CEEMDAN combined wavelet denoising improved the MW cycle slip detection algorithm.

In the preprocessing of high-precision navigation and positioning data, the most widely used MW combination cycle slip detection method is greatly affected by pseudorange noise. It has issues such as missing small cycle slips and failing to promptly reset the recursive averaging process after cycle slip detection failure, which leads to subsequent threshold divergence. This paper proposes an improved MW combination cycle slip detection method based on Complete Ensemble Empirical Mode Decomposition (CEEMDAN), permutation entropy, and wavelet denoising, which uses CEEMDAN to decompose the cycle slip signal into a series of intrinsic modal functions (IMFs) and then selects the IMFs that require denoising through the permutation entropy algorithm, and the wavelet denoising technique is combined to eliminate the residual noise further, so that the noise can be removed more accurately.

Clinical and Genetic Spectrum of Nine Cases of NLRP3-Associated Autoinflammatory Disease (NLRP3-AID) and Identification of One Novel NLRP3 Mutation by Genetic Variation Analyses.

Purpose: NLRP3-associated autoinflammatory disease (NLRP3-AID) is characterized by gain-of-function variants in the NLRP3 gene. Since there are little literature focusing on pediatric NLRP3-AID in China, we aimed to elucidate the phenotypic and genotypic profiles of Chinese patients with NLRP3-AID.

Methods: Patients with NLRP3-AID at three rheumatology centers in China were genotyped through whole exome sequencing or gene panel sequencing.

Reduction of renal interstitial fibrosis by targeting Tie2 in vascular endothelial cells.

Background: Tie2, a functional angiopoietin receptor, is expressed in vascular endothelial cells and plays an important role in angiogenesis and vascular stability. This study aimed to evaluate the effects of an agonistic Tie2 signal on renal interstitial fibrosis (RIF) and elucidate the underlying mechanisms.

Methods: We established an in vivo mouse model of folic acid-induced nephropathy (FAN) and an in vitro model of lipopolysaccharide-stimulated endothelial cell injury, then an agonistic Tie2 monoclonal antibody (Tie2 mAb) was used to intervent these processes.

Two cases of successful sirolimus treatment for patients with activated phosphoinositide 3-kinase δ syndrome 1.

Background: Activated phosphoinositide3-kinase (PI3K) δ syndrome 1 (APDS1) is a novel inborn errors of immunity (IEIs) caused by heterozygous gain of function mutations in PI3Kδ catalytic p110δ (PIK3CD). APDS1 has a spectrum of clinical manifestations. Recurrent respiratory infections, lymphoproliferation, hepatosplenomegaly, hyper-IgM syndrome and autoimmunity are the common symptoms of this disease.

Sintilimab treatment for chronic active Epstein-Barr virus infection and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children.

Background: Chronic active Epstein-Barr virus infection (CAEBV) and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) are rare but life-threatening progressive diseases triggered by EBV infection. Glucocorticoid/immunosuppressants treatment is temporarily effective; however, most patients relapse and/or progress. Hematopoietic stem cell transplantation (HSCT) is a potentially curative therapy; however, there are risks of transplantation-associated complications.

Clinical features and management of atypical hemolytic uremic syndrome patient with gene variants: a case report.

Background: Atypical hemolytic uremic syndrome (aHUS) with diacylglycerol kinase epsilon () gene variant is a rare variant of thrombotic microangiopathy (TMA). The information on the clinical features, management and long-term outcomes of -aHUS patients have not yet been fully elucidated. The aim of this study was to report a novel variant of the gene in a Chinese population with aHUS.

Multi-orbit lunar GNSS constellation design with distant retrograde orbit and Halo orbit combination.

The Moon is the closest natural satellite to mankind, with valuable resources on it, and is an important base station for mankind to enter deep space. How to establish a reasonable lunar Global Navigation Satellite System (GNSS) to provide real-time positioning, navigation, and timing (PNT) services for Moon exploration and development has become a hot topic for many international scholars. Based on the special spatial configuration characteristics of Libration point orbits (LPOs), the coverage capability of Halo orbits and Distant Retrograde Orbit (DRO) in LPOs is discussed and analyzed in detail.

The role of PD-1 signaling in health and immune-related diseases.

Programmed cell death 1 receptor (PD-1) and its ligands constitute an inhibitory pathway to mediate the mechanism of immune tolerance and provide immune homeostasis. Significantly, the binding partners of PD-1 and its associated ligands are diverse, which facilitates immunosuppression in cooperation with other immune checkpoint proteins. Accumulating evidence has demonstrated the important immunosuppressive role of the PD-1 axis in the tumor microenvironment and in autoimmune diseases.

Epstein-Barr virus-related hemophagocytic lymphohistiocytosis complicated with coronary artery dilation and acute renal injury in a boy with a novel X-linked inhibitor of apoptosis protein (XIAP) variant: a case report.

Background: X-linked lymphoproliferative disease (XLP) is a rare inherited X-linked primary immunodeficiency diseases (PID). One such disease, X-linked inhibitor of apoptosis protein (XIAP) deficiency, is characterized by Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH). However, EBV-HLH with coronary artery dilation and acute renal injury (AKI) in children is unusual.

Proteinuria as a presenting sign of combined methylmalonic acidemia and homocysteinemia: case report.

Background: Disorders of the metabolism and absorption of vitamin B12 can lead to decrease in activity of methionine synthetase and methylmalonate coenzyme A mutase (MMUT), which results in increased levels of methylmalonic acid and homocysteine in blood and urine. Often, combined methylmalonic acidemia (MMA) and homocysteinemia is misdiagnosed due to a lack of specific symptoms. The clinical manifestations are diverse, but proteinuria as the initial presentation is rare.

Acute pancreatitis and macrophage activation syndrome in pediatric systemic lupus erythematosus: case-based review.

Pancreatitis is uncommon in systemic lupus erythematosus (SLE) and is rarely reported in children, possibly being related to macrophage activation syndrome (MAS). The incidence of MAS in children with lupus pancreatitis is unknown, as is their prognosis. In this case-based review, we report a pediatric patient with SLE complicated with pancreatitis and MAS, and performed a literature review.

Analysis of breast cancer subtypes by AP-ISA biclustering.

Background: Gene expression profiling has led to the definition of breast cancer molecular subtypes: Basal-like, HER2-enriched, LuminalA, LuminalB and Normal-like. Different subtypes exhibit diverse responses to treatment. In the past years, several traditional clustering algorithms have been applied to analyze gene expression profiling.