Sequential Morphological Changes in the CNV Net after Intravitreal Anti-VEGF Evaluated with OCT Angiography

PURPOSE: To assess and describe sequential morphological changes in the choroidal neovascularization (CNV) net using optical coherence tomography angiography (OCTA) in patients undergoing treatment with intravitreal antivascular endothelial growth factor (VEGF). METHODS: Prospective cohort study. OCTA was performed sequentially: before (t0), 1 h (t1), 1 week (t2) and 1 month after the injection (t3), using Avanti RTVue XR equipped with the AngioVue® software (Optovue, Calif., USA). All images were classified by two independent graders. RESULTS: Ten eyes of 10 patients, with a mean age of 72.4 ± 10.5 years, were included. CNV morphology was described as tree-like in 5 eyes, glomerular in 1 and fragmented in 4. A fibrovascular capsule surrounding the CNV net was found in 4 eyes and a feeder trunk was noticed in 6. No changes were observed at t1. Loss of peripheral capillaries, vessel fragmentation and decreased vessel density were evident in 8 eyes at t2. The CNV capillary density and the peripheral anastomosis increased in all of these at t3. Two eyes remained unchanged through the whole length of follow-up. CONCLUSIONS: Significant changes in the CNV net can be observable in OCTA at least 1 week after intravitreal anti-VEGF. The safety of frequent examinations may provide a method of gauging treatment effects.

Opiniões sobre a visita de enfermagem em unidades de cirurgia: tradução, adaptação e validação de questionários

Enquadramento: Apesar da visita de enfermagem ou passagem de turno junto dos doentes garantir a continuidade dos cuidados de enfermagem, origina diversas questões éticas. Objetivos: Traduzir, adaptar e validar 2 questionários de colheita de dados sobre a visita de enfermagem em unidades cirúrgicas para a cultura portuguesa; identificar a opinião dos doentes e dos enfermeiros sobre a visita de enfermagem. Metodologia: Tradução, retroversão e adaptação dos questionários aplicados em 7 unidades cirúrgicas de um hospital central em Portugal de 22/08/2008 a 28/06/2009. A amostragem não probabilística acidental foi constituída por 137 enfermeiros e 96 doentes. Resultados: A visita de enfermagem promove a relação empática e de ajuda; a observação do doente e o planeamento de cuidados, no entanto é necessário clarificar o seu objetivo; incentivar à participação e ao envolvimento dos doentes; utilizar um discurso compreensível e garantir a privacidade da informação. Conclusão: Os questionários revelaram-se fidedignos e válidos na identificação das opiniões dos doentes e dos enfermeiros sobre a visita de enfermagem nas unidades de cirurgia em estudo.

Opiniões sobre a visita de enfermagem em unidades de cirurgia: tradução, adaptação e validação de questionários

Enquadramento: Apesar da visita de enfermagem ou passagem de turno junto dos doentes garantir a continuidade dos cuidados de enfermagem, origina diversas questões éticas. Objetivos: Traduzir, adaptar e validar 2 questionários de colheita de dados sobre a visita de enfermagem em unidades cirúrgicas para a cultura portuguesa; identificar a opinião dos doentes e dos enfermeiros sobre a visita de enfermagem. Metodologia: Tradução, retroversão e adaptação dos questionários aplicados em 7 unidades cirúrgicas de um hospital central em Portugal de 22/08/2008 a 28/06/2009. A amostragem não probabilística acidental foi constituída por 137 enfermeiros e 96 doentes. Resultados: A visita de enfermagem promove a relação empática e de ajuda; a observação do doente e o planeamento de cuidados, no entanto é necessário clarificar o seu objetivo; incentivar à participação e ao envolvimento dos doentes; utilizar um discurso compreensível e garantir a privacidade da informação. Conclusão: Os questionários revelaram-se fidedignos e válidos na identificação das opiniões dos doentes e dos enfermeiros sobre a visita de enfermagem nas unidades de cirurgia em estudo.Background: Although bedside nursing handover ensures the continuity of nursing care, it raises several ethical issues. Objectives: To translate, adapt and validate 2 data collection questionnaires about bedside nursing handover in surgical units into Portuguese, and identify the patients and nurses’ opinions about bedside nursing handover. Methodology: Translation, back-translation and adaptation of the questionnaires in 7 surgical units of a central hospital in Portugal between 22/08/2008 and 28/06/2009. The non- -probability sampling consisted of 137 nurses and 96 patients. Results: Bedside nursing handover promotes an empathic and helping relationship, patient observation and care planning; however, it is necessary to clarify its purpose, encourage patient participation and engagement, use clear information, and ensure information privacy. Conclusion: The questionnaires proved to be reliable and valid to identify the patients and nurses’ opinions about bedside nursing handover in the surgical units under analysis.

