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1.
Pediatr Crit Care Med ; 19(11): e618-e626, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30234741

RESUMO

BACKGROUND: The Bedside Pediatric Early Warning System score is a validated measure of severity of illness in acute care inpatient settings. Its potential as a remote assessment tool for interfacility transport has not been evaluated. We hypothesized that the Bedside Pediatric Early Warning System score was associated with need for intervention during the peritransport period and patient disposition. METHODS: We retrospectively evaluated children transported by a regional pediatric team during a 6-month period. Bedside Pediatric Early Warning System scores were calculated at the triage phone call, the transport team arrival, and at transfer of care to the hospital team. The primary outcome was the receipt of significant intervention during the peritransport period, with additional outcomes of destination (ICU, ward, emergency department) in the regional hospital. Scores are presented as median values (interquartile range). RESULTS: There were 564 children who underwent transport; 139 (25%) received interventions; and 205 (36%) were transferred to the PICU, 231 (41%) to the ward, and 127 (23%) to the emergency department. Scores were 2 (1-5; median interquartile range) in children receiving no in-transport interventions, 8 (5-11) in children receiving any intervention (p < 0.001), and 10 (7-14) in children receiving more than one intervention. Children transferred to the PICU had higher scores 6 (3-10), than children transferred to a ward 3 (1-6) or the emergency department 2 (1-3) (p < 0.001). CONCLUSIONS: The Bedside Pediatric Early Warning System score at the time of initial referral is a useful measure of severity of illness reflected by the subsequent provision of significant peritransport intervention and the transfer destination.


Assuntos
Cuidados Críticos/métodos , Transferência de Pacientes/estatística & dados numéricos , Triagem/métodos , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Equipe de Assistência ao Paciente , Curva ROC , Estudos Retrospectivos , Índice de Gravidade de Doença
2.
J Clin Monit Comput ; 32(1): 89-96, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-28258341

RESUMO

To investigate the association between low near infrared spectroscopy (NIRS) somatic oxygen saturation (<70%) at admission and the need for lifesaving interventions (LSI) in the initial 24 h of a PICU admission. Retrospective chart review of all unplanned admissions to the pediatric intensive care unit (PICU) with NIRS somatic oxygen saturation data available within 4 h of admission, excluding admissions with a cardiac diagnosis. LSI data were collected for the first 24 h after admission. Hemodynamic parameters, laboratory values, illness severity scores and diagnoses were collected. Included PICU admissions were stratified by lowest NIRS value in the first 4 h after admission: low NIRS (<70%) and normal NIRS (≥70%) groups. Rate of LSI from 4 h to 24 h was compared between the two groups. Association of LSI with NIRS saturation and other clinical and laboratory parameters was measured by univariate and multivariate methods. We reviewed 411 consecutive unplanned admissions to the PICU of which 184 (44%) patients underwent NIRS monitoring. A higher proportion of patients who underwent somatic NIRS monitoring required LSIs compared to those without NIRS monitoring (36.4 vs 5.7% respectively, p < 0.0001). The proportion of patients who required LSI was higher in the group with low NIRS (<70%) within the first 4 h compared to those with normal NIRS (≥70%) (77.1 vs 22.1%, p < 0.0001). Fluid resuscitation, blood products and vasoactive medications were the most common LSIs. Multivariable modeling showed NIRS < 70% and heart rate > 2SD for age to be associated with LSIs. ROC curve analysis of the combination of NIRS < 70% and HR >2SD for age had an area under the curve of 0.79 with 78% sensitivity and 76% specificity for association with LSI. Compared to the normal NIRS group, the low NIRS group had higher mortality (10.4 vs 0.7%, p = 0.005) and longer median hospital length of stay (2.9 vs 1.6 days, p < 0.0001). Low somatic NIRS oxygen saturation (<70%) in the first 4 h of an unplanned PICU admission is associated with need for higher number of subsequent lifesaving interventions up to 24 h after admission. Noninvasive, continuous, somatic NIRS monitoring may identify children at high risk of medical instability.


Assuntos
Unidades de Terapia Intensiva Pediátrica , Oxigênio/sangue , Espectroscopia de Luz Próxima ao Infravermelho/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Frequência Cardíaca , Hemodinâmica , Mortalidade Hospitalar , Hospitalização , Humanos , Lactente , Masculino , Análise Multivariada , Curva ROC , Respiração Artificial , Estudos Retrospectivos , Risco , Fatores de Tempo
3.
Acad Emerg Med ; 25(3): 267-274, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-28992364

RESUMO

OBJECTIVE: The purpose of this study was to determine the proportion of children undergoing procedural sedation for fracture reduction in the emergency department (ED) observed to experience negative postdischarge behaviors. Predictors of negative behaviors were evaluated, including anxiety. METHODS: This was a prospective cohort study of children receiving intravenous ketamine sedation for ED fracture reduction. The child's anxiety prior to sedation was measured with the Modified Yale Preoperative Anxiety Scale. Negative behavioral changes were measured with the Post-Hospitalization Behavior Questionnaire 1 to 2 weeks after discharge. Descriptive statistics and odds ratios (ORs) were calculated. Chi-square test was used for comparisons between groups. Multivariable logistic regression models evaluated predictors of negative behavioral change after discharge. RESULTS: Ninety-seven patients were enrolled; 82 (85%) completed follow-up. Overall, 33 (40%) children were observed to be highly anxious presedation and 18 (22%) had significant negative behavior changes after ED discharge. Independent predictors for negative behaviors were high anxiety (OR = 9.0, 95% confidence interval [CI] = 2.3-35.7) and nonwhite race (OR = 6.5, 95% CI = 1.7-25.0). CONCLUSION: For children undergoing procedural sedation in the ED, two in five children have high preprocedure anxiety and almost one in four have significant negative behaviors 1 to 2 weeks after discharge. Highly anxious and nonwhite children have increased risk of negative behavioral changes that have not been previously recognized in the ED setting.


