Management of patients with hypertension and chronic kidney disease referred to Hypertension Excellence Centres among 27 countries. On behalf of the European Society of Hypertension Working Group on Hypertension and the Kidney.

Objective Real-life management of patients with hypertension and chronic kidney disease (CKD) among European Society of Hypertension Excellence Centres (ESH-ECs) is unclear : we aimed to investigate it. Methods A survey was conducted in 2023. The questionnaire contained 64 questions asking ESH-ECs representatives to estimate how patients with CKD are managed.

Hemodynamic Bedside Monitoring Instrument with Pressure and Optical Sensors: Validation and Modality Comparison.

Results from two independent clinical validation studies for measuring hemodynamics at the patient's bedside using a compact finger probe are reported. Technology comprises a barometric pressure sensor, and in one implementation, additionally, an optical sensor for photoplethysmography (PPG) is developed, which can be used to measure blood pressure and analyze rhythm, including the continuous detection of atrial fibrillation. The capabilities of the technology are shown in several form factors, including a miniaturized version resembling a common pulse oximeter to which the technology could be integrated in.

Development and clinical validation of a miniaturized finger probe for bedside hemodynamic monitoring.

Our aim is to develop a blood pressure (BP) measurement technology that could be integrated into a finger-worn pulse oximeter, eliminating the need for a brachial cuff. We present a miniature cuffless tonometric finger probe system that uses the oscillometric method to measure BP. Our approach uses a motorized press that is used to apply pressure to the fingertip to measure BP.

Chronic kidney disease stage is associated with the number of risk factors in type 2 diabetes patients (STages Of NEphropathy in type 2 diabetes and Heart Failure - STONE HF).

Aims: To study the association between risk factors and chronic kidney disease (CKD), and characterize medication use in Finnish primary care type 2 diabetes (T2D) patients.

Methods: Data on clinical characteristics, laboratory measurements, and medications were collected from medical records. The primary outcome measure was notable CKD (stage 3-5, eGFR <60 ml/min/1.

An expert consensus on the recommendations for the use of biomarkers in Fabry disease.

Fabry disease is an X-linked lysosomal storage disorder caused by the accumulation of glycosphingolipids in various tissues and body fluids, leading to progressive organ damage and life-threatening complications. Phenotypic classification is based on disease progression and severity and can be used to predict outcomes. Patients with a classic Fabry phenotype have little to no residual α-Gal A activity and have widespread organ involvement, whereas patients with a later-onset phenotype have residual α-Gal A activity and disease progression can be limited to a single organ, often the heart.

Long-term effectiveness of enzyme replacement therapy in Fabry disease with the p.Arg227Ter variant: Fabry disease in Ostrobothnia (FAST) study.

Fabry disease (FD) is an X chromosome-linked, life-threatening lysosomal disease caused by one of more than 1000 currently known variants in the α-galactosidase A (GLA) gene. The follow-up part of the Fabry Disease in Ostrobothnia (FAST) study reports the long-term effect of enzyme replacement therapy (ERT) on a prospectively collected cohort of 12 patients, 4 males and 8 females, mean age 46 years (SD 16), with the classical variant c.679C > T p.

Late-onset and classic phenotypes of Fabry disease in males with the -Thr410Ala mutation.

Objective: To present phenotypic characteristics and biomarkers of a family with the rare mutation Thr410Ala of the gene (T410A/) causing Fabry disease (FD).

Methods And Results: In a woman in her 60s with hypertrophic cardiomyopathy, T410A/ was found in screening for variants in 59 cardiomyopathy-related genes. Her son in his 40s, two granddaughters and two great grandsons carried T410A/.

Clinical outcomes among young patients with Fabry disease who initiated agalsidase beta treatment before 30 years of age: An analysis from the Fabry Registry.

Background: Clinical manifestations of classic Fabry disease (α-galactosidase A deficiency) usually occur in childhood, while complications involving major organs typically develop in adulthood. Outcomes of Fabry-specific treatment among young patients have not been extensively reported. Our aim was to analyze clinical outcomes among patients aged 5-30 years at initiation of treatment with agalsidase beta using data from the Fabry Registry (NCT00196742, sponsor: Sanofi).

Pulmonary manifestations and the effectiveness of enzyme replacement therapy in Fabry Disease with the p. Arg227Ter (p.R227*) mutation.

Background: Fabry disease (FD) is caused by a defect in α-galactosidase A gene (GLA) which leads to a progressive accumulation of neutral shingolipids, mainly globotriaosylceramide and its metabolites in several organs. Pulmonary manifestations of FD mimic chronic obstructive pulmonary disease and are disproportionate to smoking status. The effect of enzyme replacement therapy (ERT) on pulmonary function is inconclusive.

