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Advances in Pediatrics Aug 2024Congenital adrenal hyperplasia (CAH) is an autosomal recessive genetic condition caused by various enzyme deficiencies that result in disruptions of pathways of adrenal... (Review)
Review
Congenital adrenal hyperplasia (CAH) is an autosomal recessive genetic condition caused by various enzyme deficiencies that result in disruptions of pathways of adrenal steroidogenesis. 21-hydroxylase deficiency is the most common form of CAH and has a variable phenotype which ranges a spectrum, from the most severe salt-wasting type to the simple-virilizing type and the least severe nonclassical form. Patients with CAH are at risk for various comorbidities due to the underlying adrenal hormone production imbalance as well as the treatment of the condition, which typically includes supraphysiologic glucocorticoid dosing. Children and adults require frequent monitoring and careful medication dosing adjustment. However, there are multiple novel therapies on the horizon that offer promise to patients with CAH in optimizing their treatment regimens and reducing the risk of comorbidities.
Topics: Humans; Adrenal Hyperplasia, Congenital; Glucocorticoids; Child
PubMed: 38944479
DOI: 10.1016/j.yapd.2024.02.006 -
Journal of Anesthesia, Analgesia and... Jun 2024The presence of nucleated red blood cells (NRBCs) in the peripheral blood of critically ill patients is associated with poor outcome. Evidence regarding the predictive...
BACKGROUND
The presence of nucleated red blood cells (NRBCs) in the peripheral blood of critically ill patients is associated with poor outcome. Evidence regarding the predictive value of NRBCs in patients with SARS-CoV-2-induced acute respiratory distress syndrome (ARDS) remains elusive. The aim of this study was to evaluate the predictive validity of NRBCs in these patients.
METHODS
Daily NRBC values of adult patients with SARS-CoV-2-induced ARDS were assessed and their predictive validity for mortality was statistically evaluated. A cut-off level based on the patient's maximum NRBC value during ICU stay was calculated and further specified according to Youden's method. Based on this cut-off value, further analyses such as logistic regression models and survival were performed.
RESULTS
413 critically ill patients with SARS-CoV-2-induced ARDS were analyzed. Patients who did not survive had significantly higher NRBC values during their ICU stay compared to patients who survived (1090/µl [310; 3883] vs. 140/µl [20; 500]; p < 0.0001). Patients with severe ARDS (n = 374) had significantly higher NRBC values during ICU stay compared to patients with moderate ARDS (n = 38) (490/µl [120; 1890] vs. 30/µl [10; 476]; p < 0.0001). A cut-off level of NRBC ≥ 500/µl was found to best stratify risk and was associated with a longer duration of ICU stay (12 [8; 18] vs. 18 [13; 27] days; p < 0.0001) and longer duration of mechanical ventilation (10 [6; 16] vs. 17 [12; 26] days; p < 0.0001). Logistic regression analysis with multivariate adjustment showed NRBCs ≥ 500/µl to be an independent risk factor of mortality (odds ratio (OR) 4.72; 95% confidence interval (CI) 2.95-7.62, p < 0.0001). Patients with NRBC values below the threshold of 500/µl had a significant survival advantage over those above the threshold (median survival 32 [95% CI 8.7-43.3] vs. 21 days [95% CI 18.2-23.8], log-rank test, p < 0.05). Patients who once reached the NRBC threshold of ≥ 500/µl during their ICU stay had a significantly increased long-term mortality (median survival 489 days, log-rank test, p = 0.0029, hazard ratio (HR) 3.2, 95% CI 1.2-8.5).
CONCLUSIONS
NRBCs predict mortality in critically ill patients with SARS-CoV-2-induced ARDS with high prognostic power. Further studies are required to confirm the clinical impact of NRBCs to eventually enhance decision making.
PubMed: 38943198
DOI: 10.1186/s44158-024-00174-2 -
BMC Public Health Jun 2024Childhood obesity is increasingly recognized as a major public health challenge worldwide, and excessive sedentary screen time is emerging as a key risk factor. This...
BACKGROUND
Childhood obesity is increasingly recognized as a major public health challenge worldwide, and excessive sedentary screen time is emerging as a key risk factor. This study aimed to assess the recreational screen sedentary time of Chinese primary school-aged children and investigate the relationship between screen-related family factors and the outcome variable.
