Face-to-Face Gold Porphyrins.

The interaction of square-planar metal complexes through space is of fundamental interest and relevant for the potential cooperative catalysis of two metal complex sites. In order to elucidate gold/gold, gold/porphyrin, and porphyrin/porphyrin interactions in the formal oxidation states +III and +II (after reduction) and in the excited triplet state after light excitation, a Pacman bis(gold(III)) complex [Au(DPD)][PF] with square-planar face-to-face gold(porphyrin) moieties has been prepared and characterized. Absorption and luminescence spectroscopy, cyclic voltammetry, and EPR spectroscopy on [Au(DPD)] and a mononuclear reference are complemented by DFT and TDDFT calculations.

Intrafamilial Disease Heterogeneity in Primary Hyperoxaluria Type 1.

Introduction: Primary hyperoxaluria type 1 (PH1) is known for its variable clinical course, even within families. However, the extent of this heterogeneity has not been well-studied. We aimed to analyze intrafamilial clinical heterogeneity and disease course among siblings in a large cohort of familial PH1 cases.

Potential of Reusing 3D Printed Concrete (3DPC) Fine Recycled Aggregates as a Strategy towards Decreasing Cement Content in 3DPC.

This paper explores the new potential strategy of using fine recycled aggregates (fRA) derived from waste 3D printed concrete (3DPC) as a substitute for cement in additive manufacturing. This study hypothesizes that fRA can optimize mixture design, reduce cement content, and contribute to sustainable construction practices. Experimental programs were conducted to evaluate the fresh and hardened properties, printability window, and buildability of 3DPC mixes containing fRA.

Automatic image analysis applied to the recognition of quartz surface microtextures using neural network.

Microtextures imprinted on the surface of quartz grains provide in-depth information on the environmental conditions and sedimentary processes that affected the study sediments. Microtextural analyses are therefore widely used in the provenance studies of sediments. In order to minimize the subjectivity of microtextural recognition, we propose a new software, called DeepGrain (source codes are available at https://github.

Functional BiO/GdO Silica-Coated Structures for Improvement of Early Age and Radiation Shielding Performance of Cement Pastes.

This study presents a new approach towards the production of sol-gel silica-coated BiO/GdO cement additives towards the improvement of early mechanical performance and radiation attenuation. Two types of silica coatings, which varied in synthesis method and morphology, were used to coat BiO/GdO structures and evaluated as a cement filler in Portland cement pastes. Isothermal calorimetry studies and early strength evaluations confirmed that both proposed coating types can overcome retarded cement hydration process, attributed to BiO presence, resulting in improved one day compressive strength by 300% and 251% (depending on coating method) when compared to paste containing pristine BiO and GdO particles.

Development of a tool for predicting HNF1B mutations in children and young adults with congenital anomalies of the kidneys and urinary tract.

Background: We aimed to develop a tool for predicting HNF1B mutations in children with congenital abnormalities of the kidneys and urinary tract (CAKUT).

Methods: The clinical and laboratory data from 234 children and young adults with known HNF1B mutation status were collected and analyzed retrospectively. All subjects were randomly divided into a training (70%) and a validation set (30%).

The Outcome of Childhood Immunoglobulin A Nephropathy with Acute Kidney Injury at the Onset of the Disease-National Study.

IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide. Decreased glomerular filtration rate is a known risk factor for disease progression. We aimed to examine factors that may contribute to disease progression in children that present with impaired eGFR at the onset of IgAN.

Analysis of the Impact of Changes in Thermomechanical Properties of Annealed Semi-Crystalline Plastics on the Surface Condition after the Machining Process.

In this paper, the authors present a comparative analysis of the thermomechanical properties of plastics intended for machining before and after the annealing process. The research included the dynamic properties, thermal analysis and a study of the surface after machining. The dynamic properties were tested using the DMTA method.

Rare Single Nucleotide and Copy Number Variants and the Etiology of Congenital Obstructive Uropathy: Implications for Genetic Diagnosis.

Significance Statement: Congenital obstructive uropathy (COU) is a prevalent human developmental defect with highly heterogeneous clinical presentations and outcomes. Genetics may refine diagnosis, prognosis, and treatment, but the genomic architecture of COU is largely unknown. Comprehensive genomic screening study of 733 cases with three distinct COU subphenotypes revealed disease etiology in 10.

Excited state energy landscape of phosphorescent group 14 complexes.

Great progress has been achieved on phosphorescent or photoactive complexes of the Earth-abundant transition metals, while examples for phosphorescent heavy main group element complexes are rare, in particular for group 14 complexes in the oxidation state +II. The known compounds often show only weak phosphorescence with fast non-radiative deactivation. The underlying photophysical processes and the nature of the phosphorescent electronic states have remained essentially unexplored.

