Clinical phenotype and laboratory characteristics of 93 patients with congenital fibrinogen disorders from unrelated 36 families.

Background: Congenital fibrinogen disorders (CFDs) are rare bleeding disorders (RBDs) caused by mutations in 1 of the 3 fibrinogen genes (, , and ).

Objectives: To investigate the clinical phenotype, laboratory features, diagnosis, treatment, and prognosis of CFDs.

Methods: Clinical data of 93 subjects with CFDs identified from June 2018 to December 2023 were retrospectively analyzed.

Synergism of rMV-Hu191 with cisplatin to treat gastric cancer by acid sphingomyelinase-mediated apoptosis requiring integrity of lipid raft microdomains.

Background: DDP-based chemotherapy is one of the first-line treatment in GC. However, the therapeutic efficacy of DDP is limited due to side effects. Therefore, it is of great significance to develop novel adjuvants to synergize with DDP.

[Clinical effect of "head-nine-needle" therapy on tension headache].

Objective: To observe the effect of the "head-nine-needle" therapy on tension headache.

Methods: A total of 150 patients with tension headache were divided into a head-nine-needle therapy group, a western medicine control group and an acupuncture control group according to the random number table, 50 cases in each one. In the head-nine-needle therapy group, the head-nine-needle therapy was adopted.

Construction of Multiple-Substituted Chiral Cyclohexanes through Hydrogenative Desymmetrization of 2,2,5-Trisubstituted 1,3-Cyclohexanediones.

The construction of chiral multiple-substituted cyclohexanes motifs is a challenging topic in organic synthesis. By the combination of desymmetrization and remote stereocontrol, a ruthenium-catalyzed transfer hydrogenative desymmetrization of 2,2,5-trisubstituted 1,3-cyclohexanediones has been successfully developed for the construction of chiral multiple-substituted cyclohexanes with high enantioselectivity and diastereoselectivity. When an ester group was introduced to the two-position, a hydrogenative desymmetrization/transesterification cascade occurred, affording the bicyclic lactones bearing three stereocenters, including two discrete stereocenters and one quaternary stereogenic center, with high enantioselectivity.

A recombinant measles virus vaccine strain rMV-Hu191 has oncolytic effect against human gastric cancer by inducing apoptotic cell death requiring integrity of lipid raft microdomains.

Live-attenuated strain of measles virus (MV) has oncolytic effect. In this study, the antitumor effect of rMV-Hu191, a recombinant Chinese Hu191 MV generated in our laboratory by efficient reverse genetics system, was evaluated in gastric cancer (GC). From our data, rMV-Hu191 induced cytopathic effects and inhibited tumor proliferation both in vitro and in vivo by inducing caspase-dependent apoptosis.

Preparation of Axially Chiral 2,2'-Biimidazole Ligands through Remote Chirality Delivery and Their Application in Asymmetric Carbene Insertion into N-H of Carbazoles.

Axially chiral biimidazole ligands have been rarely synthesized and studied, in contrast to the significant achievements in the synthesis and application of central chiral imidazole ligands. Herein, a series of novel axially chiral 2,2'-biimidazole ligands were synthesized from the reaction of 2,2'-bis(bromomethyl)-1,1'-binaphthalene and 2,2'-biimidazole in one step through the strategy of remote chirality delivery. These ligands have been proven to be efficient for Cu- or Fe-catalyzed asymmetric insertion of α-aryl-α-diazoacetates into the N-H bond of carbazoles with up to 96% ee.

Ruthenium-Catalyzed Hydrogenation of Carbocyclic Aromatic Amines: Access to Chiral Exocyclic Amines.

The first highly enantioselective hydrogenation of carbocyclic aromatic amines has been successfully realized using in situ-generated chiral ruthenium complex as catalyst, affording facile access to chiral exocyclic amines with up to 98% ee.

Evaluation of a real-time method of simultaneous amplification and testing in diagnosis of Mycoplasma pneumoniae infection in children with pneumonia.

Mycoplasma pneumoniae (M. pneumoniae) infection can cause community acquired pneumonia in children. A real-time method of simultaneous amplification and testing of M.

A Hydrogenation/Oxidative Fragmentation Cascade for Synthesis of Chiral 4,5-Dihydro-1H-benzo[d]azepin-1-ones.

An iridium-catalyzed asymmetric hydrogenation/oxidative fragmentation of 6-substituted 5H-benzo[d] benzofuro[3,2-b]azepines has been developed, providing an efficient access to optically active 4-substituted 4,5-dihydro-1H-benzo[d]azepin-1-ones with up to 91% ee. A possible reaction pathway includes the asymmetric hydrogenation to furnish chiral cyclic amines and oxidative fragmentation under an air atmosphere.

Haze is an important medium for the spread of rotavirus.

