The Prevention of Maternal Phenylketonuria (PKU) Syndrome: The Development and Evaluation of a Specific Training Program.

Background: Maternal phenylketonuria (PKU) syndrome, leading to severe psychomotor retardation, microcephaly, cardiac defects and undergrowth, affects the unborn children of mothers with PKU with insufficient metabolic control during pregnancy. To improve long-term outcomes, a specific prevention program was developed.

Methods: We designed a group training program for young women with PKU (>14 years) and their partners.

Clinical phenotyping uncovers heterogeneous associations between corticosteroid treatment and survival in critically ill COVID-19 patients.

Purpose: Disease heterogeneity in coronavirus disease 2019 (COVID-19) may render the current one-size-fits-all treatment approach suboptimal. We aimed to identify and immunologically characterize clinical phenotypes among critically ill COVID-19 patients, and to assess heterogeneity of corticosteroid treatment effect.

Methods: We applied consensus k-means clustering on 21 clinical parameters obtained within 24 h after admission to the intensive care unit (ICU) from 13,279 COVID-19 patients admitted to 82 Dutch ICUs from February 2020 to February 2022.

Preclinical and first-in-human safety studies on a novel magnetism-based haemofiltration method.

Extracorporeal haemofiltration devices that selectively remove cytokines could represent an adjunctive treatment in inflammatory diseases. One such device is the "IL-6-Sieve", wherein magnetic Anti-IL-6 Beads are introduced into an extracorporeal circuit via a Bead Adapter and then removed along with any surface-bound interleukin (IL)-6 by a Filter deployed in a Magnet, before the blood is returned to the patient. We report here on a series of animal studies, and a first-in-human study, on the safety of the IL-6-Sieve.

Impaired angiotensin II signaling in septic shock.

Recent years have seen a resurgence of interest for the renin-angiotensin-aldosterone system in critically ill patients. Emerging data suggest that this vital homeostatic system, which plays a crucial role in maintaining systemic and renal hemodynamics during stressful conditions, is altered in septic shock, ultimately leading to impaired angiotensin II-angiotensin II type 1 receptor signaling. Indeed, available evidence from both experimental models and human studies indicates that alterations in the renin-angiotensin-aldosterone system during septic shock can occur at three distinct levels: 1.

Performance evaluation of two different software programs for automated ASPECTS scoring in patients with suspected stroke.

Background: The range of software available to radiologists has increased enormously with the advancement of AI. A good example of this is software to determine ASPECTS in the treatment of potential stroke patients.

Objective: In this study, two software packages (eASPECTS from Brainomix and VIA_ASPECTS from Siemens) were tested and compared for their performance in the daily clinical routine of a maximum care provider with a 24/7 stroke unit.

Associations of dipeptidyl-peptidase 3 with short-term outcome in a mixed admission ICU-cohort.

Purpose: Biomarkers independently associated with outcome of intensive care unit (ICU) patients can improve risk assessment. The cytosolic protease dipeptidyl-peptidase 3 (DPP3) is released into the circulation upon cell necrosis. We aimed to investigate the prognostic properties of cDPP3 in a mixed-admission ICU cohort.

CytoSorb hemoperfusion markedly attenuates circulating cytokine concentrations during systemic inflammation in humans in vivo.

Background: The CytoSorb hemoadsorption device has been demonstrated to be capable of clearing inflammatory cytokines, but has not yet been shown to attenuate plasma cytokine concentrations. We investigated the effects of CytoSorb hemoperfusion on plasma levels of various cytokines using the repeated human experimental endotoxemia model, a highly standardized and reproducible human in vivo model of systemic inflammation and immunological tolerance induced by administration of bacterial lipopolysaccharide (LPS).

Methods: Twenty-four healthy male volunteers (age 18-35) were intravenously challenged with LPS (a bolus of 1 ng/kg followed by continuous infusion of 0.

Circulating dipeptidyl peptidase 3 and bio-adrenomedullin levels are associated with impaired outcomes in critically ill COVID-19 patients: a prospective international multicentre study.

Introduction: Dipeptidyl peptidase-3 (DPP3) is a protease involved in the degradation of several cardiovascular mediators. Adrenomedullin (bio-ADM) is a peptide essential for regulation of endothelial barrier function. In different shock-pathologies, both biomarkers are associated with disease severity, organ dysfunction and mortality.

Effects of enrichment strategies on outcome of adrecizumab treatment in septic shock: analyses of the phase II adrenomedullin and outcome in septic shock 2 trial.

Purpose: Adrecizumab, a non-neutralizing antibody of adrenomedullin (ADM) was recently investigated regarding its potential to restore endothelial barrier function in septic shock patients with high plasma ADM levels. Circulating dipeptidyl peptidase 3 (cDPP3), a protease involved in the degradation of several cardiovascular mediators, represents another biological pathway strongly associated with outcome in septic shock, although unrelated to ADM. Therefore, the prognosis of patients with elevated cDPP3 may not be influenced by Adrecizumab.

Impact of pregnancy planning and preconceptual dietary training on metabolic control and offspring's outcome in phenylketonuria.

