Increased Intracranial Arterial Pulsatility and Microvascular Brain Damage in Pseudoxanthoma Elasticum.

Background And Purpose: Carotid siphon calcification might contribute to the high prevalence of cerebrovascular disease in pseudoxanthoma elasticum through increased arterial flow pulsatility. This study aimed to compare intracranial artery flow pulsatility, brain volumes, and small-vessel disease markers between patients with pseudoxanthoma elasticum and controls and the association between arterial calcification and pulsatility in pseudoxanthoma elasticum.

Materials And Methods: Fifty patients with pseudoxanthoma elasticum and 40 age- and sex-matched controls underwent 3T MR imaging, including 2D phase-contrast acquisitions for flow pulsatility in the assessment of ICA and MCA and FLAIR acquisitions for brain volumes, white matter lesions, and infarctions.

Determinants of Quality of Life and Treatment Satisfaction During Long-Term Involuntary In-patient Treatment of Dual-Diagnosis Patients.

Introduction: Treatment resistance and disengagement from mental health services are major obstacles in the treatment of dual diagnosis patients with Severe Mental Illness. The patients in this study were admitted to a long-term involuntary treatment facility.

Aim Of The Study: To study which patient experiences and perceptions are related to the outcome measures Subjective Quality of Life (SQOL) and Treatment Satisfaction (TS) during the long-term involuntary treatment.

Etidronate halts systemic arterial calcification in pseudoxanthoma elasticum.

Background And Aims: In pseudoxanthoma elasticum (PXE), low levels of inorganic pyrophosphate result in extensive arterial calcification. Recently, the treatment of ectopic mineralization in the PXE (TEMP) trial showed that one year of treatment with etidronate halts progression of femoral artery calcification in PXE patients. The aim of this study was to test the efficacy of etidronate on calcification in different vascular beds.

Clinical effects and treatment outcomes of long-term compulsory in-patient treatment of treatment-resistant patients with severe mental illness and substance-use disorder.

Background: The treatment of homeless dual-diagnosis patients (i.e., those with severe mental illness and substance-use disorder) is difficult and often fails.

The amount of calcifications in pseudoxanthoma elasticum patients is underestimated in computed tomographic imaging; a post-mortem correlation of histological and computed tomographic findings in two cases.

Objectives: Pseudoxanthoma elasticum (PXE) is a rare genetic disorder, characterised by elastic fibre degeneration and calcifications in multiple organ systems. Computed tomography (CT) imaging is a potential method to monitor disease progression in PXE patients; however, this method has not been validated. The aim of this study was to correlate histological and computed tomographic findings in PXE patients to investigate the ability of CT scanning to detect these alterations.

The prevalence of pseudoxanthoma elasticum: Revised estimations based on genotyping in a high vascular risk cohort.

Background: Pseudoxanthoma elasticum (PXE), an autosomal recessive systemic calcification disorder, is caused by mutations in the ABCC6-gene and associated with severe visual impairment and peripheral arterial disease. Given the progress in development of a therapy for PXE, more precise estimations of its prevalence are warranted.

Methods: We genotyped the four most common ABCC6 mutations (c.

Etidronate for Prevention of Ectopic Mineralization in Patients With Pseudoxanthoma Elasticum.

Background: In pseudoxanthoma elasticum (PXE), low pyrophosphate levels may cause ectopic mineralization, leading to skin changes, visual impairment, and peripheral arterial disease.

Objectives: The authors hypothesized that etidronate, a pyrophosphate analog, might reduce ectopic mineralization in PXE.

Methods: In the Treatment of Ectopic Mineralization in Pseudoxanthoma Elasticum trial, adults with PXE and leg arterial calcifications (n = 74) were randomly assigned to etidronate or placebo (cyclical 20 mg/kg for 2 weeks every 12 weeks).

Arterial stiffening and thickening in patients with pseudoxanthoma elasticum.

