Category: Nevin Manimala

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2024 Oct;32(5):1524-1530. doi: 10.19746/j.cnki.issn.1009-2137.2024.05.034.

ABSTRACT

OBJECTIVE: To explore the clinical application value of the Bleeding Scoring Scales (2019 Pediatric ITP Scale) in the diagnosis and treatment of children with primary immune thrombocytopenia (ITP).

METHODS: A total of 422 children with ITP were evaluated with the 2019 Pediatric ITP Scale and the 2013 ITP-BAT and their clinical data were analyzed. The correlation between the two bleeding scoring scales and disease stage, platelet count was analysed, the evaluation time, consistency of the two bleeding scoring scales was compared, and the correlation of the two methods. The changes of platelet count and the score of 2019 Pediatric ITP Scale before treatment and after treatment at 48 h and one week were analyzed to detect responsiveness of the 2019 Pediatric ITP Scale.

RESULTS: The score of the 2019 Pediatric ITP Scale was mainly one point and two points, the corresponding bleeding was skin and mucosal bleeding.404 patients (95.7%) had bleeding manifestations, including 249 patients of skin bleeding (59.0%), 144 patients of mucosal bleeding (34.1%), and 11 patients of organ bleeding (2.6%), of which 2 patients were severe bleeding. The two bleeding scoring scales were both negatively correlated with platelet counts in children with ITP (r _s=-0.5032, r _s=-0.6084) and no correlation with the stage of pediatric ITP(P ＞0.05). The 2019 Pediatric ITP Scale had good consistency with the 2013 ITP-BAT (r _s=0.7638). The average time required for the 2019 Pediatric ITP Scale was 88.64 (40-181) seconds, which was lower than that required for the 2013 ITP-BAT 104.12 (47-285) seconds (Z =17.792, P < 0.001). The 2019 Pediatric ITP Scale can well reflect the treatment of pediatric ITP. There were statistically significant differences in platelet count before treatment and after treatment at 48 h and one week among steroid group, IVIG group and steroid combined with IVIG group ( P < 0.05). There were also statistically significant differences in the score of the 2019 Pediatric ITP Scale before treatment and after treatment at one week among the three groups ( P < 0.05).

CONCLUSION: The 2019 Pediatric ITP Scale has good consistency and sensitivity in clinical application, and it takes less time to complete than the 2013 ITP-BAT, this scale can be used as an effective tool for disease assessment and efficacy determination in pediatric primary immune thrombocytopenia.

PMID:39479842 | DOI:10.19746/j.cnki.issn.1009-2137.2024.05.034

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2024 Oct;32(5):1463-1471. doi: 10.19746/j.cnki.issn.1009-2137.2024.05.025.

ABSTRACT

OBJECTIVE: To explore and analyze the incidence rate, influencing factors and impact on prognosis of pulmonary hypertension (PH) in patients with Philadelphia chromosome negative myeloproliferative neoplasms (Ph^– MPNs).

METHODS: The clinical data of 271 patients with Ph^– MPNs were retrospectively analyzed, and different disease subtypes were classified. Patients with different disease types were further divided into PH⁺ and PH^– groups according to whether HP occurred. Statistical methods were used to analyze the incidence rate, risk factors, and impact on prognosis of PH in Ph^– MPNs patients.

RESULTS: The overall incidence rate of PH among 271 patients was 26.9%, and according to the classification of disease subtypes, it was found that the incidence rate of PH in patients with primary myelofibrosis (PMF) was significantly higher than those of patients with polycythemia vera and essential thrombocythemia (both P <0.05). Multivariate regression analysis showed that advanced age, long disease course, JAK2 positive and increased hematocrit, lactate dehydrogenase, monocyte count, and uric acid level were independent risk factors for PH in Ph^– MPNs patients (OR >1, P <0.05), and there were some differences in the independent risk factors between different disease subtypes. Survival analysis results showed that the overall survival (OS) rate of PH⁺ patients was significantly lower than that of PH^– patients in other types except for PMF (all P <0.05).

CONCLUSION: The incidence rate of PH in Ph^– MPNs patients is high, and its risk factors are diverse. The OS rate of Ph^– MPNs patients with PH is low. Therefore, we should be highly alert to the occurrence of PH in Ph^– MPNs patients clinically.

PMID:39479833 | DOI:10.19746/j.cnki.issn.1009-2137.2024.05.025

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2024 Oct;32(5):1407-1413. doi: 10.19746/j.cnki.issn.1009-2137.2024.05.016.

ABSTRACT

OBJECTIVE: To explore the clinical characteristics and prognosis of patients with diffuse large B-cell lymphoma (DLBCL) of the breast.

