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1.
BMJ Open Qual ; 8(4): e000725, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31803853

RESUMO

The success of rare disease research relies heavily on robust partnerships with clinicians to help identify new patients and collect samples. Many studies for paediatric rheumatic diseases requiring pretreatment samples have suffered from slow enrolment rates due to the low incidence of disease and relative urgency to treat. Therefore, timely identification of all potentially eligible patients is crucial. The objective of this project was to apply quality improvement methods to increase the frequency and timeliness of identification of eligible patients with new paediatric rheumatic diagnoses to approach for research studies. A retrospective chart review was undertaken in our paediatric rheumatology clinic to measure the number of eligible patients identified for potential research recruitment between missed recruitment opportunities. Improvement methodology was used to integrate standardised communication between clinicians and the research team into clinic workflow, to leverage social feedback as positive reinforcement for good communication and to measure change in response to the interventions. The number of eligible patients identified between missed recruitment opportunities increased from every 0-1 patient to every 14 patients during the project period, corresponding to an increase in the overall identification rate from 32% to 91% of all eligible patients. Quality improvement methods can be used to successfully integrate research recruitment into routine clinical care and accelerate advances necessary to improve health outcomes.


Assuntos
Comunicação , Seleção de Pacientes , Pediatria , Melhoria de Qualidade , Reumatologia , Criança , Humanos , Estudos Retrospectivos , Inquéritos e Questionários
2.
Am J Obstet Gynecol ; 220(5): 494.e1-494.e7, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30885769

RESUMO

BACKGROUND: Open maternal-fetal surgery for fetal myelomeningocele results in reduction in neonatal morbidity related to spina bifida but may be associated with fetal, neonatal, and maternal complications in subsequent pregnancies. OBJECTIVE: The objective of this study was to ascertain obstetric risk in subsequent pregnancies after open maternal-fetal surgery for fetal myelomeningocele closure. STUDY DESIGN: An international multicenter prospective observational registry created to track and report maternal, obstetric, fetal/neonatal, and subsequent pregnancy outcomes following open maternal-fetal surgery for fetal myelomeningocele was evaluated for subsequent pregnancy outcome variables. Institutional Review Board approval was obtained for the registry. RESULTS: From 693 cases of open maternal-fetal surgery for fetal myelomeningocele closure entered into the registry, 77 subsequent pregnancies in 60 women were identified. The overall live birth rate was 96.2%, with 52 pregnancies delivering beyond 20 weeks gestational age and median gestational age at delivery of 37 (36.3-37.1) weeks. The uterine rupture rate was 9.6% (n = 5), resulting in 2 fetal deaths. Maternal transfusion was required in 4 patients (7.7%). CONCLUSION: The risk of uterine rupture or dehiscence in subsequent pregnancies with associated fetal morbidity after open maternal-fetal surgery is significant, but is similar to that reported for subsequent pregnancies after classical cesarean deliveries. Future pregnancy considerations should be included in initial counseling for women contemplating open maternal-fetal surgery.


Assuntos
Feto/cirurgia , Meningomielocele/cirurgia , Resultado da Gravidez , Aborto Espontâneo/epidemiologia , Adulto , Transfusão de Sangue/estatística & dados numéricos , Cesárea , Feminino , Morte Fetal , Idade Gestacional , Humanos , Nascido Vivo , Gravidez , Estudos Prospectivos , Sistema de Registros , Ruptura Uterina/epidemiologia
3.
J Pediatr Surg ; 54(1): 55-59, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30442461

RESUMO

BACKGROUND/PURPOSE: Prostaglandin E1 (PGE) has been used to maintain ductus arteriosus patency and unload the suprasystemic right ventricle (RV) in neonates with congenital diaphragmatic hernia (CDH) and severe pulmonary hypertension (PH). Here we evaluate the PH response in neonates with CDH and severe PH treated with PGE. METHODS: We performed a retrospective chart review of CDH infants treated at our center between 2011 and 2016. In a subset, PGE was initiated for echocardiographic evidence of severe PH, metabolic acidosis, or hypoxemia. To assess PH response, we evaluated laboratory data, including B-type natriuretic peptide (BNP) and echocardiograms before and after PGE treatment. Categorical and continuous data were analyzed with Fisher's exact tests and Mann-Whitney t-tests, respectively. RESULTS: Fifty-seven infants were treated with PGE a mean 17 ±â€¯2 days. BNP levels declined after 1.4 ±â€¯0.2 days of treatment and again after 5.2 ±â€¯0.6 days. After 6 ±â€¯0.8 days of treatment, echocardiographic estimates of severe PH by tricuspid regurgitation jet velocity, ductus arteriosus direction, and ventricular septum position also improved significantly. Treatment was not associated with postductal hypoxemia or systemic hypoperfusion. CONCLUSIONS: In patients with CDH and severe PH, PGE is well tolerated and associated with improved BNP and echocardiographic indices of PH, suggesting successful unloading of the RV. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.


