Objective:To analyze the clinical characteristics, treatment, and outcomes of children with complete left bundle branch block (CLBBB) mediated by maternal autoantibodies.Methods:A retrospective analysis was conducted on nine children diagnosed with maternal autoantibody-mediated CLBBB, treated at Beijing Anzhen Hospital and Fujian Provincial Hospital from March 2015 to August 2023. Their clinical characteristics, electrocardiographic and echocardiographic findings before and after treatment were reviewed. Paired sample t-test was used for inter-group comparison. Results:Among the mothers, 6 had positive antinuclear antibodies (ANA), 5 had anti-Sjogren syndrome antigen A antibodies, and 3 had anti-Ro-52 antibodies. The cohort included one female and eight male children, diagnosed with CLBBB at the age of 1 (2, 13) months. The positive autoantibodies in the infants, consisted with maternal antibodies, were detected within the first 3 months of life among 3 cases. Treatments included anti-heart failure therapy, myocardial nutritional support, intravenous immunoglobulin (IVIG) and glucocorticoids. Before treatment, the levels of troponin I (0.175 (0.060, 10.270) μg/L) and N-terminal pro-B-type natriuretic peptide (420 (327, 12 865) ng/L) were elevated, which normalized in most cases after treatment. Post-treatment, the QRS duration significantly shortened compared to pre-treatment ((137±15) vs.(169±25) ms, t=3.76, P<0.001), and the QTc interval significantly decreased ((433±41) vs. (514±27) ms, t=4.95, P=0.001). Before treatment, varying degrees of mitral and tricuspid regurgitation and marked interventricular septal dyskinesia were observed in echocardiography. After treatment, valve regurgitation and ventricular septum motion significantly improved, with a marked increase in left ventricular ejection fraction ((51±13)% vs. (27±6)%, t=-6.66, P<0.001). Conclusions:Maternal autoantibody-mediated CLBBB in children presents with chronic heart failure in infancy. Early treatment with anti-heart failure medications, IVIG and glucocorticoids can improve clinical symptoms.

Abstract: Objective To investigate the expression of T cell immunoglobulin and mucin domain-containing protein 3 (Tim-3) on the surface of T cells in patients with brucellosis (Bm), as well as the expression of interleukin-10 (IL-10) and transforming growth factor beta (TGF-β) in serum, and to analyze the differential expression of these indicators in patients with acute and chronic brucellosis, in order to provide new approaches for the differential diagnosis of acute and chronic brucellosis. Methods A total of 56 patients diagnosed with brucellosis at the First Affiliated Hospital of Xinjiang Medical University from April 2023 to September 2023 were selected, including 31 patients in the acute phase and 25 patients in the chronic phase. Additionally, 35 healthy individuals underwent routine physical examinations within the same period served as healthy controls. Flow cytometry was used to detect and compare Tim-3 levels on the CD4+ T cells' surface among the groups. Levels of serum IL-10 and TGF-β were measured and compared using CBA and ELISA, respectively, and the relationship of these factors with the staging of brucellosis patients was analyzed. Results The proportions of Tim-3+CD3+CD4+T cells in the control group, acute group, and chronic group were (2.56±1.25)%, (5.14±1.98)%, and (13.66±2.66)%, respectively. The Tim-3 levels in the patients with brucellosis were higher than those in the healthy control group, with the chronic group showing even higher levels, and these differences were statistically significant (P<0.05). The levels of IL-10 and TGF in the patient group were higher than those in the healthy control group, with the chronic group exhibiting significantly higher levels of IL-10 and TGF-β than the acute group, also presenting statistically significant differences (P<0.05). The areas under the ROC curve for predicting chronic brucellosis with Tim-3, IL-10, and TGF-β scores were 0.876, 0.865, and 0.663, respectively. Conclusions There are certain differences in the expression of Tim-3, serum IL-10, and TGF-β among patients with brucellosis, with high expression indicating a potential transition to the chronic phase of the disease. Tim-3 has shown the best diagnostic performance. Therefore, as a diagnostic indicator, Tim-3 may provide new ideas and strategies for the treatment and differential diagnosis of brucellosis.

