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With advances in medicine, HIV has evolved into a chronic condition for people living with HIV (PLWH). However, many PLWH do not engage in advance care planning (ACP), and it occurs primarily in acute situations. ACP may improve preparedness for end-of-life care and increase advance directive (AD) documentation. Searches of PubMed, PsycInfo, and CINHAL were conducted to synthesize current ACP-related randomized controlled trials on two separate populations: (1) PLWH and (2) older adults without HIV. Two articles met inclusion criteria for PLWH, and the intervention had a significant improvement in ACP engagement. For the latter population, thirteen of fourteen articles had interventions that led to statistically significant increases in ACP engagement. This review underscores the need to further investigate the best strategies to improve ACP among PLWH and provides insights from existing ACP interventions for the general population to be adapted to the needs of PLWH.
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BACKGROUND: A straight silicone stent can be used to treat proximal benign tracheal stenosis in non-surgical candidates. However, stent migration is a common complication when placed at a particular location and can lead to major complications. This case series of laryngotracheal stenosis reports a fixation method for straight silicone stents in the subglottic trachea (Stage 3 of the McCaffrey classification). METHODS: The medical charts of these patients scheduled for straight silicone stent placement with suture fixation between 2014 and 2020 at the CHU UCL Namur Hospital (Belgium) were retrospectively reviewed. The procedure was performed using a rigid bronchoscope. Details of the procedure were obtained from medical records. RESULTS: This case series included six patients (males: 4, females: 2). The median patient age was 59 years. Two suture fixations were placed following previous silicone stent migration episodes, whereas the others were placed proactively to avoid this risk. All fixations were performed by the device Freka® Pexact II ENFIt®, originally developed for gastropexy in endoscopic gastrostomy. The sutures were subcutaneously buried. CONCLUSIONS: During the 6-month follow-up period, complications such as fixation issues and stent migration were reported despite the off-label use of the treatment. The straight silicone stent fixation technique used in this case series was simple and effective for securing the stent in upper benign tracheal stenosis.
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Laringoestenose , Stents , Estenose Traqueal , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Estenose Traqueal/cirurgia , Laringoestenose/cirurgia , Estudos Retrospectivos , Idoso , Adulto , Técnicas de Sutura , Recidiva , Silicones , BroncoscopiaRESUMO
BACKGROUND: Physiologic narrowing of the central airway occurs during expiration. Conditions in which this narrowing becomes excessive are referred to as expiratory central airway collapse. Expiratory central airway collapse is usually managed by applying positive pressure to the airways, which acts as a pneumatic stent. The particularity of the case reported here included the patient's left main bronchus being permeable during spontaneous breathing but collapsing during general anaesthesia, despite positive pressure ventilation and positive end-expiratory pressure. CASE PRESENTATION: We present the case of a 55-year-old man admitted for the placement of a ureteral JJ stent. Rapid desaturation occurred a few minutes after the onset of anaesthesia. After excluding the most common causes of desaturation, fibreoptic bronchoscopy was performed through the tracheal tube and revealed complete collapse of the left main bronchus. The collapse persisted despite the application of positive end-expiratory pressure and several recruitment manoeuvres. After recovery of spontaneous ventilation, the collapse was lifted, and saturation increased back to normal levels. No evidence of extrinsic compression was found on chest X-rays or computed tomography scans. CONCLUSION: Cases of unknown expiratory central airway collapse reported in the literature were usually managed with positive pressure ventilation. This approach has been unsuccessful in the case described herein. Our hypothesis is that mechanical bending of the left main bronchus occurred due to loss of the patient's natural position and thoracic muscle tone under general anaesthesia with neuromuscular blockade. When possible, spontaneous ventilation should be maintained in patients with known or suspected ECAC.
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Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/fisiopatologia , Anestesia Geral , Respiração com Pressão Positiva/efeitos adversos , Traqueia/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Respiração com Pressão Positiva/métodosRESUMO
OBJECTIVE: A subset of parents of children with disorders/differences of sex development (DSD) including ambiguous genitalia experience clinically elevated levels of anxious and depressive symptoms. Research indicates that uncertainty about their child's DSD is associated with parent psychosocial distress; however, previous studies have been cross-sectional or correlational in nature. The current study is the first to examine the longitudinal trajectory of the relationship between caregiver-perceived uncertainty about their child's DSD and caregiver anxious and depressive symptoms across the first 12 months following genital surgery in young children, or if surgery was not performed, the first 12 months following study entry. METHODS: One hundred and thirteen caregivers (Mage = 32.12; 57.5% mothers; 72.6% Caucasian) of children (N = 70; Mage = 9.81 months; 65.7% female) with DSD were recruited from 12 DSD specialty clinics in the United States. Caregivers completed psychosocial measures at baseline, 6 and 12 months following genitoplasty, or study entry if parents elected not to have surgery for their child. RESULTS: Caregiver illness uncertainty and both anxious and depressive symptoms were highest at baseline and decreased over time (ps < .05). Caregiver illness uncertainty predicted symptoms of anxious and depressive symptoms across all time points (ps < .05). CONCLUSIONS: Caregivers' perceptions of uncertainty about their child's DSD are highest soon after diagnosis, and uncertainty continues to predict both anxious and depressive symptoms across time. Thus, the initial diagnostic period is a critical time for psychological assessment and intervention, with parent illness uncertainty being an important clinical target.
