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Clinical features of 102 patients with different types of Herlyn-Werner-Wunderlich syndrome

Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2023 Apr 28;48(4):550-556. doi: 10.11817/j.issn.1672-7347.2023.220336.

ABSTRACT

OBJECTIVES: Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare female genital tract malformation, and there are few large sample studies reported at home and abroad. The clinical manifestations of this syndrome are diverse, and insufficient understanding may delay the diagnosis and treatment of the patients. This study aims to analyze the clinical characteristics of different types of HWWS patients, and to improve the diagnosis and treatment of HWWS.

METHODS: The clinical data of patients with HWWS who were hospitalized in the Department of Obstetrics and Gynecology, Third Xiangya Hospital of Central South University from October 1, 2009 to April 5, 2022 were retrospectively analyzed. The age, medical history, physical examination, imaging examination and treatment of the patients were collected for statistical analysis. The patients were divided into an imperforate oblique vaginal septum type, a perforate oblique vaginal septum type, and an imperforate oblique vaginal septum and cervical fistula type. The clinical characteristics of different types of HWWS patients were compared.

RESULTS: A total of 102 HWWS patients were enrolled, with age of 10-46 years old, including 37 (36.27%) patients with type I, 50 (49.02%) type II, and 15 (14.71%) type III. All patients were diagnosed after menarche, with an average age of (20.5±7.4) years. There were significant differences in the age of diagnosis and course of disease among the 3 types of HWWS patients (both P<0.05). Patients with type I had the youngest age of diagnosis [(18.0±6.0) years] and the shortest course of disease (median course of 6 months), while patients with type III had the oldest age of diagnosis [(22.9±9.8) years] and the longest course of disease (median course of 48 months). The main clinical manifestation of type I was dysmenorrhea, and the main clinical manifestation of type II and type III was abnormal vaginal bleeding. Of the 102 patients, 67 (65.69%) patients had double uterus, 33 (32.35%) had septate uterus, and 2 (1.96%) had bicornuate uterus. The vast majority of patients had renal agenesis on the oblique septum, and only 1 patient had renal dysplasia on the oblique septum. The oblique septum located on the left side in 45 (44.12%) patients and on the right side in 57 (55.88%) patients. There were no significant differences in uterine morphology, urinary system malformation, pelvic mass, and oblique septum among the 3 types of HWWS patients (all P>0.05). Six (5.88%) patients had ovarian chocolate cyst, 4 (3.92%) patients had pelvic abscess, and 5 (4.90%) patients had hydrosalpinx. All patients underwent vaginal oblique septum resection. Among them, 42 patients underwent hysteroscopic incision of the oblique vaginal septum without destroying the intact hymen because they had no sexual life history, and the remaining 60 patients underwent traditional oblique vaginal septum resection. Among the 102 patients, 89 patients were followed up for 1 month to 12 years. The symptoms of vaginal oblique septum in 89 patients such as dysmenorrhea, abnormal vaginal bleeding and vaginal discharge were improved after operation. Among the 42 patients who underwent hysteroscopic incision of the oblique vaginal septum without destroying the intact hymen, 25 patients underwent hysteroscopies again 3 months after operation, and there was no obvious scar formation at the oblique septum incision site.

CONCLUSIONS: Different types of HWWS have different clinical manifestations, but all can be manifested as dysmenorrhea. The patient’s uterine morphology can be manifested as double uterus, septate uterus, or bicornuate uterus. The possibility of HWWS should be considered if uterine malformation is combined with renal agenesis. Vaginal oblique septum resection is an effective treatment.

PMID:37385617 | DOI:10.11817/j.issn.1672-7347.2023.220336

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Association of insulin signaling pathway-related gene polymorphisms and gene-gene interactions with MAFLD in obese children

Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2023 Apr 28;48(4):516-525. doi: 10.11817/j.issn.1672-7347.2023.220295.

ABSTRACT

OBJECTIVES: Insulin signaling pathway plays an important role in metabolic associated fatty liver disease (MAFLD), however, the association between polymorphisms of genes related to insulin signaling pathway and MAFLD remains unclear. This study aims to investigate the association between insulin signaling pathway-related gene polymorphisms and gene-gene interactions with MAFLD susceptibility in obese children so as to provide scientific basis for further study of genetic mechanism.

