To explore the clinical features, treatment, and prognosis of patients with lymphoma-associated hemophagocytic syndrome (LAHS) in a real-world clinical setting. We retrospectively examined LAHS patients diagnosed at our center between January 2016 and August 2023, focusing primarily on their clinical features, therapeutic approaches, overall response rate (ORR), and overall survival (OS). A combination of univariate and multivariate analyses was conducted to identify potential prognostic factors. A total of 86 patients diagnosed with LAHS were included to evaluate clinical characteristics and prognostic factors. Patients with T/NK cell lymphoma had a higher probability of developing hemophagocytic syndrome (HPS) during the clinical process than those with B cell lymphoma. The median survival time was 55 days for all patients, and 47 and 81 days for the T/NK cell LAHS and B cell LAHS cohorts, respectively (P = 0.025). Among the patients evaluated, the ORR was 42.2%. Patients starting with anti-lymphoma treatment had a better, albeit not significant, ORR than those beginning with anti-HPS treatment. In the univariate analysis, T/NK cell LAHS (P = 0.027), HPS onset at relapse (P = 0.036), higher baseline plasma EBV-DNA levels (> 4,000 copies/mL, P = 0.034), and treatments including cytokine adsorption and ruxolitinib (P < 0.001 and P = 0.017, respectively) were potentially associated with worse OS, while corticosteroid therapy benefited OS. In the multivariate analysis, T/NK cell LAHS (adjusted hazard ratio (aHR) = 2.007), cytokine adsorption therapy (aHR = 4.547), and corticosteroid therapy (aHR = 0.118) were independently associated with mortality. T/NK cell lymphoma was the main cause of LAHS and carried a worse prognosis. Whether anti-lymphoma or anti-HPS treatment should start first still requires prospective studies with larger sample sizes. The key point in controlling HPS is to block the cytokine storm promptly. Corticosteroid therapy is both effective and accessible and should be used early and in sufficient quantities.

暂无摘要。

BACKGROUND: Polycystic ovary syndrome (PCOS) is a prevalent endocrine disorder affecting women of reproductive ages. Our previous study has implicated a possible link between RNA editing and PCOS, yet the actual role of RNA editing, its association with clinical features, and the underlying mechanisms remain unclear.
METHODS: Ten RNA-Seq datasets containing 269 samples of multiple tissue types, including granulosa cells, T helper cells, placenta, oocyte, endometrial stromal cells, endometrium, and adipose tissues, were retrieved from public databases. Peripheral blood samples were collected from twelve PCOS and ten controls and subjected to RNA-Seq. Transcriptome-wide RNA-Seq data analysis was conducted to identify differential RNA editing (DRE) between PCOS and controls. The functional significance of DRE was evaluated by luciferase reporter assays and overexpression in human HEK293T cells. Dehydroepiandrosterone and lipopolysaccharide were used to stimulate human KGN granulosa cells to evaluate gene expression.
RESULTS: RNA editing dysregulations across multiple tissues were found to be associated with PCOS in public datasets. Peripheral blood transcriptome analysis revealed 798 DRE events associated with PCOS. Through weighted gene co-expression network analysis, our results revealed a set of hub DRE events in PCOS blood. A DRE event in the eukaryotic translation initiation factor 2-alpha kinase 2 (EIF2AK2:chr2:37,100,559) was associated with PCOS clinical features such as luteinizing hormone (LH) and the ratio of LH over follicle-stimulating hormone. Luciferase assays, overexpression, and knockout of RNA editing enzyme adenosine deaminase RNA specific (ADAR) showed that the ADAR-mediated editing cis-regulated EIF2AK2 expression. EIAF2AK2 showed a higher expression after dehydroepiandrosterone and lipopolysaccharide stimulation, triggering changes in the downstrean MAPK pathway.
CONCLUSIONS: Our study presented the first evidence of cross-tissue RNA editing dysregulation in PCOS and its clinical associations. The dysregulation of RNA editing mediated by ADAR and the disrupted target EIF2AK2 may contribute to PCOS development via the MPAK pathway, underlining such epigenetic mechanisms in the disease.