Assuntos
Anestésicos Dissociativos/efeitos adversos , Ansiedade/etiologia , Comportamento Infantil , Sedação Consciente/efeitos adversos , Fixação de Fratura/psicologia , Ketamina/efeitos adversos , Anestésicos Dissociativos/administração & dosagem , Ansiedade/diagnóstico , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Sedação Consciente/métodos , Serviço Hospitalar de Emergência/estatística & dados numéricos , Feminino , Fixação de Fratura/efeitos adversos , Fixação de Fratura/métodos , Humanos , Ketamina/administração & dosagem , Modelos Logísticos , Masculino , Estudos Prospectivos , Inquéritos e Questionários
4.
J Neurooncol ; 136(1): 13-21, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28900832

RESUMO

The goal of this study is to spatially discriminate tumor from treatment effect (TE), within the contrast-enhancing lesion, for brain tumor patients at all stages of treatment. To this end, the diagnostic accuracy of MRI-derived diffusion and perfusion parameters to distinguish pure TE from pure glioblastoma (GBM) was determined utilizing spatially-correlated biopsy samples. From July 2010 through June 2015, brain tumor patients who underwent pre-operative DWI and DSC-MRI and stereotactic image-guided biopsy were considered for inclusion in this IRB-approved study. MRI-derived parameter maps included apparent diffusion coefficient (ADC), normalized cerebral blood flow (nCBF), normalized and standardized relative cerebral blood volume (nRCBV, sRCBV), peak signal-height (PSR) and percent signal-recovery (PSR). These were co-registered to the Stealth MRI and median values extracted from the spatially-matched biopsy regions. A ROC analysis accounting for multiple subject samples was performed, and the optimal threshold for distinguishing TE from GBM determined for each parameter. Histopathologic diagnosis of pure TE (n = 10) or pure GBM (n = 34) was confirmed in tissue samples from 15 consecutive subjects with analyzable data. Perfusion thresholds of sRCBV (3575; SN/SP% = 79.4/90.0), nRCBV (1.13; SN/SP% = 82.1/90.0), and nCBF (1.05; SN/SP% = 79.4/80.0) distinguished TE from GBM (P < 0.05), whereas ADC, PSR, and PH could not (P > 0.05). The thresholds for CBF and CBV can be applied to lesions with any admixture of tumor or treatment effect, enabling the identification of true tumor burden within enhancing lesions. This approach overcomes current limitations of averaging values from both tumor and TE for quantitative assessments.


Assuntos
Neoplasias Encefálicas/diagnóstico por imagem , Imagem de Difusão por Ressonância Magnética/métodos , Glioblastoma/diagnóstico por imagem , Lesões por Radiação/diagnóstico por imagem , Adulto , Idoso , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/terapia , Meios de Contraste , Feminino , Glioblastoma/patologia , Glioblastoma/terapia , Humanos , Aumento da Imagem , Masculino , Pessoa de Meia-Idade , Lesões por Radiação/patologia , Sensibilidade e Especificidade
5.
J Pediatr ; 183: 80-86.e1, 2017 04.
Artigo em Inglês | MEDLINE | ID: mdl-28081891

RESUMO

OBJECTIVE: To describe preschool neurodevelopmental outcomes of children with complex congenital heart disease (CHD), who were evaluated as part of a longitudinal cardiac neurodevelopmental follow-up program, as recommended by the American Heart Association and the American Academy of Pediatrics, and identify predictors of neurodevelopmental outcomes in these children. STUDY DESIGN: Children with CHD meeting the American Heart Association/American Academy of Pediatrics high-risk criteria for neurodevelopmental delay were evaluated at 4-5 years of age. Testing included standardized neuropsychological measures. Parents completed measures of child functioning. Scores were compared by group (single ventricle [1V]; 2 ventricles [2V]; CHD plus known genetic condition) to test norms and classified as: normal (within 1 SD of mean); at risk (1-2 SD from mean); and impaired (>2 SD from mean). RESULTS: Data on 102 patients were analyzed. Neurodevelopmental scores did not differ based on cardiac anatomy (1V vs 2V); both groups scored lower than norms on fine motor and adaptive behavior skills, but were within 1 SD of norms. Patients with genetic conditions scored significantly worse than 1V and 2V groups and test norms on most measures. CONCLUSIONS: Children with CHD and genetic conditions are at greatest neurodevelopmental risk. Deficits in children with CHD without genetic conditions were mild and may not be detected without formal longitudinal testing. Parents and providers need additional education regarding the importance of developmental follow-up for children with CHD.