METHODS: A total of 502 obese children with MAFLD who admitted to Hunan Provincial Children’s Hospital from September 2019 to October 2021, were recruited as a case group, and 421 obese children with non-MAFLD admitted during the same period were recruited as a control group. Socio-demographic information, preterm birth history, eating habits, and exercise status of the subjects were collected by inquiry survey, and anthropometric information was collected by physical measurement. At the same time, 2 mL of venous blood was collected to extract DNA, and the polymorphism of insulin signaling pathway-related genes (5 representative candidate genes, 12 variants) was detected. Multivariate Logistic regression analysis was used to investigate the association between insulin signaling pathway-related gene polymorphisms and MAFLD in obese children.

RESULTS: After adjusting for confounder factors, INS rs3842748 was significantly associated with the risk of MAFLD in obese children in allele, heterozygous, and dominant models [OR and 95% CI 1.749 (1.053 to 2.905), 1.909 (1.115 to 3.267), 1.862 (1.098 to 3.157), all P<0.05]; INS rs3842752 was significantly associated with the risk of MAFLD in obese children in heterozygous and dominant models [OR and 95% CI 1.736 (1.028 to 2.932), 1.700 (1.015 to 2.846), all P<0.05]. NR1H3 rs3758674 was significantly correlated with the risk of MAFLD in obese children in allele model [OR and 95% CI 0.716 (0.514 to 0.997), P<0.05]. SREBP-1c rs2297508 was significantly associated with the risk of MAFLD in obese children in allele and dominant models [OR and 95% CI 0.772 (0.602 to 0.991) and 0.743 (0.557 to 0.991), all P<0.05]. SREBP-1c rs8066560 was significantly associated with the risk of MAFLD in obese children in allele, heterozygous, and dominant models [OR and 95% CI 0.759 (0.589 to 0.980), 0.733 (0.541 to 0.992), 0.727 (0.543 to 0.974), all P<0.05]. NR1H3 rs3758674 mutant C and SREBP-1c rs2297508 mutant G had interaction in the development of MAFLD in obese children [OR and 95% CI 0.407 (0.173 to 0.954), P<0.05].

CONCLUSIONS: The INS, NR1H3, and SREBP-1c gene polymorphisms in the insulin signaling pathway are associated with the susceptibility of MAFLD in obese children, but the functions and mechanisms of these genes need to be further studied.

PMID:37385614 | DOI:10.11817/j.issn.1672-7347.2023.220295

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Agreement of cardiac output measurements by esophageal Doppler and transesophageal echocardiography with intermittent pulmonary artery thermodilution during pharmacologic manipulation of hemodynamics in anesthetized dogs

Am J Vet Res. 2023 Jul 3:1-11. doi: 10.2460/ajvr.23.05.0101. Online ahead of print.

ABSTRACT

OBJECTIVE: To compare cardiac output (CO) measurements by transesophageal echocardiography (TEECO) and esophageal Doppler monitor (EDMCO) with pulmonary artery thermodilution (PATDCO) in anesthetized dogs subjected to pharmacological interventions. The effect of treatments on EDM-derived indexes was also investigated.

ANIMALS: 6 healthy male dogs (10.8 ± 0.7 kg).

METHODS: Dogs were anesthetized with propofol and isoflurane, mechanically ventilated, and monitored with invasive mean arterial pressure (MAP), end-tidal isoflurane concentration (ETISO), PATDCO, TEECO, EDMCO, and EDM-derived indexes. Four treatments were administered to all dogs by randomization. Baseline data were collected before each treatment: (1) dobutamine infusion; (2) esmolol infusion; (3) phenylephrine infusion; and (4) ETISO > 3%. Data were collected after 10-minute stabilization and after 30 minutes of washout between treatments. Statistical tests were pairwise t test, Bland-Altman analysis, Lin’s concordance correlation (ρc), and polar plot analysis with P < .05 set as significance.

RESULTS: The mean ± SD relative bias (limits of agreement) for TEECO was 0.35 ± 25.2% (-49.1% to 49.8%) and for EDMCO was -27.2 ± 22.5% (-71.4% to 17%) versus PATDCO. The percent error for TEECO and EDMCO was 27.6% and 44.1%, respectively. The ρc value was 0.82 for TEECO and 0.66 for EDMCO. TEECO and EDMCO showed good trending ability. EDM-derived indexes displayed significant changes specific to the drug administered (P < .001).

CLINICAL RELEVANCE: For minimally invasive CO monitoring, TEE may provide more favorable performance than EDM in clinical settings; however, EDM-derived indexes yield valuable hemodynamic information that reliably follows trends in CO, thus supporting critical decision-making in canine patients.

PMID:37385599 | DOI:10.2460/ajvr.23.05.0101

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Analysis of Palliative Care Knowledge and Symptom Burden Among Female Veterans With Serious Illness: A Cross-Sectional Study

Am J Hosp Palliat Care. 2023 Jun 29:10499091231187341. doi: 10.1177/10499091231187341. Online ahead of print.