UNASSIGNED: Bloodstream infection (BSI) is characterized by high mortality, especially among these increasing super-elderly patients (≥85 years), and this study was conducted to understand the species distribution, typical clinical features and risk factors for poor prognosis of super-elderly patients with BSI.
UNASSIGNED: Based on previous work, this retrospective study was performed by reviewing an ongoing prospective medical database in a comprehensive tertiary center in China, and all super-elderly patients with BSI in the past 6 years were enrolled in this study.
UNASSIGNED: Out of 5944 adult-patients with BSI, there were totally 431 super-elderly patients (≥85 years old) enrolled in this study and age ≥90 years accounted for 31.1% (134/431). Among these 431 super-elderly patients with BSI, 40 patients (9.3%) were diagnosed with BSI and the remained 401 super-elderly patients (90.7%) were defined as hospital-acquired BSI. The typical feature of these super-elderly patients with BSI was the high proportion of patients with various comorbidities, such as cardiovascular disease (83.8%), ischemic cerebrovascular disease (63.3%) and pulmonary infection (61.0%). The other typical feature was that most (60.1%) of these patients had been hospitalized for long time (≥28 days) prior to the onset of BSI, and most patients had received various invasive treatments, such as indwelling central venous catheter (53.1%) and indwelling urinary catheter (47.1%). Unfortunately, due to these adverse features above, both the 7-day short-term mortality (13.2%, 57/431) and the 30-day long-term mortality (24.8%, 107/431) were high. The multivariate analysis showed that both chronic liver failure (OR 7.9, 95% CI 2.3-27.8, P=0.001) and indwelling urinary catheter (OR 2.3, 95% CI 1.1-4.7, P=0.023) were independent risk factors for 7-day short-term mortality, but not for 30-day long-term mortality. In addition, the microbiology results showed that the most common species were associated with nosocomial infection or self-opportunistic infection, such as Staphylococcus hominis (18.3%), Staphylococcus epidermidis (11.8%), Escherichia coli (9.7%), Klebsiella pneumoniae (9.3%) and Candida albicans (8.6%, fungi).
UNASSIGNED: Super-elderly patients with BSI had typical features, regardless of the pathogenic species distribution and their drug resistance, or clinical features and their risk factors for poor prognosis. These typical features deserved attention and could be used for the prevention and treatment of BSI among super-elderly patients.