Assuntos
Alprostadil/uso terapêutico , Hérnias Diafragmáticas Congênitas/complicações , Hipertensão Pulmonar/tratamento farmacológico , Vasodilatadores/uso terapêutico , Ecocardiografia/métodos , Feminino , Humanos , Hipertensão Pulmonar/complicações , Recém-Nascido , Masculino , Peptídeo Natriurético Encefálico/sangue , Philadelphia , Sistema de Registros , Estudos Retrospectivos , Resultado do Tratamento
4.
J Perinatol ; 38(7): 834-843, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29887609

RESUMO

OBJECTIVE: To evaluate whether infants with congenital diaphragmatic hernia (CDH) can be safely resuscitated with a reduced starting fraction of inspired oxygen (FiO2) of 0.5. STUDY DESIGN: A retrospective cohort study comparing 68 patients resuscitated with starting FiO2 0.5 to 45 historical controls resuscitated with starting FiO2 1.0. RESULTS: Reduced starting FiO2 had no adverse effect upon survival, duration of intubation, need for ECMO, duration of ECMO, or time to surgery. Furthermore, it produced no increase in complications, adverse neurological events, or neurodevelopmental delay. The need to subsequently increase FiO2 to 1.0 was associated with female sex, lower gestational age, liver up, lower lung volume-head circumference ratio, decreased survival, a higher incidence of ECMO, longer time to surgery, periventricular leukomalacia, and lower neurodevelopmental motor scores. CONCLUSION: Starting FiO2 0.5 may be safe for the resuscitation of CDH infants. The need to increase FiO2 to 1.0 during resuscitation is associated with worse outcomes.


Assuntos
Hérnias Diafragmáticas Congênitas/terapia , Recém-Nascido Prematuro , Oxigenoterapia/métodos , Oxigênio/uso terapêutico , Ressuscitação/métodos , Análise de Variância , Estudos de Casos e Controles , Oxigenação por Membrana Extracorpórea/métodos , Feminino , Hérnias Diafragmáticas Congênitas/diagnóstico , Hérnias Diafragmáticas Congênitas/mortalidade , Hospitais Pediátricos , Humanos , Recém-Nascido , Unidades de Terapia Intensiva Neonatal , Masculino , Análise Multivariada , Consumo de Oxigênio/fisiologia , Segurança do Paciente , Philadelphia , Pressão , Prognóstico , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Taxa de Sobrevida , Resultado do Tratamento
5.
J Pediatr ; 200: 44-49, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-29784517

RESUMO

OBJECTIVE: To evaluate the effect of continuous treprostinil in infants with severe pulmonary hypertension associated with congenital diaphragmatic hernia (CDH) on specific markers of pulmonary hypertension severity and to report the safety and tolerability of treprostinil. STUDY DESIGN: We conducted a retrospective cohort study of infants with CDH-associated pulmonary hypertension treated with treprostinil from January 2011 to September 2016. Severity of pulmonary hypertension was assessed by echocardiogram and serum B-type natriuretic peptide (BNP) by using time points before initiation and 24 hours, 1 week, and 1 month after treprostinil initiation. Fisher exact tests, Wilcoxon-rank sum tests, and mixed-effects models were used for analysis. RESULTS: Seventeen patients were treated with treprostinil for a median of 54.5 days (IQR 44.3-110 days). Compared with the concurrent CDH population (n = 147), infants treated with treprostinil were more likely to require extracorporeal support (76.5% vs 25.2%, P < .0001), to have a longer hospital stay (144 vs 60 days, P < .0001), and to need longer mechanical ventilator support (76.5 vs 30.9 days, P < .0001). Following treprostinil initiation, there was a significant reduction in BNP at 1 week (1439 vs 393 pg/mL, P < .01) and 1 month (1439 vs 242 pg/mL, P = .01). Severity of pulmonary hypertension by echocardiogram improved at 1 month (OR 0.14, CI 95% 0.04-0.48, P = .002). Despite these improvements, overall mortality remained high (35%). There were no adverse events related to treprostinil, including no hypotension, hypoxia, or thrombocytopenia. CONCLUSIONS: In this cohort, treprostinil use was associated with improved severity of pulmonary hypertension assessed by echocardiogram and decreased BNP, with no significant side effects.