ABSTRACT

Background: The female Veteran population is rapidly growing, as is their use of Veterans Affairs (VA) medical centers (VAMCs). Additionally, 90% of female Veterans are under 65 years old, meaning healthcare providers at VAMCs must be ready to manage the complex serious illnesses that affect female Veterans as they age. These serious illnesses require proper medical management, which can include palliative care. However, little palliative care research includes female Veterans. Aims: The aims of this cross-sectional study were to examine palliative care knowledge and symptom burden among female Veterans’ and examine factors associated a symptom burden scale. Methods: Consenting participants completed online questionnaires, including the Palliative Care Knowledge Scale (PaCKS), Condensed Memorial Symptom Assessment Scale (CMSAS), and demographics. Descriptive statistics characterized the sample, bivariate association were carried out with a Chi-square and t test. A generalized linear model explored associations between CMSAS and its subscales with sociodemographic, number of serious illnesses, and facility type (VAMC vs civilian facility). Results: 152 female Veterans completed the survey. PaCKS scores were consistent across our sample. Physical symptoms were rated higher for those receiving care at VAMCs compared to civilian facilities (P = .02) in the bivariate analysis. The factors associated with CMSAS were age, employment status and number of serious illnesses (all P < .05). Conclusions: Palliative care can assist female Veterans with serious illness. More research is needed to further explore variables associated with symptom burden among female Veterans such as age, employment status, and number of serious illnesses.

PMID:37385594 | DOI:10.1177/10499091231187341

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Association Between Marijuana Laws and Suicide Among 12- to 25-Year-Olds in the United States From 2000 to 2019

J Am Acad Child Adolesc Psychiatry. 2023 Jun 23:S0890-8567(23)00341-6. doi: 10.1016/j.jaac.2023.06.014. Online ahead of print.

ABSTRACT

OBJECTIVE: Cannabis use is associated with suicide-related outcomes in both adolescents and adults and may be increasing amidst shifting cannabis policies. However, little is known about the impact of medical marijuana legalization (MML) and recreational marijuana legalization (RML) policies on youth suicide. Using 20 years of national data, we examined associations between MML, RML, and suicide-related mortality among US individuals aged 12 to 25; and assessed whether they varied based on age and sex.

METHOD: Suicide deaths (N=113,512) from the 2000-2019 National Vital Statistics System Multiple Cause of Death files for age groups 12-13, 14-16, 17-19, 20-22, and 23-25 years were examined in relation to time-varying cannabis law status using a staggered adoption difference-in-difference (DiD) approach with a negative binomial regression to determine associations between MML, RML, and suicide rates, controlling for individual- and state-level covariates and accounting for the varying effective dates of MML and RML by state.

RESULTS: The overall unadjusted annual suicide rate was 10.93/100,000, varying from 9.76 (no ML states) to 12.78 (MML states) to 16.68 (RML states). In multivariable analysis, both MML (incidence rate ratio (IRR) = 1.10, 95% CI 1.05-1.15) and RML (IRR=1.16, 95% CI 1.06-1.27) were associated with higher suicide rates among female youth compared to states without ML. Youth aged 14-16 had higher rates of suicide in states with RML compared to states with MML (IRR=1.14, 95% CI 1.00-1.30) and states without ML (IRR=1.09, 95% CI 1.00-1.20). Findings were consistent across sensitivity analyses.

CONCLUSION: MML and RML were associated with increased suicide-related mortality in female youth and 14-to-16-year-olds of both sexes. Mechanisms through which cannabis policies are related to increased youth suicide warrant further study and should inform legislative reform.

PMID:37385585 | DOI:10.1016/j.jaac.2023.06.014

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Structural Racism and Well-Being Among Young People in the United States

Am J Prev Med. 2023 Jun 27:S0749-3797(23)00280-5. doi: 10.1016/j.amepre.2023.06.017. Online ahead of print.

ABSTRACT

INTRODUCTION: Structural racism has clear and pernicious effects on population health. However, there is limited understanding of how structural racism impacts young peoples’ well-being. The objective of this ecological cross-sectional study was to assess the relationship between structural racism and well-being for 2,009 United States counties from 2010-2019.

METHODS: Population-based data on demographics, health, and other variables related to young peoples’ ability to thrive are used to construct a previously validated composite index that serves as a proxy of young peoples’ well-being. The index is regressed on several forms of structural racism (segregation, economic, and educational) both independently and jointly, while accounting for county fixed effects, time trends, and state-specific trends, as well as weighting for child population. Data was analyzed from November 2021 through March 2023.

