Long-term safety and influence on growth in patients receiving sirolimus: a pooled analysis.

Background: Sirolimus is increasingly utilized in treating diseases associated with mTOR pathway overactivation. Despite its potential, the lack of evidence regarding its long-term safety across all age groups, particularly in pediatric patients, has limited its further application. This study aims to assess the long-term safety of sirolimus, with a specific focus on its impact on growth patterns in pediatric patients.

Novel pH-sensitive gellan gum-ε-polylysine hydrogel microspheres for sulforaphene delivery.

Background: This study aimed to improve the stability and utilization of sulforaphene (SFE) and to enhance the intestinal stability and pH-sensitive release of SFE in the gastrointestinal tract. To achieve this objective, calcium chloride (CaCl) was used as a crosslinking agent to fabricate novel SFE-loaded gellan gum (GG)-ε-polylysine (ε-PL) pH-sensitive hydrogel microspheres by using the ionic crosslinking technique.

Results: The molecular docking results of GG, ε-PL, and SFE were good and occurred in the natural state.

Reduced myelin content in bipolar disorder: A study of inhomogeneous magnetization transfer.

Background: Previous neuroimaging and pathological studies have found myelin-related abnormalities in bipolar disorder (BD), which prompted the use of magnetic resonance (MR) imaging technology sensitive to neuropathological changes to explore its neuropathological basis. We holistically investigated alterations in myelin within BD patients by inhomogeneous magnetization transfer (ihMT), which is sensitive and specific to myelin content.

Methods: Thirty-one BD and 42 healthy controls (HC) were involved.

Decreased tubulin-binding cofactor B was involved in the formation disorder of nascent astrocyte processes by regulating microtubule plus-end growth through binding with end-binding proteins 1 and 3 after chronic alcohol exposure.

Fetal alcohol syndrome (FAS) is a neurological disease caused by excessive drinking during pregnancy and characterized by congenital abnormalities in the structure and function of the fetal brain. This study was proposed to provide new insights into the pathogenesis of FAS by revealing the possible mechanisms of alcohol-induced astrocyte injury. First, a chronic alcohol exposure model of astrocytes was established, and the formation disorder was found in astrocyte processes where tubulin-binding cofactor B (TBCB) was decreased or lost, accompanied by disorganized microtubules (MT).

Preliminary Feasibility Study on Diffusion Kurtosis Imaging to Monitor the Early Functional Alterations of Kidneys in Streptozocin-Induced Diabetic Rats.

Rationale And Objectives: The aim of this study was to investigate the potential of diffusion kurtosis imaging (DKI) to assess the early renal functional undulation of diabetic mellitus (DM).

Materials And Methods: Fifty-seven Sprague-Dawley (SD) rats were randomly divided into two groups and eventually 48 rats were included in this study: the normal control (CON) group and diabetic mellitus (DM) group. Weeks 0, 4, 8, and 12 after the diabetes model was successfully established, all the rats were scanned on the 3.

Imaging characteristics of ovarian sclerosing stromal tumor.

Objectives: This study was designed to evaluate the specific imaging features of ovarian sclerosing stromal tumor (SST), improve its accuracy as well as the specificity of imaging diagnosing, and prevent overestimation of malignancy to reduce unnecessary surgical procedures.

Methods: Eight patients with magnetic resonance imaging (MRI) and six with computed tomography (CT) images were analyzed in this retrospective observational study. All the cases were confirmed by postoperative pathological examination as those of ovarian SST.

ERK1/2 Signalling Pathway Regulates Tubulin-Binding Cofactor B Expression and Affects Astrocyte Process Formation after Acute Foetal Alcohol Exposure.

Foetal alcohol spectrum disorders (FASDs) are a spectrum of neurological disorders whose neurological symptoms, besides the neuronal damage caused by alcohol, may also be associated with neuroglial damage. Tubulin-binding cofactor B (TBCB) may be involved in the pathogenesis of FASD. To understand the mechanism and provide new insights into the pathogenesis of FASD, acute foetal alcohol exposure model on astrocytes was established and the interference experiments were carried out.

Minimal gene set discovery in single-cell mRNA-seq datasets with ActiveSVM.