METHODS
Our study used data from a cross-sectional survey collected from fifth-grade students and their parents in Beijing, China, from April to May 2018 (n = 2,373). The questions included basic demographic information, family socioeconomic status, students' and parents' sedentary and exercising habits, within-family communicational factors, and health belief patterns. The recreational screen sedentary time of the children was compared across demographic groups. The study employed multivariate linear regression models to examine associations between children's screen time and various family factors, as well as the moderating effect of overall family communication.
RESULTS
Our findings revealed an average daily recreational screen sedentary time of 2.4 h among participants. Screen time significantly varied across demographic categories, including children's sex, age, residence, parents' education, household income, family size, and primary family member. After adjustment, the proportion of child-owned digital devices (p < 0.01), child's personal room (p < 0.05), family screen-viewing together (p < 0.01), and parental screen time (p < 0.01) were positively related to children's recreational sedentary screen time. Parental restrictions on screen time (p < 0.001) and attitudes toward reducing sitting time (p < 0.01) were correlated with a decrease in children's screen time. The overall family communication environment significantly moderated the effects of parental practice of restricting children's screen time (p < 0.001), positive reinforcement by parents (p < 0.05), and parents' recreational sedentary screen time (p < 0.001).
CONCLUSIONS
Our findings underscored the significance of family dynamics, parental practices, and communication in shaping children's screen time behaviors, providing valuable insights for tailored interventions and strategies to reduce childhood obesity.
Topics: Humans; Cross-Sectional Studies; Screen Time; Male; Female; Sedentary Behavior; Child; Communication; China; Beijing; Parents; Pediatric Obesity; Surveys and Questionnaires; Exercise
PubMed: 38943094
DOI: 10.1186/s12889-024-19128-y -
BMC Cardiovascular Disorders Jun 2024Pulmonary transit time (PTT) can be measured automatically from arterial input function (AIF) images of dual sequence first-pass perfusion imaging. PTT has been...
BACKGROUND
Pulmonary transit time (PTT) can be measured automatically from arterial input function (AIF) images of dual sequence first-pass perfusion imaging. PTT has been validated against invasive cardiac catheterisation correlating with both cardiac output and left ventricular filling pressure (both important prognostic markers in heart failure). We hypothesized that prolonged PTT is associated with clinical outcomes in patients with heart failure.
METHODS
We recruited outpatients with a recent diagnosis of non-ischaemic heart failure with left ventricular ejection fraction (LVEF) < 50% on referral echocardiogram. Patients were followed up by a review of medical records for major adverse cardiovascular events (MACE) defined as all-cause mortality, heart failure hospitalization, ventricular arrhythmia, stroke or myocardial infarction. PTT was measured automatically from low-resolution AIF dynamic series of both the LV and RV during rest perfusion imaging, and the PTT was measured as the time (in seconds) between the centroid of the left (LV) and right ventricle (RV) indicator dilution curves.
RESULTS
Patients (N = 294) were followed-up for median 2.0 years during which 37 patients (12.6%) had at least one MACE event. On univariate Cox regression analysis there was a significant association between PTT and MACE (Hazard ratio (HR) 1.16, 95% confidence interval (CI) 1.08-1.25, P = 0.0001). There was also significant association between PTT and heart failure hospitalisation (HR 1.15, 95% CI 1.02-1.29, P = 0.02) and moderate correlation between PTT and N-terminal pro B-type natriuretic peptide (NT-proBNP, r = 0.51, P < 0.001). PTT remained predictive of MACE after adjustment for clinical and imaging factors but was no longer significant once adjusted for NT-proBNP.
CONCLUSIONS
PTT measured automatically during CMR perfusion imaging in patients with recent onset non-ischaemic heart failure is predictive of MACE and in particular heart failure hospitalisation. PTT derived in this way may be a non-invasive marker of haemodynamic congestion in heart failure and future studies are required to establish if prolonged PTT identifies those who may warrant closer follow-up or medicine optimisation to reduce the risk of future adverse events.
