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1.
Pharmacogenet Genomics ; 34(4): 135-138, 2024 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-38372405

RESUMO

CYP2D6 is a highly polymorphic gene with clinically important structural variations. Commonly, only exon 9 is assayed on clinical pharmacogenomics panels, as it allows for accurate functional characterization even in the presence of a CYP2D6::CYP2D7 conversion. However, this method does not capture CYP2D7::CYP2D6 (CYP2D6*13) conversions, possibly leading to inaccurate phenotype assignment. The study's purpose was to determine the frequency of structural variations in CYP2D6 utilizing multiple copy number variation (CNV) assay locations to quantify the potential impact on clinical phenotype classification. A retrospective analysis was conducted of de-identified pharmacogenomics data submitted through the Translational Software, Inc. platform. Samples with CYP2D6 CNV data for exon 9 and at least one additional CNV location (5'UTR, exon 1, intron 2, exon 5 or intron 6) were included. CYP2D7::CYP2D6 and CYP2D6::CYP2D7 conversions were classified according to PharmVar nomenclature. The CYP2D6 copies were capped at four total copies to account for assay limitations in detecting more than four copies. A total of 106,474 samples were included for analysis. CYP2D7::CYP2D6 conversions were present in approximately 2.44% of samples, and 5.84% of samples had CYP2D6::CYP2D7 conversions. Many samples did not have a CYP2D7 conversion detected (91.5%; 97,462/106,474). A full gene deletion was detected in 0.15%, and 5.98% had a duplication or multiplication present. This retrospective study underscores the importance of testing more than one CNV site for CYP2D6 . Over 2% of patients were found to have a CYP2D7::CYP2D6 conversion. This translates into potentially misclassified phenotype classification and incongruent clinical recommendations.


Assuntos
Citocromo P-450 CYP2D6 , Variações do Número de Cópias de DNA , Citocromo P-450 CYP2D6/genética , Humanos , Estudos Retrospectivos , Fenótipo , Éxons/genética , Farmacogenética
2.
Pediatr Crit Care Med ; 25(5): 407-415, 2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38329381

RESUMO

OBJECTIVES: Nudging, a behavioral economics concept, subtly influences decision-making without coercion or limiting choice. Despite its frequent use, the specific application of nudging techniques by clinicians in shared decision-making (SDM) is understudied. Our aim was to analyze clinicians' use of nudging in a curated dataset of family care conferences in the PICU. DESIGN: Between 2019 and 2020, we retrospectively studied and coded 70 previously recorded care conference transcripts that involved physicians and families from 2015 to 2019. We focused on decision-making discussions examining instances of nudging, namely salience, framing, options, default, endowment, commission, omission, recommend, expert opinion, certainty, and social norms. Nudging instances were categorized by decision type, including tracheostomy, goals of care, or procedures. SETTING: Single-center quaternary pediatric facility with general and cardiac ICUs. PATIENTS: None. INTERVENTIONS: None. MEASUREMENTS: We assessed the pattern and frequency of nudges in each transcript. MAIN RESULTS: Sixty-three of the 70 transcripts contained SDM episodes. These episodes represented a total of 11 decision categories based on the subject matter of nudging instances, with 308 decision episodes across all transcripts (median [interquartile range] 5 [4-6] per conference). Tracheostomy was the most frequently discussed decision. A total of 1096 nudging instances were identified across the conferences, with 8 (6-10) nudge types per conference. The most frequent nudging strategy used was gain frame (203/1096 [18.5%]), followed by loss frame (150/1096 [13.7%]). CONCLUSIONS: Nudging is routinely employed by clinicians to guide decision-making, primarily through gain or loss framing. This retrospective analysis aids in understanding nudging in care conferences: it offers insight into potential risks and benefits of these techniques; it highlights ways in which their application has been used by caregivers; and it may be a resource for future trainee curriculum development.


Assuntos
Tomada de Decisão Compartilhada , Unidades de Terapia Intensiva Pediátrica , Humanos , Estudos Retrospectivos , Unidades de Terapia Intensiva Pediátrica/organização & administração , Criança , Família/psicologia , Relações Profissional-Família , Masculino , Feminino , Cuidados Críticos
3.
Pediatr Emerg Care ; 40(5): 382-385, 2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38194682