Purpose: This study aimed to identify the altered pathways and genes associated with freezing damage in human sperm during cryopreservation by multiomics analysis. Materials and Methods: Fifteen fresh human semen samples were collected for transcriptomic analysis, and another 5 fresh human semen samples were obtained for metabolomic analysis. For each semen sample, 1 mL was cryopreserved, and another 1 mL was left untreated for paired design. The results were then combined with previously published proteomic results to identify key genes/pathways. Results: Cryopreservation significantly reduced sperm motility and mitochondrial structure. Transcriptomic analysis revealed altered mitochondrial function, including changes in tRNA-methyltransferase activity and adenosine tri-phosphate/adenosine di-phosphate transmembrane transporter activity. Metabolomic analysis showed that the citrate cycle in mitochondria was significantly altered. Combining transcriptomic, proteomic, and metabolomic analyses revealed 346 genes that were altered in at least two omics analyses. Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis showed that metabolic pathways were significantly altered and strongly associated with mitochondria. Five genes were altered in all three omics analyses: COL11A1, COL18A1, LPCAT3, NME1, and NNT. Conclusions Five genes were identified by multiomics analysis in human cryopreserved sperm. These genes might have specific functions in cryopreservation. Explorations of the functions of these genes will be helpful for sperm cryopreservation and sperm motility improvement or even for reproduction in the future.

OBJECTIVE: To explore the genetic basis for two children with sporadic neurofibromatosis type 1 (NF1) complicated with nephrotic syndrome (NS). METHODS: Clinical data of the children were collected. Both children were subjected to high-throughput sequencing, and candidate variants were verified by Sanger sequencing. RESULTS: Both children had café-au-lait macules, subaxillary freckle and Lisch nodules. Child 1 also had congenital tibiofibular pseudarthrosis on the left side. Genetic testing revealed that child 1 has harbored a heterozygous c.844C>T variant in the exon 8 of the NF1 gene, whilst child 2 has harbored a heterozygous c.1246C>T variant in the exon 11 of the NF1 gene. Both children were diagnosed with NF1 and have developed pronounced proteinuria, hypoalbuminemia, hypercholesterolemia and pitting edema at the ages of 3 and 10, respectively. Renal biopsy of child 2 has revealed minimal change nephropathy, and the diagnosis of nephrotic syndrome was established. Child 1 was treated with glucocorticoid, and child 2 was treated with glucocorticoid in combination with mycophenolate mofetil. The NS was relieved with no recurrence during 1 year's follow-up. CONCLUSION NF1 combined with NS is rare in the clinical settings. The prognosis of children with NF1 combined with minimal change nephropathy is relatively good. Detection of NF1 gene variant can facilitate early identification and diagnosis of NF1.
Child ; Humans ; Neurofibromatosis 1/genetics* ; Nephrotic Syndrome/genetics* ; Nephrosis, Lipoid ; Glucocorticoids ; Genetic Testing

Objective:To examine the association between self-rated health status (SRH) and all-cause and cardiovascular mortality.Methods:A total of 512 713 adults aged 30-79 years from 10 areas of China were followed from baseline (2004-2008) until 31 December 2016 in the China Kadoorie Biobank study. Global and age-comparative SRH [general self-rated health status (GSRH) and age-comparative self-rated health status (ASRH), respectively] were asked in baseline questionnaires. Causes for mortality were monitored through linkage with established Disease Surveillance Point system and health insurance records. Multivariable Cox proportional regression models were used to estimate the HRs and 95% CIs for the association between SRH measures and all-cause or cardiovascular mortality. Results:During an average of 9.9 years' follow-up, 44 065 deaths were recorded, among which 17 648 were from cardiovascular disease. Compared with excellent GSRH, the HR(95% CI) for all-cause and cardiovascular mortality associated with poor GSRH was 1.84(1.78-1.91) and 1.94(1.82-2.06), respectively. Relative to better ASRH, the HR(95% CI) for all-cause and cardiovascular mortality associated with worse ASRH was 1.75(1.70-1.81) and 1.83(1.73-1.92), respectively. Conclusion:In this large prospective cohort study in China, participants reporting poor GSRH or worse ASRH had significantly higher risk of all-cause and cardiovascular mortality.

