ABSTRACT This retrospective cohort study preliminarily investigated the feasibility of implementing primary aldosteronism (PA) screening without discontinuing dihydropyridine calcium channel blockers (DHP‐CCBs). We screened all patients undergoing diagnostic testing for secondary hypertension from January 2017 to May 2022 at authors’ center. For inclusion, patients must be on DHP‐CCBs monotherapy for hypertension. Aldosterone and renin concentration were measured pre‐ and post‐washout. Confirmatory tests were conducted in patients with positive screen [combination of ARR >2.5 (ng/dL)/(μIU/mL), aldosterone ≥10 ng/dL, and renin ≤8.2 μIU/mL] after washout. The final analysis included a total of 198 patients (median age: 49.5 years and 117 men). Confirmatory tests identified PA in 31 (15.7%) patients. Aldosterone‐to‐renin ratio (ARR) increased in 158 (79.8%) patients after washout. In 168 patients with a negative pre‐washout screen, 22 turned positive after washout, and 14 were diagnosed PA. Consequently, the missed diagnosis rate of PA was 45.2% (14/31) when using standard positive screen criteria before washout of DHP‐CCBs monotherapy. Exploratory analysis indicated that a pre‐washout ARR cutoff of >1.2 (ng/dL)/(μIU/mL) demonstrated 0.97 sensitivity and 0.75 specificity. In conclusions, caution is warranted when interpretating screening results from patients on DHP‐CCBs monotherapy due to the potential risk of missed diagnosis. Preliminary data suggest that ARR ≤1.2 (ng/dL)/(μIU/mL) before washout may serve as a candidate threshold for excluding PA in these patients, though this observation remains a hypothesis‐generating finding that requires further investigation.

Background:Heart failure (HF) is a serious clinical syndrome with substantial health threats. Emerging studies link intestinal flora dysbiosis to HF onset and progression. Although probiotics are thought to regulate gut microbiota, the specific impact of probiotics on HF remains unclear, highlighting the need for systematic evaluation. Methods:As of 9 September 2025, we searched eight major academic databases using a predefined protocol for data extraction and quality assessment. Subsequently, a meta-analysis was conducted using Review Manager 5.4 and Stata 18. Forest plots were used to analyse the effect size, and publication bias was evaluated through funnel plots. Results:Ultimately, 11 of the studies met the inclusion criteria for the systematic review. The results showed that probiotics have a slight beneficial effect on cardiac function indicators (LVEF, LVESV), reduced the levels of inflammatory factors (hs-CRP, IL-6, TNF-α), regulated the proportion of dominant gut bacteria, and decreased the readmission rates of patients with HF. However, no beneficial effects were found on NT-proBNP, activity endurance, TMAO, and mortality. Conclusion:Probiotics exert cardioprotective effects and can serve as adjunctive therapy for HF management. Future high-quality, large-sample clinical studies are needed to further clarify their long-term efficacy and optimal intervention strategies. Systematic review registration:Details of the protocol for this systematic review were registered on PROSPERO (CRD420251083960).

Patients who undergo adrenalectomy for unilateral primary aldosteronism (PA) may still develop post-surgery hypertension; however, the clinical characteristics and etiology of patients developing recurrent hypertension after adrenalectomy are unclear. We analyzed the records of 43 patients with recurrent elevated blood pressure after adrenalectomy, who were treated at our center. Standard routine clinical screening workup was used to identify the cause of recurrent hypertension. Causes of recurrent hypertension after adrenalectomy included essential hypertension, primary aldosteronism, obstructive sleep apnea, renal artery stenosis, and Takayasu arteritis. Before adrenalectomy, 39.5% of patients were diagnosed with confirmed or suspected PA, primarily through CT imaging. Adrenal venous sampling (AVS) tests were not conducted on any patients, and 72.1% patients underwent partial adrenalectomy. Among all patients, elevated blood pressure was observed in 44.2% immediately post-operation, 18.6% within 1 month, 16.3% in 1–6 months, and 20.9% >6 months after operation. Most patients had hypertension of grade 2 and above. Standard endocrine functional assessment and AVS tests should be performed before adrenalectomy to ensure more accurate diagnosis and favorable post-operative outcomes. Additionally, individuals often develop essential hypertension regardless of past adrenal disease.