Topics: Humans; Heart Failure; Male; Female; Middle Aged; Aged; Predictive Value of Tests; Time Factors; Prognosis; Ventricular Function, Left; Myocardial Perfusion Imaging; Stroke Volume; Risk Factors; Pulmonary Circulation; Natriuretic Peptide, Brain; Peptide Fragments; Pulmonary Artery; Risk Assessment; Ventricular Function, Right; Magnetic Resonance Imaging
PubMed: 38943084
DOI: 10.1186/s12872-024-04003-w -
Scientific Reports Jun 2024Circulating amyloid-beta 1-40 (Αb40) has pro-atherogenic properties and could serve as a biomarker in atherosclerotic cardiovascular disease (ASCVD). However, the...
Circulating amyloid-beta 1-40 (Αb40) has pro-atherogenic properties and could serve as a biomarker in atherosclerotic cardiovascular disease (ASCVD). However, the association of Ab40 levels with morphological characteristics reflecting atherosclerotic plaque echolucency and composition is not available. Carotid atherosclerosis was assessed in consecutively recruited individuals without ASCVD (n = 342) by ultrasonography. The primary endpoint was grey scale median (GSM) of intima-media complex (IMC) and plaques, analysed using dedicated software. Vascular markers were assessed at two time-points (median follow-up 35.5 months). In n = 56 patients undergoing carotid endarterectomy, histological plaque features were analysed. Plasma Αb40 levels were measured at baseline. Ab40 was associated with lower IMC GSM and plaque GSM and higher plaque area at baseline after multivariable adjustment. Increased Ab40 levels were also longitudinally associated with decreasing or persistently low IMC and plaque GSM after multivariable adjustment (p < 0.05). In the histological analysis, Ab40 levels were associated with lower incidence of calcified plaques and plaques without high-risk features. Ab40 levels are associated with ultrasonographic and histological markers of carotid wall composition both in the non-stenotic arterial wall and in severely stenotic plaques. These findings support experimental evidence linking Ab40 with plaque vulnerability, possibly mediating its established association with major adverse cardiovascular events.
Topics: Humans; Male; Female; Plaque, Atherosclerotic; Aged; Middle Aged; Biomarkers; Amyloid beta-Peptides; Carotid Arteries; Ultrasonography; Carotid Intima-Media Thickness; Carotid Artery Diseases; Endarterectomy, Carotid
PubMed: 38942831
DOI: 10.1038/s41598-024-64906-8 -
Hydroxychloroquine in recurrent pregnancy loss: data from a French prospective multicenter registry.Human Reproduction (Oxford, England) Jun 2024What are the outcomes of pregnancies exposed to hydroxychloroquine (HCQ) in women with a history of recurrent pregnancy loss (RPL), and what factors predict the course...
STUDY QUESTION
What are the outcomes of pregnancies exposed to hydroxychloroquine (HCQ) in women with a history of recurrent pregnancy loss (RPL), and what factors predict the course of these pregnancies beyond the first trimester?
SUMMARY ANSWER
In our cohort of pregnancies in women with a history of RPL exposed to HCQ early in pregnancy, we found that the only factor determining the success of these pregnancies was the number of previous miscarriages.
WHAT IS KNOWN ALREADY
Dysregulation of the maternal immune system plays a role in RPL. HCQ, with its dual immunomodulating and vascular protective effects, is a potential treatment for unexplained RPL.
STUDY DESIGN, SIZE, DURATION
The FALCO (Facteurs de récidive précoce des fausses couches) registry is an ongoing French multicenter infertility registry established in 2017 that includes women (aged from 18 to 49 years) with a history of spontaneous RPL (at least three early miscarriages (≤12 weeks of gestation (WG)) recruited from several university hospitals.
PARTICIPANTS/MATERIALS, SETTING, METHODS
Spontaneous pregnancies enrolled in the FALCO registry with an exposure to HCQ (before conception or at the start of pregnancy) were included. Pregnancies concomitantly exposed to tumor necrosis factor inhibitors, interleukin-1 and -2 inhibitors, intravenous immunoglobulin, and/or intravenous intralipid infusion, were excluded. Concomitant treatment with low-dose aspirin (LDA), low-molecular weight heparin (LMWH), progesterone, and/or prednisone was allowed. All patients underwent the recommended evaluations for investigating RPL. Those who became pregnant received obstetric care in accordance with French recommendations and were followed prospectively. The main endpoint was the occurrence of a pregnancy continuing beyond 12 WG, and the secondary endpoint was the occurrence of a live birth.
