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1.
Pediatr Res ; 94(2): 803-810, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-36755188

RESUMEN

BACKGROUND: Individuals of minority race/ethnicity have lower rates of participation in genomic research. This study evaluated sociodemographic characteristics associated with decisions to enroll in a pediatric critical care biorepository. METHODS: Parents of children admitted to the PICU between November 2014 and May 2017 were offered to enroll their child in a biorepository using a single-page opt-in consent. Missed enrollment was assessed by failure to complete the form or declining consent on the form. We conducted a retrospective chart review for sociodemographic and clinical information. Bivariate and multivariable regression analyses were performed. RESULTS: In 4055 encounters, representing 2910 patients with complete data, 1480 (50%) completed the consent form and 1223 (83%) enrolled. We found higher odds of incomplete consent for non-English-speaking parents (OR = 2.1, p < 0.0001) and parents of children of all races except non-Hispanic white (OR = 1.27-1.99, p < 0.0001). We found higher odds of declined consent in patients with Medicaid (OR = 1.67, p = 0.003) and parents of children of all races except non-Hispanic white (OR = 1.32-2.9, p < 0.0001). CONCLUSION: Inability to enroll patients in a critical care biorepository may be associated with several sociodemographic factors at various points in recruitment/enrollment. IMPACT: Individuals of minority race/ethnicity are less likely to enroll in genomic research and in critical care research. This study evaluated sociodemographic characteristics associated with decisions to enroll a child in a pediatric critical care biorepository. Sociodemographic factors including race/ethnicity, primary language, and insurance status and patient clinical characteristics are associated with differential enrollment into a pediatric critical care biorepository. More research is needed to understand how study team-participant interactions may play a role in differential enrollment. Barriers to enrollment occur both at the time of approaching and consenting for enrollment.


Asunto(s)
Formularios de Consentimiento , Etnicidad , Estados Unidos , Niño , Humanos , Estudios Retrospectivos , Grupos Minoritarios , Cuidados Críticos
2.
J Cyst Fibros ; 22(1): 89-97, 2023 01.
Artículo en Inglés | MEDLINE | ID: mdl-35871976

RESUMEN

OBJECTIVE: We evaluated whether implementation of cystic fibrosis (CF) newborn screening (NBS) leads to equitable timeliness of initial evaluation. We compared age at first event (AFE, age at sweat test, encounter and/or care episode) between infants categorized as Black/African American, American Indian/ Native Alaskan, Asian, and/or Hispanic and/or other (Group 1) to White and not Hispanic infants (Group 2). METHODS: This retrospective cohort study from the Cystic Fibrosis Foundation Patient Registry (CFFPR) included infants born 2010-2018. Race and ethnicity categories followed US Census definitions. The primary outcome was AFE; the secondary outcome was weight for age (WFA) z-score averaged 12 to < 24 months. We compared distributions by Wilcoxon rank-sum test and proportions by Chi-square or Fisher's exact tests. A nested cohort study used a linear mixed effects model of variables that affect WFA, chosen a priori, to evaluate associations with 1-year WFA z-score. RESULTS: Among 6354 infants, 21% were in Group 1. Group 1 median AFE was 31 days (IQR 19, 49) and Group 2 was 22 days (IQR 14,36) (p< .001). Median WFA z-score at 1-2 years was lower in Group 1. In 3017 infants with complete data on variables of interest, AFE, Black race, CFTR variant class I-III, prematurity and public insurance were associated with lower 1-year WFA z-score. CONCLUSIONS: Differences in AFE for infants with CF from historically marginalized groups may exacerbate long standing health disparities. We speculate that inequitable identification of CFTR gene variants and/or bias may influence timeliness of evaluation after an out-of-range NBS.


Asunto(s)
Fibrosis Quística , Recién Nacido , Lactante , Humanos , Fibrosis Quística/diagnóstico , Tamizaje Neonatal , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Estudios Retrospectivos , Estudios de Cohortes
3.
JAMA Netw Open ; 5(11): e2241513, 2022 11 01.
Artículo en Inglés | MEDLINE | ID: mdl-36367725