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Cuidadores , Pais , Ansiedade/diagnóstico , Criança , Pré-Escolar , Estudos Transversais , Depressão/diagnóstico , Feminino , Humanos , Masculino , IncertezaRESUMO
PURPOSE: We evaluated demographic, financial and support predictors of distress for parents of young children with disorders of sex development including atypical genital development, and characterized early parental experiences. This work extends our previous findings to identify those parents at risk for distress. MATERIALS AND METHODS: Participants included mothers (76) and fathers (63) of a child (78) diagnosed with disorders of sex development characterized by moderate to severe genital atypia. Parents completed a demographic questionnaire, measures of anxious and depressive symptoms, quality of life, illness uncertainty and posttraumatic stress symptoms, and rated their satisfaction with the appearance of their child's genitalia. RESULTS: Depressive and posttraumatic stress symptoms of caregivers were comparable to standardized norms while levels of anxious symptoms were below norms. A subset of parents reported clinically elevated symptoms. Overall 26% of parents reported anxious symptoms, 24% reported depressive symptoms and 17% reported posttraumatic stress symptoms. Levels of illness uncertainty were lower than those of parents of children with other chronic illnesses. Differences by parent sex emerged, with mothers reporting greater distress. Lower income, increased medical care and travel expenses, and having no other children were related to increased psychosocial distress. CONCLUSIONS: Early psychosocial screening is recommended for parents of children with disorders of sex development. Clinicians should be aware that financial burden and lack of previous parenting experience are risk factors for distress.
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Transtornos do Desenvolvimento Sexual/psicologia , Pais/psicologia , Qualidade de Vida , Estresse Psicológico/etiologia , Adulto , Pré-Escolar , Transtornos do Desenvolvimento Sexual/complicações , Feminino , Humanos , Incidência , Lactente , Masculino , Prognóstico , Fatores de Risco , Estresse Psicológico/epidemiologia , Estresse Psicológico/psicologia , Estados Unidos/epidemiologiaRESUMO
Parents of children with disorders of sex development (DSD) report significant psychological distress, including posttraumatic stress symptoms (PTSS), with mothers consistently reporting higher rates of psychological distress than fathers. However, psychological factors contributing to PTSS in both parents are not well understood. The present study sought to fill this gap in knowledge by examining PTSS and illness uncertainty, a known predictor of psychological distress, in parents of children recently diagnosed with DSD. Participants were 52 mothers (Mage = 32.55 years, SD = 5.08) and 41 fathers (Mage = 35.53 years, SD = 6.78) of 53 infants (Mage = 9.09 months, SD = 6.19) with DSD and associated atypical genital development. Participants were recruited as part of a larger, multisite study assessing parents' psychosocial response to their child's diagnosis of DSD. Parents completed measures of illness uncertainty and PTSS. Mothers reported significantly greater levels of PTSS, but not illness uncertainty, than fathers, and were more likely than fathers to report clinical levels of PTSS (21.2% compared to 7.3%). Hierarchical regression revealed that parent sex, undiagnosed or unclassified DSD status, and illness uncertainty were each associated with PTSS. The overall model accounted for 23.5% of the variance associated with PTSS. Interventions targeting illness uncertainty may be beneficial for parents of children with newly diagnosed DSD.