RESULTS: Higher levels of structural racism are associated with lower well-being. A one-standard-deviation increase in Black-White child poverty disparity is associated with a -0.034 [95% CI: -0.019 – -0.050] standard-deviation change in index score. When accounting for multiple structural racism measures, associations remain statistically significant. In joint models, only estimates for economic racism measures remain significant when additionally controlling for demographic, socioeconomic, and adult health measures [β=-0.015; 95% CI: -0.001 – -0.029]. These negative associations are heavily concentrated in counties where Black and Latinx children are overrepresented.

CONCLUSIONS: Structural racism-particularly of the kind that produces racialized poverty outcomes-has a meaningful adverse association with child and adolescent well-being, which may produce lifelong effects. Studies of structural racism among adults should consider a lifecourse perspective.

PMID:37385571 | DOI:10.1016/j.amepre.2023.06.017

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Extent of surgical resection for radiologically subsolid T1N0 invasive lung adenocarcinoma: When is a wedge resection acceptable?

J Thorac Cardiovasc Surg. 2023 Jun 27:S0022-5223(23)00536-6. doi: 10.1016/j.jtcvs.2023.06.010. Online ahead of print.

ABSTRACT

OBJECTIVE: To evaluate whether wedge resection (WR) was appropriate for the patients with peripheral T1N0 solitary subsolid invasive lung adenocarcinoma (LUAD).

METHODS: Patients with peripheral T1N0 solitary subsolid invasive LUAD who received sublobar resection were retrospectively reviewed. Clinicopathologic characteristics, 5-year recurrence-free survival (RFS) and 5-year lung cancer-specific overall survival (LCS-OS) were analyzed. Cox regression model was used to elucidate risk factors for recurrence.

RESULTS: 258 patients receiving WR and 1245 patients receiving segmentectomy (SEG) were included. The mean follow-up time was 36.87±16.21 months. 5-year RFS following WR was 96.89% for patients with ground-glass nodule (GGN) ≤2cm and 0.25< consolidation to tumor ratio (CTR) ≤0.5, not statistically different from 100% for those with GGN≤2cm and CTR≤0.25 (p=0.231). The 5-year RFS was 90.12% for patients with GGN between 2-3cm and CTR≤0.5, significantly lower than that of patients with GGN≤2cm and CTR≤0.25 (p=0.046). For patients with GGN≤2cm and 0.25<CTR≤0.5, 5-year RFS and LCS-OS were 97.87% and 100% following WR versus 97.73% and 92.86% following SEG (RFS: p=0.987; LCS-OS: p=0.199), respectively. For patients with GGN between 2-3cm and CTR≤0.5, 5-year RFS following WR was significantly lower than that following SEG (90.61% vs. 100%, p=0.043). Multi-variable Cox regression analysis showed that STAS, visceral pleural invasion and nerve invasion remained independent risk factors for recurrence of patients with GGN between 2-3cm and CTR≤0.5 following WR.

CONCLUSIONS: WR might be appropriate for patients with invasive LUAD appearing as peripheral GGN≤2cm and CTR≤0.5, but inappropriate for those with invasive LUAD appearing as peripheral GGN between 2-3cm and CTR≤0.5.

PMID:37385528 | DOI:10.1016/j.jtcvs.2023.06.010

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Hotspots of microplastic accumulation at the land-sea transition and their spatial heterogeneity: The Po River prodelta (Adriatic Sea)

Sci Total Environ. 2023 Jun 27:164908. doi: 10.1016/j.scitotenv.2023.164908. Online ahead of print.

ABSTRACT

Deltas are the locus of river-borne sediment accumulation, however, their role in sequestering plastic pollutants is still overlooked. By combining geomorphological, sedimentological, and geochemical analyses, which include time-lapse multibeam bathymetry, sediment provenance, and μFT-IR analyses, we investigate the fate of plastic particles after a river flood event providing an unprecedented documentation of the spatial distribution of sediment as well as of microplastics (MPs), including particles fibers, and phthalates (PAEs) abundances in the subaqueous delta. Overall sediments are characterized by an average of 139.7 ± 80 MPs/kg d.w., but display spatial heterogeneity of sediment and MPs accumulation: MPs are absent within the active sandy delta lobe, reflecting dilution by clastic sediment (ca. 1.3 Mm3) and sediment bypass. The highest MP concentration (625 MPs/kg d.w.) occurs in the distal reaches of the active lobe where flow energy dissipates. In addition to MPs, cellulosic fibers are relevant (of up to 3800 fibers/kg d.w.) in all the analyzed sediment samples, and dominate (94 %) with respect to synthetic polymers. Statistically significant differences in the relative concentration of fiber fragments ≤0.5 mm in size were highlighted between the active delta lobe and the migrating bedforms in the prodelta. Fibers were found to slightly follow a power law size distribution coherent with a one-dimensional fragmentation model and thus indicating the absence of a size dependent selection mechanism during burial. Multivariate statistical analysis suggests traveling distance and bottom-transport regime as the most relevant factors controlling particle distribution. Our findings suggest that subaqueous prodelta should be considered hot spots for the accumulation of MPs and associated pollutants, albeit the strong lateral heterogeneity in their abundances reflects changes in the relative influence of fluvial and marine processes.

