Distinct ultrastructural phenotypes of glial and neuronal alpha-synuclein inclusions in multiple system atrophy.

Multiple system atrophy is characterized pathologically by the accumulation of alpha-synuclein (aSyn) into glial cytoplasmic inclusions (GCIs). The mechanism underlying the formation of GCIs is not well understood. In this study, correlative light and electron microscopy was employed to investigate aSyn pathology in the substantia nigra and putamen of post-mortem multiple system atrophy brain donors.

Association of Exclusive Breast Milk Intake and Outcomes in Infants With Uncomplicated Gastroschisis: A National Cohort Study.

Background: Enteral feeding is an essential part of the management of infants with gastroschisis. We hypothesized that exclusive breast milk is associated with improved neonatal outcomes.

Methods: We conducted a retrospective review of infants with uncomplicated gastroschisis through the Canadian Pediatric Surgery Network (CAPSNet) and Canadian Neonatal Network (CNN).

Interference of bovine hemoglobin-based oxygen carrier-201 (Hemopure) on four hematology analyzers.

Introduction: Hemoglobin-based oxygen carriers, for example HBOC-201 (Hemopure), are aimed to bridge acute anemia when blood transfusion is not available or refused by the patient. However, since HBOC-201 appears free in plasma, it interferes with laboratory tests. This study presents an overview of HBOC-201 interference on four commonly used hematology analyzers and suggests treatment monitoring possibilities.

Evaluating the Clinical and Economic Outcomes Associated with Poor Glycemic Control in People with Type 1 Diabetes in the Netherlands.

Introduction: Achieving and maintaining glycemic control is the cornerstone of type 1 diabetes management, with the aim of reducing the incidence of diabetes-related complications over the long term. However, many individuals fail to reach glycemic targets. The present study evaluated the clinical and economic burden associated with poor glycemic control in people with type 1 diabetes in the Netherlands, and the improvements in outcomes that can be achieved by improving treatment.

Proteomic landscapes of inherited platelet disorders with different etiologies.

Background: Inherited platelet disorders (IPDs) are a heterogeneous group of rare diseases that are caused by the defects in early megakaryopoiesis, proplatelet formation, and/or mature platelet function. Although genomic sequencing is increasingly used to identify genetic variants underlying IPD, this technique does not disclose resulting molecular changes that impact platelet function. Proteins are the functional units that shape platelet function; however, insights into how variants that cause IPDs impact platelet proteomes are limited.

Enteral supplementation with probiotics in preterm infants: A retrospective cohort study and 6-year follow-up.

The objective of this retrospective cohort study was to assess the impact of an enteral probiotics supplementation protocol on the incidence of necrotizing enterocolitis (NEC) in infants born <33 weeks gestational age (GA) or birth weight (BW) <1,500 g. In addition, a 6-year follow-up is presented after instigation of probiotic use. In October 2014, our NICU introduced an enteral probiotics supplementation protocol for infants born <33 weeks GA or BW <1,500 g.

Patients with Chromosome 11q Deletions Are Characterized by Inborn Errors of Immunity Involving both B and T Lymphocytes.

Disorders of the long arm of chromosome 11 (11q) are rare and involve various chromosomal regions. Patients with 11q disorders, including Jacobsen syndrome, often present with a susceptibility for bacterial and prolonged viral and fungal infections partially explained by hypogammaglobulinemia. Additional T lymphocyte or granular neutrophil dysfunction may also be present.

Histopathological characteristics are instrumental to distinguish monomorphic from polymorphic maculopapular cutaneous mastocytosis in children.

Background: Mastocytosis is characterized by the accumulation of mast cells (MCs) in the skin or other organs, and can manifest at any age. A significant number of paediatric mastocytosis cases persist after puberty. In particular, monomorphic maculopapular cutaneous mastocytosis (mMPCM) is often persistent and associated with systemic mastocytosis.

Diagnostic Value of a Protocolized In-Depth Evaluation of Pediatric Bone Marrow Failure: A Multi-Center Prospective Cohort Study.

Background: Severe multilineage cytopenia in childhood caused by bone marrow failure (BMF) often represents a serious condition requiring specific management. Patients are at risk for invasive infections and bleeding complications. Previous studies report low rates of identifiable causes of pediatric BMF, rendering most patients with a descriptive diagnosis such as aplastic anemia (AA).