Abstract Background The triglyceride-glucose (TyG) index is a reliable marker of insulin resistance that is involved in the progression of hypertension. This study aimed to evaluate the association of the TyG index with the risk for major cardiovascular events (MACE) in young adult hypertension. Methods A total of 2,651 hypertensive patients aged 18–40 years were consecutively enrolled in this study. The TyG index was calculated as Ln [triglycerides × fasting plasma glucose/2]. The cutoff value for an elevated TyG index was determined to be 8.43 by receiver-operating characteristic curve analysis. The primary endpoint was MACE, which was a composite of all-cause death, non-fatal myocardial infarction, coronary revascularization, non-fatal stroke, and end-stage renal dysfunction. The secondary endpoints were individual MACE components. Results During the median follow-up time of 2.6 years, an elevated TyG index was associated with markedly increased risk of MACE (adjusted hazard ratio [HR] 3.440, P < 0.001) in young hypertensive adults. In subgroup analysis, the elevated TyG index predicted an even higher risk of MACE in women than men (adjusted HR 6.329 in women vs. adjusted HR 2.762 in men, P for interaction, 0.001); and in patients with grade 2 (adjusted HR 3.385) or grade 3 (adjusted HR 4.168) of hypertension than those with grade 1 (P for interaction, 0.024). Moreover, adding the elevated TyG index into a recalibrated Systematic COronary Risk Evaluation 2 model improved its ability to predict MACE. Conclusions An elevated TyG index is associated with a higher risk of MACE in young adult hypertension, particularly in women and those with advanced hypertension. Regular evaluation of the TyG index facilitates the identification of high-risk patients. Graphical abstract

OBJECTIVE:The aim of this study is to evaluate performance of aldosterone-to-renin ratio (ARR) before washout of antihypertensive drugs as a screening test for primary aldosteronism (PA). METHODS:This retrospective analysis included consecutive patients screening for secondary hypertension during a period from January 2017 to May 2022 at the authors' institute. For inclusion in the final analysis, ARR had to be available prior to as well as after discontinuation of antihypertensives. Patients with ARR ≥2.4(ng/dL)/(μIU/mL) after washout proceeded to confirmatory tests. Diagnosis of PA was established based on a positive result of the confirmatory test. The diagnostic accuracy of ARR prior to the washout in predicting PA is shown as sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). RESULTS:The analysis included a total of 1306 patients [median age of 50.2 (41.0-59.0) years, 64.0% male]. Confirmatory tests showed PA in 215(16.5%) patients and essential hypertension (EH) in the remaining 1091(83.5%) patients. In comparison to the second screening test, the first screening test (before washout of antihypertensives) yielded lower plasma aldosterone and higher renin and consequently lower ARR in both the PA and EH groups. At a cutoff of .7(ng/dL)/(μIU/mL), ARR before washout had 96.3% sensitivity, 61.2% specificity, .33 PPV, and .99 NPV. At a lower cutoff of .5(ng/dL)/(μIU/mL), the sensitivity, specificity, PPV, and NPV were 97.7%, 52.0%, .29, and .99, respectively. CONCLUSION:ARR prior to washout of antihypertensives is a sensitive screening test for PA. Washout of antihypertensives could be omitted and further investigation for PA is not warranted if ARR is ≤ .7(ng/dL)/(μIU/mL) before washout.

Cardiac paragangliomas (PGLs) are rare neuroendocrine tumors, and data regarding the features of nonfunctioning PGLs are limited. These tumors are extensively vascularized and have high risk of hemorrhage for surgery and even biopsy. Differential diagnosis including biochemical analysis of these PGLs is important for further management. In this case report, we present the clinical, laboratory, imaging, and radionuclide presentations of a rare primary nonfunctioning cardiac PGL with a coronary aneurysm. Echocardiography initially showed a large echogenic mass in the left atrioventricular groove. The mass presented a diffuse hyperenhancement pattern with a central perfusion defect on contrast echocardiography. The tumor enclosed the left coronary artery from the coronary orifice, and an aneurysm was found in the left circumflex artery, with significantly increased flow velocity. These echocardiographic features and its susceptible location are indicative of the presence of a cardiac PGL. Although all biochemical evaluations of catecholamines from blood and urine samples were negative, positron emission tomography and scintigraphy finally confirmed the diagnosis of a primary cardiac PGL. Therefore, when imaging features are indicative of the presence of PGLs, the implementation of radionuclide imaging for final diagnosis is required even if the biochemical results are negative. Recognizing these uncommon Doppler and contrast echocardiographic characteristics is important for early diagnosing these nonfunctioning PGLs.