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Transtornos do Desenvolvimento Sexual/psicologia , Transtornos de Estresse Pós-Traumáticos/diagnóstico , Incerteza , Adulto , Feminino , Humanos , Lactente , Masculino , Relações Pais-FilhoRESUMO
Studies have associated electroencephalogram (EEG) suppression with postoperative delirium (POD) and postoperative cognitive decline (POCD). Otherwise, improving cerebral tissue oxygen saturation (rScO2) seems beneficial. No study has evaluated the impact of EEG suppression and decreased rScO2 on the incidence of POD and POCD when the intraoperative management of patients is performed with a depth-of-anesthesia (DOA) monitor and a cerebral oximetry. In this prospective study patients undergoing cardiac interventions were monitored with the NeuroSENSE® DOA monitor and bilateral cerebral oximetry. An algorithm was used to optimize cerebral oxygenation. EEG suppression was presented as total area under the curve (AUC) of suppression ratio (SR) > 0 s (AUCEEGSR>0s). Cerebral desaturation was defined as AUC of 25% drop of oximetry values as compared to baseline. POD was evaluated by the chart review method. POCD was defined as a Z-score ≤ 2 based on Mini Mental State Examination at baseline and day 5 or if the patient reported any cognitive decline at 3 and at 6 months postoperatively. Among the 1616 patients, 1513 underwent normothermic surgery and were further analyzed. POD and POCD were respectively evaluated in 1504 and 1350 patients of whom 303 (20%) and 270 (20%) were respectively diagnosed positive. Having experienced high magnitudes of EEG suppression (fourth quartile of AUCEEGSR>0s) was significantly associated with POD (OR = 2.247; 95% CI = 1.414-3.571; P = 0.001). Low rScO2 at the end of surgery was statistically associated with POCD (OR = 0.981; 95% CI = 0.965-0.997; P = 0.018). The results of our study show that the degree of intraoperative EEG suppression on one hand, and low rScO2 at the end of procedure on the other hand, are associated with respectively POD and POCD in patients undergoing cardiac interventions.
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Disfunção Cognitiva/diagnóstico , Delírio do Despertar/diagnóstico , Monitorização Intraoperatória/métodos , Complicações Pós-Operatórias/diagnóstico , Espectroscopia de Luz Próxima ao Infravermelho/métodos , Idoso , Idoso de 80 Anos ou mais , Área Sob a Curva , Procedimentos Cirúrgicos Cardíacos , Ponte Cardiopulmonar , Circulação Cerebrovascular , Eletrodos , Eletroencefalografia , Feminino , Humanos , Masculino , Testes de Estado Mental e Demência , Pessoa de Meia-Idade , Oximetria/métodos , Estudos Prospectivos , Centros de Atenção TerciáriaRESUMO
PURPOSE: We examined the psychological adjustment of parents of children born with moderate to severe genital atypia 12 months after their child underwent genitoplasty. MATERIALS AND METHODS: Parents were recruited longitudinally from a multicenter collaboration of 10 pediatric hospitals with specialty care for children with disorders/differences of sex development and/or congenital adrenal hyperplasia. Parents completed measures of depressive and anxious symptoms, illness uncertainty, quality of life, posttraumatic stress and decisional regret. RESULTS: Compared to levels of distress at baseline (before genitoplasty) and 6 months after genitoplasty, data from 25 mothers and 20 fathers indicated significant improvements in all psychological distress variables. However, a subset of parents continued endorsing clinically relevant distress. Some level of decisional regret was endorsed by 28% of parents, although the specific decision that caused regret was not specified. CONCLUSIONS: Overall the majority of parents were coping well 1 year after their child underwent genitoplasty. Level of decisional regret was related to having a bachelor's level of education, increased levels of illness uncertainty preoperatively and persistent illness uncertainty at 12 months after genitoplasty but was unrelated to postoperative complications.
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Transtornos do Desenvolvimento Sexual/cirurgia , Ajustamento Emocional , Genitália/anormalidades , Pais/psicologia , Procedimentos de Cirurgia Plástica/efeitos adversos , Complicações Pós-Operatórias/psicologia , Adulto , Pré-Escolar , Tomada de Decisões , Transtornos do Desenvolvimento Sexual/psicologia , Feminino , Genitália/cirurgia , Procedimentos Cirúrgicos em Ginecologia/efeitos adversos , Procedimentos Cirúrgicos em Ginecologia/métodos , Humanos , Lactente , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Período Pós-Operatório , Qualidade de Vida , Procedimentos de Cirurgia Plástica/métodos , Estresse Psicológico/diagnóstico , Estresse Psicológico/psicologia , Resultado do Tratamento , Procedimentos Cirúrgicos Urológicos Masculinos/efeitos adversos , Procedimentos Cirúrgicos Urológicos Masculinos/métodosRESUMO
PURPOSE: We examined the psychosocial characteristics of parents of children with disorders of sex development at early presentation to a disorders of sex development clinic. Parental anxiety, depression, quality of life, illness uncertainty and posttraumatic stress symptoms were assessed. Additionally we evaluated the relationship of assigned child gender to parental outcomes. MATERIALS AND METHODS: A total of 51 parents of children with ambiguous or atypical genitalia were recruited from 7 centers specializing in treatment of disorders of sex development. At initial assessment no child had undergone genitoplasty. Parents completed the Cosmetic Appearance Rating Scale, Beck Anxiety Inventory, Beck Depression Inventory, SF-36, Parent Perception of Uncertainty Scale and Impact of Event Scale-Revised. RESULTS: A large percentage of parents (54.5%) were dissatisfied with the genital appearance of their child, and a small but significant percentage reported symptoms of anxiety, depression, diminished quality of life, uncertainty and posttraumatic stress. Few gender differences emerged. CONCLUSIONS: Although many parents function well, a subset experience significant psychological distress around the time of diagnosis of a disorder of sex development in their child. Early screening to assess the need for psychosocial interventions is warranted.