METHODS: The clinical data of 28 DLBCL patients admitted to Shanxi Provincial Cancer Hospital from January 2013 to January 2023 were retrospectively analysed, including 13 cases of primary breast DLBCL (PB-DLBCL) and 15 cases of secondary breast DLBCL (SB-DLBCL), and the data of their clinical manifestations, laboratory tests, pathological examinations, treatment protocols, and follow-up were statistically analyzed.

RESULTS: There were significant differences in IPI score, LDH level and β₂– microglobulin between PB-DLBCL and SB-DLBCL patients (P < 0.05). Among the 23 patients with breast DLBCL who received regular treatment, 13 patients achieved complete remission (9 patients with PB-DLBCL and 4 patients with SB-DLBCL) after initial treatment. By the end of follow-up, 11 patients relapsed or progressed (5 patients with PB-DLBCL and 6 patients with SB-DLBCL) and 9 patients died (3 patients with PB-DLBCL and 6 patients with SB-DLBCL). The 5-year OS rate was (75.0±15.3)% in PB-DLBCL group and (32.3±17.1)% in SB-DLBCL group. The 5-year PFS rate was （59.1±19.8）% in PB-DLBCL and 0% in SB-DLBCL group. The 5-year OS rate and PFS rate of PB-DLBCL patients were higher than those of SB-DLBCL patients (P < 0.05); the 5-year OS rate of the combined central preventive treatment group was higher than that of the chemotherapy group (P < 0.05).

CONCLUSION: Breast DLBCL is divided into two categories: PB-DLBCL and SB-DLBCL. Compared with SB-DLBCL, PB-DLBCL has the characteristics of lower IPI score, LDH, and β₂-microglobulin levels. PB-DLBCL patients have a longer survival period. In addition, the prognosis of patients receiving central preventive treatment is more optimistic.

PMID:39479824 | DOI:10.19746/j.cnki.issn.1009-2137.2024.05.016

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2024 Oct;32(5):1381-1387. doi: 10.19746/j.cnki.issn.1009-2137.2024.05.012.

ABSTRACT

OBJECTIVE: To investigate the clinical characteristics and prognosis of single center adult chronic myeloid leukemia in chronic phase (CML-CP).

METHODS: Clinical data of 41 adult CML-CP patients in Department of Hematology, Shanghai Fengxian District Central Hospital from January 2015 to May 2021 were retrospectively analyzed. The clinical characteristics and prognosis of patients between <60 years group and ≥60 years group were compared.

RESULTS: The 41 patients included 27 (65.9%) males and 14 (34.1%) females. The median age of the patients was 56(19-84) years, with 22 cases (53.7%) <60 years and 19 cases (46.3%) ≥60 years. Univariate analysis indicated that the proportions of patients with comorbidities, intermediate/high-risk Sokal score, myelofibrosis, and lactate dehydrogenase ≥1 000 U/L were significantly increased in ≥60 years group compared with <60 years group at initial diagnosis (all P <0.05). There were no statistical differences in the distribution of sex, ELST score, white blood cell count, platelet count, peripheral blood basophil percentage, peripheral blood eosinophil percentage and bone marrow primitive cell percentage between the two groups (P >0.05). The proportion of patients taking reduced-dose imatinib in ≥60 years group significantly increased (P <0.001). Patients <60 years had a higher proportion of molecular biological remission after treatment of tyrosine kinase inhibitors (TKIs) than patients ≥60 years (P <0.001). The incidence of non-hematologic adverse reactions to TKI therapy significantly increased in patients ≥60 years (P <0.001). Multivariate analysis showed that no adverse factors affecting the efficacy and prognosis of TKI.

CONCLUSION: Compared with adult CML-CP patients <60 years, patients ≥60 years gain fewer benefits from TKI treatment and increased adverse reactions.

PMID:39479820 | DOI:10.19746/j.cnki.issn.1009-2137.2024.05.012

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2024 Oct;32(5):1356-1364. doi: 10.19746/j.cnki.issn.1009-2137.2024.05.008.

ABSTRACT

OBJECTIVE: To analyze the clinical characteristics and prognosis of children with hypodiploid B-cell precursor acute lymphoblastic leukemia (BCP-ALL).

METHODS: The clinical data of 1 287 children with BCP-ALL admitted to five hospital in Fujian province from April 2011 to December 2020 were retrospectively analyzed. According to the results of chromosome karyotype, all the patients were grouped into hypodiploid subgroup and non-hypodiploid subgroup. The clinical characteristics, early treatment response ［minimal residual disease (MRD) on middle stage of induction chemotherapy and end of induction chemotherapy］ and long-term efficacy ［overall survival (OS) and event-free survival (EFS)］ were compared. The prognostic factors of hypodiploid BCP-ALL were further explored.