血小板低下患者置管后发生持续渗血易导致感染等并发症.文章综述了PICC置管前、中、后相关预防穿刺点持续渗血的护理进展,包括全面评估渗血风险、提高置管技术、使用有效止血辅助措施等,旨在为降低PICC置管渗血率、保证置管安全提供参考.

Background: Mid-aortic syndrome (MAS), characterized by segmental stricture of the distal thoracic and abdominal aorta, is a heterogeneous clinical syndrome with multiple etiologies. Methods: We retrospectively analyzed 143 consecutive patients (99 females and 44 males, mean age 40.93 +/- 15.31 years) with MAS seen from January 1, 2010 to January 1, 2019. Results: Takayasu arteritis (76.9%, 110/143) and atherosclerosis (19.6%, 28/143) were the most-common causes. There were also one patient with Beh,cet's disease and one with congenital MAS in the cohort. Hypertension was the most -com-mon manifestation. Constitutional symptoms were mainly seen in Takayasu arteritis, and neurological, gastrointestinal and vascular symptoms were common in both Takayasu arteritis and atherosclerosis. The infrarenal segment was the most -commonly involved in atherosclerosis (89.3%, 25/28), whereas lesions were more distributed in Takayasu arteritis. The mean length of involved segments was longer (43.45 +/- 23.64 mm vs. 30.68 +/- 12.66 mm; P = 0.018) and the degree of ste-nosis was lower (80.20 +/- 13.36% vs. 87.50 +/- 13.95%, P = 0.004) in Takayasu arteritis than atherosclerosis. The most -common concurrently involved branch was the renal artery, followed by the celiac trunk and mesenteric arteries, in both Takayasu arteritis (51.8%, 32.7% and 27.3%, respectively) and atherosclerosis (53.6%, 25.0% and 17.9%, respectively). Concurrent artery involvement and coexisting lesions were absent in MAS caused by congenial coarctation of the abdomi-nal aorta and Beh,cet's disease. Conclusions: Takayasu arteritis and atherosclerosis were the most-common causes of MAS among these adults. Imaging tests provided evidence of involved segments and luminal and mural changes, aiding conclusive diagnoses and etiological dif-ferentiation of MAS. [Am J Med Sci 2023;365(5):420-428.]

ObjectiveTo examine the consistency of plasma aldosterone concentration at 1 and 2 h in the captopril challenge test (CCT) and to explore the possibility of replacing 2-h aldosterone concentration with 1-h aldosterone concentration for diagnosis of primary aldosteronism (PA).MethodsThis retrospective analysis included a total of 204 hypertensive patients suspected of having PA. Subjects received oral captopril challenge at 50 mg (25 mg if the systolic blood pressure was <120 mmHg), and plasma aldosterone concentration and direct renin concentration were measured at 1 and 2 h afterward (chemiluminescence immunoassay Liaison® DiaSorin, Italy). Sensitivity and specificity were used to reflect the diagnostic performance of 1-h aldosterone concentration using 2-h aldosterone concentration (11 ng/dl as the cutoff) as the reference. A receiver operating characteristic curve analysis was also conducted.ResultsAmong the 204 included patients [median age of 57.0 (48.0–61.0) years, 54.4% men], a diagnosis of PA was established in 94 patients. Aldosterone concentration in the patients with essential hypertension was 8.40 (interquartile range 7.05–11.00) ng/dl at 1 h and 7.65 (5.98–9.30) ng/dl at 2 h (P < 0.001). In patients with PA, aldosterone concentration was 16.80 (12.58–20.50) ng/dl at 1 h and 15.55 (12.60–20.85) ng/dl at 2 h (P > 0.999). At a cutoff of 11 ng/dl, the sensitivity and specificity of using 1-h aldosterone concentration to diagnose PA were 87.2% and 78.2%, respectively. A higher cutoff of 12.5 ng/ml increased specificity to 90.0% but decreased sensitivity to 75.5%. A lower cutoff of 9.3 ng/ml increased sensitivity to 97.9% but decreased specificity to 65.4%.ConclusionsWhen diagnosing PA with CCT, 1-h aldosterone concentration could not be used to replace 2-h aldosterone concentration.