RESUMEN

Importance: Readmission is often considered a hospital quality measure, yet no validated risk prediction models exist for children. Objective: To develop and validate a tool identifying patients before hospital discharge who are at risk for subsequent readmission, applicable to all ages. Design, Setting, and Participants: This population-based prognostic analysis used electronic health record-derived data from a freestanding children's hospital from January 1, 2016, to December 31, 2019. All-cause 30-day readmission was modeled using 3 years of discharge data. Data were analyzed from June 1 to November 30, 2021. Main Outcomes and Measures: Three models were derived as a complementary suite to include (1) children 6 months or older with 1 or more prior hospitalizations within the last 6 months (recent admission model [RAM]), (2) children 6 months or older with no prior hospitalizations in the last 6 months (new admission model [NAM]), and (3) children younger than 6 months (young infant model [YIM]). Generalized mixed linear models were used for all analyses. Models were validated using an additional year of discharges. Results: The derivation set contained 29 988 patients with 48 019 hospitalizations; 50.1% of these admissions were for children younger than 5 years and 54.7% were boys. In the derivation set, 4878 of 13 490 admissions (36.2%) in the RAM cohort, 2044 of 27 531 (7.4%) in the NAM cohort, and 855 of 6998 (12.2%) in the YIM cohort were followed within 30 days by a readmission. In the RAM cohort, prior utilization, current or prior procedures indicative of severity of illness (transfusion, ventilation, or central venous catheter), commercial insurance, and prolonged length of stay (LOS) were associated with readmission. In the NAM cohort, procedures, prolonged LOS, and emergency department visit in the past 6 months were associated with readmission. In the YIM cohort, LOS, prior visits, and critical procedures were associated with readmission. The area under the receiver operating characteristics curve was 83.1 (95% CI, 82.4-83.8) for the RAM cohort, 76.1 (95% CI, 75.0-77.2) for the NAM cohort, and 80.3 (95% CI, 78.8-81.9) for the YIM cohort. Conclusions and Relevance: In this prognostic study, the suite of 3 prediction models had acceptable to excellent discrimination for children. These models may allow future improvements in tailored discharge preparedness to prevent high-risk readmissions.


Asunto(s)
Alta del Paciente , Readmisión del Paciente , Masculino , Niño , Lactante , Humanos , Adolescente , Femenino , Estudios Retrospectivos , Tiempo de Internación , Hospitalización
4.
Am J Perinatol ; 2022 Dec 07.
Artículo en Inglés | MEDLINE | ID: mdl-36096150

RESUMEN

OBJECTIVE: This study aimed to establish the degree of variability in thresholds for discussing withdrawal of life-sustaining therapies (WLST) in periviable infants among neonatal intensive care unit (NICU) personnel. STUDY DESIGN: A vignette-style survey was administered to NICU personnel at two urban NICUs assessing likelihood of discussing WLST or support for discussing WLST (on a scale from 1, not at all likely/supportive to 10, extremely likely/supportive) in 10 clinical scenarios. RESULTS: Response rates ranged by clinical role from 26 to 89%. Participant responses ranged from 1 to 10 in 5 out of 10 vignettes for NICU attendings, and 9 out of 10 vignettes for bedside nurses. Lower gestational age (22-23 vs. 24-25 weeks) was associated with increased likelihood to discuss WLST in some but not all scenarios. CONCLUSION: NICU personnel have widely variable criteria for discussing WLST which threatens the informed consent process surrounding resuscitation decisions in a "trial of therapy" framework. KEY POINTS: · NICU personnel have variable criteria for WLST.. · Parents have little say in whether WLST is offered.. · Disclosure of variable criteria is not routine..

5.
Children (Basel) ; 9(9)2022 Aug 26.
Artículo en Inglés | MEDLINE | ID: mdl-36138595

RESUMEN

BACKGROUND: Social workers (SWs) and chaplains are trained to support families facing challenges associated with critical illness and potential end-of-life issues. Little is known about how parents view SW/chaplain involvement in care for critically ill children with cancer. METHODS: We studied parent perceptions of SW/chaplain involvement in care for pediatric intensive care unit (PICU) patients with cancer or who had a hematopoietic cell transplant. English- and Spanish-speaking parents completed surveys within 7 days of PICU admission and at discharge. Some parents participated in an optional interview. RESULTS: Twenty-four parents of 18 patients completed both surveys, and six parents were interviewed. Of the survey respondents, 66.7% and 75% interacted with SWs or chaplains, respectively. Most parents described SW/chaplain interactions as helpful (81.3% and 72.2%, respectively), but few reported their help with decision making (18.8% and 12.4%, respectively). Parents described SW/chaplain roles related to emotional, spiritual, instrumental, and holistic support. Few parents expressed awareness about SW/chaplain interactions with other healthcare team members. CONCLUSIONS: Future work is needed to determine SWs'/chaplains' contributions to and impact on parental decision making, improve parent awareness about SW/chaplain roles and engagement with the healthcare team, and understand why some PICU parents do not interact with SWs/chaplains.

6.
Pediatr Crit Care Med ; 23(11): 929-935, 2022 11 01.
Artículo en Inglés | MEDLINE | ID: mdl-35894600

RESUMEN

OBJECTIVES: We sought to describe the prevalence of midazolam treatment failure in children with refractory status epilepticus (RSE) and define a threshold dose associated with diminishing frequency of seizure cessation. DESIGN: Single center retrospective cohort study. SETTING: Single-center, quaternary-care PICU. PATIENTS: Children younger than 18 years old admitted to the PICU from 2009 to 2018 who had RSE requiring a continuous midazolam infusion. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We identified individuals with RSE through a data analytics inquiry. Receiver operating characteristic (ROC) curve analysis and Youden's index were used to assess the midazolam dose threshold associated with the highest sensitivity and specificity in identifying seizure cessation. A logistic regression model was used to determine if there was an association between maximum midazolam dose and seizure cessation. Of the 45 patients who met inclusion criteria for this study, 27 (60%) had seizure cessation with a midazolam infusion, whereas 18 (40%) required an additional pentobarbital infusion for seizure cessation. There was an association between maximum midazolam dose and seizure cessation, with patients more likely to fail treatment when midazolam was administered at higher doses. The maximum midazolam dose displayed high area under the ROC curve value for seizure cessation, and the Youden's J index cut-off point was 525 µg/kg/hr. Treatment above this dose was associated with diminishing frequency of seizure cessation. The median time spent titrating midazolam above 500 µg/kg/hr for those patients who required pentobarbital for seizure cessation was 3.83 hours (interquartile range, 2.28-5.58 hr). CONCLUSIONS: In pediatric patients with RSE requiring high dose midazolam, considerable time is spent titrating doses in a range (above 500 µg/kg/hr) that is associated with diminishing frequency of seizure cessation.