MAIN RESULTS AND THE ROLE OF CHANCE
One hundred pregnancies with HCQ exposure in 74 women were assessed. The mean age of the women was 34.2 years, and the median number of previous miscarriages was 5. Concomitant exposure was reported in 78 (78%) pregnancies for prednisone, 56 (56%) pregnancies for LDA, and 41 (41%) pregnancies for LMWH. Sixty-two (62%) pregnancies ended within 12 WG, the other 38 (38%) continuing beyond 12 WG. The risk of experiencing an additional early spontaneous miscarriage increased with the number of previous miscarriages, but not with age. The distributions of anomalies identified in RPL investigations and of exposure to other drugs were similar between pregnancies lasting ≤12 WG and those continuing beyond 12WG. The incidence of pregnancies progressing beyond 12 WG was not higher among pregnancies with at least one positive autoantibody (Ab) (i.e. antinuclear Ab titer ≥1:160, ≥1 positive conventional and/or non-conventional antiphospholipid Ab, and/or positive results for ≥1 antithyroid Ab) without diminished ovarian reserve (18/51, 35.3%) than among those without such autoantibody (18/45, 40.0%) (P = 0.63). Multivariate analysis showed that having ≤4 prior miscarriages was the only factor significantly predictive for achieving a pregnancy > 12 WG, after adjustment for age and duration of HCQ use prior to conception (adjusted odds ratio (OR) = 3.13 [1.31-7.83], P = 0.01).
LIMITATIONS, REASONS FOR CAUTION
Our study has limitations, including the absence of a control group, incomplete data for the diagnostic procedure for RPL in some patients, and the unavailability of results from endometrial biopsies, as well as information about paternal age and behavioral factors. Consequently, not all potential confounding factors could be considered.
WIDER IMPLICATIONS OF THE FINDINGS
Exposure to HCQ in early pregnancy for women with a history of RPL does not seem to prevent further miscarriages, suggesting limited impact on mechanisms related to the maternal immune system.
STUDY FUNDING/COMPETING INTEREST(S)
The research received no specific funding, and the authors declare no competing interests.
TRIAL REGISTRATION NUMBER
clinicaltrial.gov NCT05557201.
PubMed: 38942601
DOI: 10.1093/humrep/deae146 -
The Lancet. Public Health Jul 2024Overdose is the leading cause of death for people released from prison, and opioid agonist treatment is associated with reductions in mortality after imprisonment....
Estimated effects of opioid agonist treatment in prison on all-cause mortality and overdose mortality in people released from prison in Norway: a prospective analysis of data from the Norwegian Prison Release Study (nPRIS).
BACKGROUND
Overdose is the leading cause of death for people released from prison, and opioid agonist treatment is associated with reductions in mortality after imprisonment. However, few studies have explored the interplay of the potential modifiable risk factors and protective factors for mortality after release from prison. We aimed to describe all-cause mortality and overdose mortality among individuals released from Norwegian prisons during 2000-22 and to identify pre-existing risk factors associated with both types of mortality among these individuals for 6 months.
METHODS
For this prospective analysis, we used data from the Norwegian Prison Release Study (nPRIS), which includes all people in prison in Norway between Jan 1, 2000, and Dec 31, 2022; the Norwegian Cause of Death Registry; the Norwegian Prison Registry; the Norwegian Patient Registry; and Statistics Norway. All prisons in Norway that were open during this period were included. People who did not have a Norwegian personal identification number or were serving their sentence outside of prison units were excluded from this analysis. To identify pre-existing risk factors associated with all-cause and overdose mortality among people released from prison, we left-censored the observation period on Jan 1, 2010, creating a subsample of individuals. We calculated crude mortality rates (CMRs) and corresponding 95% CIs as the number of deaths per 100 000 person-years for several time periods after release. The primary outcomes were all-cause mortality and overdose mortality according to the ICD-10, assessed in all participants and analysed via two separate Cox proportional-hazards models.
