中华内科杂志最新文献

Wake-promoting agents mainly excite the cerebral cortex and promote wakefulness. They are clinically used to treat excessive daytime sleepiness (EDS) caused by narcolepsy and other diseases, or to maintain alertness during special tasks. In recent years, a variety of wake-promoting agents have been approved for the treatment of EDS related to narcolepsy, obstructive sleep apnea and other diseases at home and abroad. However, a dedicated expert consensus on the clinical application of wake-promoting agents remains lacking. To facilitate the rational and standardized clinical application of wake-promoting agents in China, the Sleep Disorders Committee of the Chinese Sleep Research Society convened a multidisciplinary panel of domestic experts. This assembly developed this consensus based on the latest clinical evidence sourced from both domestic and international research, as well as relevant guidelines and their collective clinical expertise. The consensus comprehensively reviews the mechanisms of action, approved indications, clinical studies, dosages, and adverse reactions of various wake-promoting agents. It also provides recommendations on drug selection, titration, and the use of wake-promoting agents in special populations for excessive daytime sleepiness caused by various etiologies. It has strong clinical guidance and clinical application value.

Becker muscular dystrophy (BMD) is an X-linked recessive myopathy caused by pathogenic variants of the dystrophin-encoding DMD gene. The clinical presentation of BMD encompasses four clinical subtypes: limb-girdle muscle weakness, quadriceps myopathy, isolated cramp-pain syndrome, and asymptomatic hyper-creatine kinase-emia. Patients may also present with cardiopulmonary involvement, neuropsychological issues, joint contractures, and spinal deformities. Diagnosis primarily relies on genetic testing and/or muscle biopsies. The treatment and management of BMD requires comprehensive and multidisciplinary interventions involving specialists in neurology, cardiology, respiratory medicine, rehabilitation medicine, orthopedics, gastroenterology, anesthesiology, clinical nutrition, psychology, and medical genetics. This coordinated approach aims to preserve patients' motor function, bone/joint function, cardiopulmonary function, and digestive function, while maintaining a good nutritional status and psychological well-being. To standardize the diagnosis, treatment, and management of BMD in China, a joint committee comprising multidisciplinary experts from the Chinese Medical Association Rare Disease Branch and the Beijing Medical Association Rare Disease Branch was established. This committee formulated the Chinese expert consensus on the diagnosis and treatment of BMD, with the primary objectives of improving patient quality of life and reducing the disease burden.

Objective: To evaluate the diagnostic utility of confirmatory tests [captopril challenge test (CTT) and seated saline infusion test (SIT)] for primary aldosteronism (PA) using liquid chromatography-tandem mass spectrometry (LC-MS/MS) and establish optimized diagnostic cutoffs. Methods: This prospective multicenter diagnostic trial enrolled patients at high risk for PA from seven tertiary clinical centers in China between April 2023 and December 2024. All participants underwent aldosterone-to-renin ratio (ARR) screening, the CCT, and the seated SIT. Plasma aldosterone concentrations (PACs) and plasma renin levels were measured via LC-MS/MS and chemiluminescence immunoassay (CLIA). PA diagnosis was defined as a CLIA-based post-SIT PAC ≥8.5 ng/dl (235.8 pmol/L) combined with an upright plasma renin concentration of <15 mU/L. Receiver operating characteristic (ROC) curves based on LC-MS/MS data from the CCT and the SIT were plotted to evaluate diagnostic performance. The DeLong test was employed for area under the ROC curve (AUC) comparisons among subgroups. Optimal diagnostic cutoffs for PA were determined based on sensitivity and specificity. Results: The study included 117 patients diagnosed with PA [age: (48±10) years; 58 (49.57%) males] and 354 patients diagnosed with essential hypertension [age: (45±12) years; 207 (58.47%) males]. Two hours post-CCT using mass spectrometry, the AUCs for the ARR and PAC were 0.858 and 0.837, respectively, both superior to PAC suppression percentage (AUC=0.718, both P<0.001). Additionally, the diagnostic efficacy of ARR was significantly better than that of plasma renin activity (PRA; AUC=0.784, P<0.001). At a diagnostic cutoff of PAC ≥5.7 ng/dl (158.1 pmol/L) or ARR ≥9.3 (ng/dl)/(ng·ml⁻¹·h⁻¹), sensitivity was 61% and specificity was 85%. For the seated SIT using mass spectrometry, the AUCs for the ARR and PAC were 0.828 and 0.852, respectively, significantly better than PRA (AUC=0.680, both P<0.001). At a PAC cutoff of ≥5.0 ng/dl (138.7 pmol/L), sensitivity was 68% and specificity was 85%, while an ARR cutoff of ≥ 19.0 (ng/dl)/(ng·ml⁻¹·h⁻¹) yielded a sensitivity of 64% and a specificity of 85%. Conclusions: Both the CCT and the SIT, based on LC-MS/MS, demonstrated excellent diagnostic performance. The recommended optimal cutoff for the ARR 2 hours post-CCT was 9.3 (ng/dl)/(ng·ml⁻¹·h⁻¹), while that for the PAC was 5.7 ng/dl. For the seated SIT, the optimal ARR cutoff was 19.0 (ng/dl)/(ng·ml⁻¹·h⁻¹) and the optimal PAC cutoff was 5.0 ng/dl.