RESUMO

BACKGROUND: Pediatric acute ischemic stroke is a rare diagnosis that requires timely recognition and definitive management to prevent morbidity and mortality. Children often present to primary care offices, urgent care clinics, and adult emergency departments for evaluation of symptoms that may be signs and symptoms of stroke. Currently, there are no published prehospital or transport protocols specific to pediatric acute ischemic stroke. The Children's Mercy Hospital Critical Care Transport Team (CMCCT) created a pediatric-specific clinical practice guideline (CPG) for suspected acute ischemic stroke. METHODS: This retrospective, descriptive study reports pediatric patients aged younger than 18 years who met criteria for the pediatric stroke CPG and required interfacility transport by CMCCT over a 4- year period. Large vessel occlusion (LVO) scores used in adults were calculated retrospectively. RESULTS: Seventeen patients met inclusion criteria. Four (24%) of 17 had radiographic evidence of acute thrombus, 3 of whom received alteplase and/or endovascular clot retrieval. Median age of confirmed stroke was 83 months (interquartile range, 65-148) compared with 177 months for nonstroke (interquartile range, 169-191), P = 0.126. The most common presenting symptom was hemiplegia in the confirmed stroke group. The confirmed stroke group scored significantly lower on the Glasgow Coma Scale (median of 8 vs 15, P = 0.014), significantly higher on the Los Angeles Motor Scale LVO score (median 4 vs 0, P = 0.021), and significantly higher on the Rapid Arterial Occlusion Evaluation LVO (median 4 vs 0, P = 0.036). CONCLUSIONS: To our knowledge, the CMCCT CPG is the first pediatric transport protocol aimed at recognition and management of pediatric stroke described in the literature. Retrospective calculation of LVO scores show that they may be helpful in application to pediatric patients.


Assuntos
Guias de Prática Clínica como Assunto , Humanos , Estudos Retrospectivos , Criança , Feminino , Pré-Escolar , Masculino , Adolescente , Lactente , Acidente Vascular Cerebral/terapia , Transporte de Pacientes , Transferência de Pacientes/estatística & dados numéricos , Escala de Coma de Glasgow , AVC Isquêmico/terapia , AVC Isquêmico/diagnóstico , Ativador de Plasminogênio Tecidual/uso terapêutico , Ativador de Plasminogênio Tecidual/administração & dosagem
4.
Am J Hum Biol ; 35(2): e23815, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36196910

RESUMO

INTRODUCTION: Both the triglyceride to HDL cholesterol (TG/HDL) ratio and timing of pubertal maturation have been identified as independent contributors to the development of atherosclerosis. OBJECTIVE: The purpose of our study was to determine the relationship between the TG/HDL ratio and measures of vascular health in children and adolescents with dyslipidemia stratified by somatic maturity. We hypothesized that somatic maturity would have a significant interaction with TG/HDL ratio and vascular health. METHODS: This was a longitudinal analysis of 120 children and adolescents (age 8-14 years) with dyslipidemia recruited from a pediatric preventive cardiology clinic. At baseline and each follow-up visit, a non-fasting serum lipid panel was collected and vascular health (carotid artery intima--media thickness, pulse wave velocity, augmentation index) was assessed. Peak height velocity (PHV) was calculated at each visit, and participants were stratified into groups by maturity offset (pre-PHV, mid-PHV, post-PHV). A mixed model design permitted baseline and follow-up visits to be classified as discrete data points. RESULTS: Of the n = 235 data points (pre-PHV = 23%, mid-PHV = 19%, and post-PHV = 58%), we identified no significant interaction between TG/HDL ratio, maturity offset, and measures of vascular structure or function. There was also no significant relationship found between TG/HDL and maturity group. Within the mid-pubertal group, there was weak relationship found between TG/HDL and augmentation index. CONCLUSION: Despite the well-described relationship between early pubertal maturation and development of cardiovascular risk factors in adulthood, we found that vascular damage resulting from an elevated TG/HDL ratio is not independently associated with somatic maturity.


Assuntos
Dislipidemias , Lipoproteínas HDL , Humanos , Criança , Adolescente , Triglicerídeos , Análise de Onda de Pulso , HDL-Colesterol , Dislipidemias/etiologia
5.
BMC Pediatr ; 23(1): 471, 2023 09 19.
Artigo em Inglês | MEDLINE | ID: mdl-37726654

RESUMO

BACKGROUND: Childhood obesity rates have continued to increase with the COVID-19 pandemic. However, data are limited on the impact of increasing obesity on associated comorbidities. METHODS: We evaluated the progression of overweight- or obesity-associated comorbidities by investigating change in laboratory results pre-COVID-19 pandemic and post-COVID-19 pandemic onset in youth with overweight or obesity. We defined progression of comorbidities based on increase in category rather than absolute change in value. RESULTS: HbA1c progression was seen in 19%, and LDL cholesterol progression was seen in 26%, as defined by categories. HbA1c progression and LDL cholesterol progression were significantly correlated. HbA1c and LDL cholesterol progression were significantly associated with older age and Hispanics, respectively. CONCLUSION: The results indicate youths with overweight or obesity have experienced progression of comorbidities during the COVID-19 pandemic. This study emphasizes the importance of early detection of comorbidities among a high-risk pediatric population.


