The clinical phenotypes and therapeutic strategies for stiff skin syndrome: a case series with literature review.

Background: Stiff skin syndrome (SSS) is a rare, non-inflammatory skin disease with a pronounced restriction in joint mobility. In this study, we aim to report Chinese pediatric patients with SSS in our center and summarize the clinical features of the disease through literature review.

Results: A retrospective study was conducted on 16 pediatric patients diagnosed with SSS at Peking Union Medical College Hospital between January 2014 and January 2024, based on clinical manifestations, laboratory tests, and skin biopsy findings.

Application of the Scleroderma Clinical Trials Consortium Damage Index in patients with juvenile systemic sclerosis.

Background: Juvenile systemic sclerosis (jSSc) can lead to permanent and irreversible anatomical or physiological dysfunction. The Scleroderma Clinical Trials Consortium-Damage Index (SCTC-DI), which has been employed and validated in adult patients, can quantify organ damage and predict mortality and morbidity. However, its application in paediatric patients remains unexplored.

A novel STING1-activating mutation is identified in a patient with childhood-onset systemic lupus erythematosus.

Gain-of-function variants in stimulator of interferon genes (STING1) are known to cause STING-associated vasculopathy with onset in infancy (SAVI), a disorder characterized by cutaneous vasculopathy, interstitial lung disease (ILD), and systemic inflammation. Here, we report a novel STING1 N188H variant in a patient who met the classification criteria for systemic lupus erythematosus (SLE) but lacked typical SAVI features. In vitro assays demonstrated that the N188H variant drives constitutive STING activation and enhances type I interferon signaling.

Efficacy and safety of thalidomide in children with monogenic autoinflammatory diseases: a single-center, real-world-evidence study.

Background: Monogenic autoinflammatory diseases (AIDs) are rare inflammatory diseases caused by genetic variants. The pathogenesis is complex and treatment options are limited. This study aimed to describe the safety and efficacy of thalidomide in the treatment of monogenic AIDs.

Capsaicin Alleviates Vascular Endothelial Dysfunction and Cardiomyopathy via TRPV1/eNOS Pathway in Diabetic Rats.

Background: Endothelial dysfunction and cardiomyopathy are considered to be important vascular complications associated with diabetes. This study was designed to investigate whether capsaicin (CAP), a selective TRPV1 agonist, could prevent diabetes-induced endothelial dysfunction and cardiomyopathy.

Methods: Male Sprague Dawley rats aged 8 weeks were injected intraperitoneally with streptozotocin (STZ, 50 mg/kg) to establish the diabetes model.

Effects of Dexmedetomidine on Postoperative Nausea and Vomiting in Adult Patients Undergoing Ambulatory Thyroidectomy: A Randomized Clinical Trial.

Postoperative nausea and vomiting (PONV) is a common and disturbing problem in patients undergoing ambulatory thyroidectomy. This prospective trial aimed to explore whether dexmedetomidine (DEX) combined with azasetron (AZA) can further drop the incidence of PONV in patients undergoing ambulatory thyroidectomy compared with AZA. This single-center, randomized, double-blind trial involved 172 adult patients undergoing ambulatory thyroidectomy.

Local Anesthetic Lidocaine and Cancer: Insight Into Tumor Progression and Recurrence.

Cancer is a leading contributor to deaths worldwide. Surgery is the primary treatment for resectable cancers. Nonetheless, it also results in inflammatory response, angiogenesis, and stimulated metastasis.

Parthenolide ameliorates tweak-induced podocytes injury.

Parthenolide (PTL) is a natural product from the shoots of Tanacetum parthenium, which has immunomodulatory effects in multiply type of diseases. This study aimed to explore the effect and the underlying mechanism of PTL on the anti-apoptotic and anti- inflammatory ability of tweak-induced podocytes. Conditionally immortalized mouse podocytes were incubated with Tumor necrosis factor-like weak inducer of apoptosis (Tweak, 100 ng/ml), PTL(10 µM) or Tweak + PTL for 12 h, 24 and 48 h, respectively.