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Adaptação Psicológica , Transtornos de Ansiedade/psicologia , Transtorno Depressivo/psicologia , Transtornos do Desenvolvimento Sexual/psicologia , Pais/psicologia , Qualidade de Vida/psicologia , Transtornos de Estresse Pós-Traumáticos/psicologia , Transtornos 46, XX do Desenvolvimento Sexual/psicologia , Hiperplasia Suprarrenal Congênita/psicologia , Adulto , Transtornos de Ansiedade/diagnóstico , Transtorno Depressivo/diagnóstico , Feminino , Identidade de Gênero , Disgenesia Gonadal 46 XY/psicologia , Humanos , Cariotipagem , Masculino , Programas de Rastreamento , Transtornos de Estresse Pós-Traumáticos/diagnóstico , Inquéritos e Questionários , Síndrome de Turner/psicologiaRESUMO
Acute psychological stress has primarily been investigated regarding its effects on conventional lymphocytes such as natural killer (NK) cells and CD4(+) and CD8(+) T cells. However, it might be important to focus on more "specialized" lymphocyte subsets, playing a role, for instance, in allergic conditions and autoimmunity, to identify links between stress, the immune system and somatic diseases. Using flow cytometry we determined frequencies of circulating T helper (Th)1-type (CD226(+)) and Th2-type (CRTH2(+)) T cells, γδ T cells, conventional CD56(+) natural killer T (NKT) cells and invariant NKT cells (iNKT) in healthy young males (N = 31; median age 26 years) undergoing a laboratory computer-based stressor lasting 12 min. We found that acute psychological stress induced a prolonged increase in CD4(+) and CD8(+) T cells expressing a Th2 phenotype. We also detected an acute increase in CD4(-) and CD8(-) double negative γδ T cells. Finally, we found that the well-known increase in NK cells under stressful conditions was paralleled by a significant increase in numbers of conventional CD56(+) NKT cells. In contrast, numbers of iNKT was not altered by stress. This study adds further evidence to a psychoneuroimmunological model proposing that under stressful conditions certain lymphocyte subsets, including iNKT and less mature T cells, are retained in lymphoid tissues while antigen-experienced effector-type T cells with a Th2 phenotype, γδ T cells and conventional CD56(+) NKT cells are mobilized into the peripheral blood. We suggest that in the case of frequent stress exposure, this might result in the promotion of, for example, allergic conditions.
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Doenças Autoimunes/etiologia , Complexo CD3/imunologia , Hipersensibilidade/etiologia , Subpopulações de Linfócitos/imunologia , Células T Matadoras Naturais/imunologia , Adulto , Antígenos de Diferenciação de Linfócitos T/imunologia , Pressão Sanguínea/fisiologia , Antígeno CD56/imunologia , Linfócitos T CD8-Positivos/imunologia , Frequência Cardíaca/fisiologia , Humanos , Masculino , Receptores de Antígenos de Linfócitos T gama-delta/biossíntese , Estresse Psicológico/imunologia , Células Th2/imunologiaRESUMO
OBJECTIVE: The impact of parent-reported stigma due to their child's disorder/difference of sex development (DSD) on parent psychosocial adjustment is poorly understood. In other pediatric populations, perceived interference of medical conditions into daily activities (i.e., illness intrusiveness ) mediates the relationship of stigma to adjustment. This study assessed relationships between parent-focused and child-focused stigma â illness intrusiveness â depressive and anxious symptoms . Exploratory analyses sought to identify patient characteristics associated with stigma. METHOD: Caregivers (59 women and 43 men) of 63 children diagnosed with a DSD up to age 4 years completed measures of demographics, parent-focused and child-focused stigma, illness intrusiveness, and depressive and anxious symptoms. RESULTS: Increased parent-focused and child-focused stigma were associated with increased illness intrusiveness, which, in turn, was associated with increased depressive and anxious symptoms for parents nested within dyads. Among children with DSD family histories, parents reported greater child-focused stigma. CONCLUSION: Parents who experience DSD-related stigma report greater interference of their child's DSD into their daily activities, which is associated with poorer psychosocial adjustment. Findings support developing clinical interventions related to parents' perceptions of stigma and illness intrusiveness to improve parent adjustment.