Assuntos
Epoprostenol/análogos & derivados , Hérnias Diafragmáticas Congênitas/complicações , Hipertensão Pulmonar/tratamento farmacológico , Pressão Propulsora Pulmonar/efeitos dos fármacos , Sistema de Registros , Anti-Hipertensivos/administração & dosagem , Relação Dose-Resposta a Droga , Epoprostenol/administração & dosagem , Feminino , Seguimentos , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Resultado do Tratamento
6.
J Autism Dev Disord ; 48(6): 2112-2121, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29383650

RESUMO

To determine the rate and predictors of autism spectrum disorder (ASD) in congenital diaphragmatic hernia (CDH). Between 06/2004 and 09/2015 a total of 110 CDH survivors underwent neurodevelopmental (ND) testing and screening for ASD, followed by a full autism diagnostic evaluation if indicated at our institution. We found a 9 time higher rate of ASD in CDH children compared to the general population (P = 0.0002). Multiple patient-related and clinical variables risk factors of ASD were identified by univariate analysis. However, only short-term and long-term neurodevelopmental delays were strongly associated with ASD in CDH by multivariate comparisons. There is a striking prevalence of ASD in CDH survivors and our findings suggest that all CDH children should be regularly screened for ASD.


Assuntos
Transtorno do Espectro Autista/diagnóstico , Transtorno do Espectro Autista/epidemiologia , Hérnias Diafragmáticas Congênitas/diagnóstico , Hérnias Diafragmáticas Congênitas/epidemiologia , Criança , Estudos de Coortes , Feminino , Seguimentos , Humanos , Recém-Nascido , Masculino , Testes Neuropsicológicos/normas , Estudos Prospectivos , Estudos Retrospectivos , Fatores de Risco
7.
Ann Thorac Surg ; 105(6): 1827-1834, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29438655

RESUMO

BACKGROUND: This study sought to evaluate neurodevelopmental outcome in survivors of high-risk congenital lung lesions (CLLs) who underwent prenatal intervention or postnatal surgery within the first month of life. METHODS: Forty-five high-risk CLL survivors underwent assessment using the Bayley Scales of Infant Development, 3rd Edition between July 2004 and December 2016. Scores were grouped as average, at-risk, and delayed based on SD intervals. Correlations between outcome and risk factors were analyzed by Fisher's exact test or two-sided t test as appropriate, with significant p values <0.05. RESULTS: Open prenatal intervention was required in 13 (28.9%) children (fetal surgical resection, n = 4 , ex utero intrapartum treatment, n = 9), whereas 32 (71.1%) children had respiratory distress postnatally and required resection within the first month of life. Mean age at follow-up was 19.3 ± 10.3 months. Mean composite scores were within the expected average range. A total of 62.2% scored within the average range for all domains. At-risk scores were found in 26.7% of children in at least one domain, and 11.1% had delays in at least one domain. Neurodevelopmental outcome was similar between treatment groups. Prolonged ventilator support and neonatal intensive care unit stay, need for supplemental oxygen at day of life 30, gastroesophageal reflux disease, and delayed enteral feeding were associated with neurologic delays (all p < 0.05). CONCLUSIONS: Neurodevelopmental scores for high-risk CLL survivors in infancy and toddlerhood are age appropriate. Neither fetal intervention nor the need for postnatal resection within the first month of life increases the risk of delays. Surrogate markers of a complicated neonatal course are predictive of adverse outcome.


Assuntos
Doenças Fetais/cirurgia , Pulmão/anormalidades , Pulmão/cirurgia , Transtornos do Neurodesenvolvimento/fisiopatologia , Procedimentos Cirúrgicos Pulmonares/métodos , Anormalidades do Sistema Respiratório/cirurgia , Fatores Etários , Estudos de Coortes , Feminino , Seguimentos , Humanos , Recém-Nascido , Unidades de Terapia Intensiva Neonatal , Masculino , Transtornos do Neurodesenvolvimento/epidemiologia , Transtornos do Neurodesenvolvimento/etiologia , Gravidez , Cuidado Pré-Natal/métodos , Procedimentos Cirúrgicos Pulmonares/efeitos adversos , Anormalidades do Sistema Respiratório/diagnóstico por imagem , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Fatores Sexuais , Sobreviventes , Fatores de Tempo , Resultado do Tratamento
8.
Pediatr Crit Care Med ; 19(1): 64-74, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-29303891