FINDINGS
The total nPRIS cohort included 112 877 individuals released from prison in Norway between 2000 and 2022, 11 995 (10·6%) of whom were female and 100 865 (89·4%) of whom were male. We identified 13 004 instances of all-cause mortality and 3085 instances of overdose mortality during the 1 463 035 person-years. The estimated CMR for all-cause mortality was 889 (95% CI 874-904) per 100 000 person-years and for overdose mortality was 211 (203-218) per 100 000 person-years. Among people diagnosed with opioid use disorder before entering prison during 2010-22 (n=6830), provision of opioid agonist treatment was estimated to be associated with reductions in both all-cause mortality (hazard ratio 0·58, 95% CI 0·39-0·85) and overdose mortality (0·51, 0·31-0·82) in the 6 months after leaving prison after adjustment for sociodemographic, prison-related, and clinical characteristics.
INTERPRETATION
In people diagnosed with opioid use disorder released from Norwegian prisons, opioid agonist treatment provided while in prison was a protective factor for both all-cause and overdose mortality at 6 months. Provision of opioid agonist treatment while in prison is crucial in reducing mortality for 6 months after release and should be available to all people in prison who have treatment needs.
FUNDING
South-Eastern Norway Regional Health Authority and the Research Council of Norway.
Topics: Humans; Norway; Male; Prospective Studies; Female; Adult; Drug Overdose; Prisoners; Middle Aged; Cause of Death; Prisons; Risk Factors; Analgesics, Opioid; Young Adult; Mortality; Registries; Opiate Substitution Treatment; Adolescent
PubMed: 38942554
DOI: 10.1016/S2468-2667(24)00098-7 -
Journal of the American Board of Family... Jun 2024Social risk data collection is expanding in community health centers (CHCs). We explored clinicians' practices of adjusting medical care based on their awareness of...
BACKGROUND
Social risk data collection is expanding in community health centers (CHCs). We explored clinicians' practices of adjusting medical care based on their awareness of patients' social risk factors-that is, changes they make to care plans to mitigate the potential impacts of social risk factors on their patients' care and health outcomes-in a set of Texas CHCs.
METHODS
Convergent mixed methods. Surveys/interviews explored clinician perspectives on adjusting medical care based on patient social risk factors. Survey data were analyzed with descriptive statistics; interviews were analyzed using thematic analysis and inductive coding.
RESULTS
Across 4 CHCs, we conducted 15 clinician interviews and collected 97 surveys. Interviews and surveys overall indicated support for adjustment activities. Two main themes emerged: 1) clinicians reported making frequent adjustments to patient care plans based on their awareness of patients' social contexts, while simultaneously expressing concerns about adjustment; and 2) awareness of patients' social risk factors, and clinician time, training, and experience all influenced clinician adjustments.
CONCLUSIONS
Clinicians at participating CHCs described routinely adjusting patient care plans based on their patients' social contexts. These adjustments were being made without specific guidelines or training. Standardization of adjustments may facilitate the contextualization of patient care through shared decision making to improve outcomes.
PubMed: 38942447
DOI: 10.3122/jabfm.2023.230289R1 -
Gynecologie, Obstetrique, Fertilite &... Jun 2024In France, embryo thawing concern 45.8% of attempts at Assisted Reproductive Technologies excluding artificial inseminations. This proportion is constantly increasing...
OBJECTIVES
In France, embryo thawing concern 45.8% of attempts at Assisted Reproductive Technologies excluding artificial inseminations. This proportion is constantly increasing for various reasons. The main objective of this study is to compare the live birth rate following frozen blastocyst transfer (FBT) according to the initial indication for freezing.
METHODS
This is a retrospective study including patients who underwent FBT between 01/01/2020 and 06/30/2022 at the Regional University Hospital Center of Tours. The results were compared (univariate and multivariate analyses) between the three main indications for freezing: freezing of the complete cohort of blastocysts for risk of ovarian hyperstimulation (=OHS), freezing of supernumerary blastocysts after fresh blastocyst transfer (BT) with pregnancy (=second request) or without pregnancy (=BT failure). Results have also been described for other indications.