Asunto(s)
Midazolam , Estado Epiléptico , Niño , Humanos , Adolescente , Estudios Retrospectivos , Pentobarbital/uso terapéutico , Anticonvulsivantes/uso terapéutico , Estado Epiléptico/tratamiento farmacológico
7.
BMC Cardiovasc Disord ; 22(1): 139, 2022 04 01.
Artículo en Inglés | MEDLINE | ID: mdl-35365073

RESUMEN

BACKGROUND: Ideal "cardiovascular health" (CVH)-optimal diet, exercise, nonsmoking, BMI, BP, lipids, and glucose-is associated with healthy longevity in adults. Pediatric heart transplant (HT) patients may be at risk for suboptimal CVH. METHODS: Single-center retrospective study of HT patients 2003-2014 who survived 1 year post-transplant. Five CVH metrics were collected at listing, 1, 3 and 5 years post-transplant (diet and exercise were unavailable). CVH was scored by summing individual metrics: ideal = 2, intermediate = 1, and poor = 0 points; total scores of 8-10 points were considered high (favorable). CVH was compared between HT patients and the US pediatric population (GP) utilizing NHANES 2007-2016. Logistic regression was performed to examine the association of CVH 1 year post-transplant with a composite adverse outcome (death, re-listing, coronary vasculopathy, or chronic kidney disease) 3 years post-transplant. RESULTS: We included 110 HT patients (median age at HT: 6 years [range 0.1-21]) and 19,081 NHANES participants. CVH scores among HT patients were generally high at listing (75%), 1 (74%), 3 (87%) and 5 (76%) years post-transplant and similar to GP, but some metrics (e.g., glucose) were worse among HT patients. Among HT patients, CVH was poorer with older age and non-Caucasian race/ethnicity. Per 1-point higher CVH score, the demographic-adjusted OR for adverse outcomes was 0.95 (95% CI, 0.7-1.4). CONCLUSIONS: HT patients had generally favorable CVH, but some metrics were unfavorable and CVH varied by age and race/ethnicity. No significant association was detected between CVH and adverse outcomes in this small sample, but study in a larger sample is warranted.


Asunto(s)
Enfermedades Cardiovasculares , Trasplante de Corazón , Adolescente , Adulto , Enfermedades Cardiovasculares/epidemiología , Niño , Preescolar , Estudios Transversales , Trasplante de Corazón/efectos adversos , Humanos , Lactante , Encuestas Nutricionales , Estudios Retrospectivos , Factores de Riesgo , Adulto Joven
8.
J Perinatol ; 42(6): 788-795, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35397644

RESUMEN

OBJECTIVE: To describe the association between echocardiographic measures of pulmonary vascular disease and time to respiratory improvement among infants with Type I severe bronchopulmonary dysplasia (sBPD). STUDY DESIGN: We measured the pulmonary artery acceleration time indexed to the right ventricular ejection time (PAAT/RVET) and right ventricular free wall longitudinal strain (RVFWLS) at 34-41 weeks' postmenstrual age. Cox-proportional hazards models were used to estimate the relationship between the PAAT/RVET, RVFWLS, and the outcome: days from 36 weeks' postmenstrual age to room-air or discharge with oxygen (≤0.5 L/min). RESULT: For 102 infants, the mean PAAT/RVET and RVFWLS were 0.27 ± 0.06 and -22.63 ± 4.23%. An abnormal measurement was associated with an increased time to achieve the outcome (PAAT/RVET: 51v24, p < 0.0001; RVFWLS; 62v38, p = 0.0006). A normal PAAT/RVET was independently associated with a shorter time to outcome (aHR = 2.04, 1.11-3.76, p = 0.02). CONCLUSION: The PAAT/RVET may aid in anticipating timing of discharge in patients with type I severe BPD.