Purpose This study aimed to explore the impact of ABL1–tyrosine kinase inhibitors (TKIs) adherence on the survival of chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL) children and clarify the potential predictors of patients’ prognosis from TKIs intake practices.Materials and Methods Ninety newly diagnosed Ph+ ALL patients who received TKIs were enrolled. We collected the baseline characteristics and adverse events in all children; moreover, TKIs adherence was measured by an eight-item Morisky medication adherence scale (MMAS-8). Progression-free survival (PFS) and overall survival (OS) analysis were performed, and risk factors for PFS and OS were evaluated.Results Among all patients, 69 cases were regarded as adherers, while 21 were non-adherers. The median duration of TKIs interruption was significantly prolonged in the non-adherence group than in the adherence group (13 [0-101] vs. 56 [11-128], p < 0.001). Additionally, dose reduction occurred in 55.2% of non-adherers versus 23.0% of adherers (p=0.002). The PFS and OS in adherers were significantly higher versus non-adherers (p=0.020 and p=0.039). MMAS-8 score was an independent risk factor for PFS (p=0.010) and OS (p=0.031). Among non-adherers, the median OS was only 23.1% (4.2%-42%) in patients aged ≤ 10 years versus 54.4% (38.8%-70%) in adolescents. Most of the patients who experienced TKIs non-adherence suffered pancytopenia.Conclusion TKIs adherence during treatment significantly influenced the survival of pediatric Ph+ ALL patients, and non-adherers with age ≤ 10 years were more vulnerable to TKIs disruption. The cumulative TKIs dose should be especially emphasized to patients with age ≤ 10 years, which may result in an inferior achievement of relevant treatment milestones.

Background: Clinical data on the correlation of dyslipidaemia with the long-term outcomes of ulcerative colitis (UC) are limited. This study aimed to evaluate the impact of lipid levels on disease activity and prognosis in UC. Methods: The retrospective data of UC patients who had detailed lipid profiles were collected from January 2003 to September 2020. All patients were followed-up to 30 September 2021. The long-term outcomes were UC-related surgery and tumorigenesis. Results: In total, 497 patients were included in the analysis. Compared to patients with normal lipid levels, those with dyslipidaemia commonly presented with more serious disease activity. Low high-density lipoprotein cholesterol (p < 0.05) levels were associated with higher risks of severe disease activity in UC. Regarding the long-term outcomes, patients with persistent dyslipidaemia were at higher risks of UC-related surgery (HR: 3.27, 95% CI: 1.86–5.75, p < 0.001) and tumorigenesis (HR: 7.92, 95% CI: 3.97–15.78, p < 0.001) and had shorter surgery- and tumour-free survival (p < 0.001) than patients with transient dyslipidaemia and normal lipid levels. Low levels of high-density lipoprotein cholesterol (p < 0.001) and apolipoprotein A1 (p < 0.05) were associated with higher risks of surgery and tumorigenesis. Conclusion: Persistent dyslipidaemia was associated with a higher risk of serious disease activity and worse long-term outcomes among patients with UC. Lipid patterns should be assessed to improve the management of high-risk patients with UC in the early phase.