RESULTS
Among the 963 FBT cycles selected, 28% of live births by thawing were obtained, all indications of freezing combined. A significantly lower rate was identified in the FBT failure group compared to the OHS group. However, after adjustment, the results remained significant for the age of the patient on the freezing cycle but not for the indication for freezing.
CONCLUSIONS
The outcome of a FBT doesn't seem significantly impacted by the indication of freezing considering the confounding factors. The prospective analysis of more data from a multicenter study would be necessary to confirm these results.
PubMed: 38942237
DOI: 10.1016/j.gofs.2024.06.002 -
JAMA Network Open Jun 2024Air pollution is a recognized risk factor associated with chronic diseases, including respiratory and cardiovascular conditions, which can lead to physical and cognitive...
IMPORTANCE
Air pollution is a recognized risk factor associated with chronic diseases, including respiratory and cardiovascular conditions, which can lead to physical and cognitive impairments in later life. Although these losses of function, individually or in combination, reduce individuals' likelihood of living independently, little is known about the association of air pollution with this critical outcome.
OBJECTIVE
To investigate associations between air pollution and loss of independence in later life.
DESIGN, SETTING, AND PARTICIPANTS
This cohort study was conducted as part of the Environmental Predictors Of Cognitive Health and Aging study and used 1998 to 2016 data from the Health and Retirement Study. Participants included respondents from this nationally representative, population-based cohort who were older than 50 years and had not previously reported a loss of independence. Analyses were performed from August 31 to October 15, 2023.
EXPOSURES
Mean 10-year pollutant concentrations (particulate matter less than 2.5 μm in diameter [PM2.5] or ranging from 2.5 μm to 10 μm in diameter [PM10-2.5], nitrogen dioxide [NO2], and ozone [O3]) were estimated at respondent addresses using spatiotemporal models along with PM2.5 levels from 9 emission sources.
MAIN OUTCOMES AND MEASURES
Loss of independence was defined as newly receiving care for at least 1 activity of daily living or instrumental activity of daily living due to health and memory problems or moving to a nursing home. Associations were estimated with generalized estimating equation regression adjusting for potential confounders.
RESULTS
Among 25 314 respondents older than 50 years (mean [SD] baseline age, 61.1 [9.4] years; 11 208 male [44.3%]), 9985 individuals (39.4%) experienced lost independence during a mean (SD) follow-up of 10.2 (5.5) years. Higher exposure levels of mean concentration were associated with increased risks of lost independence for total PM2.5 levels (risk ratio [RR] per 1-IQR of 10-year mean, 1.05; 95% CI, 1.01-1.10), PM2.5 levels from road traffic (RR per 1-IQR of 10-year mean, 1.09; 95% CI, 1.03-1.16) and nonroad traffic (RR per 1-IQR of 10-year mean, 1.13; 95% CI, 1.03-1.24), and NO2 levels (RR per 1-IQR of 10-year mean, 1.05; 95% CI, 1.01-1.08). Compared with other sources, traffic-generated pollutants were most consistently and robustly associated with loss of independence; only road traffic-related PM2.5 levels remained associated with increased risk after adjustment for PM2.5 from other sources (RR per 1-IQR increase in 10-year mean concentration, 1.10; 95% CI, 1.00-1.21). Other pollutant-outcome associations were null, except for O3 levels, which were associated with lower risks of lost independence (RR per 1-IQR increase in 10-year mean concentration, 0.94; 95% CI, 0.92-0.97).
CONCLUSIONS AND RELEVANCE
This study found that long-term exposure to air pollution was associated with the need for help for lost independence in later life, with especially large and consistent increases in risk for pollution generated by traffic-related sources. These findings suggest that controlling air pollution could be associated with diversion or delay of the need for care and prolonged ability to live independently.
Topics: Humans; Male; Aged; Female; Air Pollution; Middle Aged; United States; Particulate Matter; Environmental Exposure; Air Pollutants; Cohort Studies; Ozone; Independent Living; Nitrogen Dioxide; Aged, 80 and over; Risk Factors
PubMed: 38941096
DOI: 10.1001/jamanetworkopen.2024.18460