Asunto(s)
Displasia Broncopulmonar , Hipertensión Pulmonar , Enfermedades Vasculares , Displasia Broncopulmonar/complicaciones , Ecocardiografía , Humanos , Hipertensión Pulmonar/complicaciones , Lactante , Recién Nacido , Arteria Pulmonar/diagnóstico por imagen , Enfermedades Vasculares/complicaciones
9.
Neuroradiology ; 64(9): 1879-1885, 2022 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-35437634

RESUMEN

PURPOSE: This study investigates the feasibility of ultrafast fluid sensitive techniques for evaluation of pediatric spinal cord syrinx. Rapid imaging could obviate the need for sedation, which is often required for children undergoing lengthier standard spine imaging. METHODS: Children undergoing standard spine imaging for Chiari malformation, suspected Chiari malformation, or syrinx were included. Patients who provided informed consent were imaged with rapid acquisition sagittal and axial T2 HASTE spine sequences in addition to standard spine imaging. Standard and rapid spine imaging were then reviewed separately by a pediatric neuroradiologist. The presence or absence of syrinx, syrinx diameter, and length were assessed. The degree of cerebellar tonsillar ectopia, conus position, and evaluation of the filum were also recorded. RESULTS: Seventy-six patients aged 1 month to 18 years (mean 7 years) met the inclusion criteria. The sensitivity and specificity of rapid spine imaging for syrinx was 87.8% and 94.7% respectively. All syrinxes > 2.3 mm in diameter were identified with the rapid spine sequences. There was no statistically significant difference between rapid and standard spine imaging in assessment of syrinx diameter or length. Compared with standard spine imaging, rapid spine sequences demonstrated a 100% sensitivity for low-lying conus and a 98.2% sensitivity for cerebellar tonsillar ectopia. The filum was identified on only 31.6% of the rapid spine studies. CONCLUSION: Rapid T2 imaging demonstrated a high sensitivity for the presence and extent of spinal cord syrinx and may provide an alternative to traditional, lengthier standard spine imaging in selected patients.


Asunto(s)
Malformación de Arnold-Chiari , Siringomielia , Malformación de Arnold-Chiari/patología , Niño , Estudios de Factibilidad , Humanos , Imagen por Resonancia Magnética , Espectroscopía de Resonancia Magnética , Estudios Retrospectivos , Médula Espinal/diagnóstico por imagen , Médula Espinal/patología , Siringomielia/diagnóstico por imagen
10.
J Craniofac Surg ; 33(5): 1413-1417, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35275855

RESUMEN

ABSTRACT: Many patients with cleft palate in developing countries never receive postoperative speech assessment or therapy. The use of audiovisual recordings could improve access to post-repair speech care. The present study evaluated whether English-speaking speech-language pathologists (SLPs) could assess cleft palate patients speaking an unfamiliar language (Tamil) using recorded media. Recordings obtained from Tamil-speaking participants were rated by 1 Tamil-speaking SLP and 3 English-speaking SLPs. Ratings were analyzed for inter-rater reliability and scored for percent correct. Accuracy of the English SLPs was compared with independent t tests and Analysis of Variance. Sixteen participants (mean age 14.5 years, standard deviation [SD] 7.4 years; mean age of surgery of 2.7 years, SD 3.7 years; time since surgery: 10.8 years, SD 5.7 years) were evaluated. Across the 4 SLPs, 5 speech elements were found to have moderate agreement, and the mean kappa was 0.145 (slight agreement). Amongst the English-speaking SLPs, 10 speech elements were found to have substantial or moderate agreement, and the mean kappa was 0.333 (fair agreement). Speech measures with the highest inter-rater reliability were hypernasality and consonant production errors. The average percent correct of the English SLPs was 60.7% (SD 20.2%). English SLPs were more accurate if the participant was female, under eighteen, bilingual, or had speech therapy. The results demonstrate that English SLPs without training in a specific language (Tamil) have limited potential to assess speech elements accurately. This research could guide training interventions to augment the ability of SLPs to conduct cross-linguistic evaluations and improve international cleft care by global health teams.


Asunto(s)
Fisura del Paladar , Adolescente , Preescolar , Fisura del Paladar/cirugía , Estudios de Factibilidad , Femenino , Humanos , India , Lingüística , Reproducibilidad de los Resultados , Habla
11.
Phys Sportsmed ; 50(5): 454-460, 2022 10.
Artículo en Inglés | MEDLINE | ID: mdl-35135415

RESUMEN

OBJECTIVES: Rhythmic gymnastics injuries have not been studied thoroughly especially in the United States. Existing research studies are predominantly from Europe or Canada or from more than 15 years ago. The purpose of our study was to provide an updated description of injury patterns among rhythmic gymnasts in the United States. METHODS: A retrospective chart review was conducted of 193 rhythmic gymnastics injuries in 79 females, ages 6-20. Patients were seen between January 2010 and March 2020 in a hospital-based pediatric sports medicine clinic. Gymnast demographics, injury locations, and injury types were collected as available. Descriptive and bivariate statistical analysis was performed using general linear mixed models. RESULTS: Our cohort had a mean age of 14.61 ± 2.61 years. Overuse injuries (76.7%) were more common than acute injuries (23.3%). The most common injury types were strain (20.7%), nonspecific pain (15.5%), and tendinitis/tenosynovitis (10.36%). The most frequently injured body regions were lower extremity (75.1%), followed by trunk/back (19.2%), upper extremity (4.7%), and head/neck (1.0%). The most common injured body parts were foot (24.9%), ankle (15.5%), knee (15.0%), lower back (14.0%), and hip (13.0%). General linear mixed models revealed that older age (p = 0.001) and higher competitive level (p = 0.016) were associated with a greater number of diagnoses. Gymnasts with foot injuries were older than gymnasts with ankle (p = 0.026), hip (p < 0.0001), and knee (p = 0.002) injuries. Gymnasts with higher BMI-for-age percentile were more likely to have acute injuries than overuse (p = 0.035). CONCLUSION: Our data showed that injuries among rhythmic gymnasts were most frequently located in the lower extremities, specifically the foot, followed by trunk/back. Additionally, the most frequent injury types were strains and nonspecific pain, and overuse was the most prevalent mechanism. Gymnasts with foot injuries were older than gymnasts with ankle, hip, and knee injuries. Higher BMI is a predictor of acute injuries.