ObjectiveMounting evidence has linked microbiome and metabolome to systemic autoimmunity and cardiovascular diseases (CVDs). Takayasu arteritis (TAK) is a rare disease that shares features of immune‐related inflammatory diseases and CVDs, about which there is relatively limited information. This study was undertaken to characterize gut microbial dysbiosis and its crosstalk with phenotypes in TAK.MethodsTo address the discriminatory signatures, we performed shotgun sequencing of fecal metagenome across a discovery cohort (n = 97) and an independent validation cohort (n = 75) including TAK patients, healthy controls, and controls with Behçet's disease (BD). Interrogation of untargeted metabolomics and lipidomics profiling of plasma and fecal samples were also used to refine features mediating associations between microorganisms and TAK phenotypes.ResultsA combined model of bacterial species, including unclassified Escherichia, Veillonella parvula, Streptococcus parasanguinis, Dorea formicigenerans, Bifidobacterium adolescentis, Lachnospiraceae bacterium 7 1 58FAA, Escherichia coli, Streptococcus salivarius, Klebsiella pneumoniae, Bifidobacterium longum, and Lachnospiraceae Bacterium 5 1 63FAA, distinguished TAK patients from controls with areas under the curve (AUCs) of 87.8%, 85.9%, 81.1%, and 71.1% in training, test, and validation sets including healthy or BD controls, respectively. Diagnostic species were directly or indirectly (via metabolites or lipids) correlated with TAK phenotypes of vascular involvement, inflammation, discharge medication, and prognosis. External validation against publicly metagenomic studies (n = 184) on hypertension, atrial fibrillation, and healthy controls, confirmed the diagnostic accuracy of the model for TAK.ConclusionThis study first identifies the discriminatory gut microbes in TAK. Dysbiotic microbes are also linked to TAK phenotypes directly or indirectly via metabolic and lipid modules. Further explorations of the microbiome–metagenome interface in TAK subtype prediction and pathogenesis are suggested.

1 病例资料 患者,女,60岁,以"胸背部疼痛5年,加重4个月"入院.患者5年前逐渐出现持重物或重体力劳动后胸骨后、背部疼痛,每次持续数小时至数天不等,自行缓解,当地医院检查未见异常.4个月前疼痛症状加重,呈撕裂样,不能耐受.

目的 分析近2年高血压住院患者中内分泌性高血压的病因构成及临床特点.方法 回顾性分析阜外医院高血压病房2016年1月至2017年12月因高血压首次住院的患者中内分泌性高血压的病因构成及基本临床情况.结果 高血压患者4782例,其中内分泌性高血压371例,占7.76％.内分泌性高血压的病因构成为:原发性醛固酮增多症247例(66.58％),甲状腺功能减退症52例(14.02％),甲状腺功能亢进症38例(10.24％),多囊卵巢综合征22例(5.93％),皮质醇增多症8例(2.16％),嗜铬细胞瘤4例(1.08％).与原发性高血压组[(50.31±15.10)岁]相比,原发性醛固酮增多症组年龄稍小[(48.21±11.63)岁],甲状腺功能异常组年龄较大[(55.20±14.68)岁],多囊卵巢综合征组明显年轻[(33.03±3.0l)岁],差异均有统计学意义(P＜0.05).原发性醛固酮增多症组中男性占59.92％,甲状腺功能异常组中女性占56.67％,多囊卵巢综合征组均为育龄期年轻女性,皮质醇增多症组以青年女性为主(占75％).与原发性高血压组相比,内分泌性高血压组血压高[收缩压:(150.23±20.51) mmHg比(143.21±20.01)mmHg,舒张压:(91.38±15.72) mmHg比(87.20±15.03) mmHg],尿微量白蛋白/肌酐高[(43.12±79.41) mg/g比(39.41±134.57) mg/g],并发外周动脉狭窄/闭塞者多(9.43％比4.13％),差异均有统计学意义(P＜0.001).结论 内分泌性高血压并不少见,原发性醛固酮增多症是最常见病因.内分泌性高血压多为中青年患者,且血压高,靶器官损害重.

