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1.
Int J Offender Ther Comp Criminol ; : 306624X241246098, 2024 Apr 15.
Article in English | MEDLINE | ID: mdl-38622829

ABSTRACT

Prior literature highlights the effectiveness of the Risk-Need-Responsivity (RNR) risk principle when providing community supervision and treatment to general justice-involved individuals and special populations such as individuals convicted of a sexual offense. Individuals deemed high-risk, per risk assessment, should receive the most intensive levels of community supervision and treatment, while individuals classified as low risk should receive the lowest intensity. Research in support for the risk principle finds adherence decreased recidivism rates and increased probation compliance. The current study assesses the effects of adhering (or not) to the risk principle in supervision levels and treatment dosage on the compliance of individuals on probation for a sexual offense (N = 133). Overall, results support risk principle adherence for individuals, with increasing adherence levels associated with significantly more compliance, and non-adherence resulting in adverse outcomes. Implications for policy and practice in the supervision and treatment of individuals with a sexual offense are discussed.

2.
Nat Commun ; 15(1): 3258, 2024 Apr 18.
Article in English | MEDLINE | ID: mdl-38637498

ABSTRACT

Viral infections remain a major risk in immunocompromised pediatric patients, and virus-specific T cell (VST) therapy has been successful for treatment of refractory viral infections in prior studies. We performed a phase II multicenter study (NCT03475212) for the treatment of pediatric patients with inborn errors of immunity and/or post allogeneic hematopoietic stem cell transplant with refractory viral infections using partially-HLA matched VSTs targeting cytomegalovirus, Epstein-Barr virus, or adenovirus. Primary endpoints were feasibility, safety, and clinical responses (>1 log reduction in viremia at 28 days). Secondary endpoints were reconstitution of antiviral immunity and persistence of the infused VSTs. Suitable VST products were identified for 75 of 77 clinical queries. Clinical responses were achieved in 29 of 47 (62%) of patients post-HSCT including 73% of patients evaluable at 1-month post-infusion, meeting the primary efficacy endpoint (>52%). Secondary graft rejection occurred in one child following VST infusion as described in a companion article. Corticosteroids, graft-versus-host disease, transplant-associated thrombotic microangiopathy, and eculizumab treatment correlated with poor response, while uptrending absolute lymphocyte and CD8 T cell counts correlated with good response. This study highlights key clinical factors that impact response to VSTs and demonstrates the feasibility and efficacy of this therapy in pediatric HSCT.


Subject(s)
Epstein-Barr Virus Infections , Hematopoietic Stem Cell Transplantation , Virus Diseases , Humans , Child , Herpesvirus 4, Human , Risk Factors , Hematopoietic Stem Cell Transplantation/adverse effects
3.
Proc Natl Acad Sci U S A ; 121(8): e2310051121, 2024 Feb 20.
Article in English | MEDLINE | ID: mdl-38346198

ABSTRACT

Over the last 10,000 y, humans have manipulated fallow deer populations with varying outcomes. Persian fallow deer (Dama mesopotamica) are now endangered. European fallow deer (Dama dama) are globally widespread and are simultaneously considered wild, domestic, endangered, invasive and are even the national animal of Barbuda and Antigua. Despite their close association with people, there is no consensus regarding their natural ranges or the timing and circumstances of their human-mediated translocations and extirpations. Our mitochondrial analyses of modern and archaeological specimens revealed two distinct clades of European fallow deer present in Anatolia and the Balkans. Zooarchaeological evidence suggests these regions were their sole glacial refugia. By combining biomolecular analyses with archaeological and textual evidence, we chart the declining distribution of Persian fallow deer and demonstrate that humans repeatedly translocated European fallow deer, sourced from the most geographically distant populations. Deer taken to Neolithic Chios and Rhodes derived not from nearby Anatolia, but from the Balkans. Though fallow deer were translocated throughout the Mediterranean as part of their association with the Greco-Roman goddesses Artemis and Diana, deer taken to Roman Mallorca were not locally available Dama dama, but Dama mesopotamica. Romans also initially introduced fallow deer to Northern Europe but the species became extinct and was reintroduced in the medieval period, this time from Anatolia. European colonial powers then transported deer populations across the globe. The biocultural histories of fallow deer challenge preconceptions about the divisions between wild and domestic species and provide information that should underpin modern management strategies.