Assuntos
COVID-19 , Obesidade Infantil , Criança , Adolescente , Humanos , Sobrepeso/epidemiologia , LDL-Colesterol , Hemoglobinas Glicadas , Pandemias , Obesidade Infantil/epidemiologia , COVID-19/epidemiologia
6.
Matern Child Health J ; 27(4): 621-631, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-36840786

RESUMO

INTRODUCTION: Recognition of the importance of adolescents' environments in influencing their sexual and reproductive health (SRH) decision-making necessitates a deeper understanding of the role that community stakeholders play in shaping Adolescent and Young Adults (AYA) access to SRH education and care. We describe community stakeholders' knowledge, beliefs, and attitudes about AYA's SRH needs in three rural Latino communities in Kansas. METHODS: Key stakeholders completed a written survey incorporating the theory of Planned Behavior to assess attitudes, norms, and intentions to support AYA's SRH education and access to care. RESULTS: Across three rural immigrant community settings, respondents (N = 55) included 8 community health workers, 9 health care providers, 7 public health officials, 19 school health officials, and 12 community members. More than half self-identified as Latino (55%). Six (11%) participants, half of whom were in the health sector, thought SRH education would increase the likelihood that teens would engage in sexual activities. In contrast, other stakeholders thought that providing condoms (17, 30.9%), contraception other than condoms (14, 25.5%), and providing HPV immunization (5, 9.6%) would increase the likelihood of engaging in sexual activity. Ambivalence regarding support for SRH education and service provision prevailed across sectors, reflected even in the endorsement of the distribution of less effective contraceptive methods. Obstacles to care include immigration status, discrimination, lack of confidential services, and transportation. CONCLUSION: Key stakeholders living in rural communities revealed misconceptions, negative attitudes, and ambivalent beliefs related to the delivery of SRH education and services, potentially creating barriers to AYA's successful access to care.


Little is known about the knowledge, beliefs and attitudes held by key stakeholders in rural immigrant Latino communities regarding their level of support for AYA's SRH needs. Key stakeholders may harbor influential and ambivalent opinions regarding AYA's sexuality considering existing cultural norms, therefore, stigmatizing access to SRH education and care. We examined key community stakeholders' attitudes and norms as measures of intentions to support AYA's SRH education and care. We identify actionable steps needed to eliminate existing barriers to AYA's access to SRH education and care among at-risk immigrant Latino communities in the rural Midwest.


Assuntos
Gravidez na Adolescência , Serviços de Saúde Reprodutiva , Gravidez , Feminino , Adolescente , Adulto Jovem , Humanos , População Rural , Anticoncepção , Educação Sexual , Comportamento Sexual , Saúde Reprodutiva , Hispânico ou Latino
7.
Cardiol Young ; 33(7): 1136-1142, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35864813

RESUMO

INTRODUCTION: Children with a single ventricle post-Fontan palliation are at increased risk of poor outcomes with peak oxygen consumption acting as a surrogate outcome marker. The purpose of this study is to evaluate the relationship between peak oxygen consumption and echocardiographic measures of ventricular function and deformation, including ventricular global longitudinal strain and dyssynchrony, in children and adolescents following Fontan palliation. METHODS: Patients (age 8-21 years) with single ventricle post-Fontan palliation were prospectively recruited and participated in an echocardiogram, including views optimised for two-dimensional speckle tracking, and a cardiopulmonary exercise test on a cycle ergometer to maximal volitional fatigue. RESULTS: Thirty-eight patients (mean age 13.7 ± 2.3 years) post-Fontan palliation had either a single left ventricular (n = 20), single right ventricular (n = 14), or biventricular (n = 4) morphology. Peak oxygen consumption (24.9 ± 5.6 ml/kg/minute) was correlated with global longitudinal strain (r = -0.435, p = 0.007), a strain discoordination time to peak index (r = -0.48, p = 0.003), and the presence of an electro-mechanical dyssynchrony strain pattern (p = 0.008). On multivariate regression modelling, these three variables were associated with peak oxygen consumption independently of age and sex. The single right ventricular group had evidence of possible diastolic dysfunction by E/e' compared to the single left ventricular and biventricular groups (p = 0.001). CONCLUSIONS: Strain analysis measures are correlated with peak oxygen consumption in this cohort of children, adolescents, and young adults following Fontan palliation, suggesting that ventricular mechanics may influence the efficiency of the Fontan circulation.


Assuntos
Técnica de Fontan , Adulto Jovem , Humanos , Criança , Adolescente , Adulto , Técnica de Fontan/efeitos adversos , Ventrículos do Coração , Ecocardiografia/métodos , Função Ventricular , Consumo de Oxigênio
8.
Am J Perinatol ; 39(10): 1104-1111, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-33336348

RESUMO

OBJECTIVE: This study aimed to determine the factors associated with positive infant drug screen and create a shortened screen and a prediction model. STUDY DESIGN: This is a retrospective cohort study of all infants who were tested for drugs of abuse from May 2012 through May 2014. The primary outcome was positive infant urine or meconium drug test. Multivariable logistic regression was used to identify independent risk factors. A combined screen was created, and test characteristics were analyzed. RESULTS: Among the 3,861 live births, a total of 804 infants underwent drug tests. Variables associated with having a positive infant test were (1) positive maternal urine test, (2) substance use during pregnancy, (3) ≤ one prenatal visit, and (4) remote substance abuse; each p-value was less than 0.0001. A model with an indicator for having at least one of these four predictors had a sensitivity of 94% and a specificity of 69%. Application of this screen to our population would have decreased drug testing by 57%. No infants had a positive urine drug test when their mother's urine drug test was negative. CONCLUSION: This simplified screen can guide clinical decision making for determining which infants should undergo drug testing. Infant urine drug tests may not be needed when a maternal drug test result is negative. KEY POINTS: · Many common drug screening criteria are not predictive.. · Four criteria predicted positive infant drug tests.. · No infant urine drug test is needed if the mother tests negative..