Assuntos
Deficiências do Desenvolvimento/etiologia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico , Deficiência Intelectual/etiologia , Fatores Etários , Pré-Escolar , Estudos de Coortes , Deficiências do Desenvolvimento/diagnóstico , Deficiências do Desenvolvimento/epidemiologia , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Deficiência Intelectual/diagnóstico , Deficiência Intelectual/epidemiologia , Modelos Logísticos , Estudos Longitudinais , Masculino , Análise Multivariada , Testes Neuropsicológicos , Medição de Risco , Índice de Gravidade de Doença , Fatores Sexuais , Estados Unidos/epidemiologia
6.
J Pediatr Gastroenterol Nutr ; 64(6): 979-983, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-27755343

RESUMO

OBJECTIVES: The Infant and Child Feeding Questionnaire (ICFQ) was created to facilitate early detection of feeding and swallowing problems. This is achieved by promoting effective communication between caregivers and health care providers resulting in referral for evaluation and treatment of feeding and swallowing problems by specialists. The purpose of this pilot study was to determine whether items from the ICFQ could be used to screen for differences between children with known feeding problems (FP) and without known feeding problems (NFP). METHODS: Caregivers of children ages 36 months or younger with FP and NFP were recruited to complete the ICFQ and demographic questions. T tests were completed to compare demographic characteristics of the research groups. Responses to ICFQ items were analyzed using receiver operating characteristic analysis and odds ratios to determine whether questionnaire items distinguished between study groups. RESULTS: Sixty-four caregivers of children with FP and 57 caregivers of NFP children were recruited. Three participants in the NFP group did not meet inclusion criteria and were excluded from analysis. A combination of 4 ICFQ questions distinguished between groups (receiver operating characteristic = 0.974). Significant odds ratios were also found for 9 feeding behaviors that distinguished between groups. CONCLUSIONS: A subset of items from the ICFQ showed promise for distinguishing FP from NFP groups. Future work will expand the regional representation of the participant samples and obtain equal representation of participants across all age-adjusted questionnaires to determine whether the same combination of ICFQ items continues to distinguish between FP and NFP groups.


Assuntos
Cuidadores , Transtornos de Deglutição/diagnóstico , Transtornos da Alimentação e da Ingestão de Alimentos/diagnóstico , Inquéritos e Questionários , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Razão de Chances , Projetos Piloto , Psicometria , Curva ROC
7.
Hosp Pediatr ; 7(1): 39-45, 2017 01.
Artigo em Inglês | MEDLINE | ID: mdl-27965360

RESUMO

OBJECTIVES: Local pediatric screening guidelines for venous thromboembolism (VTE) are developed from incomplete pediatric data and extrapolated from adult data in which immobility is a major risk factor. We hypothesized that screening guidelines centered on immobility are inadequate for identifying children at risk of central venous catheter (CVC)-associated VTE. METHODS: This retrospective case-control (4:1) study at an academic, quaternary-level, free-standing children's hospital applied screening guidelines for VTE risk to all cases of VTE from July 2012 to April 2014. Cases and controls were classified as "at risk" or "not at risk" of VTE by guideline criteria. These guidelines assessed VTE risk factors, including CVC, as reported in the pediatric literature. RESULTS: VTE prevalence was 0.5 per 100 admissions. Sixty-nine of 114 patients with radiographically confirmed VTE were classified as being "at risk" by the guidelines, with a sensitivity of 61%, specificity of 90.8%, a positive predictive value of 2.4%, and negative predictive value of 99.8%. There was no difference in screening guidelines sensitivity for identifying CVC-associated VTE versus non-CVC-associated VTE. Half of the 45 patients with VTE who were not captured as being "at risk" did not have decreased mobility, the entry point to the algorithm, and 80% of these patients had a CVC. CONCLUSIONS: Screening guidelines have low sensitivity for identifying hospitalized children at increased risk of both CVC-associated and other VTE events. Decreased mobility is not a requirement for CVC-associated VTE. Risk factors extrapolated from adult data are insufficient for identifying children at risk of VTE.


Assuntos
Programas de Rastreamento , Medição de Risco , Trombose Venosa Profunda de Membros Superiores , Adolescente , Estudos de Casos e Controles , Criança , Criança Hospitalizada/estatística & dados numéricos , Feminino , Guias como Assunto/normas , Hospitalização/estatística & dados numéricos , Humanos , Masculino , Programas de Rastreamento/métodos , Programas de Rastreamento/normas , Valor Preditivo dos Testes , Melhoria de Qualidade , Estudos Retrospectivos , Medição de Risco/métodos , Medição de Risco/normas , Fatores de Risco , Sensibilidade e Especificidade , Estados Unidos , Trombose Venosa Profunda de Membros Superiores/diagnóstico , Trombose Venosa Profunda de Membros Superiores/terapia
8.
Br J Haematol ; 175(2): 237-245, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27539682