Building a precision medicine infrastructure at a national level: The Swedish experience.

Precision medicine has the potential to transform healthcare by moving from one-size-fits-all to personalised treatment and care. This transition has been greatly facilitated through new high-throughput sequencing technologies that can provide the unique molecular profile of each individual patient, along with the rapid development of targeted therapies directed to the Achilles heels of each disease. To implement precision medicine approaches in healthcare, many countries have adopted national strategies and initiated genomic/precision medicine initiatives to provide equal access to all citizens.

Self-reported symptom severity, general health, and impairment in post-acute phases of COVID-19: retrospective cohort study of Swedish public employees.

This study aimed to examine current symptom severity and general health in a sample of primarily non-hospitalized persons with polymerase chain reaction (PCR) confirmed COVID-19 in comparison to PCR negative controls. During the first quarter of 2021, we conducted an online survey among public employees in West Sweden, with a valid COVID-19 test result. The survey assessed past-month severity of 28 symptoms and signs, self-rated health, the WHO Disability Assessment Schedule (WHODAS) 2.

Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I.

Patients with primary hyperoxaluria type I (PH I) are prone to develop early kidney failure. Systemic deposition of calcium-oxalate (CaOx) crystals starts, when renal function declines and plasma oxalate increases. All tissue, but especially bone, heart and eyes are affected.

Outcome of infantile nephropathic cystinosis depends on early intervention, not genotype: A multicenter sibling cohort study.

Infantile nephropathic cystinosis (INC) is an inheritable lysosomal storage disorder characterized by lysosomal cystine accumulation, progressive kidney disease, and multiple extrarenal complications (ERCs). Cysteamine postpones the onset of end-stage kidney disease (ESKD) and reduces the incidence of ERCs; however, cysteamine is generally initiated upon establishment of the renal Fanconi syndrome (FS) and partial loss of kidney function, whereas data on long-term effects of cysteamine administered from neonatal age are lacking. An international multicenter retrospective cohort study of siblings with INC was set up to investigate the outcome in relation to age at initiation of cysteamine versus CTNS genotype, with attention to patients treated with cysteamine from neonatal age.

Nephropathic cystinosis in Poland: a 40-year retrospective study.

Introduction: Nephropathic cystinosis (NC) is a rare, autosomal recessive disorder leading to lysosomal accumulation of cystine. It is caused by mutations in the CTNS gene encoding a cystine cotransporter cystinosin. The infantile (INC) and juvenile (JNC) forms are distinguished.

Optimization of cerebrospinal fluid microbial DNA metagenomic sequencing diagnostics.

Infection in the central nervous system is a severe condition associated with high morbidity and mortality. Despite ample testing, the majority of encephalitis and meningitis cases remain undiagnosed. Metagenomic sequencing of cerebrospinal fluid has emerged as an unbiased approach to identify rare microbes and novel pathogens.

Hygric Properties of Machine-Made, Historic Clay Bricks from North-Eastern Poland (Former East Prussia): Characterization and Specification for Replacement Materials.

This paper deals with the hygric characterization of early 20th century machine-made clay bricks, representative of great number of historical buildings in north-eastern Poland. Heritage buildings have a high potential for adaptive reuse, which is strictly connected with an urge for knowledge about the properties of these existing building envelopes. To better understand the hygric behavior of historic buildings, various experimental laboratory tests, including density, water absorption, compressive strength and freeze-thaw resistance, were conducted.

Relationship between Gd-IgA1 and TNFR1 in IgA nephropathy and IgA vasculitis nephritis in children - multicenter study.

Aim Of The Study: To evaluate the relationship between serum Gd-IgA1 (sGd-IgA1) and serum and urine TNFR1 (sTNFR1, uTNFR1) levels as possible prognostic factors in IgA nephropathy (IgAN) and IgA vasculitis nephritis (IgAVN).

Material And Methods: From 299 patients from the Polish Registry of Pediatric IgAN and IgAVN, 60 children (24 IgAN and 36 IgAVN) were included in the study. The control group consisted of 20 healthy children.

The Role of Complement Component C3 Activation in the Clinical Presentation and Prognosis of IgA Nephropathy-A National Study in Children.

The aim of the study was to evaluate the influence of the intensity of mesangial C3 deposits in kidney biopsy and the serum C3 level on the clinical course and outcomes of IgAN in children. The study included 148 children from the Polish Pediatric IgAN Registry, diagnosed based on kidney biopsy. Proteinuria, creatinine, IgA, C3 were evaluated twice in the study group, at baseline and the end of follow-up.