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Pais , Estigma Social , Ansiedade/psicologia , Cuidadores/psicologia , Pré-Escolar , Feminino , Humanos , Masculino , Pais/psicologia , Desenvolvimento SexualRESUMO
BACKGROUND: To date, multiple myeloma remains an incurable malignancy due to the persistence of minimal residual disease in the bone marrow. In this setting, monoclonal antibodies against myeloma-specific cell surface antigens represent a promising therapeutic approach, which is however hampered by a lack of appropriate target structures expressed across all pathogenic myeloma cell populations. We, therefore, investigated functionally relevant immunoreceptors specifically associated with myeloma cells as well as their clonogenic precursors. DESIGN AND METHODS: Potential target proteins were identified using antibody arrays against phosphorylated immunoreceptors with lysates from myeloma cell lines. CD229 expression was confirmed in primary myeloma cells by reverse transcriptase polymerase chain reaction, western blot, fluorescence-activated cell sorting, and immunohistochemistry. Apoptosis, clonogenic growth, and sensitivity to chemotherapy were determined following short-interfering RNA-mediated downregulation of CD229. Antibody-dependent cellular and complement-dependent cytotoxicity were analyzed using a monoclonal antibody against CD229 to demonstrate the antigen's immunotherapeutic potential. RESULTS: Our screening assay identified CD229 as the most strongly over-expressed/phosphorylated immunoreceptor in myeloma cell lines. Over-expression was further demonstrated in the CD138-negative population, which has been suggested to represent myeloma precursors, as well as on primary tumor cells from myeloma patients. Accordingly, CD229 staining of patients' bone marrow samples enabled the identification of myeloma cells by flow cytometry and immunohistochemistry. Down-regulation of CD229 led to a decreased number of viable myeloma cells and clonal myeloma colonies, and enhanced the anti-tumor activity of conventional chemotherapeutics. Targeting CD229 with a monoclonal antibody resulted in complement- and cell-mediated lysis of myeloma cells. CONCLUSIONS: Our results demonstrate that the immunoreceptor CD229 is specifically over-expressed on myeloma cells including their clonogenic precursors and contributes to their malignant phenotype. Monoclonal antibodies against this protein may represent a promising diagnostic and immunotherapeutic instrument in this disease.
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Antígenos CD/metabolismo , Mieloma Múltiplo/metabolismo , Antígenos CD/genética , Antineoplásicos/farmacologia , Linhagem Celular Tumoral , Sobrevivência Celular/efeitos dos fármacos , Sobrevivência Celular/genética , Citotoxicidade Imunológica/efeitos dos fármacos , Citotoxicidade Imunológica/genética , Expressão Gênica , Regulação Neoplásica da Expressão Gênica , Inativação Gênica , Humanos , Subpopulações de Linfócitos/metabolismo , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/genética , Mieloma Múltiplo/terapia , Células-Tronco Neoplásicas/metabolismo , Plasmócitos/metabolismo , Plasmócitos/patologia , Família de Moléculas de Sinalização da Ativação LinfocitáriaRESUMO
Cancer-testis antigens (CTA) represent attractive targets for tumor immunotherapy. However, a broad picture of CTA expression in acute myeloid leukemia (AML) is missing. CTA expression was analyzed in normal bone marrow (BM) as well as in AML cell lines before and after treatment with demethylating agents and/or histone acetylase inhibitors. Presence of selected CTA with a strictly tumor-restricted expression was then determined in samples of patients with AML before and after demethylating therapy. Screening AML cell lines for the expression of 20 CTA, we identified six genes (MAGE-A3, PRAME, ROPN1, SCP-1, SLLP1, and SPO11) with an AML-restricted expression. Analyzing the expression of these CTA in blast-containing samples from AML patients (N = 64), we found all samples to be negative for MAGE-A3 and SPO11 while a minority of patients expressed ROPN1 (1.6%), SCP-1 (3.1%), or SLLP1 (9.4%). The only CTA expressed in substantial proportion of patients (53.1%) was PRAME. Following demethylating treatment with 5'-aza-2'-deoxycytidine, we observed an increased or de novo expression of CTA, in particular of SSX-2, in AML cell lines. In AML patients, we detected increased expression of PRAME and induction of SSX-2 after demethylating therapy with 5-azacytidine. With the exception of PRAME, CTA are mostly absent from AML blasts. However, demethylating treatment induces strong expression of CTA, particularly of SSX-2, in vitro and in vivo. Therefore, we propose that CTA-specific immunotherapy for AML should preferentially target PRAME and/or should be combined with the application of demethylating agents opening the perspective for alternative targets like CTA SSX-2.