Sequencing costs currently prohibit the application of single-cell mRNA-seq to many biological and clinical analyses. Targeted single-cell mRNA-sequencing reduces sequencing costs by profiling reduced gene sets that capture biological information with a minimal number of genes. Here we introduce an active learning method that identifies minimal but highly informative gene sets that enable the identification of cell types, physiological states and genetic perturbations in single-cell data using a small number of genes.

Non-invasive investigation of early kidney damage in streptozotocin-induced diabetic rats by intravoxel incoherent motion diffusion-weighted (IVIM) MRI.

Background: The current study investigated the performance of intravoxel incoherent motion diffusion (IVIM) technology in monitoring early renal injury in streptozotocin rats.

Methods: Forty-eight Sprague-Dawley (SD) rats were divided into a control group and a diabetic mellitus (DM) group. Six rats in each group were randomly selected for MR scans at four different time points (0, 4, 8, and 12 weeks).

Effects of resveratrol on lipid and protein co-oxidation in fish oil-enriched whey protein isolate emulsions.

In this study, the impact of resveratrol (RES) on co-oxidation of lipid and protein in a fish oil-fortified whey protein isolate (WPI) emulsion was investigated. Oil-in-water (O/W) emulsions containing 1% fish oil, 6 mg/mL of WPI and RES (0.08 ~ 2 mM) were oxidatively stressed using a Fenton system at 25 °C for 24 h.

Sirolimus Can Increase the Disappearance Rate of Cardiac Rhabdomyomas Associated with Tuberous Sclerosis: A Prospective Cohort and Self-Controlled Case Series Study.

Objectives: To investigate the efficacy and safety of sirolimus in the treatment of cardiac rhabdomyomas associated with tuberous sclerosis complex and the specific benefits in different subgroups.

Study Design: The study was a prospective cohort and self-controlled case series study. Based on the prevalence of cardiac rhabdomyoma at different ages, we estimated the natural tumor disappearance rate.

Transaminitis in a Three-year-old Boy with Duchenne Muscular Dystrophy.

Duchenne muscular dystrophy (DMD) is a fatal X-linked genetic disease of the neuromuscular system and is the most serious type of muscular dystrophy in humans. The disease is characterized by progressive muscular atrophy and a poor prognosis. The incidence rate is 1/3500, and symptoms appear at age of 5 years-old.

Sirolimus improves seizure control in pediatric patients with tuberous sclerosis: A prospective cohort study.

Purpose: This study aimed to analyze the therapeutic effect of sirolimus on seizures in pediatric patients with tuberous sclerosis.

Methods: We first compared the efficacy of controlling seizures in all patients after they had taken sirolimus for one year, and then we performed a subgroup analysis based on whether the administered antiepileptic drugs were changed to determine whether the efficacy was associated with changes of antiepileptic drugs.

Results: A total of 91 eligible children were enrolled.

Influence of malondialdehyde-induced modifications on physicochemical and digestibility characteristics of whey protein isolate.

Impacts of lipid oxidation product malondialdehyde (MDA) on the properties of whey protein isolate (WPI) were investigated in this study. The incorporation of MDA into WPI promoted the formation of protein carbonyls, with the significant loss of protein sulfhydryls, impaired intrinsic fluorescence, and increased protein surface hydrophobicity. The visualized band profiles revealed by gel electrophoresis and immunoblotting suggested that WPI's main components β-lactoglobulin and α-lactalbumin were the targets of MDA, and the derivatives of MDA were involved in protein cross-linking and aggregation at higher molecular weights.

Coordinated DNA Methylation and Gene Expression Data for Identification of the Critical Genes Associated with Childhood Atopic Asthma.

Asthma is a chronic inflammatory disorder of airways that involves in many cells and factors. This study aimed to screen critical genes and miRNAs involved in childhood atopic asthma. DNA methylation and gene expression data (access numbers GSE65163 and GSE65204) were downloaded from Gene Expression Omnibus (GEO) database, which included 72 atopic asthmatic subject samples and 69 healthy samples.

RNF138 confers cisplatin resistance in gastric cancer cells via activating Chk1 signaling pathway.

Chemotherapy resistance represents a major issue associated with gastric cancer (GC) treatment, and arises through multiple mechanisms, including modulation of the cell-cycle check point. Several ubiquitin kinases, including RING finger protein 138 (RNF138), have been reported to mediate the G2/M phase arrest. In this study, we investigated the role of RNF138 in the development of cisplatin resistance of two GC cell lines.