Technological Applications Contributing to Relieve Care Burden or to Sleep of Caregivers and People With Dementia: A Scoping Review From the Perspective of Social Isolation.

The need for care will increase in the coming years. Most people with a disability or old age receive support from an informal caregiver. Caring for a person with dementia can be difficult because of the BPSD (Behavioral and Psychological Symptoms of Dementia).

High prevalence of non-accidental trauma among deceased children presenting at Level I trauma centers in the Netherlands.

Purpose: Between 0.1-3% of injured children who present at a hospital emergency department ultimately die as a result of their injuries. These events are typically reported as unnatural causes of death and may result from either accidental or non-accidental trauma (NAT).

Pediatric Fibrinogen PART II-Overview of Indications for Fibrinogen Use in Critically Ill Children.

Bleeding is frequently seen in critically ill children and is associated with increased morbidity and mortality. Fibrinogen is an essential coagulation factor for hemostasis and hypofibrinogenemia is an important risk factor for bleeding in pediatric and adult settings. Cryoprecipitate and fibrinogen concentrate are often given to critically ill children to prevent bleeding and improve fibrinogen levels, especially in the setting of surgery, trauma, leukemia, disseminated intravascular coagulopathy, and liver failure.

Pediatric Fibrinogen PART I-Pitfalls in Fibrinogen Evaluation and Use of Fibrinogen Replacement Products in Children.

Fibrinogen is a key coagulation protein, playing a critical role in hemostasis. It is the first factor to decrease to critical levels during bleeding. Hypofibrinogenemia is an important risk factor for bleeding in clinical settings, including pediatric surgery.

Outcome of Surgical Interventions and Deliveries in Patients with Bleeding of Unknown Cause: An Observational Study.

Background:  The most optimal management for patients with bleeding of unknown cause (BUC) is unknown, as limited data are available.

Objective:  Evaluate management and outcome of surgical procedures and deliveries in patients with BUC.

Materials And Methods:  All patients ≥12 years of age, referred to a tertiary center for a bleeding tendency, were included.

The long-term cost-effectiveness of oral semaglutide in the Netherlands based on the PIONEER 2, 3 and 4 randomized controlled trials.

Aims: To assess the long-term cost-effectiveness of novel glucagon-like peptide-1 (GLP-1) analog oral semaglutide versus sodium-glucose cotransporter-2 inhibitor empagliflozin, dipeptidyl peptidase-4 inhibitor sitagliptin and injectable GLP-1 analog liraglutide in the Netherlands, based on the results of the PIONEER clinical trials.

Methods: Outcomes were projected over patient lifetimes using the IQVIA CORE Diabetes Model. Clinical data were derived from PIONEER 2, 3 and 4.

Prophylactic fibrinogen concentrate administration in surgical correction of paediatric craniosynostosis: A double-blind placebo-controlled trial.

Background: Surgical craniosynostosis repair in children is associated with massive blood loss and significant transfusion of blood products. Fibrinogen concentrate is claimed to be useful in reducing blood loss and transfusion requirements.

Objective: We investigated whether prophylactic administration of fibrinogen concentrate will reduce blood loss and transfusion requirements during paediatric craniofacial surgery.

Contactin-1 Is Reduced in Cerebrospinal Fluid of Parkinson's Disease Patients and Is Present within Lewy Bodies.

Synaptic degeneration is an early phenomenon in Parkinson's disease (PD) pathogenesis. We aimed to investigate whether levels of synaptic proteins contactin-1 and contactin-2 in cerebrospinal fluid (CSF) of PD patients are reduced compared to dementia with Lewy bodies (DLB) patients and controls and to evaluate their relationship with α-synuclein aggregation. Contactin-1 and -2 were measured in CSF from PD patients ( 58), DLB patients ( 72) and age-matched controls ( 90).

Evaluation of thromboelastometry, thrombin generation and plasma clot lysis time in patients with bleeding of unknown cause: A prospective cohort study.

Introduction: Diagnostic evaluation of patients with a bleeding tendency remains challenging, as no disorder is identified in approximately 50% of patients. An impaired interplay of several haemostatic factors might explain bleeding phenotype in these patients.

Objective: To investigate whether global haemostasis assays are able to identify haemostatic abnormalities in patients with a bleeding tendency unexplained by current diagnostic laboratory tests.