Subject(s)
Deer , Animals , Humans , Balkan Peninsula
4.
Clin Immunol ; 261: 109942, 2024 04.
Article in English | MEDLINE | ID: mdl-38367737

ABSTRACT

Severe combined immunodeficiency (SCID) is characterized by a severe deficiency in T cell numbers. We analyzed data collected (n = 307) for PHA-based T cell proliferation from the PIDTC SCID protocol 6901, using either a radioactive or flow cytometry method. In comparing the two groups, a smaller number of the patients tested by flow cytometry had <10% of the lower limit of normal proliferation as compared to the radioactive method (p = 0.02). Further, in patients with CD3+ T cell counts between 51 and 300 cells/µL, there was a higher proliferative response with the PHA flow assay compared to the 3H-T assay (p < 0.0001), suggesting that the method of analysis influences the resolution and interpretation of PHA results. Importantly, we observed many SCID patients with profound T cell lymphopenia having normal T cell proliferation when assessed by flow cytometry. We recommend this test be considered only as supportive in the diagnosis of typical SCID.


Subject(s)
Lymphopenia , Severe Combined Immunodeficiency , Infant, Newborn , Humans , Severe Combined Immunodeficiency/diagnosis , Lymphopenia/diagnosis , Neonatal Screening/methods , T-Lymphocytes , Cell Proliferation
5.
J Autism Dev Disord ; 2024 Jan 30.
Article in English | MEDLINE | ID: mdl-38289527

ABSTRACT

PURPOSE: A high-quality primary care clinic should provide clear action points and important care coordination for a child receiving a new diagnosis of autism spectrum disorder (ASD). Unfortunately, a substantial proportion of caregivers report little-to-no post-diagnosis support from their home clinics and primary care providers often report lack of training and resources in providing these supports. METHODS: We implemented an intervention package to investigate the impact on the frequency and quality of follow-up care for children with ASD in a busy, high-volume resident continuity clinic. The package consisted of a care coordination scheduling pathway and a standardized clinical template-embedded in the electronic health record (EHR)-that guided providers through best-practice recommendations and patient resources. RESULTS: As a result of these interventions, 74% of patients had ASD-specific follow-up, a more than threefold increase from baseline with a majority of providers using the EHR-embedded template to guide their visit. Providers also indicated a high degree of usability for the system and that it aided them in following best-practice guidelines for ASD care. CONCLUSION: Through explicit scheduling pathways and a novel EHR template, we saw a significant increase in ASD-specific follow-up visits and implementation of best practices for ASD care, demonstrating a new process for training and engaging primary care providers in clear action steps for post-diagnostic care without having to rely on tertiary referrals.

6.
J Sex Res ; 61(3): 441-453, 2024.
Article in English | MEDLINE | ID: mdl-37186692

ABSTRACT

Despite research showing that many college men and women have experienced misperception of their friendliness as sexual intent, such research has focused on this type of misperception only as a correlate of men's sexual aggression. In fact, regardless of methodology used many researchers seem to suggest women do not misperceive men's sexual intent, and in some instances may actually under-perceive it. We used a hypothetical scenario to determine whether men (n = 324) and women (n = 689) college students perceived similar sexual intent from a character who is not the same gender as they are, as depicted in a story about a man and woman on a "date." Our results revealed that men and women in our sample reported similar levels of perceived sexual intent on the part of the character with a different gender as described in the scenario, even after that character clearly indicated to the partner that they "think they do not want to have sex." In addition, the perceived level of the character's sexual intent as solicited in response to this scenario design was related to sexual coercion intentions among both men and women (though it appears more strongly related among men), and these relationships remained even after controlling for other known correlates of sexual coercion (e.g., rape myth acceptance, level of sexual arousal). Implications for the study of misperception and its origins are discussed.


Subject(s)
Coercion , Rape , Male , Humans , Female , Intention , Sexual Behavior , Students , Perception
7.
Pediatr Dermatol ; 41(1): 143-144, 2024.
Article in English | MEDLINE | ID: mdl-37469225

ABSTRACT

Treatment of severe eczema in patients with primary immunodeficiencies can be particularly challenging as there are no guidelines with regards to these conditions. Dupilumab is an interleukin (IL)-4Rα antagonist that inhibits both IL-4 and IL-13 and is approved for the treatment of atopic dermatitis in pediatric patients. In this report, we describe a patient with a case of severe eczema in the context of Wiskott-Aldrich syndrome-related disorder, who was successfully treated with dupilumab.