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Antígenos de Neoplasias/genética , Azacitidina/análogos & derivados , Células da Medula Óssea/metabolismo , Ácidos Hidroxâmicos/farmacologia , Leucemia Mieloide Aguda/genética , Proteínas de Neoplasias/genética , Proteínas Repressoras/genética , Idoso , Antígenos de Neoplasias/metabolismo , Antimetabólitos Antineoplásicos/farmacologia , Azacitidina/farmacologia , Biomarcadores/análise , Células da Medula Óssea/citologia , Células da Medula Óssea/imunologia , Estudos de Casos e Controles , Linhagem Celular Tumoral , Metilação de DNA , Decitabina , Epigenômica , Feminino , Perfilação da Expressão Gênica , Regulação Neoplásica da Expressão Gênica , Inibidores de Histona Desacetilases/efeitos adversos , Inibidores de Histona Desacetilases/farmacologia , Histona Desacetilases/metabolismo , Humanos , Ácidos Hidroxâmicos/efeitos adversos , Imunoterapia/métodos , Leucemia Mieloide Aguda/imunologia , Leucemia Mieloide Aguda/metabolismo , Leucemia Mieloide Aguda/patologia , Masculino , Pessoa de Meia-Idade , Terapia de Alvo Molecular , Proteínas de Neoplasias/metabolismo , Proteínas Repressoras/metabolismo , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Regulação para CimaRESUMO
Purpose: Airway stenting offers good palliation and improves the quality of life in patients with inoperable bronchotracheal stenosis. However, in some cases, the management of stenting can be life-threatening. Hence, a strategy for maintaining oxygenation and hemodynamic stability should be anticipated to avoid critical situations. Herein, we report the use of extracorporeal membrane oxygenation (ECMO) in bronchotracheal stenting management to secure oxygenation and facilitate interventions. Methods: We retrospectively reviewed all patients who underwent rigid bronchoscopy under ECMO support for the management of bronchotracheal stenting at CHU UCL Namur hospital (Belgium), between January 2009 and December 2019. Results: We included 14 bronchoscopy cases performed on 11 patients (3 patients underwent 2 bronchoscopies) in this study; 12 were performed on males and 2 on females. The median age was 54 years. There were 11 benign and 3 malignant etiologies for the central airway obstruction/stenosis. Eight cases were supported by venovenous ECMO and six by venoarterial ECMO. The median ECMO time was 267 minutes. The weaning of ECMO support was successful in all cases. In most cases, the procedures were performed effectively and safely. Only two local complications caused by the cannulation of ECMO were reported, and anticoagulation was adapted to avoid bleeding at the operating site and clot formation in the system. Conclusion: Elective ECMO support was helpful and safe for the high-risk management of bronchotracheal stenting with rigid bronchoscopy and was not associated with any additional significant complications.
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Oxigenação por Membrana Extracorpórea , Broncoscopia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Qualidade de Vida , Estudos Retrospectivos , StentsRESUMO
BACKGROUND: Multiple myeloma is a life-threatening disease and despite the introduction of stem cell transplantation and novel agents such as thalidomide, lenalidomide, and bortezomib most patients will relapse and develop chemoresistant disease. Therefore, alternative therapeutic modes for myeloma are needed and cancer-testis antigens such as MAGE-C1/CT7 and MAGE-A3 have been suggested to represent a class of tumor-specific proteins particularly suited for targeted immunotherapies. Surprisingly, the biological role of cancer-testis genes in myeloma remains poorly understood. DESIGN AND METHODS: We performed the first investigation of the function of two cancer-testis antigens most commonly expressed in myeloma, MAGE-C1/CT7 and MAGE-A3, using an RNA interference-based gene silencing model in myeloma cell lines. Functional assays were used to determine changes in proliferation, cell adhesion, chemosensitivity, colony formation, and apoptosis resulting from gene-specific silencing. RESULTS: We show that the investigated genes are not involved in regulating cell proliferation or adhesion; however, they play an important role in promoting the survival of myeloma cells. Accordingly, knock-down of MAGE-C1/CT7 and MAGE-A3 led to the induction of apoptosis in the malignant plasma cells and, importantly, both genes were also essential for the survival of clonogenic myeloma precursors. Finally, silencing of cancer-testis genes further improved the response of myeloma cells to conventional therapies. CONCLUSIONS: Cancer-testis antigens such as MAGE-C1/CT7 and MAGE-A3 play an important role in promoting the survival of myeloma cells and clonogenic precursors by reducing the rate of spontaneous and chemotherapy-induced apoptosis and might, therefore, represent attractive targets for novel myeloma-specific therapies.