OBJECTIVE: To analyze the epidemiological characteristics of newly reported advanced schistosomiasis cases in Sichuan Province, so as to provide the evidence for analyzing the causes and formulating targeted control measures of newly reported advanced schistosomiasis cases.
METHODS: Individual case investigation forms for advanced schistosomiasis cases were collected from the Sichuan Provincial Epidemic Annual Report System from 2011 to 2022, and patients\' demographics, previous medical history and liver parenchymal grading were retrieved. All advanced schistosomiasis cases\' medical records were reviewed, and the subtypes of schistosomiasis-endemic villages where the cases\' household registration were, floating population, survival and death and time of death were collected.
RESULTS: A total of 321 newly reported advanced schistosomiasis cases were found in Sichuan Province from 2011 to 2022, with a male to female ratio of 0.99 to 1. There were 274 cases at ages of over 50 years (85.4%), with the highest proportion seen at ages of 60 to 69 years (87 cases, 27.1%), and splenomegaly was the most common type (180 cases, 56.1%), with no dwarfism type detected. The highest number of cases was reported in 2011 (78 cases), followed by in 2022 (74 cases), and the highest number of cases were reported in Meishan City (199 cases, 62.0%), Dongpo District (131 cases, 40.8%), and hilly subtype areas (136 cases, 42.4%). As of the end of 2022, there were 111 deaths due to advanced schistosomiasis, with the highest number of deaths seen in 2018 (25 deaths), and the highest mortality was seen among patients with the ascites type (41.2%). There were 47 (37.3%), 40 (59.5%) and 4 (23.5%) cases with grade III liver parenchyma among patients with splenomegaly, ascites, and colonic proliferation types, respectively, and there was a significant difference in the grading of III liver parenchyma among three types of patients (H = 12.092, P < 0.05), with more severe liver parenchyma injuries seen among patients with the ascites type than among those with splenomegaly and colonic proliferation type (Z = 24.262 and 44.738, both Padjusted values < 0.05).
CONCLUSIONS: There have been newly reported advanced schistosomiasis cases in Sichuan Province during recent years, and patients with the ascites type should be given a high priority among advanced schistosomiasis cases in Sichuan Province. Intensified clue surveys are needed for early identification and treatment of advanced schistosomiasis cases, so as to increase the survival rate and improve the quality of life.
［摘要］ 目的 分析四川省新报告晚期血吸虫病 (晚血) 病例流行病学和临床特征, 为分析新发晚血成因、制定有针对性 的防治措施提供依据。方法 收集2011—2022年四川省疫情年报系统中的晚血病例个案调查表, 获取病例个人基本信 息、既往病史、肝实质分级情况; 通过查阅晚血病例资料, 获取病例户籍所在流行村流行亚型、是否为流动人口、存活及死 亡情况、死亡时间等, 并进行描述性统计分析。结果 2011—2022年四川省累计新报告晚血病例共321例, 男女比例为 0.99:1; 274例 (85.4%) 年龄> 50岁, 以60~69岁组占比最高 (87例, 27.1%); 病例类型以巨脾型最多 (180例, 56.1%), 无侏 儒型病例; 2011年报告病例数最多 (78例), 其次为2022年 (74例)。2011—2022年报告晚血病例数最多的市 (州)、县 (区) 分别为眉山市 (199例, 62.0%) 和东坡区 (131例, 40.8%), 报告病例数最多的流行亚型为丘陵亚型地区 (136例, 42.4%)。截至2022年底, 321例晚血病例中有111例死亡, 其中2018年死亡人数最多 (25例), 腹水型病例死亡率最高 (41.2%)。巨脾型、腹水型、结肠增殖型病例中, 肝实质分级为Ⅲ级的病例数分别为47 (37.3%)、40 (59.5%)、4例 (23.5%), 3种类型晚血病例肝实质分级严重程度差异有统计学意义 (H = 12.092, P < 0.05), 腹水型晚血病例肝实质分级较巨脾型 和结肠增殖型病例严重 (Z = 24.262、44.738, P校正均< 0.05)。结论 近年来, 四川省每年仍有新报告晚血病例, 腹水型病 例是四川省晚血病例救治重点。应加强对重点人群的线索调查, 及早发现、治疗晚血病例, 提高其存活率和生命质量。.

OBJECTIVE: To explore the value of magnetic resonance imaging (MRI) and clinical features in identifying ovarian thecoma-fibroma (OTF) with cystic degeneration and ovary adenofibroma (OAF).
METHODS: A total of 40 patients with OTF (OTF group) and 28 patients with OAF (OAF group) were included in this retrospective study. Univariable and multivariable analyses were performed on clinical features and MRI between the two groups, and the receiver operating characteristic (ROC) curve was plotted to estimate the optimal threshold and predictive performance.
RESULTS: The OTF group had smaller cyst degeneration degree (P < .001), fewer black sponge sign (20% vs. 53.6%, P = .004), lower minimum apparent diffusion coefficient value (ADCmin) (0.986 (0.152) vs. 1.255 (0.370), P < .001), higher age (57.4 ± 14.2 vs. 44.1 ± 15.9, P = .001) and more postmenopausal women (72.5% vs. 28.6%, P < .001) than OAF. The area under the curve of MRI, clinical features and MRI combined with clinical features was 0.870, 0.841, and 0.954, respectively, and MRI combined with clinical features was significantly higher than the other two (P < .05).
CONCLUSIONS: The cyst degeneration degree, black sponge sign, ADCmin, age and menopause were independent factors in identifying OTF with cystic degeneration and OAF. The combination of MRI and clinical features has a good effect on the identification of the two.
CONCLUSIONS: This is the first time to distinguish OTF with cystic degeneration from OAF by combining MRI and clinical features. It shows the diagnostic performance of MRI, clinical features, and combination of the two. This will facilitate the discriminability and awareness of these two diseases among radiologists and gynaecologists.