Subject(s)
Dermatitis, Atopic , Eczema , Wiskott-Aldrich Syndrome , Humans , Child , Wiskott-Aldrich Syndrome/complications , Wiskott-Aldrich Syndrome/drug therapy , Antibodies, Monoclonal, Humanized/therapeutic use , Dermatitis, Atopic/complications , Dermatitis, Atopic/drug therapy , Eczema/complications , Eczema/drug therapy , Treatment Outcome , Severity of Illness Index
8.
Violence Against Women ; 30(1): 207-227, 2024 01.
Article in English | MEDLINE | ID: mdl-37807715

ABSTRACT

Research on pornography use and sexual coercion has provided inconsistent results. One explanation for this is the lack of a valid, reliable, and comprehensive measure of pornography use. This study seeks to address this need by developing a comprehensive pornography use instrument consisting of four domains identified in research: type of pornography used, sexual scripts, habits, and compulsivity. A sample of 324 college men was used. Results of the study yielded factor structures for these domains and evidence of reliability and validity. Findings present the initial development of a pornography use instrument that could improve research in this area and aid practitioners in treatment and supervision decisions.


Subject(s)
Erotica , Sexual Behavior , Male , Humans , Psychometrics , Reproducibility of Results , Surveys and Questionnaires
9.
Sex Abuse ; : 10790632231219238, 2023 Nov 30.
Article in English | MEDLINE | ID: mdl-38032094

ABSTRACT

The few existing typology studies on women who have sexually offended (WWSO) have largely been limited by small sample sizes, have not included scale scores from risk assessments, or used recidivism within their typology (instead of using typologies to predict recidivism). In our sample of 241 WWSO, we conducted a latent profile analysis and observed four, distinct profiles: "low-risk WWSO," characterized by fewer criminal history incidents and lower risk-assessment scores; "problem-endorsing WWSO," with higher probability of endorsing various life problems such as educational/employment and emotional/personal issues; "antisocial WWSO" with more criminal history incidents, alcohol/drug problems, and higher scores on psychopathy; and "combined WWSO" with characteristics of both the problem-endorsing and antisocial profiles. This last profile showed elevations in general and/or violent recidivism risk, but relatively low recidivism. There were no cases of sexual recidivism in our sample following an average 30-month follow-up period. Membership in the antisocial profile predicted general and/or violent recidivism and suggests that WWSO typologies may be useful in predicting non-sexual recidivism for this population.

10.
Blood ; 142(24): 2105-2118, 2023 12 14.
Article in English | MEDLINE | ID: mdl-37562003

ABSTRACT

Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by life-threatening infections and inflammatory conditions. Hematopoietic cell transplantation (HCT) is the definitive treatment for CGD, but questions remain regarding patient selection and impact of active disease on transplant outcomes. We performed a multi-institutional retrospective and prospective study of 391 patients with CGD treated either conventionally (non-HCT) enrolled from 2004 to 2018 or with HCT from 1996 to 2018. Median follow-up after HCT was 3.7 years with a 3-year overall survival of 82% and event-free survival of 69%. In a multivariate analysis, a Lansky/Karnofsky score <90 and use of HLA-mismatched donors negatively affected survival. Age, genotype, and oxidase status did not affect outcomes. Before HCT, patients had higher infection density, higher frequency of noninfectious lung and liver diseases, and more steroid use than conventionally treated patients; however, these issues did not adversely affect HCT survival. Presence of pre-HCT inflammatory conditions was associated with chronic graft-versus-host disease. Graft failure or receipt of a second HCT occurred in 17.6% of the patients and was associated with melphalan-based conditioning and/or early mixed chimerism. At 3 to 5 years after HCT, patients had improved growth and nutrition, resolved infections and inflammatory disease, and lower rates of antimicrobial prophylaxis or corticosteroid use compared with both their baseline and those of conventionally treated patients. HCT leads to durable resolution of CGD symptoms and lowers the burden of the disease. Patients with active infection or inflammation are candidates for transplants; HCT should be considered before the development of comorbidities that could affect performance status. This trial was registered at www.clinicaltrials.gov as #NCT02082353.