RESUMO

Sickle cell disease (SCD) pain transitions from acute to chronic for unknown reasons. Chronic elevation of the pain neurotransmitter substance P (SP) sensitizes pain nociceptors. We evaluated SP levels in controls and SCD patients during baseline and acute pain and investigated associations between SP and age, gender, pain history, haemolysis and hydroxycarbamide (also termed hydroxyurea) use. Plasma SP levels were measured using enzyme-linked immunosorbent assay. Independent samples t-test compared SP levels between: (i) SCD baseline and controls, and (ii) SCD baseline and acute pain. Multivariate linear regression determined associations between SP and age, gender, pain history and hydroxycarbamide use. Spearman correlation determined an association between SP and haemolysis. We enrolled 35 African American controls, 25 SCD baseline and 12 SCD pain patients. SCD patients were 7-19 years old. Mean ± standard deviation SP level (pg/ml) in SCD baseline was higher than controls (32·4 ± 11·6 vs. 22·9 ± 7·6, P = 0·0009). SP in SCD pain was higher than baseline (78·1 ± 43·4 vs. 32·4 ± 11·6, P = 0·004). Haemolysis correlated with increased SP: Hb (r = -0·7, P = 0·0002), reticulocyte count (r = 0·61, P = 0·0016), bilirubin (r = 0·68, P = 0·0216), lactate dehydrogenase (r = 0·62, P = 0·0332), aspartate aminotransferase (r = 0·68, P = 0·003). Patients taking hydroxycarbamide had increased SP (ß = 29·2, P = 0·007). SP could be a mediator of or marker for pain sensitization in SCD and a biomarker and/or target for novel pain treatment.


Assuntos
Anemia Falciforme/sangue , Anemia Falciforme/complicações , Antidrepanocíticos/efeitos adversos , Hidroxiureia/efeitos adversos , Substância P/sangue , Adolescente , Adulto , Negro ou Afro-Americano , Anemia Falciforme/diagnóstico , Anemia Falciforme/tratamento farmacológico , Antidrepanocíticos/uso terapêutico , Biomarcadores , Estudos de Casos e Controles , Criança , Feminino , Hemólise/efeitos dos fármacos , Humanos , Hidroxiureia/uso terapêutico , Masculino , Fatores de Risco , Adulto Jovem
9.
Pediatr Crit Care Med ; 17(8): e352-61, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-27362853

RESUMO

OBJECTIVES: To identify patient, hospital, and central venous catheter factors that may influence the use of low-dose heparin infusion for central venous catheter patency in critically ill children. DESIGN: Secondary analysis of an international multicenter observational study. SETTING: Fifty-nine PICUs over four study dates in 2012, involving seven countries. PATIENTS: Children less than 18 years old with a central venous catheter who were admitted to a participating unit and enrolled in the completed Prophylaxis against Thrombosis Practice study were included. All overflow patients were excluded. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of the 2,484 patients in the Prophylaxis against Thrombosis Practice study, 1,312 patients had a central venous catheter. Five hundred seven of those patients used low-dose heparin infusion. The frequency of low-dose heparin infusion was compared across various patient, hospital, and central venous catheter factors using chi-square, Mann-Whitney U, and Fisher exact tests. In the multivariate analysis, age was not a significant factor for low-dose heparin infusion use. Patients with pulmonary hypertension had decreased low-dose heparin infusion use, whereas those with active surgical or trauma diagnoses had increased low-dose heparin infusion use. All centrally inserted central venous catheters were more likely to use low-dose heparin infusion when compared with peripherally inserted central venous catheters. The Asia-Pacific region showed increased low-dose heparin infusion use, along with community hospitals and smaller ICUs (< 10 beds). CONCLUSIONS: Patient, central venous catheter, and hospital factors are associated with the use of low-dose heparin infusion in critically ill children. Further study is needed to evaluate the efficacy and persistence of low-dose heparin infusion use.


Assuntos
Anticoagulantes/administração & dosagem , Cateterismo Venoso Central/efeitos adversos , Heparina/administração & dosagem , Padrões de Prática Médica/estatística & dados numéricos , Tromboembolia Venosa/prevenção & controle , Adolescente , Anticoagulantes/uso terapêutico , Criança , Pré-Escolar , Estado Terminal , Feminino , Disparidades em Assistência à Saúde/estatística & dados numéricos , Heparina/uso terapêutico , Humanos , Lactente , Recém-Nascido , Infusões Intravenosas , Unidades de Terapia Intensiva Pediátrica , Masculino , Análise Multivariada , Estudos Retrospectivos , Tromboembolia Venosa/etiologia
10.
Pediatr Emerg Care ; 32(8): 499-503, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27331580

RESUMO

OBJECTIVE: Recent Pediatric Advanced Life Support (PALS) guidelines have deemphasized the use of advanced airways in short transport. It is unclear if guideline recommendations have altered practice. We sought to determine if a temporal change exists in the number of prehospital pediatric trauma intubations since the 2005 PALS guidelines update. METHODS: This is an institutional review board-approved, retrospective, single-center study. Reviewed all pediatric trauma activations where patients younger than 19 years were intubated at the scene, en route or at the level 1 trauma center during 2006 to 2011. Specific complications collected were esophageal intubations, mainstem intubations and need for re-intubations. RESULTS: There were 1012 trauma activations, 1009 pediatric patients, 300 (29.7%) intubated during transport to Children's Hospital of Wisconsin Pediatric Trauma Center (PTC) or upon arrival. Mean age of 9.5 ± 5.9 years. Fifty-seven percent (n = 172) were intubated before PTC, 31.7% (n = 95) field intubations, 25.7% (n = 77) outside facility intubations. 44% (n = 132) at PTC. Age was not a significant variable. There was no difference in the proportion of injured children requiring intubation who were intubated before arrival to the PTC. Those intubated in the field versus a facility had significantly increased mortality (P = 0.0002), longer hospital days (P = 0.0004) including intensive care unit days (P = 0.0003) and ventilator days (P = 0.0003) even when adjusted for illness severity. CONCLUSIONS: There was no significant change in the proportion of pretrauma room intubations following the 2005 PALS guidelines even when adjusted for illness or injury severity. Children injured farther from the PTC and more severely injured children were more likely to be intubated before arrival at the PTC.