Assuntos
Mecônio , Transtornos Relacionados ao Uso de Substâncias , Feminino , Humanos , Recém-Nascido , Programas de Rastreamento , Gravidez , Estudos Retrospectivos , Detecção do Abuso de Substâncias , Transtornos Relacionados ao Uso de Substâncias/diagnóstico , Transtornos Relacionados ao Uso de Substâncias/epidemiologia , Transtornos Relacionados ao Uso de Substâncias/urina
9.
J Pediatr Orthop ; 42(8): 408-412, 2022 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-35765866

RESUMO

BACKGROUND: A high index of suspicion for nerve palsy is essential in the setting of a displaced supracondylar humerus fracture (SCHF) with careful attention to the examination. We hypothesize that nerve injuries are more prevalent in higher energy type III and flexion SCHFs compared with type II fractures. METHODS: A retrospective review was performed of 1085 operatively treated SCHFs in pediatric patients, aged 0 to 14 years, between January 1, 2015 and December 31, 2018. There were 979 patients eligible for analysis. Exclusion criteria included follow-up <3 weeks, polytrauma, pathologic fracture, and prior fracture of the ipsilateral elbow. RESULTS: The overall rate of nerve injury was 10.6% (104/979). A significant difference in the rate of nerve palsy was identified between fracture types: 0.9% type II, 19.3% type III, and 10.7% flexion type SCHFs ( P <0.001). Median nerve palsy was the most common (n=65). An increased rate of ulnar nerve palsy was observed in flexion type SCHFs. The rate of nerve palsy was higher in the medial pin group (14.9%, P =0.035) but no significant association with iatrogenic ulnar nerve palsy was present. In type III and flexion SCHFs, operative time >60 minutes ( P =0.023) and the need for open reduction ( P =0.012) were significantly associated with postoperative nerve palsy. Referral to therapy was required in 30.8% of patients with a nerve palsy compared with 7.9% of patients without ( P <0.001). CONCLUSION: Longer operative times, need for open reduction, and significantly higher rates of nerve palsy among type III and flexion SCHFs are best explained by the unstable nature of the fracture and greater degree of soft tissue trauma. Given prolonged duration of follow-up and more frequent need for therapy demonstrated in patients with a nerve palsy, these data can be used to improve counseling for families and patients after displaced SCHF. LEVEL OF EVIDENCE: Level III-retrospective comparative study.


Assuntos
Fraturas do Úmero , Neuropatias Ulnares , Criança , Humanos , Fraturas do Úmero/complicações , Fraturas do Úmero/cirurgia , Úmero/cirurgia , Paralisia/complicações , Estudos Retrospectivos , Resultado do Tratamento
10.
J Pediatr ; 236: 284-290, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-33811870

RESUMO

OBJECTIVE: To assess justice system involvement among adolescents in the pediatric emergency department and identify associations with risk and protective factors. STUDY DESIGN: We conducted a cross-sectional, computerized survey of adolescents to assess for personal, justice system involvement, and nonhousehold justice system involvement (ie, important people outside of household). We assessed sexual behaviors, violent behaviors, substance use, school suspension/expulsion, parental supportiveness, and participant mood (score <70 indicates psychological distress). We compared differences between groups using the χ2 tests, Fisher exact tests, t tests, and performed multivariable logistic regression analyses. RESULTS: We enrolled 191 adolescents (mean age 16.1 years, 61% female). Most (68%) reported justice system involvement: personal (13%), household (42%), and nonhousehold (40%). Nearly one-half (47%) were sexually active and 50% reported school suspension/expulsion. The mean score for mood was 70.1 (SD 18); adolescents with justice system involvement had had lower mood scores (68 vs 74, P = .03) compared with those without justice system involvement. In a multivariable model, school expulsion/suspension was significantly associated with reporting any justice system involvement (OR 10.4; 95% CI 4.8-22.4). CONCLUSIONS: We identified the pediatric emergency department as a novel location to reach adolescents at risk for poor health outcomes associated with justice system involvement. Future work should assess which health promotion interventions and supports are desired among these adolescents and families.