In burn scar patients, the damage of the body surface skin and scar hyperplasia and contracture resulting in the small number of peripheral veins available for infusion in the scar area, and the poor conditions of peripheral veins. There fore it is difficult for nurses to puncture the vein. This article reviews the clinical application of intravenous indwelling needle for burn scar patients, selection of blood vessels in scars, puncture, fixation and maintenance of venous indwelling needles and health education. The article aims at providing a basis for protecting the limited peripheral veins of scar patients well and improving the quality of holistic nursing service of intravenous infusion for patients with burn scars.

Objective This study aimed to investigate the clinical characteristics of cryptogenic stroke as the first manifestation of occult systemic malignancy (OSM) and to determine whether plasma D-dimer levels and lesions in multiple vascular regions could predict occult systemic malignancy in patients with cryptogenic stroke. Methods Data from 83 patients including 9 patients with OSM and 74 patients without OSM were extracted from the stroke database of Beijing Shijitan Hospital. Clinical variables were analyzed between cryptogenic stroke patients with OSM and without OSM. The relationshiop between plasma D-dimer levels or lesions in multiple vascular regions and OSM were analyzed by multiple logistic regression analysis. Results Compare to patients without OSM, patients with OSM was older (66.8± 7.5 vs.56.4±15.7,P=0.004)and had lower hemoglobin levels (127.9±19.8 vs.143.6±17.7,P=0.015),higher conut score [3(1,3) vs. 1(0,2), P=0.011], higher plasma D-dimer levels (77.7% vs. 13.5%, P<0.001) and more common lesions in multiple vascular regions(100% vs.23.0%, P<0.001).Multiple logistic regression analysis revealed that high plasma D-dimer levels are independent risk factors for OSM (OR=26.250,95%CI:3.041-226.604,P=0.003).Conclusions Patients with OSM are elderly people and have poorer nutritional status, higher plasma D-dimer levels and more common lesions in multiple vascular regions. High plasma D-dimer levels can be useful to predict OSM in patients with cryptogenic stroke at earlier stages.

BACKGROUND AND PURPOSE: Self-rated health (SRH) is a consistent and strong predictor of all-cause and cardiovascular mortality in various populations. However, the associations between SRH measures and risk of first-ever or recurrent stroke were rarely explored. We thus aim to prospectively investigate the associations between SRH measures and risk of total and subtypes of stroke in Chinese population. METHODS: A total of 494,113 participants from the China Kadoorie Biobank without prior heart diseases or cancer (486,541 without stroke and 7,572 with stroke) were followed from baseline (2004 to 2008) until December 31, 2013. General and age-comparative SRH were obtained from baseline questionnaires. First-ever stroke or recurrent events were ascertained through linkage to disease registry system and health insurance data. RESULTS: We identified 27,662 first-ever stroke and 2,909 recurrent events during an average of 7.0 years of follow-up. Compared with excellent general SRH, the hazard ratios (HRs) and 95% confidence intervals (CIs) for first-ever stroke associated with good, fair, and poor general SRH were 1.04 (1.00 to 1.08), 1.19 (1.15 to 1.23), and 1.49 (1.42 to 1.56) in the multivariate model, respectively. Compared with better age-comparative SRH, the HRs (95% CIs) of same and worse age-comparative SRH were 1.13 (1.10 to 1.17) and 1.51 (1.45 to 1.58), respectively. The relations of SRH measures with ischemic stroke, hemorrhagic stroke, and recurrent stroke were similar to that with total first-ever stroke. However, the magnitude of associations was much stronger for fatal stroke than for non-fatal stroke. CONCLUSIONS: This large-scale prospective cohort suggests that self-perceived health status is associated with incident stroke, regardless of stroke subtype.
Adult* ; Asian Continental Ancestry Group* ; China* ; Cohort Studies ; Follow-Up Studies ; Heart Diseases ; Humans ; Insurance, Health ; Mortality ; Prospective Studies ; Stroke*