Assuntos
Manuseio das Vias Aéreas/métodos , Intubação Intratraqueal/métodos , Adolescente , Criança , Pré-Escolar , Gerenciamento Clínico , Serviços Médicos de Emergência/métodos , Feminino , Humanos , Intubação Intratraqueal/mortalidade , Masculino , Guias de Prática Clínica como Assunto , Estudos Retrospectivos , Centros de Traumatologia
11.
Am J Pathol ; 186(6): 1568-81, 2016 06.
Artigo em Inglês | MEDLINE | ID: mdl-27102768

RESUMO

Nemaline myopathies (NMs) are a group of congenital muscle diseases caused by mutations in at least 10 genes and associated with a range of clinical symptoms. NM is defined on muscle biopsy by the presence of cytoplasmic rod-like structures (nemaline rods) composed of cytoskeletal material. Myofiber smallness is also found in many cases of NM and may represent a cause of weakness that can be counteracted by treatment. We have used i.p. injection of activin type IIB receptor (ActRIIB)-mFc (an inhibitor of myostatin signaling) to promote hypertrophy and increase strength in our prior murine work; we therefore tested whether ActRIIB-mFc could improve weakness in NM mice through myofiber hypertrophy. We report a study of ActRIIB-mFc treatment in the Acta1 H40Y mouse model of NM. Treatment of Acta1 H40Y mice produced significant increases in body mass, muscle mass, quadriceps myofiber size, and survival, but other measurements of strength (forelimb grip strength, ex vivo measurements of contractile function) did not improve. Our studies also identified that the complications of urethral obstruction are associated with mortality in male hemizygote Acta1 H40Y mice. The incidence of urethral obstruction and histologic evidence of chronic obstruction (inflammation) were significantly lower in Acta1 H40Y mice that had been treated with ActRIIB-mFc. ActRIIB-mFc treatment produces a mild benefit to the disease phenotype in Acta1 H40Y mice.


Assuntos
Receptores de Activinas Tipo II/antagonistas & inibidores , Miofibrilas/efeitos dos fármacos , Miopatias da Nemalina/patologia , Animais , Western Blotting , Modelos Animais de Doenças , Masculino , Camundongos , Camundongos Mutantes , Músculo Esquelético/efeitos dos fármacos , Músculo Esquelético/patologia , Miofibrilas/patologia
12.
Blood ; 127(20): 2472-80, 2016 05 19.
Artigo em Inglês | MEDLINE | ID: mdl-26917779

RESUMO

Approximately 20% to 25% of patients with von Willebrand disease (VWD) have a qualitative defect of the von Willebrand factor (VWF) protein activities. Variant VWD typically is classified as type 1C, 2A, 2B, 2M, or 2N depending on the VWF activity defect. Traditionally, diagnosis has relied on multiple clinical laboratory assays to assign VWD phenotype. We developed an enzyme-linked immunosorbent assay (ELISA) to measure the various activities of VWF on a single plate and evaluated 160 patient samples enrolled in the Zimmerman Program for the Molecular and Clinical Biology of von Willebrand Disease with type 2 VWD. Using linear discriminate analysis (LDA), this assay was able to identify type 1C, 2A, 2B, 2M, or 2N VWD with an overall accuracy of 92.5% in the patient study cohort. LDA jackknife analysis, a statistical resampling technique, identified variant VWD with an overall accuracy of 88.1%, which predicts the assay's performance in the general population. In addition, this assay demonstrated correlation with traditional clinical laboratory VWF assays. The VWF multiplex activity assay may be useful as a same-day screening assay when considering the diagnosis of variant VWD in an individual patient.


Assuntos
Ensaio de Imunoadsorção Enzimática/métodos , Doenças de von Willebrand/classificação , Fator de von Willebrand/análise , Confiabilidade dos Dados , Análise Discriminante , Testes Genéticos , Genótipo , Hemofilia A/sangue , Humanos , Fenótipo , Probabilidade , Curva ROC , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Fatores de Tempo , Doenças de von Willebrand/sangue , Doenças de von Willebrand/diagnóstico , Doenças de von Willebrand/genética , Fator de von Willebrand/genética
13.
Blood ; 127(20): 2481-8, 2016 05 19.
Artigo em Inglês | MEDLINE | ID: mdl-26862110