Assuntos
Comportamento do Adolescente , Transtornos Relacionados ao Uso de Substâncias , Adolescente , Criança , Estudos Transversais , Serviço Hospitalar de Emergência , Feminino , Promoção da Saúde , Humanos , Masculino
11.
Am J Kidney Dis ; 78(1): 96-102.e1, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-33421455

RESUMO

RATIONALE & OBJECTIVE: The preferred vascular access for hemodialysis recipients is an arteriovenous fistula in the nondominant arm. Prior placement of a peripheral intravenous (PIV) catheter can lead to vascular injury and limit options for arteriovenous fistula creation, a particular problem for children, who may need hemodialysis for their entire lifetime. We instituted an initiative to increase the frequency of PIV catheter placement in the dominant arm for hospitalized pediatric patients with advanced chronic kidney disease (CKD). STUDY DESIGN: Quality improvement initiative. SETTING & PARTICIPANTS: Children with CKD stage 3-5, receiving dialysis, and/or following kidney transplantation who were hospitalized at one children's hospital between September 2018 and August 2020. QUALITY IMPROVEMENT ACTIVITIES: Retrospective data on PIV catheter location for patients from January 1 to June 30, 2017, served as baseline data. Quality improvement activities consisted of: 1) education of the multidisciplinary treatment team, patients, and parents regarding importance of vein preservation; 2) placement of individualized notes in the electronic medical record identifying the preferred arm for PIV catheter placement; 3) use of "restricted extremity" arm bands; and 4) vascular access team participation to minimize attempts for PIV catheter placement. OUTCOME: Monthly compliance with placement of PIV catheters in dominant arms. ANALYTICAL APPROACH: Location of PIV catheter placements were determined monthly and used to create run charts describing compliance. RESULTS: At baseline and before institution of this initiative, 34 of 72 (47%) PIV catheters were placed in patients' dominant arms, with only 2 of 8 (25%) PIV catheters placed in the dominant arm for children aged<5 years. After instituting the initiative, 345 of 371 (93%) PIV catheters were placed in the dominant arm of 93 children; in children aged<5 years, 58 of 62 (94%) PIV catheters were placed in the dominant arm. Only 38 of 371 (10%) PIV catheters were placed in the antecubital vein. LIMITATIONS: Single-center study. CONCLUSIONS: Education regarding the importance of vein preservation, along with implementation of a standardized process for identifying children for whom vein preservation is important, can help direct PIV catheter placement and potentially preserve vasculature in pediatric patients with CKD.


Assuntos
Cateterismo Periférico/normas , Melhoria de Qualidade , Insuficiência Renal Crônica/terapia , Adolescente , Criança , Feminino , Humanos , Masculino , Estudos Retrospectivos
12.
Pediatr Blood Cancer ; 68(9): e29085, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-33913590

RESUMO

BACKGROUND/OBJECTIVES: Rhabdomyosarcoma (RMS) is characterized by the expression of the myogenic regulatory protein MYOD1. Histologic types include alveolar, embryonal (ERMS), and spindle cell sclerosing RMS (SRMS). SRMS harbors MYOD1 mutations in a subset of adult cases in association with poor prognosis. DESIGN/METHODS: To study the level of MYOD1 protein expression and its clinical significance, we have analyzed variable numbers of pediatric (<18 years of age) and adult (age range ≥18 to 35 years) ERMS and SRMS cases for presence or absence of MYOD1 immunoreactivity in correlation with clinical outcome and MYOD1 L122R mutations. RESULTS: Lack of MYOD1 immunoreactivity, identified in 23.8% of nonalveolar RMS (non-ARMS) cases, was more prevalent in SRMS (44%) than ERMS (17.2%) and was significantly associated with low overall survival and unfavorable tumor sites (p < .05). Lack of MYOD1 immunoreactivity was not associated with MYOD1 L122R mutations, which were identified in 3/37 (8%) cases including only two of 31 (6.5%) pediatric cases, one of 11 or 9% pediatric SRMS, and one case of infant ERMS. CONCLUSION: These studies highlight the prognostic role of MYOD1 in non-ARMS. Lack of MYOD1 immunoreactivity is associated with poor prognosis in ERMS and SRMS. MYOD1 gene mutations are generally infrequent in pediatric RMS. Although mutations are predominant in SRMS, they may exceptionally occur in infantile ERMS.


Assuntos
Rabdomiossarcoma Alveolar , Rabdomiossarcoma Embrionário , Rabdomiossarcoma , Adolescente , Adulto , Criança , Humanos , Lactente , Mutação , Proteína MyoD/genética , Prognóstico , Rabdomiossarcoma/genética , Adulto Jovem
13.
Pediatr Nephrol ; 36(8): 2349-2360, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33693990

RESUMO

BACKGROUND: Eculizumab is approved for the treatment of atypical hemolytic uremic syndrome (aHUS). Its use off-label is frequently reported. The aim of this study was to describe the broader use and outcomes of a cohort of pediatric patients exposed to eculizumab. METHODS: A retrospective, cohort analysis was performed on the clinical and biomarker characteristics of eculizumab-exposed patients < 25 years of age seen across 21 centers of the Pediatric Nephrology Research Consortium. Patients were included if they received at least one dose of eculizumab between 2008 and 2015. Traditional summary statistics were applied to demographic and clinical data. RESULTS: A total of 152 patients were identified, mean age 9.1 (+/-6.8) years. Eculizumab was used "off-label" in 44% of cases. The most common diagnoses were aHUS (47.4%), Shiga toxin-producing Escherichia coli HUS (12%), unspecified thrombotic microangiopathies (9%), and glomerulonephritis (9%). Genetic testing was available for 60% of patients; 20% had gene variants. Dosing regimens were variable. Kidney outcomes tended to vary according to diagnosis. Infectious adverse events were the most common adverse event (33.5%). No cases of meningitis were reported. Nine patients died of noninfectious causes while on therapy. CONCLUSIONS: This multi-center retrospective cohort analysis indicates that a significant number of children and young adults are being exposed to C5 blockade for off-label indications. Dosing schedules were highly variable, limiting outcome conclusions. Attributable adverse events appeared to be low. Cohort mortality (6.6%) was not insignificant. Prospective studies in homogenous disease cohorts are needed to support the role of C5 blockade in kidney outcomes.