Objective To describe the clinical features and magnetic resonance imaging (MRl) findings of brainstem infarction producing internuclear ophthalmoplegia (INO) as an predominant clinical manifestation.Methods The clinical data of 9 patients diagnosed with brainstem infarction presenting with INO by angiography and MRI diagnosis in our hospital were collected from January 2010 to October 2016.The clinical features and imaging findings of these patients were analysed,and the pathogenesis mechanisms was analysed by literature research.Results The median age of the 9 patients was 56 years old,and there were 5 young and middle aged cases (under 60 years old),accounted for 55.6％.Among them,8 cases (88.9％) with INO typically presenting with horizontal diplopia,the most common accompanied symptom was dizzness (accounted for 55.6％),and most of them (accounted for 77.8％) were unilateral.A total of 6 cases were anterior INO,3 cases were both anterior and posterior INO.Five cases (55.6％) retained converging movement.The MRI showed that paramedian ventral infarcts located in the the cerebral aqueduct were responsible for INO in 1 case,and paramedian dorsal infarcts located in the pons were responsible for INO in 8 cases.The magnetic resonance angiography (MRA) indicated that vascular occlusion or stenosis was found in posterior circulation in 6 patients,there were 5 cases with stenosis of the posterior cerebral artery which was the most common.The ocular symptoms,including diplopia and blurred vision,of 6 cases (66.7％) disappeared during their hospital stay (range:13-23 d,median:14 d).During following up,ocular symptoms of 1 case finally disappeared within 3 months.Conclusion Brainstem infarction presenting with INO is mainly caused by paramedian dorsal infarcts located in the pons,and most of the patients complicated with posterior circulation atherosclerotic lesions.The pathogenesis varies,and the patients have a good clinical prognosis.For the young and middle-aged patients presenting with diplopia as an initial symptom,it should pay more attention to differential diagnosis.

Objective To observe the pathological changes of pituitary tissue in rats with acute necrotizing pancreatitis and to explore the mechanism of pituitary tissue injury in rats.Methods 24 SD rats were randomly divided into normal group (N group,n=8),sham operation group (SO group,n=8),and acute necrotizing pancreatitis group (ANP group,n=8).ANP model was established by retrograde injection of 5％ sodium taurocholate into the biliopancretic duct.The serum levels of amylase(AMY) and lipase (LIP) were detected by automatic biochemical.The serum levels of growth hormone (GH),adrenocorticotropic hormone (ACTH),thyroid stimulating hormone (TSH) and follicle-stimulating hormone (FSH) were measured by radioimmunoassay.The pathological changes of pancreatic tissue and pituitary tissue were observed by the light microscope.The expression of Casepase3,Caspasel2 and CHOP in pituitary tissue were determined by immunohistochemical method.Results Compared to SO group,the serum levels of AMY(8679.16±307.60) U/L and LIP(9376.83±380.92) U/L were significantly higher in ANP group (P＜0.05).The serum levels of ACTH (0.92±0.41) pg/ml,TSH (0.14±0.06) pg/ml,and FSH (2.01±0.38) pg/ml were significantly lower in ANP group(P＜0.05).The expression of Caspse 3 (65.66±7.58),Caspase12(70.66±4.76) and CHOP(143.16±19.05) in pituitary tissue were significantly increased in ANP group (P＜0.05).The pancreatic injury was more severe in ANP group under light microscope (P＜0.05).The degree of hyperemia of pituitary tissue of ANP group was aggravated.Conclusion Pathological changes occur in rat pituitary tissues and endoplasmic reticulum stress injury plays a role in pituitary injury during ANP.