The majority of type 2 diabetic patients in Finnish primary care are at very high risk of cardiovascular events: A cross-sectional chart review study (STONE HF).

Aims: To characterize clinical profiles, prevalence of chronic kidney disease (CKD), and treatment patterns in type 2 diabetes (T2D) and heart failure (HF) patients in Finnish primary care.

Methods: A total of 1385 patients (1196 with T2D, 50 with HF, and 139 with T2D and HF) in 60 Finnish primary care centers were recruited to this cross-sectional study. Data on demographic and clinical characteristics, laboratory measurements, and medications were collected retrospectively from medical records.

Unsupervised hierarchical clustering identifies a metabolically challenged subgroup of hypertensive individuals.

The current classification of hypertension does not reflect the heterogeneity in characteristics or cardiovascular outcomes of hypertensive individuals. Our objective was to identify distinct phenotypes of hypertensive individuals with potentially different cardiovascular risk profiles using data-driven cluster analysis. We performed clustering, a procedure that identifies groups with similar characteristics, in 3726 individuals (mean age 59.

An instrument for measuring blood pressure and assessing cardiovascular health from the fingertip.

Despite blood pressure being one the leading modifiable risk factors for cardiovascular disease and death, it is severely under-monitored. For this challenge we propose a finger artery non-invasive tono-oscillometric monitor (FANTOM) which is an automated low-cost instrument for measuring blood pressure and hemodynamic parameters from the fingertip. The sensing technology is highly scalable and could be integrated to a pulse oximeter probe for increased patient comfort.

Cardiomyopathy associated with the Ala143Thr variant of the gene.

Objective: To investigate whether the Ala143Thr variant of the gene (A143T/), with conflicting interpretations of pathogenicity, is associated with Fabry cardiomyopathy.

Methods: The index patient, a woman in her 60s with cardiomyopathy, was screened for variants in 59 cardiomyopathy-related genes. A143T/, the only rare variant found, was screened in 10 relatives.

Natural course of Fabry disease with the p. Arg227Ter (p.R227*) mutation in Finland: Fast study.

Background: Fabry disease is caused by a deficient or an absent alfa-galactosidase A activity and is an X-linked disorder that results in organ damage and a shortened life span, especially in males. The severity of the disease depends on the type of mutation, gender, skewed X-chromosome inactivation, and other still unknown factors.

Methods: In this article, we describe the natural course of a common classic Fabry disease mutation, p.

Agreement Between Ambulatory and Home Blood Pressure Monitoring in Detecting Nighttime Hypertension and Nondipping Patterns in the General Population.

Background: Nighttime blood pressure (BP) and nondipping pattern are strongly associated with hypertensive end-organ damage. However, no previous studies have compared the diagnostic agreement between ambulatory and home monitoring in detecting these BP patterns in the general population.

Methods: We studied a population-based sample of 180 persons aged 32-80 years.

The effect of enzyme replacement therapy on clinical outcomes in male patients with Fabry disease: A systematic literature review by a European panel of experts.

Background: Enzyme replacement therapy (ERT) with recombinant human α-galactosidase has been available for the treatment of Fabry disease since 2001 in Europe and 2003 in the USA. Treatment outcomes with ERT are dependent on baseline patient characteristics, and published data are derived from heterogeneous study populations.

Methods: We conducted a comprehensive systematic literature review of all original articles on ERT in the treatment of Fabry disease published up until January 2017.

European expert consensus statement on therapeutic goals in Fabry disease.

Background: Fabry disease, an inherited lysosomal storage disorder, causes multi-organ pathology resulting in substantial morbidity and a reduced life expectancy. Although Fabry disease is an X-linked disorder, both genders may be affected, but generally to a lesser extent in females. The disease spectrum ranges from classic early-onset disease to non-classic later-onset phenotypes, with complications occurring in multiple organs or being confined to a single organ system depending on the stage of the disease.

Relation of blood pressure and organ damage: comparison between feasible, noninvasive central hemodynamic measures and conventional brachial measures.

Objective: The present cross-sectional study investigated whether central SBP and pulse pressure (PP) measured noninvasively with a novel cuff-based stand-alone monitor are more strongly associated with hypertensive end-organ damage than corresponding brachial measures.

Methods: We investigated the cross-sectional association of central versus brachial SBP and PP with echocardiographic left ventricular mass index (LVMI), LV hypertrophy (LVH), carotid intima-media thickness (IMT), and increased IMT (IMT ≥ 75th percentile) among 246 participants drawn from the general population (mean age 57.2 years, 55.