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Antígenos de Neoplasias/fisiologia , Mieloma Múltiplo/metabolismo , Mieloma Múltiplo/patologia , Proteínas de Neoplasias/fisiologia , Neoplasias Testiculares , Linhagem Celular Tumoral , Sobrevivência Celular/fisiologia , Humanos , MasculinoRESUMO
Disorders/differences of sex development (DSD) are congenital conditions in which there is atypical chromosomal, gonadal and/or phenotypic sex. While there remains controversy around the traditionally binary concept of sex, most patients with DSD are reared either male or female depending on their genetic sex, gonadal sex, genital phenotype and status of their internal genital tract. This study uses prospective data from 12 institutions across the United States that specialize in DSD care. We focused on patients raised female. Eligible patients had moderate to severe genital atypia (defined as Prader score >2), were ≤2 years of age at entry, and had no prior genitoplasty. The aim of this study is to describe early post operative complications for young patients undergoing modern approaches to feminizing genitoplasty. Of the 91 participants in the cohort, 57 (62%) were reared female. The majority had congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (n = 52), 1 had ovo-testicular syndrome, 2 had mixed gonadal dysgenesis and 2 had partial androgen insensitivity syndrome (PAIS). Of the 50 participants who received early genitoplasty, 43 (86%) had follow-up at 6-12 months post-surgery. Thirty-two participants (64%) received a clitoroplasty, 31 (62%) partial urogenital mobilization and 4 (8%) total urogenital sinus mobilization. Eighteen percent (9/50) experienced post-surgical complications with 7 (14%) being rated as Clavien-Dindo grade III. Both parents and surgeons reported improved satisfaction with genital appearance of participants following surgery compared to baseline. This information on post-operative complications associated with contemporary approaches to feminizing genitoplasty performed in young children will help guide families when making decisions about whether or not to proceed with surgery for female patients with moderate to severe genital atypia.
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Hiperplasia Suprarrenal Congênita , Transtornos do Desenvolvimento Sexual , Hiperplasia Suprarrenal Congênita/cirurgia , Criança , Pré-Escolar , Estudos de Coortes , Transtornos do Desenvolvimento Sexual/cirurgia , Feminino , Genitália/cirurgia , Humanos , Masculino , Estudos Prospectivos , Procedimentos Cirúrgicos Urogenitais/efeitos adversosRESUMO
PURPOSE: Little is known about the phenotypes, diagnoses, and sex of rearing of infants with atypical genital development in the United States. As part of a multicenter study of these infants, we have provided a baseline report from US difference/disorder of sex development clinics describing the diagnoses, anatomic features, and sex of rearing. We also determined whether consensus guidelines are followed for sex designation in the United States. METHODS: Eligible participants had moderate-to-severe genital atypia, were aged <3 years, and had not undergone previous genitoplasty. Karyotype, genetic diagnosis, difference/disorder of sex development etiology, family history, and sex of rearing were collected. Standardized examinations were performed. RESULTS: Of 92 subjects, the karyotypes were 46,XX for 57%, 46,XY for 34%, and sex chromosome abnormality for 9%. The median age at the baseline evaluation was 8.8 months. Most 46,XX subjects (91%) had congenital adrenal hyperplasia (CAH) and most 46,XY subjects (65%) did not have a known diagnosis. Two individuals with CAH underwent a change in sex of rearing from male to female within 2 weeks of birth. The presence of a uterus and shorter phallic length were associated with female sex of rearing. The most common karyotype and diagnosis was 46,XX with CAH, followed by 46,XY with an unknown diagnosis. Phenotypically, atypical genitalia have been most commonly characterized by abnormal labioscrotal tissue, phallic length, and urethral meatus location. CONCLUSIONS: An increased phallic length was positively associated with rearing male. Among the US centers studied, sex designation followed the Consensus Statement recommendations. Further study is needed to determine whether this results in patient satisfaction.