Subject(s)
Graft vs Host Disease , Granulomatous Disease, Chronic , Hematopoietic Stem Cell Transplantation , Humans , Granulomatous Disease, Chronic/genetics , Granulomatous Disease, Chronic/therapy , Retrospective Studies , Prospective Studies , Transplantation, Homologous , Hematopoietic Stem Cell Transplantation/adverse effects , Genotype , Transplantation Conditioning/adverse effects , Graft vs Host Disease/prevention & control
11.
Violence Against Women ; 29(12-13): 2348-2371, 2023 10.
Article in English | MEDLINE | ID: mdl-37501575

ABSTRACT

The current study explores the relationship between psychopathy, cognitive distortions that could promote sexual coercion, i.e., rape myth acceptance (RMA) and misperception of sexual intent (MPSI), and one's level of sexual arousal on the intentions to use sexual coercion. Using a sample of 218 college men (aged 18-69) and a hypothetical vignette design, we examine potential direct and indirect effects of psychopathy on coercion intentions. Psychopathy directly related to coercion intentions and also operated indirectly through RMA. Furthermore, RMA was related to coercion intentions through MPSI. Implications for understanding sexual coercion pathways and prevention efforts are discussed.


Subject(s)
Rape , Male , Humans , Rape/psychology , Coercion , Sexual Behavior/psychology , Intention , Arousal
12.
Pediatr Blood Cancer ; : e30493, 2023 Jun 19.
Article in English | MEDLINE | ID: mdl-37337128

ABSTRACT

BACKGROUND: While racial disparities in the clinical outcomes of hematopoietic stem cell transplant (HSCT) patients have been explored, racial disparities in quality of life (QoL) during the re-adjustment phase after transplant are yet to be investigated in pediatric patients. The objective of this study was to examine the role of patient race in QoL at least 2 years after pediatric HSCT. PROCEDURE: We conducted a retrospective chart review of patients under 21 years of age at diagnosis who received an allogeneic transplant at our institution between January 2007 and December 2017. Patient QoL was assessed using the Pediatric Quality-of-Life Inventory Generic Score Scales (PedsQL TM 4.0) at least 2 years post transplant. Patient demographic, treatment, and transplant outcome data were obtained for subsequent analysis, where patient race was categorized as either Black, White, Hispanic, or Native American. RESULTS: Data were collected on 86 pediatric patients who underwent HSCT. Forty patients (46.5%) were non-Hispanic White, 29 (33.7%) Hispanic, 10 (11.6%) Black, and seven (8.1%) Native American. Where preliminary analyses indicated a difference in QoL by patient race, there were no significant differences in physical, emotional, social, and school functioning by patient race after adjusting for transplant characteristics (age at transplant, sex, diagnosis, donor type, and conditioning regimen) and determinants of socioeconomic status (insurance type, estimated household income). CONCLUSIONS: Pediatric patients had comparable QoL, regardless of race, at a median of 3 years after HSCT in our study cohort.

13.
PLoS Biol ; 21(6): e3002151, 2023 06.
Article in English | MEDLINE | ID: mdl-37310918

ABSTRACT

The 2022 multicountry mpox outbreak concurrent with the ongoing Coronavirus Disease 2019 (COVID-19) pandemic further highlighted the need for genomic surveillance and rapid pathogen whole-genome sequencing. While metagenomic sequencing approaches have been used to sequence many of the early mpox infections, these methods are resource intensive and require samples with high viral DNA concentrations. Given the atypical clinical presentation of cases associated with the outbreak and uncertainty regarding viral load across both the course of infection and anatomical body sites, there was an urgent need for a more sensitive and broadly applicable sequencing approach. Highly multiplexed amplicon-based sequencing (PrimalSeq) was initially developed for sequencing of Zika virus, and later adapted as the main sequencing approach for Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2). Here, we used PrimalScheme to develop a primer scheme for human monkeypox virus that can be used with many sequencing and bioinformatics pipelines implemented in public health laboratories during the COVID-19 pandemic. We sequenced clinical specimens that tested presumptively positive for human monkeypox virus with amplicon-based and metagenomic sequencing approaches. We found notably higher genome coverage across the virus genome, with minimal amplicon drop-outs, in using the amplicon-based sequencing approach, particularly in higher PCR cycle threshold (Ct) (lower DNA titer) samples. Further testing demonstrated that Ct value correlated with the number of sequencing reads and influenced the percent genome coverage. To maximize genome coverage when resources are limited, we recommend selecting samples with a PCR Ct below 31 Ct and generating 1 million sequencing reads per sample. To support national and international public health genomic surveillance efforts, we sent out primer pool aliquots to 10 laboratories across the United States, United Kingdom, Brazil, and Portugal. These public health laboratories successfully implemented the human monkeypox virus primer scheme in various amplicon sequencing workflows and with different sample types across a range of Ct values. Thus, we show that amplicon-based sequencing can provide a rapidly deployable, cost-effective, and flexible approach to pathogen whole-genome sequencing in response to newly emerging pathogens. Importantly, through the implementation of our primer scheme into existing SARS-CoV-2 workflows and across a range of sample types and sequencing platforms, we further demonstrate the potential of this approach for rapid outbreak response.