To prevent maternal phenylketonuria (PKU) syndrome low phenylalanine concentrations (target range, 120-360 μmol/L) during pregnancy are recommended for women with PKU. We evaluated the feasibility and effectiveness of current recommendations and identified factors influencing maternal metabolic control and children's outcome. Retrospective study of first successfully completed pregnancies of 85 women with PKU from 12 German centers using historical data and interviews with the women.

The value of bioactive adrenomedullin and dipeptidyl peptidase 3 to predict short-term unfavourable outcomes after cardiac surgery: A prospective cohort study.

Background: Adrenomedullin (ADM) is a key regulator of endothelial barrier function and vascular tone. Dipeptidyl peptidase 3 (DPP3) is a protease involved in the degradation of several cardiovascular mediators. Increased levels of bioactive ADM (bio-ADM) and circulating DPP3 (cDPP3) were found to predict short-term outcome in cardiogenic shock patients.

Preventing maternal phenylketonuria (PKU) syndrome: important factors to achieve good metabolic control throughout pregnancy.

Background: Insufficient metabolic control during pregnancy of mothers with phenylketonuria (PKU) leads to maternal PKU syndrome, a severe embryo-/fetopathy. Since maintaining or reintroducing the strict phenylalanine (Phe) limited diet in adults with PKU is challenging, we evaluated the most important dietary and psychosocial factors to gain and sustain good metabolic control in phenylketonuric women throughout pregnancy by a questionnaire survey with 38 questions concerning therapy feasibility. Among them, the key questions covered 5 essential items of PKU care as follows: General information about maternal PKU, PKU training, diet implementation, individual metabolic care, personal support.

Mechanical thrombectomy in ischemic stroke: Parameters affecting the TICI outcome.

Background: Mechanical thrombectomy (MT) is a standard stroke treatment for patients with large vessel occlusions (LVOs). A decisive factor for a successful outcome is, among other things, timely treatment.

Objective: The objective was to analyze several time points in relation to outcomes and/or surrogate parameters.

The neurological and neuropsychiatric spectrum of adults with late-treated phenylketonuria.

Introduction: Phenylketonuria (PKU) is a rare, treatable inborn error of metabolism with frequent neurological and neuropsychiatric complications, especially in undiagnosed or insufficiently treated individuals. Given the wide range of clinical presentations and the importance of treatment implications, we here delineate the neurological and neuropsychiatric symptom spectrum in a large cohort of previously unreported adults with late-treated PKU.

Methods: We consecutively evaluated late-treated PKU cases and pooled clinical and paraclinical data, including video-material, from three centers with expertise in complex movement disorders, inborn errors of metabolism and pediatrics.

How rural is rural? The relationship between rural background of medical students and their career location intentions.

Objective: Rural background is associated with greater interest in rural practice. However, there is no universally agreed definition of 'rural' background used in medical school selection. This study explored the association between definitions of 'rural' background and students' intended career locations.

Circulating biomarkers to assess cardiovascular function in critically ill.

Purpose Of Review: Circulatory shock is one of the most common reasons for ICU admission. Mortality rates in excess of 40% necessitate the rapid identification of high-risk patients, as well as the early assessment of effects of initiated treatments. There is an unmet medical need for circulating biomarkers that may improve patient stratification, predict responses to treatment interventions and may even be a target for novel therapies, enabling a better biological rationale to personalize therapy.

Increased blood angiotensin converting enzyme 2 activity in critically ill COVID-19 patients.

https://bit.ly/2MU1z4z.

Hepatorenal Tyrosinaemia: Impact of a Simplified Diet on Metabolic Control and Clinical Outcome.

: Tyrosinaemia type 1 is a rare inherited metabolic disease caused by an enzyme defect in the tyrosine degradation pathway. It is treated using nitisinone and a low-protein diet. In a workshop in 2013, a group of nutritional specialists from Germany, Switzerland and Austria agreed to advocate a simplified low-protein diet and to allow more natural protein intake in patients with tyrosinaemia type 1.

Promotion of vascular integrity in sepsis through modulation of bioactive adrenomedullin and dipeptidyl peptidase 3.

Sepsis represents one of the major medical challenges of the 21st century. Despite substantial improvements in the knowledge on pathophysiological mechanisms, this has so far not translated into novel adjuvant treatment strategies for sepsis. In sepsis, both vascular tone and vascular integrity are compromised, and contribute to the development of shock, which is strongly related to the development of organ dysfunction and mortality.

Correction to: A 3i hip fracture liaison service with nurse and physician co-management is cost-effective when implemented as a standard clinical program.

The original version of this article, published on 22 July 2020, unfortunately contained a mistake.

A 3i hip fracture liaison service with nurse and physician co-management is cost-effective when implemented as a standard clinical program.

Unlabelled: A hip fracture liaison service that was implemented in 2 hospitals in Alberta, Canada, co-managed by a nurse and physician, was cost-effective and improved initiation of osteoporosis medication following hip fracture.

Purpose/introduction: To determine cost-effectiveness of a 3i hip fracture liaison service (H-FLS) with 12-month follow-up, co-managed by a nurse and physician, when implemented into standard practice.

Methods: The cost-effectiveness analysis compared those receiving the H-FLS to a simulated usual care group using a decision analytic model that incorporated Markov processes.