Asunto(s)
Traumatismos en Atletas , Traumatismos de los Pies , Medicina Deportiva , Adolescente , Adulto , Traumatismos en Atletas/epidemiología , Niño , Femenino , Gimnasia/lesiones , Humanos , Dolor , Estudios Retrospectivos , Estados Unidos/epidemiología , Adulto Joven
12.
Otolaryngol Head Neck Surg ; 167(5): 869-876, 2022 11.
Artículo en Inglés | MEDLINE | ID: mdl-35133903

RESUMEN

OBJECTIVE: Traditionally, data regarding thyroidectomy were extracted from billing databases, but information may be missed. In this study, a multi-institutional pediatric thyroidectomy database was used to evaluate recurrent laryngeal nerve (RLN) injury and hypoparathyroidism. STUDY DESIGN: Retrospective multi-institutional cohort study. SETTING: Tertiary care pediatric hospital systems throughout North America. METHODS: Data were individually collected for thyroidectomies, then entered into a centralized database and analyzed using univariate and multivariable regression models. RESULTS: In total, 1025 thyroidectomies from 10 institutions were included. Average age was 13.9 years, and 77.8% were female. Average hospital stay was 1.9 nights and 13.5% of patients spent at least 1 night in the pediatric intensive care unit. The most frequent pathology was papillary thyroid carcinoma (42%), followed by Graves' disease (20.1%) and follicular adenoma (18.2%). Overall, 1.1% of patients experienced RLN injury (0.8% permanent), and 7.2% experienced hypoparathyroidism (3.3% permanent). Lower institutional volume (odds ratio [OR], 3.57; 95% CI, 1.72-7.14) and concurrent hypoparathyroidism (OR, 3.51; 95% CI, 1.64-7.53) correlated with RLN injury on multivariable analysis. Graves' disease (OR, 2.27; 95% CI, 1.35-3.80), Hashimoto's thyroiditis (OR, 4.67; 95% CI, 2.39-9.09), central neck dissection (OR, 3.60; 95% CI, 2.36-5.49), and total vs partial thyroidectomy (OR, 7.14; 95% CI, 4.55-11.11) correlated with hypoparathyroidism. CONCLUSION: These data present thyroidectomy information and complications pertinent to surgeons, along with preoperative risk factor assessment. Multivariable analysis showed institutional volume and hypoparathyroidism associated with RLN injury, while hypoparathyroidism associated with surgical indication, central neck dissection, and extent of surgery. Low complication rates support the safety of thyroidectomy in pediatric tertiary care centers.


Asunto(s)
Enfermedad de Graves , Hipoparatiroidismo , Traumatismos del Nervio Laríngeo Recurrente , Neoplasias de la Tiroides , Humanos , Femenino , Niño , Adolescente , Masculino , Tiroidectomía/métodos , Estudios Retrospectivos , Estudios de Cohortes , Traumatismos del Nervio Laríngeo Recurrente/cirugía , Neoplasias de la Tiroides/cirugía , Enfermedad de Graves/complicaciones , Enfermedad de Graves/cirugía , Complicaciones Posoperatorias/cirugía
13.
J Am Heart Assoc ; 11(1): e022854, 2022 01 04.
Artículo en Inglés | MEDLINE | ID: mdl-34935411

RESUMEN

Background Pediatric dilated cardiomyopathy (DCM) is a well-known clinical entity; however, phenotype-genotype correlations are inadequately described. Our objective was to provide genotype associations with life-threatening cardiac outcomes in pediatric DCM probands. Methods and Results We performed a retrospective review of children with DCM at a large pediatric referral center (2007-2016), excluding syndromic, chemotherapy-induced, and congenital heart disease causes. Genetic variants were adjudicated by an expert panel and an independent clinical laboratory. In a cohort of 109 pediatric DCM cases with a mean age at diagnosis of 4.2 years (SD 5.9), life-threatening cardiac outcomes occurred in 47% (42% heart transplant, 5% death). One or more pathogenic/likely pathogenic variants were present in 40/109 (37%), and 36/44 (82%) of pathogenic/likely pathogenic variants occurred in sarcomeric genes. The frequency of pathogenic/likely pathogenic variants was not different in patients with familial cardiomyopathy (15/33 with family history versus 25/76 with no family history, P=0.21). TTN truncating variants occurred in a higher percentage of children diagnosed as teenagers (26% teenagers versus 6% younger children, P=0.01), but life-threatening cardiac outcomes occurred in both infants and teenagers with these TTN variants. DCM with left ventricular noncompaction features occurred in 6/6 patients with MYH7 variants between amino acids 1 and 600. Conclusions Sarcomeric variants were common in pediatric DCM. We demonstrated genotype-specific associations with age of diagnosis and cardiac outcomes. In particular, MYH7 had domain-specific association with DCM with left ventricular noncompaction features. Family history did not predict pathogenic/likely pathogenic variants, reinforcing that genetic testing should be considered in all children with idiopathic DCM.


