The PROPKD Score: A New Algorithm to Predict Renal Survival in Autosomal Dominant Polycystic Kidney Disease

The course of autosomal dominant polycystic kidney disease (ADPKD) varies among individuals, with some reaching ESRD before 40 years of age and others never requiring RRT. In this study, we developed a prognostic model to predict renal outcomes in patients with ADPKD on the basis of genetic and clinical data. We conducted a cross-sectional study of 1341 patients from the Genkyst cohort and evaluated the influence of clinical and genetic factors on renal survival. Multivariate survival analysis identified four variables that were significantly associated with age at ESRD onset, and a scoring system from 0 to 9 was developed as follows: being male: 1 point; hypertension before 35 years of age: 2 points; first urologic event before 35 years of age: 2 points; PKD2 mutation: 0 points; nontruncating PKD1 mutation: 2 points; and truncating PKD1 mutation: 4 points. Three risk categories were subsequently defined as low risk (0–3 points), intermediate risk (4–6 points), and high risk (7–9 points) of progression to ESRD, with corresponding median ages for ESRD onset of 70.6, 56.9, and 49 years, respectively. Whereas a score ≤3 eliminates evolution to ESRD before 60 years of age with a negative predictive value of 81.4%, a score >6 forecasts ESRD onset before 60 years of age with a positive predictive value of 90.9%. This new prognostic score accurately predicts renal outcomes in patients with ADPKD and may enable the personalization of therapeutic management of ADPKD.     

Why do adults with palliative care needs present to the emergency department? A narrative review of the literature

Background: Given the increasing emphasis on community-based provision of palliative care and the view that good dying should occur at home, it is unsurprising that policy in many countries has started focusing on preventing hospital admissions at the end of life. However, little attention has been paid to the role of the emergency department (ED) in this regard, despite the fact that a high proportion of hospital admissions among patients with palliative care needs originate in the ED. This paper presents the next logical step in improving understanding of ED use within a palliative care context; as to determine what constitutes an avoidable ED presentation initially requires recognition of the reasons that such patients are presenting.

Methods: A systematic, narrative approach was used to appraise the relevant studies. From August to December 2014, electronic databases, gray literature and guidelines were searched, using MeSH headings and keywords. As it was anticipated that reasons for presentation would be differently described, all papers addressing presentations to the ED among patients with palliative care needs were identified; information regarding reasons for presentation were then examined.

Results: Twelve papers ultimately met the inclusion criteria and were accessible. The ‘reason’ for a patient's presentation was largely defined as their ‘presenting complaint’ or ‘underlying diagnosis.’ There was consistency in the studies in this respect; the majority found that people with lung cancer and those experiencing distressing respiratory symptoms were most likely the present to the ED. Subsequently, pain and gastrointestinal symptoms such as nausea and vomiting were uniformly found to be most common. The three prospective studies gathered information about reason for presentation from the patient perspective using structured questionnaires, but no study collected qualitative data from patients and families.

Discussion: This systematic narrative review is the first to explore reasons for ED presentation among patients with palliative care needs. However, the data does not facilitate a detailed discussion about the difficulties that this population of patients face, and whether they may be best managed in an acute or community setting. A deep understanding of the perspectives of patients is urgently needed, so as to both understand their reasons for presentation and implement relevant patient-directed changes in service provision.     

Brain regions and genes affecting postural control

Postural control is integrated in all facets of motor commands. The role of cortico-subcortical pathways underlying postural control, including cerebellum and its afferents (climbing, mossy, and noradrenergic fibers), basal ganglia, motor thalamus, and parieto-frontal neocortex has been identified in animal models, notably through the brain lesion technique in rats and in mice with spontaneous and induced mutations. These studies are complemented by analyses of the factors underlying postural deficiencies in patients with cerebellar atrophy. With the gene deletion technique in mice, specific genes expressed in cerebellum encoding glutamate receptors (Grid2 and Grm1) and other molecules (Prkcc, Cntn6, Klf9, Syt4, and En2) have also been shown to affect postural control. In addition, transgenic mouse models of the synucleinopathies and of Huntington's disease cause deficiencies of motor coordination resembling those of patients with basal ganglia damage.     

The Effect of Media on Body Image in Pregnant and Postpartum Women

Much research has found that exposure to certain types of media portrayals of women can be related to body image concerns among women. The current paper focuses on the impact of certain messages on pregnant and postpartum women. These women are rarely examined in a media research context but are particularly vulnerable to body image concerns. This experimental study involved 192 pregnant or postpartum women who read a magazine containing glamorized media portrayals of pregnant/postpartum women or a control magazine. Pregnant women reported lower body image after only five minutes of exposure to the magazine with pregnant/postpartum women compared to the control group. There was no immediate effect on postpartum women. Implications for the media industry, health professionals, and women are discussed.     

Use of a case-mix approach to study the trends in the incidence of second primary cancers

Purpose

To analyze trends in second primary cancer (SPC) incidence by using a case-mix approach to standardize on first cancer site distribution.

CX3CR1-dependent subretinal microglia cell accumulation is associated with cardinal features of age-related macular degeneration

The role of retinal microglial cells (MCs) in age-related macular degeneration (AMD) is unclear. Here we demonstrated that all retinal MCs express CX3C chemokine receptor 1 (CX3CR1) and that homozygosity for the CX3CR1 M280 allele, which is associated with impaired cell migration, increases the risk of AMD. In humans with AMD, MCs accumulated in the subretinal space at sites of retinal degeneration and choroidal neovascularization (CNV). In CX3CR1-deficient mice, MCs accumulated subretinally with age and albino background and after laser impact preceding retinal degeneration. Raising the albino mice in the dark prevented both events. The appearance of lipid-bloated subretinal MCs was drusen-like on funduscopy of senescent mice, and CX3CR1-dependent MC accumulation was associated with an exacerbation of experimental CNV. These results show that CX3CR1-dependent accumulation of subretinal MCs evokes cardinal features of AMD. These findings reveal what we believe to be a novel pathogenic process with important implications for the development of new therapies for AMD.     

The Association Between Bariatric Surgery and Psychiatric Disorders: a National Cohort Study

Obesity Surgery - Previous studies have shown that bariatric surgery reduces the risk of cardiovascular outcomes. Less is known about the effects of bariatric surgery on psychiatric disorders. This...     

Pulmonary artery compression by hematoma,a rare but life-threatening complication of Stanford type A aortic dissection

Hemorrhagic extension along the pulmonary arteries should be recognized as a complication of Stanford type A aortic dissection. Radiologists and other physicians with an interest in cardiothoracic imaging should be aware of this rare but life-threatening pathology. The anatomical concept of the pathology is outlined in this text, through high-quality electrocardiogram-gated computerized tomography images.