Assuntos
Nefrologia , Adolescente , Anticorpos Monoclonais Humanizados/efeitos adversos , Síndrome Hemolítico-Urêmica Atípica/tratamento farmacológico , Síndrome Hemolítico-Urêmica Atípica/genética , Criança , Humanos , Estudos Prospectivos , Estudos Retrospectivos
14.
Pediatr Emerg Care ; 37(1): e37-e41, 2021 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-29596282

RESUMO

OBJECTIVES: This study aimed to determine acceptability of intimate partner violence (IPV) materials and effect of the materials on caregiver attitudes toward IPV screening. METHODS: We used an anonymous computerized survey to assess approval of IPV materials and screening, and willingness to disclose IPV in response to screening among mothers bringing a child to an urban pediatric emergency department or 2 suburban pediatric urgent care centers affiliated with a free-standing academic children's hospital. We compared responses between participants recruited before and after display of the IPV materials, as well as between subgroups, using χ2 tests. RESULTS: A total of 522 participated (predisplay, n = 261; postdisplay, n = 261). More subjects in the postdisplay group approved of display of IPV materials in pediatric emergency department/urgent care center restrooms (94% pre vs 98% post, P = 0.04) and examination rooms (94% pre vs 98% post, P = 0.01). We found no differences in acceptability of IPV screening between the pre and post groups (73% pre and post, P = 0.92). Willingness to disclose IPV in response to screening was higher in the postdisplay group for those with a personal IPV history (55% pre, 73% post; P = 0.02), African Americans (60% pre, 78% post; P = 0.02), and those with a high school degree or less education (66% pre, 77% post; P = 0.04). CONCLUSIONS: Intimate partner violence materials in this study were acceptable to most participants and did not negatively impact attitudes toward IPV screening or willingness to disclose IPV. Display of IPV materials should be considered as a component of IPV intervention.


Assuntos
Atitude , Cuidadores , Violência por Parceiro Íntimo , Programas de Rastreamento , Educação de Pacientes como Assunto , Cuidadores/psicologia , Criança , Feminino , Humanos , Mães/psicologia , Inquéritos e Questionários
15.
BMC Gastroenterol ; 19(1): 26, 2019 Feb 11.
Artigo em Inglês | MEDLINE | ID: mdl-30744574

RESUMO

BACKGROUND: In adults, there is a consensus for standards to diagnose gastroparesis utilizing a gastric emptying study as the key diagnostic modality but there is no consensus for a standard in pediatrics. Additionally, some cost savings might be achieved if symptoms could be utilized to predict patients with gastroparesis. The aims of the current study were to confirm the sensitivity of a 4 h study in the pediatric population and to assess whether the severity of symptoms were predictive of delayed gastric emptying. STUDY: This was a single site, two part study. In the first part, results were reviewed for all patients who had completed a 4-h, solid gastric emptying study over the course of a 3 year period. In the second portion of the study, participants scheduled for a gastric emptying study, completed a modified GCSI questionnaire. RESULTS: Out of a total of 109 participants, at 2 h, 14 participants (12.8%) had abnormal studies as compared to 26 (23.85%) participants who had abnormal studies at 4 h (p = .0027). Of the 95 participants with normal studies at 2 h, 15% (14/95) were abnormal at 4 h. There were no differences in symptom severity scores between those with slow and those with normal emptying at either 2 h or 4 h. CONCLUSIONS: Our study adds independent confirmation that extending studies from 2 to 4 h increases the diagnostic yield and should be the standard in children and adolescents as it is in adults.


Assuntos
Esvaziamento Gástrico , Gastroparesia/diagnóstico por imagem , Gastroparesia/fisiopatologia , Adolescente , Criança , Pré-Escolar , Redução de Custos , Feminino , Humanos , Masculino , Estudos Prospectivos , Cintilografia/economia , Compostos Radiofarmacêuticos , Inquéritos e Questionários , Coloide de Enxofre Marcado com Tecnécio Tc 99m , Fatores de Tempo
16.
Pediatr Nephrol ; 34(2): 261-267, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30194664