Subject(s)
COVID-19 , Mpox (monkeypox) , Zika Virus Infection , Zika Virus , Humans , COVID-19/epidemiology , Pandemics , SARS-CoV-2/genetics , Genomics
14.
Animals (Basel) ; 13(12)2023 Jun 12.
Article in English | MEDLINE | ID: mdl-37370474

ABSTRACT

American pet owners spend billions of dollars on food and treats so it is important to understand what products they want and what they think their dog would enjoy. This study analyzed video recordings of dogs engaging in dental chews in their home environment and compared the observed appetitive behaviors to owner preference and owner-reported dog preference. Overall, appetitive behavior differed significantly between some dental chews. Owner preference for the chews correlated significantly with dog appetitive behavior, but the effect was small (r (702) = 0.22, p = 0.001), whereas owner-reported dog preference correlated significantly with dog appetitive behavior and showed a moderate effect size (r (702) = 0.43, p = 0.001)-similar in magnitude to findings when parents are asked to report on their children's behavior. By merging objective behavioral observation of owner-recorded videos with their survey responses, we were able to preliminarily parse out what factors owners may use to assess preference and encourage the future use of in-home video recordings to better understand dog and owner engagement and interaction with pet products.

15.
Transplant Cell Ther ; 29(5): 330.e1-330.e7, 2023 05.
Article in English | MEDLINE | ID: mdl-36804931

ABSTRACT

Pediatric patients with acute myeloid leukemia (AML) who undergo allogeneic hematopoietic stem cell transplantation (HSCT) continue to have high rates of relapse. In 2018, Phoenix Children's Hospital started using post-HSCT maintenance therapy in patients with AML in attempt to decrease the number of relapses after HSCT. This therapy consisted of the hypomethylating agent azacitidine (AZA; 6 cycles starting on day +60) and prophylactic donor lymphocyte infusion (DLI; 3 escalating doses beginning after day +120). We aimed to compare 2-year leukemia-free survival (LFS) post-HSCT between patients with AML who received post-HSCT maintenance therapy with AZA and prophylactic DLI and historical control patients who did not receive post-HSCT therapy. This retrospective pre-post study was conducted at Phoenix Children's Hospital and included patients with AML who underwent HSCT between January 1, 2008, and May 31, 2022. We compared LFS, overall survival (OS), and immune reconstitution patterns post-HSCT between patients with AML who received post-HSCT maintenance therapy with AZA and prophylactic DLI (postintervention group) and historical control patients who did not receive this post-HSCT maintenance therapy (preintervention group). Sixty-three patients were evaluable. After excluding 7 patients who died or relapsed prior to day +60, 56 patients remained, including 39 in the preintervention group and 17 in the postintervention group. The median age at transplantation was 9.1 years in the preintervention group and 11 years in the postintervention group (P = .33). The 2-year LFS was 61.5% in the preintervention group, compared to 88.2% in the postintervention group (P = .06). The 2-year OS was 69.2% in the preintervention group and 88.2% in the postintervention group (P = .15). The rates of CD3+CD4+ T cell and CD19+ B cell recovery were faster in the preintervention group compared to the postintervention group (P = .004 and .0006, respectively). In this limited retrospective study, post-HSCT maintenance therapy using AZA and prophylactic DLI was well tolerated; however, its efficacy is yet to be fully determined.