OBJECTIVE: To describe the clinical, electrophysiological, and genetic features of an unusual case with an RDH12 homozygous pathogenic variant and reviewed the characteristics of the patients reported with the same variant.
METHODS: The patient underwent a complete ophthalmologic examination including best-corrected visual acuity, anterior segment and dilated fundus, visual field, spectral-domain optical coherence tomography (OCT) and electroretinogram (ERG). The retinal disease panel genes were sequenced through chip capture high-throughput sequencing and Sanger sequencing was used to confirm the result. Then we reviewed the characteristics of the patients reported with the same variant.
RESULTS: A 30-year male presented with severe early retinal degeneration who complained night blindness, decreased visual acuity, vitreous floaters and amaurosis fugax. The best corrected vision was 0.04 OD and 0.12 OS, respectively. The fundus photo and OCT showed bilateral macular atrophy but larger areas of macular atrophy in the left eye. Autofluorescence shows bilateral symmetrical hypo-autofluorescence. ERG revealed that the amplitudes of a- and b-wave were severely decreased. Multifocal ERG showed decreased amplitudes in the local macular area. A homozygous missense variant c.146C>T (chr14:68191267) was found. The clinical characteristics of a total of 13 patients reported with the same pathologic variant varied.
CONCLUSIONS: An unusual patient with a homozygous pathogenic variant in the c.146C>T of RDH12 which causes late-onset and asymmetric retinal degeneration are reported. The clinical manifestations of the patient with multimodal retinal imaging and functional examinations have enriched our understanding of this disease.

UNASSIGNED: The highly infectious Omicron variant of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have caused large-scale transmission from Dec 2022 to Feb 2023 in China. After this event, a remarkable surge of influenza A (Flu A) occurred from March to May 2023, especially in pediatric patients.
UNASSIGNED: This study aimed to investigate the differences between pediatric patients infected with COVID-19 Omicron and Flu A virus.
UNASSIGNED: A total of 1,063 hospitalized children who admitted into two tertiary general hospital of Guangdong province of China were included. Medical records were compared retrospectively in these patients during the pandemic periods of SARS-CoV-2 omicron and Flu A.
UNASSIGNED: A total of 592 Patients with Flu A were mostly preschool and school-aged (>3y, 76.0%), they showed higher ratio of high fever (≥39°C), cough, rhinorrhea, and vomiting than patients with SARS-CoV-2 omicron. Most of the 471 Omicron patients were young children (0-3y, 74.5%) and had more poor appetite and dyspnea symptom. Benign acute children myositis (BACM) was only observed in patients with Flu A, and a significant male predominance. Multisystem inflammatory syndrome in children (MIS-C) was only found in patients with SARS-CoV-2 Omicron. Compared to the SARS-CoV-2 Omicron group, for both age groups (0-3 years and > 3 years), the Flu A group showed significantly reduced lymphocyte (Lym) counts (P < 0.001), and elevated levels of aspartate aminotransferase (AST), lactate dehydrogenase (LDH), and creatinine kinase-MB (CK-MB) in laboratory indexes (all P < 0.001). Additionally, it was found that more children hospitalized with COVID-19 had increased C-reactive protein (CRP) levels compared to those with Flu A.
UNASSIGNED: Influenza A infections have notably surged in children, coinciding with the relaxation of COVID-19 related social restrictions. During the epidemic periods of Omicron and Flu A virus infection, different clinical and laboratory characteristics were observed in hospitalized children.