RESULTS: Among 1 287 BCP-ALL patients， 28 patients (2.2%) were hypodiploid BCP-ALL. The proportion of patients with white blood cell count （WBC）≥50×10⁹/L in the hypodiploid subgroup was significantly higher than that in the non-hypodiploid subgroup (P =0.004), while there was no statistically significant difference in gender ratio, age group at initial diagnosis, and early treatment response between the two groups (all P >0.05). The 5-year EFS and OS rate of the hypodiploid subgroup were 75.0%(95%CI ：66.8%-83.2%) and 77.8%(95%CI ：69.8%-85.8%), respectively, which were lower than those of non-hypodiploid subgroup ［EFS: 79.6%(95%CI ：78.4%-80.8%); OS: 86.4%(95%CI ：85.4%-87.5%)］, but the difference was not statistically significant (all P >0.05). Further subgroup analysis by risk stratification showed that the 5-year EFS and OS rates of the hypodiploid subgroup were significantly lower than those in the low-risk (LR) group ［LR group EFS: 91.4% (95%CI ：88.4%-93.6% ), P < 0.001; OS: 94.7% (95%CI ：92.1%-96.4%), P < 0.001］ ; it was similar to that of BCP-ALL children stratified into intermediate-risk (IR) excluding hypodiploid ［IR group EFS: 79.4%(95%CI ：74.9%-83.2%), P =0.343; OS: 87.3%(95%CI ：83.6%-90.2%), P =0.111］; while was higher than that of EFS in HR group, but the difference was not statistically significant ［HR group EFS: 58.7%(95%CI ：52.6%-64.8%), P =0.178. OS: 69.9%(95%CI ：63.5%-75.4%), P =0.417］. Univariate analysis showed that gender, age, white blood cell count, and MRD on middle stage of induction chemotherapy had no significant impact on OS and EFS; chromosome count< 40 was a risk factor for lower OS (P =0.026), but has no significant effect on EFS; MRD≥0.01% after induction therapy was a risk factor for lower OS and EFS (P =0.002, and 0.001, respectively).

CONCLUSION: Children with hypodiploid BCP-ALL have an intermediate prognosis, and MRD ≥0.01% after induction chemotherapy may be a risk factors for poor prognosis.

PMID:39479816 | DOI:10.19746/j.cnki.issn.1009-2137.2024.05.008

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2024 Oct;32(5):1334-1342. doi: 10.19746/j.cnki.issn.1009-2137.2024.05.005.

ABSTRACT

OBJECTIVE: To establish a morphologic classification system for characterizing blast cells in patients with acute promyelocytic leukemia (APL) and analyze the correlation of different APL morphologic characteristics with conventional tests and genetic variants.

METHODS: Based on the morphological characteristics of APL blast cells, a classification system of 14 categories was established to characterize the inter- and intra-individual cellular morphological heterogeneity of patients. The classification system was used for the morphological analysis of 40 APL patients, and the classification results were statistically analyzed with the patients’ conventional test indexes and gene variant characteristics to analyze the correlation of different APL blast cell morphological features with conventional test indexes and gene variants.

RESULTS: In the FLT3-ITD mutation-positive group, there were significantly fewer cells with regular nuclear shape, hyper granularity, and missing Auer rods (category 1) than in the FLT3 mutation-negative group (P < 0.05). The activated partial thromboplastin (APTT) was significantly longer in the group with regular nucleus compared to the group with irregular nucleus (P < 0.05). In the hypo-granular group, the APTT was also significantly longer compared to the hyper-granular group (P < 0.01), and the proportion of myeloid blast cells was relatively lower (P < 0.05). The peripheral blood white blood cell counts, D-dimer, lactate dehydrogenase and proportion of bone marrow blast cells were significantly higher in the Auer rods (-) group than Auer rods increasing group (all P < 0.05).

CONCLUSION: The newly established morphologic classification system in this study can objectively characterize different types of APL blast cells, which helps to better assess the intra- and inter-individual heterogeneity of APL blast cells, and further use in accurately analyzing the correlation of morphological phenotypes with biological properties of APL.

PMID:39479813 | DOI:10.19746/j.cnki.issn.1009-2137.2024.05.005

Hum Reprod. 2024 Oct 30:deae243. doi: 10.1093/humrep/deae243. Online ahead of print.

ABSTRACT

STUDY QUESTION: What are the reproductive outcomes of patients who cryopreserved oocytes or embryos in the context of fertility preservation in the Netherlands?