Alterations of White Matter Connectivity in Preschool Children with Autism Spectrum Disorder.

Purpose To investigate the topologic architecture of white matter connectivity networks in preschool-aged children with a diagnosis of autism spectrum disorder (ASD) versus typical development (TD). Materials and Methods Forty-two participants were enrolled, including 21 preschool children with ASD (14 male children and seven female children; mean age, 4.56 years ± 0.

Intravoxel incoherent motion (IVIM) at 3.0 T: evaluation of early renal function changes in type 2 diabetic patients.

Purpose: The purpose of this study was to evaluate the utility of intravoxel incoherent motion diffusion-weighted imaging (IVIM DWI) parameters in identifying early renal function changes in diabetics.

Methods: A total of 40 patients with type 2 diabetes mellitus and 20 healthy control subjects underwent multiple b value DWI. The diabetic patients were stratified into two groups based on albuminuria category: NAU (normal to mildly increased albuminuria; ACR < 30 mg/g) and MAU (moderately increased albuminuria; 30 ≤ ACR < 300 mg/g).

Two de novo variations identified by massively parallel sequencing in 13 Chinese families with children diagnosed with autism spectrum disorder.

Autism spectrum disorder (ASD) is a genetically heterogeneous neurodevelopmental disorder characterized by impairments in social interaction and communication, and by restricted and repetitive behaviors. The genetic architecture of ASD has been elucidated, including chromosomal rearrangements, de novo or inherited rare variants, and copy number variants. However, the genetic mechanism of Chinese families with ASD children is explored rarely.

Improved Li-storage performance with PEDOT-decorated MnO nanoboxes.

In this paper, MnO nanoboxes coated with poly(3,4-ethylenedioxythiophene) film (denoted as MnO@PEDOT) are investigated as an anode material in lithium-ion batteries. The MnO nanoboxes are developed through the surface chemical oxidation decomposition of MnCO cubes and the subsequent removal of their remaining cores. PEDOT is coated on the surface of MnO nanoboxes via in situ polymerization of 3,4-ethylenedioxythiophene.

Role of quantitative intravoxel incoherent motion parameters in the preoperative diagnosis of nodal metastasis in patients with rectal carcinoma.

Purpose: To evaluate the diagnostic value of intravoxel incoherent motion imaging (IVIM) in differentiating metastatic and nonmetastatic lymph nodes in patients with rectal carcinoma.

Materials And Methods: In all, 68 patients with histologically proven rectal carcinoma underwent an IVIM sequence (b = 0, 25, 50, 75, 100, 150, 200, 400, 600, 800, 1000, 1200, 1500, and 2000 s/mm(2) ) on a 3.0T MRI scanner.

Syndrome of Electrical Status Epilepticus During Sleep: Epileptic Encephalopathy Related to Brain Development.

Background: Epileptic encephalopathy with electrical status epilepticus during sleep is an age-related and self-limited disorder. The present study analyzed the etiology, demographics, and pathogenesis of patients with electrical status epilepticus during sleep to provide information on the diagnosis and therapy of this syndrome.

Methods: The etiologies of epileptic encephalopathy with electrical status epilepticus during sleep in patients admitted in Chinese People's Liberation Army General Hospital from 2009 to 2014 were retrospectively analyzed.

[Efficacy and safety of rapamycin in treatment of children with epilepsy complicated with tuberous sclerosis].

Objective: To evaluate the therapeutic effect and safety of rapamycin in treatment of children with tuberous sclerosis complex (TSC) complicated with epilepsy.

Method: This was an open-label, prospective, self-controlled study. From Sep.

[A family with two children diagnosed with aspartylglucosaminuria-case report and literature review].

Objective: The authors sought to investigate the clinical features and characteristics of genetic mutation in patients with aspartylglucosaminuria.

Method: Clinical data of two pediatric siblings in a family were analyzed retrospectively and relative literature was reviewed in order to study the clinical features, imaging and enzymatic characteristics and genetic mutations.

Result: Case 1, the proband, male, he was hospitalized at 20 months of age because of fever and hepatosplenomegaly for nine days.