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The decision about unsupervised privileges for sexual offenders against children (SOC) is one of the most difficult decisions for practitioners in forensic high-security hospitals. Facing the possible consequences of the decision for the society, a valid and reliable risk management of SOCs is essential. Some risk management approaches provide frameworks for the construction of relevant future risk situations. Due to ethical reasons, it is not possible to evaluate the validity of constructed risk situations in reality. The aim of the study was to test if behavioral monitoring of SOCs in high-immersive virtual risk situations provides additional information for risk management. Six SOCs and seven non-offender controls (NOC) walked through three virtual risk situations, confronting the participant with a virtual child character. The participant had to choose between predefined answers representing approach or avoidance behavior. Frequency of chosen answers were analyzed in regards to knowledge of the participants about coping skills and coping skills focused during therapy. SOCs and NOCs behavior differed only in one risk scenario. Furthermore, SOCs showed in 89% of all cases a behavior not corresponding to their own belief about adequate behavior in comparable risk situations. In 62% of all cases, SOCs behaved not corresponding to coping skills they stated that therapists focused on during therapy. In 50% of all cases, SOCs behaved in correspondence to coping skills therapists stated that they focused on during therapy. Therapists predicted the behavior of SOCs in virtual risk situations incorrect in 25% of all cases. Thus, virtual risk scenarios provide the possibility for practitioners to monitor the behavior of SOCs and to test their decisions on unsupervised privileges without endangering the community. This may provide additional information for therapy progress. Further studies are necessary to evaluate the predictive and ecological validity of behavioral monitoring in virtual risk situations for real life situations.
RESUMO
BACKGROUND/AIMS: Parents of children born with disorders of sex development (DSD) often experience anxiety, but risk factors, including parental perception of the severity of their child's DSD, have not been examined. We hypothesized that severity of illness (SOI) ratings would relate to parental anxiety, and would be higher for parents of children with a potentially life-threatening DSD (e.g., 21-hydroxylase deficiency). METHODS: Eighty-nine parents (Mage = 33.0, 56.2% mothers) of 51 children (Mage in months = 8.7) with a DSD including ambiguous genitalia were recruited from 12 specialized DSD clinics. Parents completed questionnaires prior to genitoplasty, 6 months post-genitoplasty, and 12 months post-genitoplasty (if completed). Data were analyzed with linear mixed modeling. RESULTS: Parental anxiety decreased over time, χ2(1) = 10.14, p < 0.01. A positive relationship between SOI and anxiety was found, with SOI being a strong predictor of anxiety (b = 0.53, p < 0.01; χ2[1] = 5.33, p < 0.05). An SOI by time interaction indicated SOI had an increasing effect on anxiety over time, b = 0.06, p < 0.05; χ2(1) = 6.30, p < 0.05. There was no diagnosis by SOI interaction. CONCLUSION: Parental anxiety decreased over time, but those with higher SOI ratings reported greater initial anxiety followed by slower resolution over time. Underlying etiology of DSD had no effect on the relationship between SOI and anxiety.
Assuntos
Ansiedade/psicologia , Cuidadores/psicologia , Transtornos do Desenvolvimento Sexual/diagnóstico , Pais/psicologia , Percepção , Adulto , Feminino , Humanos , Masculino , Qualidade de Vida , Fatores de Risco , Índice de Gravidade de Doença , Inquéritos e QuestionáriosRESUMO
BACKGROUND: The birth of a child with a disorder of sex development (DSD) and atypical genitalia can be traumatizing and isolating for families. Parents of children with DSD are at risk for increased levels of psychological distress, including depression, anxiety, illness uncertainty (IU), post-traumatic stress symptoms (PTSS), and impairments in quality of life (QOL). Our previous report indicated that although the majority of parents of children with atypical genitalia were coping well prior to the child's genitoplasty, approximately 25% of them reported experiencing some type of psychological distress. OBJECTIVE: The current study sought to examine the trajectory of parental psychological distress prior to, and 6 months after their child underwent genitoplasty. METHODS: Parents were recruited as part of an ongoing, prospective, multi-site study involving 10 pediatric hospitals with specialized care for children with atypical genitalia. Results from 49 parents (27 mothers, 22 fathers) of 28 children (17 female sex of rearing, 11 male sex of rearing) born with atypical genitalia (Prader rating of 3-5 in 46,XX DSD or by a Quigley rating of 3-6 in 46,XY DSD or 45,XO/46,XY) were included in the study. RESULTS: There were no significant changes in level of depressive or anxious symptoms or quality of life between baseline and 6-month post-operative follow-up, although mothers continued to report significantly higher levels of depressive symptoms than fathers, and as a group, these parents reported lower QOL than published norms. The level of PTSS significantly decreased for all parents, suggesting that parents may have come through the acute stress phase associated with their child's diagnosis. Finally, while there were no significant changes in IU over the time period, the level of IU for parents of boys actually increased, while parents of girls reported no change (Figure). CONCLUSION: Six months after their child has undergone genitoplasty, the majority of parents report minimal levels of psychological distress. However, a subset of these parents continue to experience significant distress related to their child's diagnosis. Specifically, parents of boys appear to be at increased risk for difficulties, which may be related to either the lack of clinical diagnosis for almost half of these children or the necessity of two-stage surgeries for the majority of them. We will continue collecting data on these families to better understand the trajectory of these adjustment variables.