Anticholinergic Exposure, Drug Dose and Postoperative Delirium: Comparison of Dose-Related and Non-Dose-Related Anticholinergic Burden Scores in a Retrospective Cohort Study of Older Orthopaedic and Trauma Surgery Patients.

Purpose: Postoperative delirium (POD) is a common complication in older adult patients after surgery. A patient's preoperative anticholinergic (AC) burden is a potentially modifiable risk factor for POD. As the influence of the drug dose remains unknown, we aimed to compare three AC burden scores in relation to POD, two of which were dose-related.

Development and validation of a new drug-focused predictive risk score for postoperative delirium in orthopaedic and trauma surgery patients.

Background: Postoperative delirium (POD) is the most common complication following surgery in elderly patients. During pharmacist-led medication reconciliation (PhMR), a predictive risk score considering delirium risk-increasing drugs and other available risk factors could help to identify risk patients.

Methods: Orthopaedic and trauma surgery patients aged ≥ 18 years with PhMR were included in a retrospective observational single-centre study 03/2022-10/2022.

Aminoacylase 1 deficiency: case report on three affected siblings.

Background: Aminoacylase 1 (ACY1, EC 3.5.1.

[The old person in road traffic].

Due to the demographic change and the rising mobility of older adults in comparison with former generations, the number of old persons involved in road traffic has been continuously growing. The safety of all participating parties in road traffic necessitates a successful interaction of each individual's visual, motor and cognitive functions. These might be negatively affected by age-related physiological changes with a large individual variation.

The Influence of Adherence to Orthosis and Physiotherapy Protocol on Functional Outcome after Proximal Humeral Fracture in the Elderly.

In the treatment of proximal humeral fractures (PHF), patients are often recommended to wear a sling or orthosis and to perform physiotherapy. However, some patients, especially elderly ones, struggle to comply with these rehabilitation regimens. Therefore, the aim of the study was to evaluate whether these incompliant patients have a worse functional outcome than those who adhere to the rehabilitation protocol.

Benefit of medication reviews by renal pharmacists in the setting of a computerized physician order entry system with clinical decision support.

What Is Known And Objective: A 'renal pharmacist consultant service' (RPCS) reviewing patients' charts with renal impairment (RI) for drug-related problems (DRP) can foster patient safety. However, the benefit of this service in the new setting of a computerized physician order entry (CPOE)-system with a clinical decision support (CDS)-system is unknown. The aim of our study was to evaluate the general need for an RPCS on wards with a CPOE-CDS-system already in use and its effectiveness on prescription changes to ensure in-hospital patient safety.

Etiology and classification of acute pancreatitis in children admitted to ICU using the Pediatric Sequential Organ Failure Assessment (pSOFA) score.

Background: Pediatric acute pancreatitis (AP) is rare but increasing. Severe AP is associated with higher morbidity and mortality. However, there are no universally accepted prognostic criteria for AP.

Impact of Newborn Screening and Early Dietary Management on Clinical Outcome of Patients with Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency and Medium Chain Acyl-CoA Dehydrogenase Deficiency-A Retrospective Nationwide Study.

Long chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD/MTPD) and medium chain acyl-CoA dehydrogenase deficiency (MCADD) were included in the expanded neonatal screening program (ENBS) in Czechia in 2009, allowing for the presymptomatic diagnosis and nutritional management of these patients. The aim of our study was to assess the nationwide impact of ENBS on clinical outcome. This retrospective study analysed acute events and chronic complications and their severity in pre-ENBS and post-ENBS cohorts.

Recurrence of Primary Sclerosing Cholangitis After Liver Transplant in Children: An International Observational Study.

Background And Aims: Recurrent primary sclerosing cholangitis (rPSC) following liver transplant (LT) has a negative impact on graft and patient survival; little is known about risk factors for rPSC or disease course in children.

Approach And Results: We retrospectively evaluated risk factors for rPSC in 140 children from the Pediatric PSC Consortium, a multicenter international registry. Recipients underwent LT for PSC and had >90 days of follow-up.

Morphogenetic systems: Models and experiments.

M systems are mathematical models of morphogenesis developed to gain insights into its relations to phenomena such as self-assembly, self-controlled growth, homeostasis, self-healing and self-reproduction, in both natural and artificial systems. M systems rely on basic principles of membrane computing and self-assembly, as well as explicit emphasis on geometrical structures (location and shape) in 2D, 3D or higher dimensional Euclidean spaces. They can be used for principled studies of these phenomena, both theoretically and experimentally, at a computational level abstracted from their detailed implementation.

Severe linezolid-induced lactic acidosis in a child with acute lymphoblastic leukemia: A case report.

Linezolid is an antibiotic increasingly used for treatment of resistant Gram-positive infections, which blocks bacterial proteosythesis through direct inhibition of mitochondrial ribosomes. The most common adverse effects of linezolid include gastrointestinal symtoms, peripheral neuropathy, bone marrow depression and lactic acidosis. Here we present a rare case of a 9-year-old female, a survivor of acute lymphoblastic leukemia (ALL) and a hematopoietic stem cell transplant (HSCT), who developed life-threatening lactic acidosis with vomiting, impaired consciousness and Kussmaul breathing after 51 days of intravenous linezolid administration due to mycobacterial infection.

The Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) Index: A Prognostic Tool for Children.

Background And Aims: Disease progression in children with primary sclerosing cholangitis (PSC) is variable. Prognostic and risk-stratification tools exist for adult-onset PSC, but not for children. We aimed to create a tool that accounts for the biochemical and phenotypic features and early disease stage of pediatric PSC.

Assessing the Validity of Adult-derived Prognostic Models for Primary Sclerosing Cholangitis Outcomes in Children.

Background: Natural history models for primary sclerosing cholangitis (PSC) are derived from adult patient data, but have never been validated in children. It is unclear how accurate such models are for children with PSC.

Methods: We utilized the pediatric PSC consortium database to assess the Revised Mayo Clinic, Amsterdam-Oxford, and Boberg models.

Acute onset of autoimmune hepatitis in children and adolescents.

Background: Autoimmune hepatitis (AIH) is a rare progressive liver disease, which manifests as acute hepatitis in 40%-50% of pediatric cases. This refers predominantly to spontaneous exacerbations of previously unrecognized subclinical AIH with laboratory and histological signs of chronic hepatitis, or to acute exacerbations of known chronic disease. Only a few of these patients fulfill criteria for acute liver failure (ALF).

Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis: Predictors of Gamma Glutamyltransferase Normalization and Favorable Clinical Course.

Objective: To investigate patient factors predictive of gamma glutamyltransferase (GGT) normalization following ursodeoxycholic acid (UDCA) therapy in children with primary sclerosing cholangitis.

Study Design: We retrospectively reviewed patient records at 46 centers. We included patients with a baseline serum GGT level ≥50 IU/L at diagnosis of primary sclerosing cholangitis who initiated UDCA therapy within 1 month and continued therapy for at least 1 year.

Gamma Glutamyltransferase Reduction Is Associated With Favorable Outcomes in Pediatric Primary Sclerosing Cholangitis.

Adverse clinical events in primary sclerosing cholangitis (PSC) happen too slowly to capture during clinical trials. Surrogate endpoints are needed, but no such validated endpoints exist for children with PSC. We evaluated the association between gamma glutamyltransferase (GGT) reduction and long-term outcomes in pediatric PSC patients.

The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration.

Unlabelled: There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death.

Chemical angioplasty with spasmolytics for vasospasm after subarachnoid hemorrhage.

Background: Clinically symptomatic vasospasm leading to delayed ischemic neurological deficits occurs in up to 30% of patients with subarachnoid hemorrhage (SAH). Vasospasm can result in a serious decline in clinical conditions of patients with SAH, yet the algorithm for vasospasm treatment and prevention remains unclear. Intra-arterial administration of vasodilators is one of the modalities used for vasospasm therapy.

Prospective study of hypothalamo-hypophyseal dysfunction in children and adolescents following traumatic brain injury.

Background And Aims: Retrospective studies of TBI have found a neuroendocrine dysfunction following traumatic brain injury in 23 to 60% of adults and 15 to 21% of children. Our aims were to determine the prevalence of hypothalamo-hypophyseal dysfunction in children following brain injury, assess its relationship to the type of injury and the course of the acute post-traumatic phase.

Patients And Methods: Body development (growth, pubertal development, and skeletal maturity) were evaluated in 58 patients (21 girls) after a brain injury rated 3 to 12 on the Glasgow Coma Scale (GCS).

Long-term follow-up of children and adolescents with primary sclerosing cholangitis and autoimmune sclerosing cholangitis.

Background: Sclerosing cholangitis (SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate long-term results in children with SC according to the types of SC.

Methods: We retrospectively followed up 25 children with SC over a period of 4-17 years (median 12).

Significant phenotype variability of congenital central hypoventilation syndrome in a family with polyalanine expansion mutation of the PHOX2B gene.

Background: Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder resulting from mutations in the PHOX2B gene located on chromosome 4p12.3, characterized by hypoventilation secondary to missing responses to both hypercapnia and hypoxia.

Case Report: Proband.

Muscular dystrophies and myopathies: the spectrum of mutated genes in the Czech Republic.

Inherited neuromuscular disorder (NMD) is a wide term covering different genetic disorders affecting muscles, nerves, and neuromuscular junctions. Genetic and clinical heterogeneity is the main drawback in a routine gene-by-gene diagnostics. We present Czech NMD patients with a genetic cause identified using targeted next-generation sequencing (NGS) and the spectrum of these causes.

Enantioseparation of d,l-2-hydroxyglutaric acid by capillary electrophoresis with tandem mass spectrometry-Fast and efficient tool for d- and l-2-hydroxyglutaracidurias diagnosis.

A novel capillary electrophoresis-tandem mass spectrometry method for the enantioseparation and identification of 2-hydroxyglutaric acid enantiomers without derivatization for clinical purposes was described. Vancomycin chloride was used as an efficient chiral selector for the discrimination of 2-hydroxyglutaric acid enantiomers by capillary electrophoresis employed complete capillary filling method. The obtained resolution was 2.