Objective: Evidence-based studies on endovascular approaches for childhood Takayasu arteritis(c-TA) are limited. This study presented the real-world scenario of largest cohort up-to-date for c-TA patients undergoing interventions and their post-interventional outcomes. Design and method: Patients with c-TA satisfying the 1990 ACR or 2010 EULAR/PRINTO/PReS criteria were recruited from January 2002 to December 2017. Data on clinical, laboratory, imaging features, treatment and post-interventional outcomes were collected. Statistical analysis was performed based on data distribution. Complication-free survival and Re-intervention-free survival were projected by Kaplan-Meier methods and compared by Log-rank tests. Associated factors for intervention (or stenting) and predictors for post-interventional complications (or re-interventions) were assessed via Logistic regression and COX regression models, respectively. Results: Among 101 patients enrolled, 69(68.3%) underwent 121 interventions (Angioplasty 95; Stenting 26) during a median 3.1 years of follow-up. Compared with the medical treatment group, the intervention group appeared with fewer male population(18.8% vs 39.3%, OR = 0.2, p = 0.017) and more type IV disease(47.8% vs. 17.9%, OR = 10.60, p = 0.002). Male sex also indicated the risk for re-intervention(HR = 4.72, p = 0.016). Retinopathy(OR = 4.8, p = 0.027), time of delay in diagnosis per year(OR = 1.76, p = 0.016) and descending thoracic aorta involvement(OR = 10.19, p = 0.003) associated with stent insertion. Hypertension secondary to renal artery(59.4%) or mid-aorta(14.5%) stenosis, heart failure(21.7%), claudication(21.7%) served as leading clinical hints for interventions. Anti-inflammatory and anti-platelet therapies covered 89.9% and 91.3% patients, respectively. The technical success rate was 96.7%. Over median 2.88 years since intervention, 36 lesions occurred complications in 28 patients and 22 lesions in 17 patients, majorly on renal artery or mid-aorta. The 5-year complication-free and re-intervention survivals were 50.7% and 65.8%, separately. Dual antiplatelet therapy(HR = 0.28, p = 0.011), concurrent surgery(HR = 14.84, p = 0.002), retinopathy secondary to hypertension(HR = 3.54, p = 0.004), and pulmonary artery hypertension(HR = 3.0, p = 0.024) were independent predictors for complications. Conclusions: Over two-thirds c-TA patients require interventions and the 5-year complication-free survival is 50.7%. Male sex, retinopathy, and pulmonary artery hypertension alert unfavorable outcomes. Dual anti-platelet therapy appears to protect c-TA patients from post-interventional complications. Endovascular approaches can be a choice for mid-aorta and/or renal artery lesions caused by c-TA.

Objective To analyze the causes of renal artery stenosis (RAS) and compare the clinical characteristics in accordance with the primary disease among patients aged from 30 to 50. Methods Patients were grouped by etiologies of RAS. Groups were retrospectively examined and compared regarding demographic data, clinical manifestations, laboratory findings, and imaging findings. Results A total of 152 patients (74 females, 78 males; mean age: 40.70 ± 6.01 years) were enrolled, including 84 patients (55.3%) with atherosclerosis (AS), 46 patients (30.3%) with Takayasu arteritis (TA), 18 patients (11.8%) with fibromuscular dysplasia (FMD), and four patients (2.6%) with other etiologies. Patients in AS group had greater body mass index, higher prevalence of comorbidities and higher rate of smoking and drinking history. TA patients showed more constitutional symptoms and vascular findings, and higher erythrocyte sedimentation rate. RAS in both AS group and TA group mainly located on ostia and proximal segments, but RAS in FMD group mainly involved middle to distal segment of renal artery. The AS group had significantly lesser stenosis than the other groups. Although renal function evaluated by the estimated glomerular filtration rate did not significantly differ among the groups, the incidence of kidney shrinkage was significantly higher in the TA and FMD groups (39.1% and 50%, respectively) than in the AS group (8.3%). The FMD group had milder cardiac damage than other groups. Conclusions AS was the most common cause of RAS in patients aged from 30 to 50, followed by TA and FMD. The etiology of RAS should be carefully distinguished based on clinical manifestations, laboratory findings, and imaging to ensure that proper treatment is provided.

Background Pheochromocytoma and paraganglioma is a rare disease with a prevalence of 0.2-0.6% in hypertensive patients from outpatient. Case summary A 21-year-old man complained of blood pressure elevation over one year and persistent hyperhidrosis near 5 years. In local hospital, a mass in the pericardial space with abundant blood flow was observed via echocardiography and confirmed under minimally invasive thoracotomy. With suspicion of malignant cardiac mass, the patient was recommended to transfer for further diagnosis and treatment. Combining evaluation for blood and urinary catecholamine levels, somatostatin receptor imaging, and iodine-131 metaiodobenzylguanidine scintigraphy, he was confirmed with the diagnosis of cardiac paraganglioma with blood supply from the right coronary artery identified via angiography. The cardiac tumour was then surgically resected and confirmed with a pathological diagnosis of paraganglioma. Subsequent genetic test suggested succinate dehydrogenase complex iron sulfur subunit B (SDHB) gene mutation. At 5-month follow-up, the patient was recovered with normal levels of blood catecholamines and catecholamine metabolites. Discussion Cardiac paraganglioma should be considered and evaluated in hypertensive patients with cardiac mass, even in non-typical population. Given a potential risk of developing malignancies, close follow-up is significant in patients with SDHB gene mutations.