[Application of iodine contrast agent optimization protocol with fixed injection time in triple-rule-out CT examination of chest pain].

Objective: To investigate the feasibility of using optimized protocol of iodine contrast agent with fixed injection time in triple-rule-out CT examination of acute chest pain patients.

Methods: A prospective study was conducted. The patients who underwent triple-rule-out CT examination of acute chest pain at the Second Hospital of Shanxi Medical University from September 2017 to June 2018 were enrolled.

Blocking P2X7 receptor ameliorates oxidized LDL-mediated podocyte apoptosis.

The purpose of our research is to elucidate whether oxLDL activates P2X7R in cultured human podocytes and if the activation of P2X7R leads to podocyte apoptosis. Additionally, we explore the underlying mechanism involved in podocyte apoptosis. Immortalized human podocytes were incubated with oxLDL (80 µg/ml), P2X7R antagonist A438079 (10 µM), or the compound of A438079 and oxLDL for 48 h, respectively.

Bioactivation and Regioselectivity of Pig Cytochrome P450 3A29 towards Aflatoxin B₁.

Due to unavoidable contaminations in feedstuff, pigs are easily exposed to aflatoxin B₁ (AFB₁) and suffer from poisoning, thus the poisoned products potentially affect human health. Heretofore, the metabolic process of AFB₁ in pigs remains to be clarified, especially the principal cytochrome P450 oxidases responsible for its activation. In this study, we cloned CYP3A29 from pig liver and expressed it in Escherichia coli, and its activity has been confirmed with the typical P450 CO-reduced spectral characteristic and nifedipine-oxidizing activity.

Trp266 determines the binding specificity of a porcine aflatoxin B₁ aldehyde reductase for aflatoxin B₁-dialdehyde.

Aflatoxin B₁ (AFB₁) is a severe threat to human and animal health. The aflatoxin B₁ aldehyde reductase (AFAR) family specifically catalyzes AFB₁-dialdehyde, a toxic metabolic intermediate of AFB₁, producing a nontoxic dialcohol. Although several AFARs have been found and characterized, the binding specificity of the family for AFB₁-dialdehyde remains unclear.

An efficient PIFA-mediated synthesis of a directly linked zinc chlorin dimer via regioselective oxidative coupling.

The synthesis of a directly linked zinc chlorin dimer was first achieved by a facile and efficient oxidative coupling of zinc chlorin monomers with phenyliodine bis(trifluoroacetate) (PIFA). The reaction shows high regioselectivity at the 20-position near the hydrogenated pyrrole ring producing selective dichlorin in 74% yield.

Carbonyl reduction of mequindox by chicken and porcine cytosol and cloned carbonyl reductase 1.

Mequindox (MEQ) is a novel synthetic quinoxaline 1,4-dioxides derivative, which is widely used as a veterinary drug and animal feed additive. However, the metabolic mechanism of MEQ is rarely reported. The N-oxide reduction mechanism of MEQ was reported in our previous work.

Mequindox induced cellular DNA damage via generation of reactive oxygen species.

Mequindox, a quinoxaline-N-dioxide derivative that possesses antibacterial properties, has been widely used as a feed additive in the stockbreeding industry in China. While recent pharmacological studies have uncovered potential hazardous effects of mequindox, exactly how mequindox induces pathological changes and the cellular responses associated with its consumption remain largely unexplored. In this study, we investigated the cellular responses associated with mequindox treatment.

An efficient PIFA-mediated synthesis of fused diporphyrin and triply-singly interlacedly linked porphyrin array.

An efficient and metal-free oxidative method was reported for synthesis of triply linked diporphyrins with 2.5 equiv of phenyliodine bis(trifluoroacetate) (PIFA). This reaction showed high selectivity for Zn(II) porphyrins and had been successfully applied in the synthesis of a novel triply-singly interlacedly linked porphyrin array with lower energy gap.