Asunto(s)
Cardiomiopatía Dilatada , Adolescente , Niño , Estudios de Asociación Genética , Pruebas Genéticas , Genotipo , Humanos , Mutación , Sarcómeros
14.
Allergy Asthma Clin Immunol ; 17(1): 102, 2021 Oct 09.
Artículo en Inglés | MEDLINE | ID: mdl-34627374

RESUMEN

BACKGROUND: Knowledge is limited about the relationship between clinical reactivity to foods through breastfeeding and long-term food allergy outcomes. We explored parent-perceived symptoms of food allergy via breastfeeding and the association with future tolerance. METHODS: Subjects identified from the Chicago Food Allergy Study (2005-2011) were categorized by parent-reported reactions to maternally ingested foods via breastfeeding (50/898 peanut-allergic, 69/620 egg-allergic, and 153/589 milk-allergic). The primary outcome was tolerance [passed oral food challenge (OFC) or consumption of previously implicated food]. Secondary outcomes included severe reactions (anaphylaxis and/or cardiovascular/respiratory symptoms) and additional concomitant food allergies. Univariate chi-square analyses were performed to assess for association between variables, followed by logistic regression models. RESULTS: Of the 50 subjects with parent-reported peanut-associated symptoms with breastfeeding, none gained tolerance. There were no significant associations between parent-reported breastfeeding symptoms and development of tolerance for egg and milk (egg: OR 0.46, 95% CI 0.21-1.01, p = 0.053; milk: OR 1.13, 95% CI 0.70-1.81, p = 0.614). All egg-allergic subjects with parent-perceived symptoms while breastfeeding also reported multiple food allergies (n = 69), but milk- and peanut-allergic subjects were not more likely to have multiple allergies (milk: OR 1.89, 95% CI 0.88-4.02, p = 0.10; peanut: OR 2.36, 95% CI 0.72-7.76, p = 0.16). There were no significant associations between parent-reported breastfeeding symptoms and subsequent reaction severity. CONCLUSIONS: A significant proportion of parents perceive symptoms of food allergy attributable to indirect breastfeeding exposures. Our exploratory analysis suggests that infants with parent-perceived clinical reactivity to peanut via breastmilk may be less likely to gain tolerance. Infants with parent-reported reactivity to egg via breastmilk exposure were more likely to report multiple food allergies. Further rigorous prospective studies are needed to clarify the true prevalence of IgE-mediated food allergy symptoms attributable to indirect breastfeeding exposures and the association with development of tolerance.

15.
J Clin Anesth ; 75: 110493, 2021 12.
Artículo en Inglés | MEDLINE | ID: mdl-34482261

RESUMEN

STUDY OBJECTIVE: This study assessed whether implementation of an enhanced recovery-based pathway decreased length of stay without increasing readmissions among patients with adolescent idiopathic scoliosis undergoing posterior spinal fusion. DESIGN: Retrospective observational before-and-after study. SETTING: A tertiary children's hospital. PATIENTS: A total of 117 patients were studied, 78 in the pre-intervention group and 39 in the post-intervention group. All patients underwent posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS) in the same institution with one of two spine surgeons. Age, sex, American Society of Anesthesiologists physical status, and Cobb angle were comparable between the two groups. INTERVENTIONS: Between the pre- and post-intervention groups an enhanced recovery protocol was developed. The pathway included standardized use of nonopioid analgesics, proactive transition to oral analgesics, scheduled antiemetics, plans for diet advancement, and specific physical therapy goals. MEASUREMENTS: Outcome measurements included hospital length of stay, cumulative opioid doses in the first two postoperative days, and time to discontinuation of urinary catheter and patient-controlled analgesia. Postoperative emergency department visits, hospital readmissions and chronic pain management referrals were also measured. Pain scores on postoperative days one through four were recorded. MAIN RESULTS: Hospital length of stay decreased from 4.6 days to 3.8 days. Patient-controlled analgesia (PCA) was discontinued one day earlier on average following pathway implementation. Average cumulative postoperative opioid use, in morphine equivalents, decreased in the first two postoperative days from 2.5 to 2.2 mg/kg. There was no change in hospital readmission rate or postoperative chronic pain referral. CONCLUSIONS: Patients undergoing PSF for AIS experienced shorter hospital stays without increased readmissions following the implementation of an enhanced recovery pathway. Development of this pathway required buy-in from multiple stakeholders and significant coordination among services. The principles used to develop this pathway may be applied in other institutions and to other patient populations using the model outlined here.