Glucocorticoid-remediable aldosteronism (GRA) is an autosomal-dominant inherited aldosteronism that is often accompanied by early-onset hypertension. GRA is caused by the unequal crossover of the 11β-hydroxylase (CYP11B1) and aldosterone synthase (CYP11B2) genes. As a result of chimeric gene duplication, aldosterone is ectopically synthesized in the adrenal zona fasciculata under the control of adrenocorticotropic hormone (ACTH). Here, we describe a Chinese pedigree with three affected subjects. Both the uncle and nephew were hospitalized in our hospital due to early-onset hypertension (onset <20 years old) and were diagnosed with primary aldosteronism (PA). Their laboratory test results revealed hyperaldosteronism, hyporeninemia, a high plasma aldosterone to renin (ARR) ratio, and normal serum potassium (K+). Captopril failed to suppress aldosterone secretion. This family had a strong paternal history of hypertension. Thirteen members underwent gene testing, and three of them were found to be GRA positive. Through long-extension PCR (XL-PCR) and direct sequencing, we identified the CYP11B1/CYP11B2 chimeric gene, and with unequal crossover breakpoints located between intron 2 of CYP11B1 and exon 3 of CYP11B2 in the three patients. Low-dose dexamethasone was effective. This is the first family report of GRA in northern China. Moreover, a case of GRA combined with a CACNA1H gene mutation is reported for the first time. We found that dihydropyridine calcium channel blockers (CCBs) combined with aldosterone receptor antagonists exerted good therapeutic effects in controlling blood pressure in GRA patients for whom glucocorticoid therapy was not an option.

Background: Chronic heart failure (CHF) is a serious complication and a major cause of mortality in patients with Takayasu arteritis (TA). We aimed to explore the clinical features and long-term outcomes in TA patients with CHF. Methods and results: Adult TA patients admitted to our hospital between January 2009 to April 2018 were classified as Hr and non-HF group. The adverse events were defined as a composite of all-cause mortality and hospitalization for HF. The outcome of the HF-group was further analyzed. A total of 61 HF patients and 102 non-HF patients were identified. In the HF group, the median age at assessment was 41.9 years, and female was predominant (82.0%). The multivariable logistic regression model revealed that pulmonary hypertension, aortic regurgitation, mitral regurgitation, level albumin, and uric acid were independently associated with CHI. After a median follow-up of 1347 days, 25 adverse events occurred in HI patients, and the 5-year event-free rate was 54.7%. The Cox model showed that coronary artery involvement, aortic regurgitation, without interventional treatment were related to adverse events. Conclusions: The 5-year event-free rate was not satisfying. Aggressive intervention may decreased the likelihood of adverse events in patients with CHF. (C) 2020 Elsevier B.V. All rights reserved.

目的 基于单中心临床治疗成本数据,对英夫利西单抗(Infliximab,IFX)纳入医保前后和沙利度胺(Thalidomide,THA)、甲氨蝶呤(Methotrexate,MTX)在复发克罗恩病(Crohn's disease,CD)治疗中的成本-效用进行比较分析.方法 纳入2009年10月1日至2018年9月30日采用THA、MTX和IFX治疗的复发CD患者,收集其直接医疗成本和CD疾病活动度评分(Crohn's disease activity index,CDAI).IFX纳入医保后的相应成本由IFX医保前后费用差价算得.质量调整寿命年(quality adjusted life year,QALY)由CDAI换算得来,QALY原始算法基于EQ-5D量表换算得来.以THA治疗方案为参照进行成本-效用分析,2018年人均GDP为成本-效用评估阈值标准.结果 在IFX纳入医保前后,对复发CD患者而言,THA方案人均直接医疗成本最低(0.80万元).而相对于THA治疗方案,增加单位QALY的不同治疗方案费用:MTX治疗方案花费100万元,IFX纳入医保前需花费133.67万元,但在IFX纳入医保后仅需消耗1.87万元,远低于2018年人均GDP.结论 IFX纳入医保后成为复发CD患者极具成本-效用优势的药物治疗方案.