The present study aimed to investigate the association between the expression profiles of long non-coding RNAs (lncRNAs) and the clinical characteristics or prognosis of patients with nasopharyngeal carcinoma (NPC). The findings presented in the present review may provide novel strategies for the prevention and treatment of NPC. For the analyses, medical databases, including PubMed, Web of Science and Cochrane library were searched using specific search terms, search strategies and screening strategies. Endnote X9 document management software was then employed to extract documents from January, 2010 to May, 2023. Data were extracted following the prescribed standards. Review Manager 5.4 and STATA 12.0 data analysis software were used for data analysis. A total of 490 publications were analyzed for inclusion. In total, 29 publications, composed of 24 studies with upregulated lncRNAs and 5 studies with downregulated lncRNAs, were included in the final analysis. The analysis revealed that the upregulation of lncRNAs was significantly associated with T stage, N stage and clinical stage, as well as with the overall survival (OS) and disease-free survival (DFS) of patients with NPC (P<0.05). However, there was no significant association between the upregulated lncRNAs and sex, M stage or relapse-free survival (RFS) (P>0.05). On the other hand, the suppression of lncRNA expression was significantly associated with N stage, M stage, clinical stage and the OS of patients with NPC (P<0.05), but not with T stage and RFS (P>0.05). Taken together, the present review demonstrates that the up- and downregulation of different lncRNAs was associated with an advanced clinical stage and a shorter OS of patients with NPC. Therefore, lncRNAs may serve as potential prognostic factors in NPC.

Clear cell meningiomas are a rare histological subtype of World Health Organization (WHO) grade II meningioma. Despite its relatively low frequency, clear cell meningioma has attracted considerable attention because of its unique pathological characteristics, clinical behavior, and challenging management considerations. The purpose of our systematic review is to provide clinicians with a better understanding of this rare disease. PubMed was searched for articles in the English language published from 1988 to 2023 June. The keywords were as follows: \"clear cell meningioma,\" \"clear cell\" and \"meningioma.\" We analyzed clinical manifestations, radiological manifestations, pathological features, comprehensive treatment strategies, and prognosis to determine the factors influencing recurrence-free survival (RFS). Recurrence-free survival curves of related factors were calculated by the Kaplan‒Meier method. The log-rank test and Cox univariate analysis were adopted to assess the intergroup differences and seek significant factors influencing prognosis and recurrence. Fifty-seven papers met the eligibility criteria, including 207 cases of clear cell meningioma (CCM), which were confirmed by postoperative pathology. The fifty-seven articles involved 84 (40.6%) males and 123 (59.4%) females. The average age at diagnosis was 27.9 years (range, 14 months to 84 years). Among the symptoms observed, headache, neurologic deficit, and hearing loss were the most commonly reported clinical manifestations. Most tumors (47.8%) were located in the skull base region. Most tumors showed significant enhancement, and homogeneous enhancement was more common. A total of 152 (74.1%) patients underwent gross total resection (GTR), and 53 (25.9%) patients underwent subtotal resection (STR). During the follow-up, the tumor recurred in 80 (39.4%) patients. The log-rank test and the Cox univariate analysis revealed that tumor resection range (GTR vs. STR) and adjuvant treatment (YES vs. NO) were significant predictors of recurrence-free survival (RFS). Clear cell meningioma is a rare type of meningioma with challenging diagnosis and therapy. The prognosis of this disease is different from that of regular meningiomas. Recurrence remains a possibility even after total tumor resection. We found that the surgical resection range and adjuvant treatment affected the recurrence period. This finding provides significant guidance for the treatment of clear cell meningioma.