Subject(s)
Hematopoietic Stem Cell Transplantation , Leukemia, Myeloid, Acute , Humans , Child , Azacitidine/therapeutic use , Retrospective Studies , Leukemia, Myeloid, Acute/therapy , Lymphocytes
16.
medRxiv ; 2023 Jan 13.
Article in English | MEDLINE | ID: mdl-36299420

ABSTRACT

The 2022 multi-country monkeypox (mpox) outbreak concurrent with the ongoing COVID-19 pandemic has further highlighted the need for genomic surveillance and rapid pathogen whole genome sequencing. While metagenomic sequencing approaches have been used to sequence many of the early mpox infections, these methods are resource intensive and require samples with high viral DNA concentrations. Given the atypical clinical presentation of cases associated with the outbreak and uncertainty regarding viral load across both the course of infection and anatomical body sites, there was an urgent need for a more sensitive and broadly applicable sequencing approach. Highly multiplexed amplicon-based sequencing (PrimalSeq) was initially developed for sequencing of Zika virus, and later adapted as the main sequencing approach for SARS-CoV-2. Here, we used PrimalScheme to develop a primer scheme for human monkeypox virus that can be used with many sequencing and bioinformatics pipelines implemented in public health laboratories during the COVID-19 pandemic. We sequenced clinical samples that tested presumptive positive for human monkeypox virus with amplicon-based and metagenomic sequencing approaches. We found notably higher genome coverage across the virus genome, with minimal amplicon drop-outs, in using the amplicon-based sequencing approach, particularly in higher PCR cycle threshold (lower DNA titer) samples. Further testing demonstrated that Ct value correlated with the number of sequencing reads and influenced the percent genome coverage. To maximize genome coverage when resources are limited, we recommend selecting samples with a PCR cycle threshold below 31 Ct and generating 1 million sequencing reads per sample. To support national and international public health genomic surveillance efforts, we sent out primer pool aliquots to 10 laboratories across the United States, United Kingdom, Brazil, and Portugal. These public health laboratories successfully implemented the human monkeypox virus primer scheme in various amplicon sequencing workflows and with different sample types across a range of Ct values. Thus, we show that amplicon based sequencing can provide a rapidly deployable, cost-effective, and flexible approach to pathogen whole genome sequencing in response to newly emerging pathogens. Importantly, through the implementation of our primer scheme into existing SARS-CoV-2 workflows and across a range of sample types and sequencing platforms, we further demonstrate the potential of this approach for rapid outbreak response.

17.
J Allergy Clin Immunol ; 151(2): 547-555.e5, 2023 02.
Article in English | MEDLINE | ID: mdl-36456360

ABSTRACT

BACKGROUND: Shearer et al in 2014 articulated well-defined criteria for the diagnosis and classification of severe combined immunodeficiency (SCID) as part of the Primary Immune Deficiency Treatment Consortium's (PIDTC's) prospective and retrospective studies of SCID. OBJECTIVE: Because of the advent of newborn screening for SCID and expanded availability of genetic sequencing, revision of the PIDTC 2014 Criteria was needed. METHODS: We developed and tested updated PIDTC 2022 SCID Definitions by analyzing 379 patients proposed for prospective enrollment into Protocol 6901, focusing on the ability to distinguish patients with various SCID subtypes. RESULTS: According to PIDTC 2022 Definitions, 18 of 353 patients eligible per 2014 Criteria were considered not to have SCID, whereas 11 of 26 patients ineligible per 2014 Criteria were determined to have SCID. Of note, very low numbers of autologous T cells (<0.05 × 109/L) characterized typical SCID under the 2022 Definitions. Pathogenic variant(s) in SCID-associated genes was identified in 93% of patients, with 7 genes (IL2RG, RAG1, ADA, IL7R, DCLRE1C, JAK3, and RAG2) accounting for 89% of typical SCID. Three genotypes (RAG1, ADA, and RMRP) accounted for 57% of cases of leaky/atypical SCID; there were 13 other rare genotypes. Patients with leaky/atypical SCID were more likely to be diagnosed at more than age 1 year than those with typical SCID lacking maternal T cells: 20% versus 1% (P < .001). Although repeat testing proved important, an initial CD3 T-cell count of less than 0.05 × 109/L differentiated cases of typical SCID lacking maternal cells from leaky/atypical SCID: 97% versus 7% (P < .001). CONCLUSIONS: The PIDTC 2022 Definitions describe SCID and its subtypes more precisely than before, facilitating analyses of SCID characteristics and outcomes.