RESUMO

von Willebrand disease (VWD) is the most common inherited bleeding disorder, and type 1 VWD is the most common VWD variant. Despite its frequency, diagnosis of type 1 VWD remains the subject of debate. In order to study the spectrum of type 1 VWD in the United States, the Zimmerman Program enrolled 482 subjects with a previous diagnosis of type 1 VWD without stringent laboratory diagnostic criteria. von Willebrand factor (VWF) laboratory testing and full-length VWF gene sequencing was performed for all index cases and healthy control subjects in a central laboratory. Bleeding phenotype was characterized using the International Society on Thrombosis and Haemostasis bleeding assessment tool. At study entry, 64% of subjects had VWF antigen (VWF:Ag) or VWF ristocetin cofactor activity below the lower limit of normal, whereas 36% had normal VWF levels. VWF sequence variations were most frequent in subjects with VWF:Ag <30 IU/dL (82%), whereas subjects with type 1 VWD and VWF:Ag ≥30 IU/dL had an intermediate frequency of variants (44%). Subjects whose VWF testing was normal at study entry had a similar rate of sequence variations as the healthy controls (14%). All subjects with severe type 1 VWD and VWF:Ag ≤5 IU/dL had an abnormal bleeding score (BS), but otherwise BS did not correlate with VWF:Ag. Subjects with a historical diagnosis of type 1 VWD had similar rates of abnormal BS compared with subjects with low VWF levels at study entry. Type 1 VWD in the United States is highly variable, and bleeding symptoms are frequent in this population.


Assuntos
Doença de von Willebrand Tipo 1/sangue , Adolescente , Testes de Coagulação Sanguínea , Hibridização Genômica Comparativa , Feminino , Variação Genética , Hemorragia/etiologia , Humanos , Masculino , Fenótipo , Análise de Sequência de DNA , Inquéritos e Questionários , Estados Unidos/epidemiologia , Adulto Jovem , Doença de von Willebrand Tipo 1/diagnóstico , Doença de von Willebrand Tipo 1/epidemiologia , Fator de von Willebrand/análise , Fator de von Willebrand/genética
14.
Am J Perinatol ; 33(6): 600-4, 2016 05.
Artigo em Inglês | MEDLINE | ID: mdl-26731179

RESUMO

Objective This study aims to determine the frequency that umbilical venous catheters (UVCs) and peripherally inserted central catheters (PICCs) migrate into the cardiothymic silhouette after initial verification of correct placement. Study Design This is a single-center, retrospective study in neonates in whom a PICC or UVC was placed. The frequency of catheter tip migration into the cardiothymic silhouette requiring catheter manipulation was determined radiographically at 1 and 24 hours, respectively, after insertion. Results At 1 and 24 hours, 36 and 23% of UVCs (n = 41) migrated into the cardiothymic silhouette, respectively. At 1 and 24 hours, 23 and 11% of PICCs (n = 63) migrated into the cardiothymic silhouette, respectively. Migration was not associated with birth weight, weight at insertion, or postnatal age at insertion. Conclusion UVCs and PICCs frequently migrate into the cardiothymic silhouette increase the risk for development of a pericardial effusion. Serial radiographic assessment of catheter tip location is needed to assess catheter migration within the first 24 hours of line placement.


Assuntos
Cateterismo Venoso Central/efeitos adversos , Cateterismo Periférico/efeitos adversos , Cateteres Venosos Centrais/efeitos adversos , Migração de Corpo Estranho/diagnóstico por imagem , Migração de Corpo Estranho/epidemiologia , Cateterismo Venoso Central/métodos , Cateterismo Periférico/métodos , Feminino , Migração de Corpo Estranho/terapia , Humanos , Recém-Nascido , Unidades de Terapia Intensiva Neonatal , Masculino , Derrame Pericárdico/etiologia , Radiografia Torácica , Estudos Retrospectivos , Centros de Atenção Terciária , Fatores de Tempo , Veias Umbilicais , Wisconsin
15.
Arch Pathol Lab Med ; 140(1): 51-65, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26132600

RESUMO

CONTEXT: There is no current standard among myopathologists for reporting muscle biopsy findings. The National Institute of Neurological Disorders and Stroke has recently launched a common data element (CDE) project to standardize neuromuscular data collected in clinical reports and to facilitate their use in research. OBJECTIVE: To develop a more-uniform, prospective reporting tool for muscle biopsies, incorporating the elements identified by the CDE project, in an effort to improve reporting and educational resources. DESIGN: The variation in current biopsy reporting practice was evaluated through a study of 51 muscle biopsy reports from self-reported diagnoses of genetically confirmed or undiagnosed muscle disease from the Congenital Muscle Disease International Registry. Two reviewers independently extracted data from deidentified reports and entered them into the revised CDE format to identify what was missing and whether or not information provided on the revised CDE report (complete/incomplete) could be successfully interpreted by a neuropathologist. RESULTS: Analysis of the data highlighted showed (1) inconsistent reporting of key clinical features from referring physicians, and (2) considerable variability in the reporting of pertinent positive and negative histologic findings by pathologists. CONCLUSIONS: We propose a format for muscle-biopsy reporting that includes the elements in the CDE checklist and a brief narrative comment that interprets the data in support of a final interpretation. Such a format standardizes cataloging of pathologic findings across the spectrum of muscle diseases and serves emerging clinical care and research needs with the expansion of genetic-testing therapeutic trials.