SUMMARY ANSWER: This study shows that after a 10-year follow-up period, the utilization rate to attempt pregnancy using cryopreserved oocytes or embryos was 25.5% and the cumulative live birth rate after embryo transfer was 34.6% per patient.

WHAT IS KNOWN ALREADY: Fertility preservation by freezing oocytes or embryos is an established treatment for women with a risk of premature ovarian failure (caused by a benign or oncological disease) or physiological age-related fertility decline. Little is known about the success of cryopreservation, the utilization rate of oocytes or embryos, or the live birth rates.

STUDY DESIGN, SIZE, DURATION: A retrospective observational study was performed in the Netherlands. Data were collected between 2017 and 2019 from 1112 women who cryopreserved oocytes or embryos more than 2 years ago in the context of fertility preservation in 10 IVF centers in the Netherlands.

PARTICIPANTS/MATERIALS, SETTING, METHODS: A total of 1112 women were included in this study. Medical files and patient databases were used to extract data. Women were categorized based on indication of fertility preservation: oncological, benign, or non-medical. To indicate statistical differences the t-test or Mann-Whitney U test was used. Kaplan-Meier analyses were used for time endpoints, and log-rank analyses were used to assess statistical differences. The study protocol was approved by the medical ethics committee.

MAIN RESULTS AND THE ROLE OF CHANCE: Fertility preservation cycles have been performed increasingly over the years in the Netherlands. In the first years, less than 10 cycles per year were performed, increasing to more than 300 cycles per year 10 years later. Initially, embryos were frozen in the context of fertility preservation. In later years, cryopreservation of oocytes became the standard approach. Cryopreservation of oocytes versus embryos resulted in comparable numbers of used embryos (median of 2) for transfer and comparable live birth rates (33.9% and 34.6%, respectively). The 5-year utilization rate was 12.3% and the 10-year utilization rate was 25.5%. The cumulative clinical pregnancy rate was 35.6% and the cumulative live birth rate was 34.6% per patient. Those who had fertility preservation due to benign diseases returned earlier to use their cryopreserved embryos or oocytes.

LIMITATIONS, REASONS FOR CAUTION: The follow-up period after the fertility preservation procedure varied between patients in this study and not all frozen oocytes or embryos had been used at the end of this study. This might have led to underestimated outcomes reported in this study. Furthermore, intention to treat cannot be fully determined since women who started the fertility preservation procedure without success (cancellation due to low response) were not included in this study.

WIDER IMPLICATIONS OF THE FINDINGS: This study provides data on the reproductive outcomes after various indications of fertility preservation. This knowledge can be informative for professionals and future patients to improve counseling and informed decision making regarding ovarian stimulation in the context of fertility preservation.

STUDY FUNDING/COMPETING INTEREST(S): No funding was obtained for this study. The authors have no conflicts of interest to declare related to this study. V.T.H. received grants paid to the institute for studies outside the present work from AstraZeneca, Gilead, Novartis, Eli Lily, Pfizer, and Daiichi Sankyo. V.T.H. received consulting fees from Eli Lily outside the present work. M.G. received grants paid to the institute for studies outside the present work from Guerbet and Ferring. E.M.E.B. received a grant from The Dutch Network of Fertility Preservation for a study outside the present work.

TRIAL REGISTRATION NUMBER: N/A.

PMID:39479806 | DOI:10.1093/humrep/deae243

Am J Epidemiol. 2024 Oct 30:kwae411. doi: 10.1093/aje/kwae411. Online ahead of print.

ABSTRACT

The COVID-19 pandemic’s global impact has been devastating, causing millions of deaths. Our study investigates excess sepsis-related mortality trends over three years during the pandemic. Using CDC’s National Vital Statistics System data from January 2018 to March 2023, we projected sepsis-related deaths during the pandemic using a Poisson log-linear regression model. We compared observed versus predicted deaths and analyzed temporal trends by demographics and regions. Among the 753,160 deaths documented between March 2020 and March 2023, a significant downward trend was noted in sepsis-related mortality rates from March 2022 to March 2023, coinciding with the surge of the Omicron variant. The excess mortality rates were 170.6 per million persons (95% CI: 168.2-172.6), 167.5 per million persons (95% CI: 163.6-170.9), and 73.3 per million persons (95% CI: 69.4-76.6) in the first, second, and third years, respectively. Increased sepsis-related mortality was observed across all age subgroups, with the greatest increase noted in those aged 85 years and above compared to middle- and young-aged decedents. Disparities were also observed across racial/ethnic, sex/gender subgroups, and geographic regions. This study highlights the effectiveness of current policies and prevention measures in response to the long-term circulating of SARS-CoV-2 in the community.