Objective: To investigate the prognostic utility of the endothelial activation and stress index (EASIX) combined with the serum albumin concentration in patients with hematologic neoplasms complicated by bloodstream infection (BSI). Methods: This single-center retrospective cohort study analyzed the clinical data of patients with hematologic neoplasms and comorbid BSI admitted to the Department of Hematology of the Affiliated Hospital of Xuzhou Medical University between January 2020 and June 2023. Patients were categorized into survivor and non-survivor groups based on 30-day mortality post-BSI diagnosis. Univariate analysis was used to compare clinical indicators between groups, while multivariate logistic regression was employed to identify independent prognostic factors. The predictive efficacy of the EASIX and serum albumin was evaluated using receiver operating characteristic (ROC) curve analysis. Results: A total of 221 BSI patients were included [113 males (51.1%), 108 females (48.9%)], with a median age of 54 (40, 64) years. The survivor group comprised 158 patients (71.5%) and the non-survivor group 63 patients (28.5%). Univariate analysis revealed that the non-survivor group had significantly higher EASIX scores [3.92 (2.87, 6.05)] and lactate dehydrogenase levels [341 (222, 918) U/L] than the survivor group [2.37 (1.12, 3.93) and 216 (146, 330) U/L, respectively] (both P<0.001). Non-survivors presented with lower hemoglobin levels [63 (57, 72) g/L vs. 70 (58, 96) g/L; P<0.05] and significantly lower platelet counts [16 (10, 30)×10⁹/L vs. 27 (12, 75)×10⁹/L] and serum albumin concentrations [30.9 (27.8, 34.2) g/L vs. 36.6 (32.9, 39.9) g/L] (both P<0.01). Multivariate logistic regression confirmed that both the EASIX score (OR=1.401, 95%CI 1.161 to 1.691, P<0.001) and the serum albumin concentration (OR=0.851, 95%CI 0.782 to 0.926, P<0.001) are independent prognostic factors. ROC curve analysis yielded an area under the curve (AUC) of 0.717 (95%CI 0.642 to 0.792) for the EASIX in predicting mortality, and an AUC of 0.764 (95%CI 0.701 to 0.827) for serum albumin. Their combined use increased the AUC to 0.803 (95%CI 0.744 to 0.863), with a sensitivity of 93.4%. Conclusions: Elevated EASIX scores and hypoalbuminemia serve as independent predictors of a poor prognosis in patients with hematologic neoplasms complicated by BSI. The combined detection of both indicators significantly improves predictive efficacy and may facilitate early clinical risk stratification.

Objective: To evaluate the clinical value of magnetic navigation ultrasound (MNU) combined with digital subtraction angiography (DSA) for dual-guided portal vein stent placement (PTPS) in the treatment of portal vein tumor thrombus. Methods: This retrospective study analyzed clinical data from 30 patients with portal vein tumor thrombus who underwent PTPS at the Hepatobiliary Center of the First Affiliated Hospital of Nanjing Medical University (Jiangsu Province Hospital) between December 2019 and December 2022. The observation group (15 patients) received dual-guided puncture using novel MNU combined with DSA, while the control group (15 patients) underwent conventional DSA-guided puncture. Parameters compared included operation time, number of puncture attempts, radiation dose (displayed by DSA), patient tolerance, procedural success rate, and postoperative complications. Statistical analysis was performed using SPSS 26.0. Measurement data were expressed as mean±standard deviation (x¯±s) or median, and intergroup comparisons were conducted using t-tests or Mann-Whitney U tests. Results: The observation group showed significantly shorter operation time [(22.3±9.7) min vs. (41.6±12.9) min, t=-4.63, P<0.001], fewer puncture attempts [(1.9±0.7) vs. (8.3±4.1), t=-5.99, P<0.001], and lower radiation dose [228 (180, 285) vs. 815 (650, 1 050) mGy, Z=-4.67, P<0.001]. Two cases in the control group required procedure termination due to pain or prolonged operation time from multiple punctures, whereas all observation group patients successfully completed the procedure. The success rate was higher in the observation group (15/15) than in the control group (13/15). The incidence of puncture site bleeding (1/15), stent thrombosis (2/15), and postoperative infection (2/15) in the observation group were lower than those in the control group (5/15,4/15,and 3/15, respectively). Conclusion: Preliminary data suggest that dual-guided PTPS with MNU and DSA demonstrates improved puncture safety and success rates, indicating potential clinical value.