzyme levels. Conclusions: Children with sarcoidosis seem torespond well to systemic steroids and low dose methotrexate. Delayed diagnosis and ocularinvolvement are probably associated with poor outcome.

Uma forma simplificada de determinar a direção da osteotomia de corte único para a correção de deformidade combinada rotacional e angular dos ossos longos / A simplified way of determining the direction of a single-cut osteotomy to correct combined rotational and angular deformities of long bones

A direção da obliquidade do corte para a execução da osteotomia de corte único, para correção de deformidades angulares e rotacionais combinadas, é de difícil determinação. A obliquidade adequada, se no sentido horário ou anti-horário em relação à transversal ao eixo do osso, é usualmente determinada através de ensaios em modelos ósseos plásticos imitando a deformidade ou em bananas, onde diferentes simulações podem ser feitas. Isto é muito confuso e difícil. Neste trabalho propomos uma tabela, com entradas para as deformidades angular e rotacional e o lado afetado. Na tabela é indicada diretamente a obliquidade correta do corte em relação à perpendicular ao eixo diafisário. Uma revisão passo a passo da osteotomia de corte único é também apresentada, com vistas ao planejamento pré-operatório, com ênfase na contribuição proposta.

The direction of the obliquity of the cut for performing single-cut osteotomy to correct combined angular and rotational deformities is difficult to determine. The appropriate obliquity, i.e. whether clockwise or anticlockwise in relation to the perpendicular to the bone axis, is usually determined through trials using plastic bone models to imitate the deformity, or on bananas, on which different simulations can be made. This is very confusing and difficult. In this study, we propose a table, with entries for angular and rotational deformities and the affected side. The correct obliquity of the cut in relation to the perpendicular to the diaphyseal axis is directly indicated in the table. A step-by-step revision of the preoperative planning of the single-cut osteotomy is also presented, with emphasis on the proposed contribution.