RESUMO

BACKGROUND: Nephrotic syndrome (NS) results in hypercoagulability and increased risk of infection. Furthermore, infection increases the risk of venous thromboembolism (VTE). Our objective was to determine the prevalence of infection, VTE, and the associated outcomes among a cohort of hospitalized children with NS. METHODS: All children with NS admitted to 17 pediatric hospitals across North America from 2010 to 2012 were included. Prevalence of infection and VTE was determined. Wilcoxon rank-sum and logistic regression were performed. RESULTS: Seven-hundred thirty hospitalizations occurred among 370 children with NS. One-hundred forty-eight children (40%) had ≥ 1 infection (211 episodes) and 11 (3%) had VTE. Those with VTE had infection more frequently (p = 0.046) and were younger at NS diagnosis (3.0 vs. 4.0 years; p = 0.008). The most common infectious pathogen identified was Streptococcus pneumoniae. The median hospital length of stay for those with infection [10 vs 5 days (p < 0.0001)] or VTE [22 vs 6 days (p < 0.0001)] was longer than those without either complication. Of those with infection, 13% had an intensive care unit (ICU) stay compared with 3.3% of those without infection. Median ICU stay was 4 days in those with VTE compared to 0 days in those without (p < 0.001). By logistic regression, only the number of ICU days was associated with VTE (OR 1.074, 95% CI 1.013-1.138). CONCLUSIONS: Hospitalized children with NS have high rates of infection. Presence of VTE was associated with infection. Both were associated with longer hospitalizations and ICU stays.


Assuntos
Síndrome Nefrótica/complicações , Infecções Pneumocócicas/epidemiologia , Streptococcus pneumoniae/isolamento & purificação , Tromboembolia Venosa/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Unidades de Terapia Intensiva/estatística & dados numéricos , Tempo de Internação/estatística & dados numéricos , Modelos Logísticos , Masculino , América do Norte/epidemiologia , Infecções Pneumocócicas/etiologia , Prevalência , Estudos Retrospectivos , Fatores de Risco , Tromboembolia Venosa/etiologia
17.
Pediatr Cardiol ; 40(4): 805-812, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30759268

RESUMO

Atherosclerosis promoting cardiovascular disease risk factors (CVDrf) are highly prevalent among youth in the U.S. Determining which standard modifiable clinical measures (SMCMs) has the greatest impact on vascular structure and function is valuable for the health care provider to help identify children at highest risk. The aim of this study was to determine modifiable outpatient clinical predictors of vascular health in youth with CVDrf. Children and adolescents with CVDrf (n = 120, 13.1 ± 1.9 years, 49% female) were recruited from a pediatric preventive cardiology clinic. The SMCMs included BMI z-score, waist-to-height ratio (WTHR), lipid panel, hemoglobin A1c, blood pressure (BP), presence of tobacco smoke exposure, and presence of hypertriglyceridemic waist (HTW) phenotype (triglycerides ≥ 110 mg/dL and waist circumference ≥ 90 percentile). Vascular function and structure were measured with pulse wave velocity (PWV), central systolic BP (CSP), augmentation index (AIx), and carotid artery intima-media thickness (cIMT). Sex and height specific z-scores for PWV, CSP, and cIMT were used. Multiple linear regression with backwards selection identified SMCMs which strongly predicted vascular function and structure. Among SMCMs, WTHR and HTW were the most frequent predictors of vascular function (PWV: R2 = 0.32; CSP: R2 = 0.35; AIx R2 = 0.13). Other predictors of vascular function included hemoglobin A1C, BP, and BMI z-score. Systolic BP and LDL cholesterol were predictors of vascular structure (cIMT: R2 = 0.14). The strongest predictors of vascular health in youth with CVDrf were related to measures of central obesity. Targeting these SMCM in lieu of vascular testing in outpatient clinic setting may be practical to identify children and adolescents at greatest risk for CVD.


Assuntos
Vasos Sanguíneos/fisiopatologia , Doenças Cardiovasculares/etiologia , Dislipidemias/fisiopatologia , Adolescente , Pressão Sanguínea/fisiologia , Doenças Cardiovasculares/diagnóstico , Espessura Intima-Media Carotídea/estatística & dados numéricos , Criança , Dislipidemias/complicações , Feminino , Humanos , Lipídeos/sangue , Masculino , Análise de Onda de Pulso/métodos , Fatores de Risco
18.
J Nurs Adm ; 49(5): 273-279, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30973430

RESUMO

OBJECTIVE: To investigate the amount and type of hospitalized children's nighttime sleep interruptions, perceptions, and efficiency. BACKGROUND: Sleep in hospitals is notoriously poor and impedes healing. Pediatric studies have been limited including breadth of diagnoses, age, or measures. METHODS: Actigraphy, sleep surveys, and nursing diaries were used to describe sleep on 2 nursing units along with environmental assessments. RESULTS: Ninety-five children from 1 month to 17 years with multiple diagnoses participated. The median number of awakenings was 2.7 per night. The median for the longest uninterrupted episode of sleep was 5.5 hours. Children need 8 to 17 hours of daily sleep, while this sample had a median of 7.5 hours of night sleep. Sensors showed talk as the predominant noise, whereas surveys showed alarms and vital signs awakened patients. CONCLUSIONS: Children are not getting essential, minimally interrupted sleep in hospitals. Disseminating results will increase awareness and accelerate environmental changes.