Asunto(s)
Escoliosis , Fusión Vertebral , Adolescente , Analgesia Controlada por el Paciente , Niño , Humanos , Tiempo de Internación , Dolor Postoperatorio/tratamiento farmacológico , Dolor Postoperatorio/epidemiología , Estudios Retrospectivos , Escoliosis/cirugía , Fusión Vertebral/efectos adversos
16.
Horm Res Paediatr ; 94(5-6): 201-210, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34425574

RESUMEN

INTRODUCTION: Short stature is a common concern that necessitates pediatric endocrinology evaluation. Growth hormone deficiency (GHD) is a commonly considered etiology. Brain and pituitary magnetic resonance imaging (MRI) with gadolinium-based contrast agents (GBCAs) is the most widely used imaging in assessing patients with GHD. Given the significant strides made in MRI technology, the need for contrast material should be reassessed. METHOD: We performed a retrospective review of healthy patients with short stature and/or GHD who underwent brain and pituitary MRI with and without contrast to assess the added value of contrast administration. RESULTS: 227/318 identified patients underwent growth hormone (GH) stimulation testing; 28 (12.3%) with normal GH response and 62 (27.3%) with severe GHD. We found a low incidence of sellar and suprasellar pathologies. When comparing noncontrast and contrast MRI, we found perfect agreement in detecting abnormal posterior pituitary bright spots (kappa:1.0) and substantial agreement in detecting pars intermedia cysts and posterior superior sellar cysts (kappa: 0.74 and 0.71, respectively). Initially, only moderate agreement was found in detecting infundibular abnormalities (kappa: 0.51), although a revised noncontrast MRI protocol with high-resolution 3D images enabled visualization of the infundibulum. CONCLUSION: The MRI evaluation of healthy patients with short stature and/or isolated GHD may be completed without the use of GBCAs. The slight overestimation of pituitary stalk interruption by noncontrast images can be overcome by adding newer high-resolution sequences.


Asunto(s)
Anomalías Múltiples , Medios de Contraste/efectos adversos , Enanismo Hipofisario , Gadolinio/administración & dosificación , Hormona de Crecimiento Humana/deficiencia , Hipotiroidismo , Imagen por Resonancia Magnética , Hipófisis/fisiopatología , Silla Turca/anomalías , Niño , Endocrinología , Femenino , Humanos , Masculino , Estudios Retrospectivos
17.
J Cardiovasc Magn Reson ; 23(1): 86, 2021 07 01.
Artículo en Inglés | MEDLINE | ID: mdl-34193197

RESUMEN

BACKGROUND: Cardiac evaluations, including cardiovascular magnetic resonance (CMR) imaging and biomarker results, are needed in children during mid-term recovery after infection with SARS-CoV-2. The incidence of CMR abnormalities 1-3 months after recovery is over 50% in older adults and has ranged between 1 and 15% in college athletes. Abnormal cardiac biomarkers are common in adults, even during recovery. METHODS: We performed CMR imaging in a prospectively-recruited pediatric cohort recovered from COVID-19 and multisystem inflammatory syndrome in children (MIS-C). We obtained CMR data and serum biomarkers. We compared these results to age-matched control patients, imaged prior to the SARS-CoV-2 pandemic. RESULTS: CMR was performed in 17 children (13.9 years, all ≤ 18 years) and 29 age-matched control patients without SARS-CoV-2 infection. Cases were recruited with symptomatic COVID-19 (11/17, 65%) or MIS-C (6/17, 35%) and studied an average of 2 months after diagnosis. All COVID-19 patients had been symptomatic with fever (73%), vomiting/diarrhea (64%), or breathing difficulty (55%) during infection. Left ventricular and right ventricular ejection fractions were indistinguishable between cases and controls (p = 0.66 and 0.70, respectively). Mean native global T1, global T2 values and segmental T2 maximum values were also not statistically different from control patients (p ≥ 0.06 for each). NT-proBNP and troponin levels were normal in all children. CONCLUSIONS: Children prospectively recruited following SARS-CoV-2 infection had normal CMR and cardiac biomarker evaluations during mid-term recovery. Trial Registration Not applicable.