Background And Aims: Patients with pseudoxanthoma elasticum (PXE), a monogenetic calcification disease, are at high vascular risk. Although the precise arterial phenotype remains unestablished, it is hypothesized that PXE predominantly affects the medial arterial layer leading to arterial stiffening. We aimed to test this hypothesis by measuring arterial wall characteristics in PXE and comparisons with the general population and diabetes mellitus type 2 (DM2), a condition typically associated with mixed intimal and medial arterial disease.

Inter-arm systolic blood pressure differences, relations with future vascular events and mortality in patients with and without manifest vascular disease.

Background: Inter-arm systolic blood pressure difference (SBPD) is an easily obtained patient characteristic which relates to vascular disease. We aimed to identify determinants of large inter-arm SBPD and to investigate the relation between inter-arm SBPD and vascular events in patients with and without manifest vascular disease.

Methods: In a cohort of 7344 patients with manifest vascular disease or vascular risk factors alone enrolled in the Second Manifestations of ARTerial disease (SMART) study, single bilateral non-simultaneous blood pressure measurements were performed.

Cerebral disease in a nationwide Dutch pseudoxanthoma elasticum cohort with a systematic review of the literature.

Background: Pseudoxanthoma elasticum (PXE) is a monogenetic disease with progressive calcification of arteries and potential risk of stroke. To gain insights in the cerebral involvement in PXE, we evaluated prevalence and determinants of cerebral disease in our PXE cohort and performed a systematic review of literature.

Methods: Systematic history taking concerning cerebral disorders was performed in our PXE cohort.

Prevalence and severity of arterial calcifications in pseudoxanthoma elasticum (PXE) compared to hospital controls. Novel insights into the vascular phenotype of PXE.

Background And Aims: Pseudoxanthoma elasticum (PXE) is a monogenetic disorder with progressive calcifications of the skin, the Bruch's membrane in the eyes and the arterial wall. Vascular disease is considered to be very prevalent, but the whole-body distribution of arterial calcifications in PXE is unknown. We aimed to systematically investigate arterial calcifications in PXE.

Bisphosphonates for cardiovascular risk reduction: A systematic review and meta-analysis.

Background And Aims: Bisphosphonates might be effective in reducing cardiovascular events due to their ability to reduce calcification in arterial walls. We aimed to investigate the effects of treatment with bisphosphonates on the prevention of atherosclerotic processes and cardiovascular disease.

Methods: Pubmed, Embase and the Cochrane Library were systematically reviewed by two independent investigators for randomized controlled studies published up to January 2016, in which the effect of bisphosphonates on arterial wall disease, cardiovascular events, cardiovascular mortality or all-cause mortality were reported.

[Pseudoxanthoma elasticum: A disorder with different manifestations].

Background: Pseudoxanthoma elasticum (PXE) is a rare, autosomal recessive inheritable disorder characterized by progressive elastic fibre calcification.

Case Description: Here we describe two patients with different presentations of PXE. Patient A, an 11-year-old girl, visited the dermatologist because of yellow papules (pseudoxanthomas) on the side of her neck.

Abnormally high failure rate for femoral angioplasty in patients with pseudoxanthoma elasticum.

Pseudoxanthoma elasticum (PXE) is an inherited disease characterized by skin lesions, central blindness, and progressive peripheral occlusive disease. Severe claudication is a frequent symptom for which angioplasty represents a possible therapeutic avenue. We report the outcomes of four patients with PXE treated by angioplasty and stenting of the superficial femoral artery in two centers.

The relation between HbA1c and cardiovascular events in patients with type 2 diabetes with and without vascular disease.

Objective: Poor glycemic control is related to vascular events in patients with type 2 diabetes, but the presence of vascular disease might influence this relation. We evaluated the relation between glycemic control (HbA1c level) and new cardiovascular events and mortality in patients with type 2 diabetes, with and without vascular disease.