Assuntos
Criança Hospitalizada/estatística & dados numéricos , Dissonias , Planejamento Ambiental , Hospitais Pediátricos/estatística & dados numéricos , Sono , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Inquéritos e Questionários
19.
J Emerg Med ; 56(3): 241-247, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30679068

RESUMO

BACKGROUND: Musculoskeletal (MSK) infections can be difficult to diagnose in acute care settings. The utility of clinical decision tools for pediatric MSK infections in an emergency department has not been well studied. OBJECTIVE: Our aim was to evaluate the performance of a septic hip clinical decision rule (CDR) in the evaluation of pediatric musculoskeletal infections. METHODS: We performed a retrospective study of children evaluated for an MSK infection in our emergency department from 2014 to 2016. Data collection included demographics, discharge diagnoses, and clinical/laboratory predictors from the CDR. A χ2 analysis and Wilcoxon rank-sum tests compared patients with and without MSK infections. Logistic regression analysis examined the predictors for MSK infections. A receiver operating characteristic (ROC) curve was calculated to evaluate the performance of the predictors. RESULTS: Of 996 evaluations included in the final analysis, 109 (10.9%) had MSK infections. In a multivariable model, an adjusted odds ratio (OR) was significant for fever (OR 3.9, 95% confidence interval [CI] 2.4-6.4), refusal to bear weight/pseudoparalysis (OR 4.4, 95% CI 2.7-7.1), and C-reactive protein (CRP) > 2.0 mg/dL (OR 5.4, 95% CI 3.2-9.1). The probability of infection was 75.1% with five predictors present, 1.9% for zero predictors, and 5.1% if one predictor was present. An ROC curve showed an area under the curve of 0.82, indicating moderate accuracy. CONCLUSIONS: A septic hip CDR demonstrates a low predicted probability of an MSK infection with zero or one clinical predictor present and moderate predictability with all five predictors. Fever, refusal to bear weight/pseudoparalysis, and CRP > 2.0 mg/dL performed best and should alert providers to consider other MSK infections in addition to septic arthritis.


Assuntos
Artrite Infecciosa/diagnóstico , Quadril/microbiologia , Pediatria/instrumentação , Adolescente , Artrite Infecciosa/fisiopatologia , Proteína C-Reativa/análise , Criança , Pré-Escolar , Sistemas de Apoio a Decisões Clínicas/instrumentação , Sistemas de Apoio a Decisões Clínicas/normas , Serviço Hospitalar de Emergência/organização & administração , Feminino , Febre/etiologia , Quadril/fisiopatologia , Humanos , Lactente , Modelos Logísticos , Masculino , Razão de Chances , Pediatria/métodos , Pediatria/normas , Valor Preditivo dos Testes , Estudos Prospectivos , Curva ROC , Estudos Retrospectivos , Suporte de Carga
20.
Pediatr Emerg Care ; 35(11): 777-781, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31689269

RESUMO

INTRODUCTION: Compassion fatigue, a product of burnout (BO), secondary traumatic stress (STS), and compassion satisfaction (CS), is reduced capacity and interest in being empathetic for suffering individuals. Our objective was to determine prevalence of compassion fatigue in the pediatric emergency department. METHODS: We administered the Professional Quality of Life instrument, including BO, STS, and CS scales, to a convenience sample of pediatric emergency department staff (physicians, nurses, technicians, social workers, child life specialists). We categorized participants as having BO (high BO, low CS, moderate-low STS scores), STS (high STS, moderate-low BO, low CS), compassion fatigue (high STS and BO, low CS), and high-risk fatigue (high STS, moderate-low BO, low CS) and low risk (moderate-high CS, moderate-low BO, low STS) of compassion fatigue. RESULTS: One hundred seventy-seven staff (50% response rate) participated. The majority were white (90%) and female (88%), with participation highest among physicians (97%). Twenty-six percent had low CS scores, 26% had high BO scores, and 20% had high STS scores. Five percent met criteria for categorization as compassion fatigue, 24% for BO, and 24% for low risk of compassion fatigue. Current personal stress was associated with higher BO scores (P = 0.008) and secondary categorization as BO (P = 0.05). Recent work stress was associated with high STS scores (P = 0.03). DISCUSSION: Five percent of participants met criteria for compassion fatigue; a significant proportion had BO, STS, or CS scores, placing them at risk of compassion fatigue. Future studies should explore factors contributing to and interventions to minimize compassion fatigue.


Assuntos
Esgotamento Profissional/epidemiologia , Fadiga de Compaixão/epidemiologia , Serviço Hospitalar de Emergência/estatística & dados numéricos , Recursos Humanos em Hospital/psicologia , Qualidade de Vida , Adulto , Estudos Transversais , Feminino , Humanos , Satisfação no Emprego , Masculino , Pessoa de Meia-Idade , Medicina de Emergência Pediátrica/estatística & dados numéricos , Índice de Gravidade de Doença , Inquéritos e Questionários
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