Síndroma compartimental aguda ou das locas: Conceitos essenciais

A síndroma compartimental aguda define-se como sendo a falência da circulação intersticial por conflito de espaço. Representa, habitualmente, uma severa complicação das lesões traumáticas, podendo envolver potencialmente qualquer compartimento do organismo. Mais frequente em fraturas de alta energia cinética e em lesões por esmagamento dos membros, é uma verdadeira emergência ortopédica, por forma a conseguir-se a redução da pressão intracompartimental e restabelecer a perfusão dos tecidos em sofrimento. Assim, a fasciotomia cirúrgica descompressiva, em tempo oportuno, pode evitar a isquémia irreversível dos tecidos mioneurais contidos nos diferentes compartimentos osteomiofasciais, isto é, as retrações fibrosas musculares, a necrose muscular, as lesões nervosas permanentes, a perda permanente da função e, como última consequência, a amputação dos membros. A definição da melhor altura para a fasciotomia nem sempre é uma tarefa fácil, envolvendo critérios clínicos e, também, a determinação do valor da pressão intracompartimental, mormente nos doentes em estado de inconsciência. Nos casos em que houver dúvidas e nos doentes em risco de desenvolver esta complicação, é defensável efetuar uma fasciotomia precoce, mesmo sabendo que pode ser desnecessária, uma vez que as complicações relacionadas com as fasciotomias tardias são devastadoras. Com efeito, o risco de infeção e de septicémia são elevados, a taxa de amputações é considerável e, por vezes, perde-se a vida. As sequelas da síndrome compartimental aguda são uma das causas frequentes de litigância contra os cirurgiões ortopedistas.

Brooke-Spiegler Syndrome - an underrecognized cause of multiple familial scalp tumors: report of a new germline mutation

BACKGROUND: Brooke-Spiegler syndrome (BSS) is probably an underdiagnosed genodermatosis that predisposes for the development of cylindromas, spiradenomas and trichoepitheliomas mainly of the head and neck. Wide phenotypic variability regarding the number and type of lesions can be observed within a family. Mutations of the CYLD gene are identified in the vast majority of cases and play a key role in BSS pathogenesis. MAIN OBSERVATIONS: Two first degree relatives with numerous erythematous telangiectatic nodules of the scalp present for decades, with recurring tendency regardless the multiple previous excisions. Histopathological review of the lesions revealed predominantly "spiradenocylindromas" in the proband and cylindromas in her sister. The suspicion of BSS was confirmed after detection of a new nonsense germline mutation of CYLD (c.1783C>T pGln 595*) in the proband. CONCLUSIONS: BSS diagnosis can be challenging and is based on clinical-pathological correlation, positive familial association and identification of CYLD mutations. CYLD exerts antineoplastic effects by downregulating intracellular NF-κB signalling pathways. The reported mutation affecting the ubiquitin-specific protease domain leads to a truncated and catalytically inactive enzyme. Despite the expanding list of CYLD mutations no firm genotype-phenotype correlation is known so far. Early recognition and treatment of BSS avoid disfiguring changes like "turban tumor".

[Carotid atherosclerosis and white matter hypodensities: a controversial relationship]