Research Design And Methods: In a cohort of 1,687 patients with type 2 diabetes enrolled in the Second Manifestations of Arterial Disease (SMART) study, the continuous relation between HbA1c and cardiovascular events (composite of myocardial infarction, stroke, and vascular mortality) and all-cause mortality was evaluated with Cox proportional hazard analyses stratified for the presence of vascular disease.

Prognostic value of continued smoking on survival and recurrence rates in patients with head and neck cancer: A systematic review.

Background: The purpose of this systematic review was to determine the prognostic value of continued smoking after diagnosis on survival and recurrence rates in head and neck cancer.

Methods: A systematic search was performed and predefined inclusion and exclusion criteria were used to screen and select the articles. The remaining articles were critically appraised.

Mortality after cardiac surgery in patients with liver cirrhosis classified by the Child-Pugh score.

Liver cirrhosis is a known risk factor for postoperative mortality in patients undergoing cardiac surgery. Clinical assessment of liver cirrhosis using the widely accepted Child-Pugh (CP) score is thus vital for evaluation of surgical options and perioperative care. However, detailed mortality rates as a consequence of liver cirrhosis are unclear.

[A woman with overlapping growth of nail plate fragments].

A 38-year-old woman presented with nail abnormalities on both of the halluces, consisting of overlapping growth of nail plate fragments, diagnosed as onychomadesis. The onychomadesis was provoked by trauma.

[Hypersomnia: a diagnostic problem].

Hypersomnia is a frequently occurring problem. When taking a medical history it is important to distinguish between fatigue and sleepiness. We present a 14-year-old girl with narcolepsy and a 59-year-old man with idiopathic hypersomnia.

Screening for frailty in primary care: a systematic review of the psychometric properties of the frailty index in community-dwelling older people.

Background: To better accommodate for the complex care needs of frail, older people, general practitioners must be capable of easily identifying frailty in daily clinical practice, for example, by using the frailty index (FI). To explore whether the FI is a valid and adequate screening instrument for primary care, we conducted a systematic review of its psychometric properties.

Methods: We searched the Cochrane, PubMed and Embase databases and included original studies focusing on the criterion validity, construct validity and responsiveness of the FI when applied in community-dwelling older people.

Identifying frailty: do the Frailty Index and Groningen Frailty Indicator cover different clinical perspectives? a cross-sectional study.

Background: Early identification of frailty is important for proactive primary care. Currently, however, there is no consensus on which measure to use. Therefore, we examined whether a Frailty Index (FI), based on ICPC-coded primary care data, and the Groningen Frailty Indicator (GFI) questionnaire identify the same older people as frail.

Habitual physical activity in daily life correlates positively with markers for mitochondrial capacity.

Physical exercise training is a powerful tool to maintain or improve mitochondrial density and function (mitochondrial capacity). This study aims to determine whether mitochondrial capacity is also associated with habitual physical activity in daily life (PADL). The capacity of classic markers for mitochondrial density, i.

Ubiquitin-proteasome-dependent proteolytic activity remains elevated after zymosan-induced sepsis in rats while muscle mass recovers.

We studied the role of the ubiquitin-proteasome system in rat skeletal muscle during sepsis and subsequent recovery. Sepsis was induced with intraperitoneal zymosan injections. This model allows one to study a sustained and reversible catabolic phase and mimics the events that prevail in septic and subsequently recovering patients.

Skeletal Muscle wasting and contractile performance in septic rats.

We investigated the temporal effects of sepsis on muscle wasting and function in order to study the contribution of wasting to the decline in muscle function; we also studied the fiber-type specificity of this muscle wasting. Sepsis was induced by injecting rats intraperitoneally with a zymosan suspension. At 2 h and at 2, 6, and 11 days after injection, muscle function was measured using in situ electrical stimulation, Zymosan injection induced severe muscle wasting compared to pair-fed and ad libitum fed controls.