PMID:37385497 | DOI:10.1016/j.scitotenv.2023.164908

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Diabetes care practices and outcomes in 40.000 children and adolescents with type 1 diabetes from the SWEET registry during the COVID-19 pandemic

Diabetes Res Clin Pract. 2023 Jun 27:110809. doi: 10.1016/j.diabres.2023.110809. Online ahead of print.

ABSTRACT

AIMS: This study aimed to provide a global insight into initiatives in type 1 diabetes care driven by the COVID-19 pandemic and associations with glycemic outcomes.

METHODS: An online questionnaire regarding diabetes care before and during the pandemic was sent to all centers (n=97, 66,985 youth with type 1 diabetes) active in the SWEET registry. Eighty-two responded, and 70 (42,798 youth with type 1 diabetes) had available data (from individuals with type 1 diabetes duration >3 months, aged ≤21 years) for all 4 years from 2018 to 2021. Statistical models were adjusted, among others, for technology use.

RESULTS: Sixty-five centers provided telemedicine during COVID-19. Among those centers naive to telemedicine before the pandemic (n=22), four continued only face-to-face visits. Centers that transitioned partially to telemedicine (n=32) showed a steady increase in HbA1c between 2018 and 2021 (p<0.001). Those that transitioned mainly to telemedicine (n=33%) improved HbA1c in 2021 compared to 2018 (p<0.001).

CONCLUSIONS: Changes to models of care delivery driven by the pandemic showed significant associations with HbA1c shortly after the pandemic outbreak and 2 years of follow-up. The association appeared independent of the concomitant increase in technology use among youth with type 1 diabetes.

PMID:37385473 | DOI:10.1016/j.diabres.2023.110809

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Abnormalities on baseline chest imaging are risk factors for immune checkpoint inhibitor associated pneumonitis

Respir Med. 2023 Jun 27:107330. doi: 10.1016/j.rmed.2023.107330. Online ahead of print.

ABSTRACT

BACKGROUND: Chronic lung disease is a proposed risk factor for immune checkpoint inhibitor pneumonitis (ICI-pneumonitis); however, data is sparse regarding the impact of pre-existing lung disease and baseline chest imaging abnormalities on the risk of developing ICI-pneumonitis.

METHODS: We conducted a retrospective cohort study of patients with ICI treatment for cancer from 2015 to 2019. ICI-pneumonitis was determined by the treating physician with corroboration via an independent physician review and exclusion of alternative etiologies. Controls were patients treated with ICI without a diagnosis of ICI-pneumonitis. Fisher’s exact tests, Student’s t-tests, and logistic regression were used for statistical analysis.

RESULTS: We analyzed 45 cases of ICI-pneumonitis and 135 controls. Patients with abnormal baseline chest CT imaging (emphysema; bronchiectasis; reticular, ground glass and/or consolidative opacities) had increased risk for ICI-pneumonitis (OR 3.41, 95%CI: 1.68-6.87, p = 0.001). Patients with gastroesophageal reflux disease (GERD) (OR 3.83, 95%CI: 1.90-7.70, p = < 0.0001) also had increased risk for ICI-pneumonitis. On multivariable logistic regression, patients with abnormal baseline chest imaging and/or GERD remained at increased risk for ICI-pneumonitis. Eighteen percent of all patients (32/180) had abnormal baseline chest CT consistent with chronic lung disease without a documented diagnosis.

CONCLUSION: Patients with baseline chest CT abnormalities and GERD were at increased risk for developing ICI-pneumonitis. The large proportion of patients with baseline radiographic abnormalities without a clinical diagnosis of chronic lung disease highlights the importance of multidisciplinary evaluation prior to ICI initiation.

PMID:37385460 | DOI:10.1016/j.rmed.2023.107330