RESUMO

OBJECTIVES: The purpose of this study was to assess the need and timing of extracorporeal membrane oxygenation in relation to congenital diaphragmatic hernia repair as modifiers of short-term neurodevelopmental outcomes. DESIGN: Retrospective study. SETTING: A specialized tertiary care center. PATIENTS: Between June 2004 and February 2016, a total of 212 congenital diaphragmatic hernia survivors enrolled in our follow-up program. Neurodevelopmental outcome was assessed at a median age of 22 months (range, 5-37) using the Bayley Scales of Infant Development, third edition. Fifty patients (24%) required extracorporeal membrane oxygenation support. Four patients (8%) were repaired prior to cannulation, 25 (50%) were repaired on extracorporeal membrane oxygenation, and 21 (42%) were repaired after decannulation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Children with congenital diaphragmatic hernia, who required extracorporeal membrane oxygenation scored on average 4.6 points lower on cognitive composite (p = 0.031) and 9.2 points lower on the motor composite (p < 0.001). Language scores were similar between groups. Mean scores for children with congenital diaphragmatic hernia repaired on extracorporeal membrane oxygenation were significantly lower for cognition (p = 0.021) and motor (p = 0.0005) outcome. Language scores were also lower, but did not reach significance. A total of 40% of children repaired on extracorporeal membrane oxygenation scored below average in all composites, whereas only 9% of the non-extracorporeal membrane oxygenation, 4% of the repaired post-extracorporeal membrane oxygenation, and 25% of the repaired pre-extracorporeal membrane oxygenation patients scored below average across all domains. Only 20% of congenital diaphragmatic hernia survivors repaired on extracorporeal membrane oxygenation support scored within the average range for all composite domains. Duration of extracorporeal membrane oxygenation support was not associated with a higher likelihood of adverse cognitive (p = 0.641), language (p = 0.147), or motor (p = 0.720) outcome. CONCLUSIONS: Need for extracorporeal membrane oxygenation in congenital diaphragmatic hernia survivors is associated with worse neurocognitive and neuromotor outcome. Need for congenital diaphragmatic hernia repair while on extracorporeal membrane oxygenation is associated with deficits in multiple domains. Overall time on extracorporeal membrane oxygenation did not impact neurodevelopmental outcome.


Assuntos
Desenvolvimento Infantil , Oxigenação por Membrana Extracorpórea/efeitos adversos , Hérnias Diafragmáticas Congênitas/complicações , Herniorrafia/efeitos adversos , Transtornos do Neurodesenvolvimento/etiologia , Pré-Escolar , Oxigenação por Membrana Extracorpórea/métodos , Feminino , Seguimentos , Hérnias Diafragmáticas Congênitas/terapia , Humanos , Lactente , Recém-Nascido , Masculino , Transtornos do Neurodesenvolvimento/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Centros de Atenção Terciária , Fatores de Tempo
9.
Fetal Diagn Ther ; 43(4): 297-303, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28768252

RESUMO

INTRODUCTION: We investigated the correlation of amniotic fluid (AF) concentrations of glial fibrillary acidic protein (GFAP) with prenatal features of myelomeningocele (MMC) and neurodevelopmental outcome after fetal MMC (fMMC) surgery. MATERIALS AND METHODS: AF was collected during fMMC surgery between December 2012 and November 2015. AF-GFAP concentration was determined by ELISA. Retrospective chart review identified the characteristics of the defect. Data regarding delivery and 1-year neurodevelopmental outcome was collected from The Children's Hospital of Philadelphia fMMC Registry. RESULTS: Eighty-two AF samples were collected from fMMC surgeries. Perinatal data were obtained from 77 subjects, and 1-year follow-up data from 65 subjects. GFAP concentrations were significantly elevated in MMC compared to myeloschisis (24.1 ± 2.9 and 10.3 ± 1.5 ng/mL; p < 0.0001). A larger percentage of subjects with myeloschisis defects delivered before their scheduled due date (myeloschisis 88.5%; MMC 55.0%; p = 0.003) and delivered at an earlier mean gestational age (34.6 ± 0.4 weeks, n = 26) compared to those with MMC defects (35.2 ± 0.4 weeks, n = 51) (p = 0.04). DISCUSSION: AF-GFAP levels differentiate between MMC and myeloschisis, and raise interesting questions regarding the clinical significance between the 2 types of defects.


Assuntos
Líquido Amniótico/metabolismo , Proteína Glial Fibrilar Ácida/metabolismo , Meningomielocele/metabolismo , Defeitos do Tubo Neural/metabolismo , Feminino , Idade Gestacional , Humanos , Masculino , Gravidez , Sistema de Registros , Estudos Retrospectivos
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