Assuntos
Doenças Neuromusculares/diagnóstico , Patologia Cirúrgica/normas , Projetos de Pesquisa/normas , Biópsia , Elementos de Dados Comuns , Humanos , National Institute of Neurological Disorders and Stroke (USA) , Estados Unidos
16.
Pediatr Crit Care Med ; 16(8): 766-71, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26237655

RESUMO

OBJECTIVES: Studies showing the changes in workflow during transition from semi to full electronic medical records are lacking. This objective study is to identify the changes in workflow in the PICU during transition from semi to full electronic health record. DESIGN: Prospective observational study. Children's Hospital of Wisconsin Institutional Review Board waived the need for approval so this study was institutional review board exempt. This study measured clinical workflow variables at a 72-bed PICU during different phases of transition to a full electronic health record, which occurred on November 4, 2012. Phases of electronic health record transition were defined as follows: pre-electronic health record (baseline data prior to transition to full electronic health record), transition phase (3 wk after electronic health record), and stabilization (6 mo after electronic health record). Data were analyzed for the three phases using Mann-Whitney U test with a two-sided p value of less than 0.05 considered significant. SETTING: Seventy-two bed PICU. PATIENTS: All patients in the PICU were included during the study periods. MEASUREMENTS AND MAIN RESULTS: Five hundred and sixty-four patients with 2,355 patient days were evaluated in the three phases. Duration of rounds decreased from a median of 9 minutes per patient pre--electronic health record to 7 minutes per patient post electronic health record. Time to final note decreased from 2.06 days pre--electronic health record to 0.5 days post electronic health record. Time to first medication administration after admission also decreased from 33 minutes pre--electronic health record and 7 minutes post electronic health record. Time to Time to medication reconciliation was significantly higher pre-electronic health record than post electronic health record and percent of medication reconciliation completion was significantly lower pre--electronic health record than post electronic health record and percent of medication reconciliation completion was significantly higher pre--electronic health record than. There was no significant change in time between placement of discharge order and physical transfer from the unit [corrected].changes clinical workflow in a PICU with decreased duration of rounds, time to final note, time to medication administration, and time to medication reconciliation completion. There was no change in the duration from medical to physical transfer.


Assuntos
Registros Eletrônicos de Saúde/estatística & dados numéricos , Unidades de Terapia Intensiva Pediátrica/estatística & dados numéricos , Avaliação de Processos em Cuidados de Saúde/estatística & dados numéricos , Fluxo de Trabalho , Adolescente , Criança , Pré-Escolar , Feminino , Hospitais Pediátricos/estatística & dados numéricos , Humanos , Lactente , Masculino , Reconciliação de Medicamentos/estatística & dados numéricos , Transferência de Pacientes/estatística & dados numéricos , Serviço de Farmácia Hospitalar/estatística & dados numéricos , Estudos Prospectivos , Fatores de Tempo
17.
Ann Emerg Med ; 66(5): 466-74, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25935844

RESUMO

STUDY OBJECTIVE: The J-Tip (National Medical Products Inc, Irvine, CA) uses air instead of a needle to push lidocaine into the skin. To our knowledge, no studies have investigated its use for venipuncture in young children. We determine whether the J-Tip decreased venipuncture pain in young children compared with vapocoolant spray. METHODS: Children aged 1 to 6 years were randomized into 3 groups: intervention (J-Tip), control (vapocoolant spray), and sham (vapocoolant spray and pop of an empty J-Tip). The procedure was videotaped and scored with the Face, Legs, Activity, Cry and Consolability (FLACC) tool at 3 points; baseline, before approach; device, at J-Tip deployment; and at venipuncture. The FLACC tool was scored 0 (none) to 10 (severe). Comparisons of pain scores over time were made with the generalized estimating equation. Venipuncture success and adverse effects were assessed and compared with χ(2). RESULTS: Two hundred five children enrolled: intervention 96, control 53, and sham 56. There were no between-group differences in baseline characteristics. There was no mean change in pain scores from device to venipuncture in the intervention group (0.26; 95% confidence interval [CI] -0.31 to 0.82), but there was an increase in pain in the control (2.82; 95% CI 1.91 to 3.74) and sham (1.68; 95% CI 0.83 to 2.52) groups. This change was greater for the control and sham compared to the intervention group. There was no difference in venipuncture success between groups. No severe adverse events occurred. Minor adverse events were the same between groups. CONCLUSION: Use of the J-Tip for children aged 1 to 6 years reduced venipuncture pain compared with vapocoolant spray or sham treatment.