Subject(s)
Severe Combined Immunodeficiency , Infant, Newborn , Humans , Infant , Severe Combined Immunodeficiency/diagnosis , Severe Combined Immunodeficiency/genetics , Retrospective Studies , Prospective Studies , Homeodomain Proteins/genetics
18.
J Interpers Violence ; 38(7-8): 5519-5541, 2023 Apr.
Article in English | MEDLINE | ID: mdl-36181240

ABSTRACT

Decades of research have examined the association between pornography use and sexual coercion, with the most significant relationships found between more deviant genres of pornography use and sexual violence. However, researchers have yet to provide a comparable body of research examining the theoretical mechanism of this association. One theory that has shown promise in explaining the association between certain variables of pornography use and sexual coercion is the sexual script theory. In this theory, scripts can best be understood as a mechanism through which society defines and disseminates what is acceptable, desirable, and pleasurable sexual conduct. Studies examining the application of the sexual script theory to the association between pornography use and sexual coercion have found that pornography use has a significant indirect effect on sexual coercion and correlates of sexual coercion through sexual scripts. The current study sought to extend this line of inquiry by examining the relationship between pornography use, sexual scripts, and sexual coercion. A structural equation model examining direct and indirect effects of sexual scripts and pornography use on sexually coercive behaviors was run using a sample of 390 college-aged males. Results of the study indicated there were significant direct and indirect effects in the model. Specifically, pornography use, while not directly related to sexually coercive behaviors, had a significant indirect effect on sexual coercion through sexual scripts. These results further support the use of the sexual scripts theory to help explain the relationship between pornography use and sexual coercion.


Subject(s)
Coercion , Sex Offenses , Male , Humans , Young Adult , Erotica , Sexual Behavior , Universities
19.
N Engl J Med ; 387(25): 2344-2355, 2022 12 22.
Article in English | MEDLINE | ID: mdl-36546626

ABSTRACT

BACKGROUND: The DNA-repair enzyme Artemis is essential for rearrangement of T- and B-cell receptors. Mutations in DCLRE1C, which encodes Artemis, cause Artemis-deficient severe combined immunodeficiency (ART-SCID), which is poorly responsive to allogeneic hematopoietic-cell transplantation. METHODS: We carried out a phase 1-2 clinical study of the transfusion of autologous CD34+ cells, transfected with a lentiviral vector containing DCLRE1C, in 10 infants with newly diagnosed ART-SCID. We followed them for a median of 31.2 months. RESULTS: Marrow harvest, busulfan conditioning, and lentiviral-transduced CD34+ cell infusion produced the expected grade 3 or 4 adverse events. All the procedures met prespecified criteria for feasibility at 42 days after infusion. Gene-marked T cells were detected at 6 to 16 weeks after infusion in all the patients. Five of 6 patients who were followed for at least 24 months had T-cell immune reconstitution at a median of 12 months. The diversity of T-cell receptor ß chains normalized by 6 to 12 months. Four patients who were followed for at least 24 months had sufficient B-cell numbers, IgM concentration, or IgM isohemagglutinin titers to permit discontinuation of IgG infusions. Three of these 4 patients had normal immunization responses, and the fourth has started immunizations. Vector insertion sites showed no evidence of clonal expansion. One patient who presented with cytomegalovirus infection received a second infusion of gene-corrected cells to achieve T-cell immunity sufficient for viral clearance. Autoimmune hemolytic anemia developed in 4 patients 4 to 11 months after infusion; this condition resolved after reconstitution of T-cell immunity. All 10 patients were healthy at the time of this report. CONCLUSIONS: Infusion of lentiviral gene-corrected autologous CD34+ cells, preceded by pharmacologically targeted low-exposure busulfan, in infants with newly diagnosed ART-SCID resulted in genetically corrected and functional T and B cells. (Funded by the California Institute for Regenerative Medicine and the National Institute of Allergy and Infectious Diseases; ClinicalTrials.gov number, NCT03538899.).


Subject(s)
Genetic Therapy , Severe Combined Immunodeficiency , Humans , Infant , Busulfan/therapeutic use , Genetic Therapy/adverse effects , Genetic Therapy/methods , Immunoglobulin M , Severe Combined Immunodeficiency/genetics , Severe Combined Immunodeficiency/immunology , Severe Combined Immunodeficiency/therapy , DNA Repair Enzymes/deficiency , DNA Repair Enzymes/genetics , Antigens, CD34/administration & dosage , Antigens, CD34/immunology , Transplantation, Autologous/adverse effects , Transplantation, Autologous/methods , Lentivirus , Genetic Vectors/administration & dosage , Genetic Vectors/adverse effects , Genetic Vectors/therapeutic use , T-Lymphocytes/immunology , B-Lymphocytes/immunology
20.
Am J Crim Justice ; 47(4): 615-616, 2022.
Article in English | MEDLINE | ID: mdl-36467594
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