Asunto(s)
COVID-19/complicaciones , Corazón/diagnóstico por imagen , Corazón/fisiología , Imagen por Resonancia Magnética/métodos , Síndrome de Respuesta Inflamatoria Sistémica/complicaciones , Adolescente , Biomarcadores/sangre , COVID-19/sangre , Niño , Femenino , Humanos , Masculino , Estudios Prospectivos , SARS-CoV-2 , Síndrome de Respuesta Inflamatoria Sistémica/sangre
18.
J Urol ; 206(5): 1291-1299, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-34251872

RESUMEN

PURPOSE: We identified the incidence of acquired cryptorchidism among patients with proximal and mid shaft hypospadias, predictors of acquired cryptorchidism, and the prevalence of testis-epididymis nonfusion with ascended testes. We hypothesized that proximal hypospadias would be associated with higher incidence of acquired cryptorchidism than mid shaft hypospadias, and that ascended testes would exhibit increased prevalence of testis-epididymis nonfusion similar to anatomical findings in an undescended testis. MATERIALS AND METHODS: A retrospective cohort study of patients who underwent primary proximal and mid shaft hypospadias repair from 2010 to 2016 was conducted. Clinical and operative notes were reviewed. Patients with congenitally undescended testes or differences of sex development were excluded. RESULTS: A total of 175 patients were identified. Those with proximal hypospadias (14/104, 13%) were more likely than those with mid shaft hypospadias (1/71, 1%) to develop acquired cryptorchidism (p=0.04). Among proximal hypospadias patients, increased risk of acquired cryptorchidism was associated with pre-term birth (p <0.01) and penoscrotal transposition (p=0.01) but not with testis position on initial examination (p >0.99). In the 14 proximal hypospadias patients with acquired cryptorchidism, 21 ascended testes underwent orchiopexy. Operative notes adequately described testis-epididymis anatomy for 8/21 ascended testes. Testis-epididymis nonfusion was described in 6/8 ascended testes. CONCLUSIONS: Risk of acquired cryptorchidism is increased among patients with proximal hypospadias. Operative notes revealed a high rate of epididymal nonfusion with ascended testes, suggesting these testes morphologically resemble undescended testes. Close followup of testis position is needed in these patients, and the threshold to perform orchiopexy may need to be lower in select patients.


Asunto(s)
Criptorquidismo/epidemiología , Hipospadias/cirugía , Orquidopexia/estadística & datos numéricos , Niño , Preescolar , Criptorquidismo/etiología , Criptorquidismo/cirugía , Estudios de Seguimiento , Humanos , Hipospadias/complicaciones , Incidencia , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos
19.
Pediatr Crit Care Med ; 22(9): 785-794, 2021 09 01.
Artículo en Inglés | MEDLINE | ID: mdl-34038067

RESUMEN

OBJECTIVES: Parents value clear communication with PICU clinicians about possible patient and family outcomes (prognostic conversations). We describe PICU parent and attending physician reports and agreement regarding the occurrence of prognostic conversations. We queried parents and physicians about prognostic conversation content, which healthcare providers had prognostic conversations, and whether parents wanted more prognostic information. DESIGN: Prospective cross-sectional survey study. SETTING: University-based 40-bed PICU. PARTICIPANTS: Parents and attending physicians of PICU patients with multiple organ dysfunction within 24 hours of PICU admission. INTERVENTIONS: Surveys administered to parents and attending PICU physicians 5-10 days after PICU admission. MEASUREMENTS AND MAIN RESULTS: Surveys asked parents and physicians to report the occurrence of prognostic conversations related to PICU length of stay, risk of PICU mortality, and anticipated post-PICU physical, neurologic, and psychologic morbidities for patients and post-PICU psychologic morbidities for parents. Of 101 participants, 87 parents and 83 physicians reported having prognostic conversations. Overall concordance between parents and physicians was fair (Kappa = 0.22). Parents and physicians most commonly reported prognostic conversations about PICU length of stay (67.3% and 63.3%, respectively) and patient post-PICU physical morbidity (n = 48; 48.5% and n = 45; 44.5% respectively). Conversations reported less often by parents and physicians were about patient post-PICU psychologic morbidity (n = 13; 12.9% and n = 20; 19.8%, respectively). Per parent report, bedside nurses and physicians provided most prognostic information. Chaplains (n = 14; 50%) and social workers (n = 17; 60%) were more involved in conversations regarding parent psychologic morbidities. Most commonly, parents requested more information about length of stay and their child's physical morbidities. Parents less frequently wanted information about their own psychologic morbidities. CONCLUSIONS: Most parents and physicians report having prognostic conversations, primarily about length of stay and post-ICU physical morbidities. Concordance between parents and physicians is suboptimal. Future studies should evaluate prognostic conversations at other timepoints, how information is delivered, and how these conversations impact the PICU experience.


Asunto(s)
Enfermedad Crítica , Médicos , Niño , Estudios Transversales , Humanos , Unidades de Cuidado Intensivo Pediátrico , Pronóstico , Estudios Prospectivos
20.
Am J Infect Control ; 49(9): 1200-1203, 2021 09.
Artículo en Inglés | MEDLINE | ID: mdl-33774103

RESUMEN

A quasi-experimental study performed in a pediatric hematology-oncology unit demonstrated that whole-room ultraviolet-C disinfection was associated with a significant reduction in hospital-onset Clostridioides difficile infection (P< .01, trend and level), but not healthcare-associated viral respiratory infections (P= .06 for trend, P= .36 for level) or central line-associated bloodstream infections (P> 0.75, trend and level).


Asunto(s)
Clostridioides difficile , Infección Hospitalaria , Niño , Infección Hospitalaria/prevención & control , Desinfección , Hospitales , Humanos , Rayos Ultravioleta
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