Assuntos
Anestésicos Locais/administração & dosagem , Lidocaína/administração & dosagem , Dor/prevenção & controle , Flebotomia/instrumentação , Criança , Pré-Escolar , Desenho de Equipamento , Feminino , Humanos , Lactente , Injeções a Jato , Masculino , Medição da Dor
18.
Blood ; 125(14): 2297-304, 2015 Apr 02.
Artigo em Inglês | MEDLINE | ID: mdl-25662333

RESUMO

Von Willebrand factor (VWF) contains binding sites for platelets and for vascular collagens to facilitate clot formation at sites of injury. Although previous work has shown that VWF can bind type IV collagen (collagen 4), little characterization of this interaction has been performed. We examined the binding of VWF to collagen 4 in vitro and extended this characterization to a murine model of defective VWF-collagen 4 interactions. The interactions of VWF and collagen 4 were further studied using plasma samples from a large study of both healthy controls and subjects with different types of von Willebrand disease (VWD). Our results show that collagen 4 appears to bind VWF exclusively via the VWF A1 domain, and that specific sequence variations identified through VWF patient samples and through site-directed mutagenesis in the VWF A1 domain can decrease or abrogate this interaction. In addition, VWF-dependent platelet binding to collagen 4 under flow conditions requires an intact VWF A1 domain. We observed that decreased binding to collagen 4 was associated with select VWF A1 domain sequence variations in type 1 and type 2M VWD. This suggests an additional mechanism through which VWF variants may alter hemostasis.


Assuntos
Colágeno Tipo IV/metabolismo , Mutação/genética , Doenças de von Willebrand/metabolismo , Fator de von Willebrand/metabolismo , Animais , Sítios de Ligação , Estudos de Casos e Controles , Células Cultivadas , Citometria de Fluxo , Humanos , Camundongos , Mutagênese Sítio-Dirigida , Ligação Proteica , Conformação Proteica , Estrutura Terciária de Proteína , Relação Estrutura-Atividade , Doenças de von Willebrand/genética , Fator de von Willebrand/química , Fator de von Willebrand/genética
19.
J Pediatr Hematol Oncol ; 37(3): 190-4, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25575295

RESUMO

Given the availability of various pain severity scales, greater understanding of the agreement between pain scales is warranted. We compared Visual Analog Scale (VAS) and Numeric Rating Scale (NRS) pain severity ratings in children with sickle cell disease (SCD) to identify the relationship and agreement between pain scale ratings. Twenty-eight patients (mean ± SD age, 14.65 ± 3.12 y, 50% female) receiving pain interventions within the emergency department completed serial VAS and NRS pain severity ratings every 30 minutes. Data were used to calculate the relationship (Spearman correlation) and agreement (Bland-Altman approach) between the VAS and NRS. One hundred twenty-eight paired VAS-NRS measurements were obtained. VAS and NRS ratings were significantly correlated for the initial assessment (rs = 0.88, P < 0.001) and all assessments (rs = 0.87, P < 0.001). Differences between VAS and NRS means were -0.52 (P = 0.006) for the initial assessment and -0.86 (P < 0.001) across all assessments. The difference between VAS and NRS ratings decreased as pain severity increased across all assessments (P = 0.027), but not the initial assessment. Within pediatric patients with SCD, VAS and NRS ratings were found to trend together; however, VAS scores were found to be significantly lower than NRS scores across assessments. The agreement between the 2 measures improved at increasing levels of pain severity. These findings demonstrate that the VAS and NRS are similar, but cannot be used interchangeably when assessing self-reported pain in SCD.


Assuntos
Anemia Falciforme/complicações , Medição da Dor/métodos , Dor/etiologia , Adolescente , Anemia Falciforme/patologia , Criança , Feminino , Seguimentos , Humanos , Masculino , Dor/patologia , Manejo da Dor , Pediatria , Prognóstico , Escala Visual Analógica
20.
Pediatrics ; 135(2): 213-23, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25560444

RESUMO

OBJECTIVES: To determine if recommending strict rest improved concussion recovery and outcome after discharge from the pediatric emergency department (ED). METHODS: Patients aged 11 to 22 years presenting to a pediatric ED within 24 hours of concussion were recruited. Participants underwent neurocognitive, balance, and symptom assessment in the ED and were randomized to strict rest for 5 days versus usual care (1-2 days rest, followed by stepwise return to activity). Patients completed a diary used to record physical and mental activity level, calculate energy exertion, and record daily postconcussive symptoms. Neurocognitive and balance assessments were performed at 3 and 10 days postinjury. Sample size calculations were powered to detect clinically meaningful differences in postconcussive symptom, neurocognitive, and balance scores between treatment groups. Linear mixed modeling was used to detect contributions of group assignment to individual recovery trajectory. RESULTS: Ninety-nine patients were enrolled; 88 completed all study procedures (45 intervention, 43 control). Postdischarge, both groups reported a 20% decrease in energy exertion and physical activity levels. As expected, the intervention group reported less school and after-school attendance for days 2 to 5 postconcussion (3.8 vs 6.7 hours total, P < .05). There was no clinically significant difference in neurocognitive or balance outcomes. However, the intervention group reported more daily postconcussive symptoms (total symptom score over 10 days, 187.9 vs 131.9, P < .03) and slower symptom resolution. CONCLUSIONS: Recommending strict rest for adolescents immediately after concussion offered no added benefit over the usual care. Adolescents' symptom reporting was influenced by recommending strict rest.


Assuntos
Repouso em Cama , Concussão Encefálica/terapia , Síndrome Pós-Concussão/prevenção & controle , Adolescente , Concussão Encefálica/diagnóstico , Criança , Convalescença , Feminino , Hospitais Pediátricos , Humanos , Modelos Lineares , Masculino , Testes Neuropsicológicos , Alta do Paciente , Síndrome Pós-Concussão/diagnóstico , Equilíbrio Postural , Estudos Prospectivos , Wisconsin , Adulto Jovem
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