Portuguese recommendations for the prevention, diagnosis and management of primary osteoporosis - 2018 update.

BACKGROUND: Advances in osteoporosis (OP)case definition, treatment options, optimal therapy duration and pharmacoeconomic evidence in the national context motivated the Portuguese Society of Rheumatology (SPR) to update the Portuguese recommendations for the diagnosis and management of osteoporosis published in 2007. METHODS: SPR bone diseases' working group organized meetings involving 55 participants (rheumatologists, rheumatology fellows and one OP specialist nurse) to debate and develop the document. First, the working group selected 11 pertinent clinical questions for the diagnosis and management of osteoporosis in standard clinical practice. Then, each question was investigated through literature review and draft recommendations were built through consensus. When insufficient evidence was available, recommendations were based on experts' opinion and on good clinical practice. At two national meetings, the recommendations were discussed and updated. A draft of the recommendations full text was submitted to critical review among the working group and suggestions were incorporated. A final version was circulated among all Portuguese rheumatologists before publication and the level of agreement was anonymously assessed using an online survey. RESULTS: The 2018 SPR recommendations provide comprehensive guidance on osteoporosis prevention, diagnosis, fracture risk assessment, pharmacological treatment initiation, therapy options and duration of treatment, based on the best available evidence. They attained desirable agreement among Portuguese rheumatologists. As more evidence becomes available, periodic revisions will be performed. Target audience and patient population: The target audience for these guidelines includes all clinicians. The target patient population includes adult Portuguese people. Intended use: These recommendations provide general guidance for typical cases. They may not be appropriate in all situations - clinicians are encouraged to consider this information together with updated evidence and their best clinical judgment in individual cases.

Esteatocistoma múltiple: reporte de caso / Steatocystoma multiplex: a case report

El esteatocistoma múltiple corresponde a una entidad cuya lesión elemental es el quiste cutáneo, lesión de carácter benigno y de muy baja frecuencia, en la mayoría de los casos su presentación es esporádico, originado en la unidad pilosebácea. Su principal diagnóstico diferencial corresponde al quiste velloso eruptivo. El diagnóstico se obtiene a través de la conjunción entre la clínica con la histopatología. Actualmente se dispone de varias alternativas para su tratamiento. En la literatura está descrita su asociación con el Síndrome de Gardner, el cual tiene un carácter autosómico dominante. Es posible encontrar: poliposis, osteomas, hipertrofía congénita del epitelio pigmentario de la retina y quistes cutáneo. Se presenta a continuación un caso de un paciente de sexo masculino de 17 años con un esteatocistoma múltiple.

Steatocystoma multiplex is a disorder of very low frequency whose principal characteristic is benign cutaneous cysts originating in the pilosebaceous unit. Most cases present sporadically. The differential diagnosis is principally eruptive villous hair cyst. Diagnosis is reached by considering both the clinical picture and the histology. There are several treatment alternatives. Its association with Gardner´s syndrome, an autosomal dominant trait, is described in the literature. It may be accompanied by polyposis, osteomas, congenital hypertrophy of the retinal pigment epithelium and cutaneous cysts. We present the case of a 17 year old male patient with steatocystoma multiplex.

Clinical features and long-term outcomes of systemic lupus erythematosus: comparative data of childhood, adult and late-onset disease in a national register.

Systemic lupus erythematosus (SLE) affects predominantly women at reproductive age but may present at any age. Age at disease onset has a modulating effect on presentation and course of disease, but controversies persist regarding its impact on long-term outcome. Our aims were to characterize clinical features, co-morbidities and cumulative damage in childhood-onset, adult-onset and late-onset SLE. Patients with childhood-onset SLE fulfilling ACR 1997 criteria were identified in a nationwide register-Reuma.pt/SLE (N = 89) and compared with adult-onset and late-onset counterparts matched 1:1:1 for disease duration. 267 SLE patients with mean disease duration of 11.9 ± 9.3 years were analyzed. Skin (62 %), kidney (58 %), neurological (11 %) and hematologic involvement (76 %) were significantly more common in childhood-onset SLE and disease activity was higher in this subset than in adult- and late-onset disease (SLEDAI-2K 3.4 ± 3.8 vs. 2.2 ± 2.7 vs. 1.6 ± 2.8, respectively; p = 0.004). Also, more childhood-onset patients received cyclophosphamide (10 %) and mycophenolate mofetil (34 %). A greater proportion of women (96 %), prevalence of arthritis (89 %) and anti-SSA antibodies (34 %) were noted in the adult-onset group. There was a significant delay in the diagnosis of SLE in older ages. Co-morbidities such as hypertension, diabetes and thyroid disease were significantly more frequent in late-onset SLE, as well as the presence of irreversible damage evaluated by the SLICC/ACR damage index (20 vs. 26 vs. 40 %; p < 0.001). Greater organ involvement as well as the frequent need for immunosuppressants supports the concept of childhood-onset being a more severe disease. In contrast, disease onset is more indolent but co-morbidity burden and irreversible damage are greater in late-onset SLE, which may have implications for patients' management.

[Nutritional status of the university student population in Galicia]. / Estado nutricional de una población de estudiantes universitarios de Galicia.

In this work we have investigated the dietary habits of 107 university students in Northwest Spain. From the different types of questionnaires that exist, the "24 hours recall" has been elected. Biochemical analysis were carried out in blood and urine to complete the nutritional study. Our results indicate that the distribution of energy intake over the day is generally acceptable though in many cases a bigger breakfast would be advisable. The results of blood and urine analysis were in all cases within the corresponding normal ranges which means that there aren't any evidence of pathological alteration.

[Consumption of lipids in the university population of Spain, evaluation of the hepatic profile and of serum cholesterol]. / Ingesta de lípidos en la población universitaria del noroeste de España, evaluación de su perfil hepático y del colesterol sérico.

In the present study we tried to characterise the diet of university students in Northwest Spain, paying particular attention to lipids and fibre intake. We administered a 24 hour recall dietary-habits questionnaire. Saturated and monounsaturated fats predominated, with the intake of polyunsaturated fatty acids being considerably lower. Mean daily cholesterol intake was in most cases in excess of the recommended maximum and mean fibre intake showed a considerable variation among individuals. The obtained hepatic profile is found within normal range. The calories provided by the diet result adapted in half of the studied population approximately, while the fourth part does not cover the recommended quantity. These results could be considered a risk in the future.

[Study of dietary habits of students in Galicia]. / Estudio de los hábitos alimentarios de estudiantes en Galicia.

The food habits of 168 university students in Galicia (a region in Northwest Spain) were investigated by means of a questionnaire. Alcohol consumption, smoking and frequency of physical exercise were also evaluated, and height, body weight and blood pressure were determined for all subjects. Our results show that in the diet of university students all groups of foods are present, and their frequency consumption is within the recommended intake by Health Authorities. Smoking and alcohol consumption is low among the study population.