Effect of an Educational Nursing Intervention on the Mental Adjustment of Patients with Chronic Arterial Hypertension: An Interventional Study.

The objective of this analytical and interventional prospective quantitative study was to assess the effect of an educational intervention performed by nurses for mental adjustment to chronic disease in patients with hypertension. A convenience sample was studied, composed of 329 participants with chronic hypertension, followed in a primary healthcare unit in the Central Region of Portugal. Data collection was carried out by applying the Mental Adjustment to Disease Scale (MADS) before and 1 month after the educational nursing intervention between September 2017 and February 2018. Prior to the application of the educational intervention, 43.5% of the participants were classified as "unadjusted" in at least one of the subscales of MADS. After the educational intervention, 21.3% of the participants classified as "unadjusted" became "adjusted" in all MADS subscales. The success rate of the intervention varied from 26.9% (in the fatalism subscale) to 44.6% (for the anxious concern subscale). Participants were more likely to be mentally "unadjusted" to hypertension if they lived with other family members, had an active professional situation before the diagnosis of hypertension, still had an active professional situation now, were under 65 years old, had a shorter time to diagnosis (1-2 years), and measured blood pressure less regularly. The educational intervention performed by nurses is relevant for the mental adjustment of hypertensive patients, contributing to increased knowledge, as well as improvement in preventive and self-care practices, facilitating the experience of the health/disease transition process.

Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis.

Pulmonary hypoplasia or agenesis is a rare congenital disorder that results in lung underdevelopment. This disease is usually found in children but rarely encountered in adults. We describe the case of an 84-year-old woman diagnosed with a unilateral pulmonary hypoplasia presenting simultaneously with left pulmonary artery hypoplasia. Due to this condition, the patient had a lifelong history of pulmonary infections that resulted in several bronchiectases in the affected lung. Moreover, the pulmonary artery hypoplasia led to the development of pulmonary hypertension and collateral circulation causing hemoptysis, giving rise to the patient attending the emergency department. Although we were able to medically manage the hemoptysis, it can be fatal and require surgical intervention. Hence, an early diagnosis is essential so that appropriate follow-up and prompt prevention and treatment of complications, such as pulmonary infections, hemoptysis and pulmonary hypertension, are achieved. LEARNING POINTS: Although rare and typically associated with childhood, lung and pulmonary artery hypoplasia can in exceptional cases be present in adult life.The diagnosis of lung and pulmonary artery hypoplasia is challenging in adults as they can easily be mistaken for more common diseases.Symptoms and complications may be life-threatening, including dyspnea, hemoptysis, recurrent pulmonary infections and pulmonary hypertension.An early diagnosis is extremely important because it allows a close follow-up with consequent timely detection and treatment of serious complications, especially pulmonary hypertension.

Exome sequencing in a consanguineous family clinically diagnosed with early-onset Alzheimer's disease identifies a homozygous CTSF mutation.

We have previously reported the whole genome genotyping analysis of 2 consanguineous siblings clinically diagnosed with early onset Alzheimer's disease (AD). In this analysis, we identified several large regions of homozygosity shared between both affected siblings, which we suggested could be candidate loci for a recessive genetic lesion underlying the early onset AD in these cases. We have now performed exome sequencing in one of these siblings and identified the potential cause of disease: the CTSF c.1243G>A:p.Gly415Arg mutation in homozygosity. Biallelic mutations in this gene have been shown to cause Type B Kufs disease, an adult-onset neuronal ceroid lipofuscinosis with some cases resembling the impairment seen in AD.

Hepatocellular Carcinoma Treatment With Sorafenib: Real-Life Evaluation of Prognostic Factors and a Practical Clue for Patient Management.

INTRODUCTION: Sorafenib chemotherapy is the first-line therapy for patients with hepatocellular carcinoma (HCC) in an advanced stage. The aim of this study was to evaluate prognostic factors of survival in HCC patients treated with sorafenib, in real-life clinical practice. MATERIAL AND METHODS: Retrospective study of HCC patients who initiated treatment with sorafenib, following assessment and indication from the multidisciplinary group. RESULTS: There were included 36 patients, mostly male (89%) and with a mean age of 65 years. The main etiologies were chronic hepatitis C (44%) and alcoholic liver disease (36%). Twenty patients (56%) were classified as Child-Pugh A and 16 patients (44%) as Child-Pugh B. Half of the patients group were staged as BCLC C and the remaining as BCLC B. Significant adverse events were observed in 15 patients (42%) and were associated with longer survival (21.5 vs. 3.2 months, p < 0.001). The most frequent adverse events were diarrhea and palmar-plantar syndrome. Median survival was 17.3 months for Child-Pugh A versus 3.2 months for Child-Pugh B patients (p = 0.001). Within Child-Pugh A, median OS was 21.5 months for BCLC B patients and 15.7 months for BCLC C patients (p = 0.001). DISCUSSION AND CONCLUSIONS: The main prognostic factors beyond Child-Pugh class and BCLC stage included the occurrence of significant adverse events. Being related to increased time of exposure to the drug, it points out the need of dose reducing instead of discontinuation whenever significant adverse events occur.

INTRODUÇÃO: O tratamento de primeira linha de doentes com carcinoma hepatocelular (HCC) em estadio avançado é a quimioterapia com sorafenib. O objetivo deste estudo foi avaliar os fatores de prognóstico de sobrevivência em doentes com HCC tratados com sorafenib, na prática clínica. MATERIAL E MÉTODOS: Estudo retrospetivo com inclusão dos doentes com HCC que iniciaram tratamento com sorafenib, após avaliação e decisão de grupo em reunião multidisciplinar. RESULTADOS: Foram incluídos 36 doentes, que eram em maioria do género masculino (89%) e com idade média de 65 anos. As principais etiologias eram a hepatite C crónica (44%) e a doença hepática alcoólica (36%). Vinte doentes (56%) foram classificados como Child-Pugh A e 16 doentes (44%) como Child-Pugh B. A amostra apresentava em metade dos casos estadio BCLC C e os restantes BCLC B. Quinze doentes (42%) desenvolveram efeitos adversos significativos, que se associaram com maior sobrevivência (21,5 vs 3,2 meses, p < 0,001). Os efeitos adversos mais frequentes foram diarreia e síndrome palmo-plantar. A sobrevivência global mediana foi de 6,8 meses (IC 95%, 3-10,6). A sobrevivência mediana foi de 17,3 meses nos doentes Child-Pugh A versus 3,2 meses nos casos Child-Pugh B (p = 0,001). Considerando os doentes Child-Pugh A, a sobrevivência mediana foi de 21,5 meses para o estadio BCLC B e 15,7 meses para o estadio BCLC C (p = 0,001). DISCUSSÃO E CONCLUSÕES: Os principais fatores prognósticos, além da classificação de Child-Pugh e do estadiamento BCLC, incluíram a ocorrência de efeitos adversos. Estes, relacionados com o tempo de exposição ao fármaco, assinalam a importância de uma estratégia de redução de dose em vez de descontinuação quando se manifestam efeitos adversos significativos.