INTRODUCTION: White matter hypodensities of presumed vascular origin, are recognized as an important cause of morbidity with established clinical and cognitive consequences. Nonetheless, many doubts remain on its physiopathology. Our goal is to clarify the potential role of carotid atherosclerosis and other vascular risk factors in the development of white matter hypodensities of presumed vascular origin. MATERIAL AND METHODS: We included patients that underwent CT brain scan and neurosonologic evaluation within a one-month period. Full assessment of vascular risks factors was performed. We seek to find independent associations between white matter hypodensities of presumed vascular origin, carotid intima-media thickness and vascular risk factors. RESULTS: 472 patients were included, mean age was 67.32 (SD: 14.75), 274 (58.1%) were male. The independent predictors of white matter hypodensities of presumed vascular origin were age (OR: 1.067, 95% IC: 1.049 - 1.086, p < 0.001) and hypertension (OR: 1.726, 95% IC: 1.097 - 2.715, p = 0.018). No association was found between IMT (OR: 2.613, 95% IC: 0.886 - 7.708, p = 0.082) or carotid artery stenosis (OR: 1.021, 95% IC: 0.785 - 1.328, p = 0.877) and white matter hypodensities of presumed vascular origin. DISCUSSION: Only age and hypertension proved to have an independent association with white matter hypodensities of presumed vascular origin. Carotid atherosclerosis, evaluated by IMT and the degree of carotid artery stenosis, showed no association with white matter hypodensities of presumed vascular origin. Since atherosclerosis is a systemic pathology, these results suggest that alternative mechanisms are responsible for the development of white matter hypodensities of presumed vascular origin. CONCLUSION: Age and hypertension seem to be the main factors in the development of white matter hypodensities of presumed vascular origin. No association was found between carotid atherosclerosis and white matter hypodensities of presumed vascular origin.

Relational and reproductive trajectories leading to adolescent pregnancy in Portugal: a national and regional characterization

INTRODUCTION: The current study aimed to describe the relational and reproductive trajectories leading to adolescent pregnancy in Portugal, and to explore whether there were differences in this process according to adolescents' place of residence. MATERIAL AND METHODS: Data were collected between 2008 and 2013 in 42 public health services using a self-report questionnaire developed by the researchers. The sample consisted of a nationally representative group of pregnant adolescents (n = 459). RESULTS: Regardless of having had one (59.91%) or multiple sexual partners (40.09%), the majority of adolescents became pregnant in a romantic relationship, using contraception at the time of the conception and knowing the contraceptive failure which led to pregnancy (39.22%). In some regions other trajectories were highly prevalent, reflecting options such as planning the pregnancy (Alentejo Region/ Azores Islands), not using contraception (Centro Region/Madeira Islands) or using it incorrectly, without identifying the contraceptive failure (Madeira Islands). On average, romantic relationships were longer than 19 months and adolescents' partners were older than themselves (> 4 years) and no longer in school (75.16%); these results were particularly significant when the pregnancy was planned. DISCUSSION: The knowledge gained in this study shows that prevention efforts must be targeted according to the adolescents' needs in each region and should include high-risk male groups. CONCLUSION: Our results may enable more efficient health policies to prevent adolescent pregnancy in different country regions and support educators and health care providers on sexual education and family planning efforts.

Accelerated age-related olfactory decline among type 1 Usher patients

Usher Syndrome (USH) is a rare disease with hearing loss, retinitis pigmentosa and, sometimes, vestibular dysfunction. A phenotype heterogeneity is reported. Recent evidence indicates that USH is likely to belong to an emerging class of sensory ciliopathies. Olfaction has recently been implicated in ciliopathies, but the scarce literature about olfaction in USH show conflicting results. We aim to evaluate olfactory impairment as a possible clinical manifestation of USH. Prospective clinical study that included 65 patients with USH and 65 normal age-gender-smoking-habits pair matched subjects. A cross culturally validated version of the Sniffin' Sticks olfaction test was used. Young patients with USH have significantly better olfactory scores than healthy controls. We observe that USH type 1 have a faster ageing olfactory decrease than what happens in healthy subjects, leading to significantly lower olfactory scores in older USH1 patients. Moreover, USH type 1 patients showed significantly higher olfactory scores than USH type 2, what can help distinguishing them. Olfaction represents an attractive tool for USH type classification and pre diagnostic screening due to the low cost and non-invasive nature of the testing. Olfactory dysfunction should be considered among the spectrum of clinical manifestations of Usher syndrome.