This study investigated whether the rotavirus infection rate in children is associated with temperature and air pollutants in Hangzhou, China. This study applied a distributed lag non-linear model (DLNM) to assess the effects of daily meteorological data and air pollutants on the rotavirus positive rate among outpatient children. There was a negative correlation between temperature and the rotavirus infection rate.

Clinical Value of Assessing Cytokine Levels for the Differential Diagnosis of Bacterial Meningitis in a Pediatric Population.

We performed a prospective observational study to evaluate the utility of measuring inflammatory cytokine levels to discriminate bacterial meningitis from similar common pediatric diseases. Inflammatory cytokine levels and other cerebrospinal fluid (CSF) physicochemical indicators were evaluated in 140 patients who were diagnosed with bacterial meningitis via microbiological culture or PCR assay. The CSF concentrations of interleukin (IL)-6 and IL-10, CSF/blood IL-6 and IL-10 ratios, CSF white blood cell count, and CSF micro total protein were significantly elevated in bacterial meningitis patients compared with healthy children or patients with viral encephalitis, epilepsy, or febrile convulsions (P < 0.

24h Urinary Protein Levels and Urine Protein/Creatinine Ratios Could Probably Forecast the Pathological Classification of HSPN.

This study aimed to assess the relevance of laboratory tests in Henoch-Schönlein purpura nephritis (HSPN) classification, and determine accurate classification factors. This prospective study included 694 HSPN patients who underwent ultrasound-guided percutaneous renal biopsy (PRB). Renal specimens were scored according to International Study of Kidney Disease in Children (ISKDC) classification.

Dual Inhibition of Cdc7 and Cdk9 by PHA-767491 Suppresses Hepatocarcinoma Synergistically with 5-Fluorouracil.

Activation of checkpoint kinase 1 (Chk1) is essential in chemoresistance of hepatocarcinoma (HCC) to 5-fluorouracil (5-FU) and other antimetabolite family of drugs. In this study, we demonstrated that PHA-767491, a dual inhibitor of two cell cycle checkpoint kinases, cell division cycle kinase 7 (Cdc7) and cyclin-dependent kinase 9 (Cdk9), has synergistic antitumor effect with 5-FU to suppress human HCC cells both in vitro and in vivo. Compared with the sole use of each agent, PHA-767491 in combination with 5-FU exhibited much stronger cytotoxicity and induced significant apoptosis manifested by remarkably increased caspase 3 activation and poly(ADP-Ribose) polymerase fragmentation in HCC cells.

Isolation of autophagosome subpopulations after induction of autophagy by calcium.

Autophagy is a dynamic process accomplished by the generation and maturation of autophagosomes. Isolation of autophagosomes and subsequent compositional analysis can provide information about their biogenesis mechanism. In this article, HEK293 cells expressing GFP-LC3 were treated by calcium phosphate precipitates (CPP) to induce autophagy.

Relationship between immune parameters and organ involvement in children with Henoch-Schonlein purpura.

Henoch-Schonlein purpura (HSP) is the most common type of connective tissue diseases which increasingly occurs in children in recent years and its pathogenesis remains unclear. In order to explore the immune parameters and underlying pathogenesis mechanism of children with HSP, the study involved 1232 patients with HSP having different clinical symptoms and their laboratory indicators were evaluated. Th1/Th2 imbalance and overactivity of Th2 cells can cause increase in the synthesis and release of immunoglobulins in children with HSP.

Absolute lymphocyte count is associated with minimal residual disease level in childhood B-cell precursor acute lymphoblastic leukemia.

The prognostic value of absolute lymphocyte count (ALC) has been a recent matter of debate in childhood acute lymphoblastic leukemia (ALL). In the current study, ALCs at the time of diagnosis (ALC-0), after 7 days of initial therapy (ALC-8) and at interim of the induction therapy (ALC-22) were examined in Chinese children with B-cell precursor (BCP) ALL and correlated with the level of minimal residual disease (MRD) at day 22 of induction therapy. Medical and laboratory records of 140 patients diagnosed with childhood BCP ALL were retrieved and analyzed.

Prognostic significance of flow cytometric minimal residual disease assessment after the first induction course in Chinese childhood acute myeloid leukemia.

Flow cytometry based minimal residual disease (MRD) was evaluated for outcome prediction in childhood acute myeloid leukemia (AML). The median levels of MRD in relapsed and nonrelapsed patients were different after the first induction (0.64% vs.

[Targeted killing of the Nalm-6 cells with 2E8-Genistein immunotoxin and its mechanism].

Objective: Leukemia is the most common hematopoietic malignancies in children. Chemotherapy is currently the primary modality of treatment for this fatal disease. Although chemotherapy is very effective in terms of cell killing, severe side effects such as severe infections, intracranial hemorrhage etc.