PMID:39479803 | DOI:10.1093/aje/kwae411

Thyroid. 2024 Oct 31. doi: 10.1089/thy.2024.0310. Online ahead of print.

ABSTRACT

Background: The coexistence of v-Raf murine sarcoma viral oncogene homolog B1 (BRAF^V600E) and telomere reverse transcriptase promoter (TERT-p) mutations is considerably associated with aggressiveness and poor prognosis in papillary thyroid carcinoma (PTC). However, the association between gross findings and genetic alterations in PTC remains unknown. We aimed to investigate the association between clinicopathologic features, including macroscopic features, and the coexistent BRAF^V600E and TERT-p mutations in patients with PTC. Methods: We retrospectively analyzed 375 cases of PTC surgically resected between January 2018 and October 2023 at a single institution, based on the presence of BRAF^V600E and TERT-p double mutation. Clinicopathologic features, including gross features on the cut surface of tumors, were evaluated. Subsequently, the association between clinicopathologic features and mutation status was statistically examined. Cox proportional hazard models were used to analyze the impact of molecular pathological features on disease-free survival (DFS). Results: The BRAF^V600E and TERT-p double mutation was identified in 78 (20.8%) patients among the PTC cases and was significantly correlated with shorter DFS. Multivariable analysis revealed that factors such as relatively older age (≥55 years) (odds ratio [OR] = 12.083, 95% confidence interval [CI] 4.498-32.456), larger tumor size (>2.0 cm) (OR = 2.722, CI 1.104-6.712), lobulated tumor margins (OR = 16.114, CI 3.155-82.296), papillary excrescences on the cut surface (OR = 17.573, CI 3.462-89.201), solid-cut surface (OR = 4.012, CI 1.084-14.849), minimal extrathyroidal extension (ETE) (OR = 4.156, CI 1.209-14.282), gross ETE (OR = 6.517, CI 1.734-24.490), and Ki-67 labeling index (LI) (≥5%, OR = 12.145, CI 4.354-33.877) were significantly associated with the double mutation. Conclusions: The BRAF^V600E and TERT-p double mutation in PTC was significantly associated with relatively old age, larger tumor size, lobulated configuration in tumor margin, papillary excrescences on the cut surface, solid-cut surface, ETE, and high Ki-67 LI. These features are suggestive of the presence of the double mutation and should be analyzed at the molecular level in patients with PTC.

PMID:39479802 | DOI:10.1089/thy.2024.0310

J Neurochem. 2024 Oct 31. doi: 10.1111/jnc.16254. Online ahead of print.

ABSTRACT

Evidence from observational and Mendelian randomization (MR) studies suggested that insulin resistance (IR) was associated with Alzheimer’s disease (AD). However, the causal effects of different indicators of IR on AD remain inconsistent. Here, we aim to assess the causal association between the insulin sensitivity index (ISI), a measure of post-prandial IR, and the risk of AD. We first conducted primary and secondary univariable MR analyses. We selected 8 independent genome-wide significant (p < 5E-08, primary analyses) and 61 suggestive (p < 1E-05, secondary analyses) ISI genetic variants from large-scale genome-wide association studies (GWAS; N = 53 657), respectively, and extracted their corresponding GWAS summary statistics from AD GWAS, including IGAP2019 (N = 63 926) and FinnGen_G6_AD_WIDE (N = 412 181). We selected five univariable MR methods and used heterogeneity, horizontal pleiotropy test, and leave-one-out sensitivity analysis to confirm the stability of MR estimates. Finally, we conducted a meta-analysis to combine MR estimates from two non-overlapping AD GWAS datasets. We further performed multivariable MR (MVMR) to assess the potential mediating role of type 2 diabetes (T2D) on the association between ISI and AD using two MVMR methods. In univariable MR, utilizing 8 genetic variants in primary analyses, we found a significant causal association of genetically increased ISI with decreased risk of AD (OR = 0.79, 95% CI: 0.68-0.92, p = 0.003). Utilizing 61 genetic variants in secondary analyses, we found consistent findings of a causal effect of genetically increased ISI on the decreased risk of AD (OR = 0.89, 95% CI: 0.82-0.96, p = 0.003). Heterogeneity, horizontal pleiotropy test, and leave-one-out sensitivity analysis ensured the reliability of the MR estimates. In MVMR, we found no causal relationship between ISI and AD after adjusting for T2D (p > 0.05). We provide genetic evidence that increased ISI is significantly and causally associated with reduced risk of AD, which is mediated by T2D. These findings may inform prevention strategies directed toward IR-associated T2D and AD.

PMID:39479764 | DOI:10.1111/jnc.16254