Bowel-associated dermatosis-arthritis syndrome in an adolescent with short bowel syndrome

Bowel-associated dermatosis-arthritis syndrome (BADAS) is a neutrophilic dermatosis, characterized by the occurrence of arthritis and skin lesions related to bowel disease with or without bowel bypass. We report an unusual case of BADAS in a 15-year-old white male with congenital aganglionosis of the colon and hypoganglionosis of the small intestine and multiple bowel surgeries in childhood complicated by short bowel syndrome. He presented with recurrent peripheral polyarthritis, tenosynovitis, and painful erythematous subcutaneous nodules located on the dorsolateral regions of the legs and on the dorsa of the feet. Histological examination disclosed a neutrophilic dermatosis confirming the diagnosis of BADAS.Although an uncommon disease, especially at pediatric age, it is important to evoke the diagnosis of BADAS in children and adolescents with bowel disease, because treatment options and prognosis are distinct from other rheumatologic conditions.

Tourette's syndrome and associated disorders: a systematic review

To compile data on Tourette's syndrome (TS), tics and associated disorders. METHODS: A systematic review of the literature was conducted using the 5S levels of organization of healthcare research evidence (systems, summaries, synopses, syntheses, studies), based on the model described by Haynes. The search keywords were Tourette, tics and comorbidity, which were cross-referenced. Studies provided by publishers and articles being processed on July 31, 2013, were also included. RESULTS: Of all studies retrieved during the search, 64 were selected because they analyzed the epidemiology, clinical features and etiopathogenesis of TS and its comorbidities. TS is classified as a hyperkinetic movement disorder, and at least 90% of the patients have neuropsychiatric comorbidities, of which attention deficit hyperactivity and obsessive-compulsive disorders are the most common. The syndrome is clinically heterogeneous and has been associated with a dysfunction of cortico-striatal-thalamic-cortical circuits involving various neurotransmitters. Although its genetic etiology has been widely studied, other factors may be important to understand this syndrome and its associated disorders. CONCLUSIONS: TS is a neurodevelopmental disorder that results from the impact of stress factors on a vulnerable biological substrate during the critical periods of neurodevelopment. The study of TS and its comorbidities may contribute, at different levels, to the understanding of several neuropsychiatric disorders of clinical and therapeutic relevance.

Gender mismatch between donor and recipient is a factor of morbidity but does not condition survival after cardiac transplantation

We intended to evaluate the influence of sex mismatch between donor and recipient, which is still under much debate, on survival and comorbidities after cardiac transplantation. From November 2003 to December 2013, a total of 258 patients were transplanted in our center. From these, 200 receptors were male (77.5%) and constituted our study population, further divided into those who received the heart from a female donor (Group A) - 44 patients (22%) and those who received it from a male donor (Group B) - 156 (78%). Median follow-up was 4.2 ± 3.0 years (1-10 years). The two groups were quite comparable with each other, except for body mass index, systolic pulmonary artery pressure, and transpulmonary gradient, which were significantly lower in Group A. A low donor/recipient weigh ratio (<0.8) was avoided whenever possible. Hospital mortality was not different in the two groups. During follow-up, global survival was similar, as was survival free from acute cellular rejection and cardiac allograft vasculopathy. However, patients in Group A had decreased survival free from serious infections and malignant tumors. Allocation of female donors to male receptors can be done safely, at least in receptors without pulmonary hypertension and when an adequate donor/recipient weigh ratio is ensured.

Cancer diagnosis disclosure from Portuguese physicians

OBJECTIVE: Doctor-patient communication in oncology, particularly concerning diagnostic disclosure, is a crucial factor related to the quality of the doctor-patient relationship and the psychological state of the patient. The aims of our study were to investigate physicians' opinions and practice with respect to disclosure of a cancer diagnosis and to explore potential related factors. METHOD: A self-report questionnaire developed for our study was responded to by 120 physicians from Coimbra University Hospital Centre and its primary healthcare units. RESULTS: Some 91.7% of physician respondents generally disclosed a diagnosis, and 94.2% were of the opinion that the patient knowing the truth about a diagnosis had a positive effect on the doctor-patient relationship. A need for training about communicating with oncology patients was reported by 85.8% of participants. The main factors determining what information to provide to patients were: (1) patient intellectual and cultural level, (2) patient desire to know the truth, and (3) the existence of family. SIGNIFICANCE OF RESULTS: Our results point to a paradigm shift in communication with cancer patients where disclosure of the diagnosis should be made part of general clinical practice. Nevertheless, physicians still experience difficulties in revealing cancer diagnoses to patients and often lack the skills to deal with a patient's emotional responses, which suggests that more attention needs to be focused on communication skills training programs.