RESUMO
BACKGROUND: Immune thrombocytopenia (ITP) and Evans syndrome (ES) are manifestations of immune dysregulation. Genetic variants in immune-related genes have been identified in patients with ITP and especially ES. We aimed to explore familial autoimmunity in patients with ITP and ES to understand possible contributions to chronicity. PROCEDURE: We assessed family history in two ways: via patient report for ITP and ES and by population-based analysis using the Utah Population Database (UPDB) for ITP. A total of 266 patients with ITP and 21 patients with ES were identified via chart review, and 252 of the 266 patients with ITP were also identified in the UPDB. RESULTS: Chart review showed familial autoimmunity in 29/182 (15.9%) and 25/84 (29.8%) of patients with newly diagnosed+persistent (nd+p) ITP and chronic ITP (cITP), respectively, (p = .009). The UPDB analysis revealed that autoimmunity in relatives of patients with nd+pITP was higher than in relatives of controls (odds ratio [OR]: 1.69 [1.19-2.41], p = .004), but was not significantly increased in relatives of patients with cITP (OR 1.10 [0.63-1.92], p = .734). Incomplete family history in medical records likely contributed to the observed discrepancy. CONCLUSIONS: The findings suggest that familial autoimmunity may have a stronger association with the development of ITP rather than its duration. Twelve (57.1%) patients with ES reported autoimmunity in their relatives. UPDB analysis was omitted due to the small number of patients with ES. The use of population databases offers a unique opportunity to assess familial health and may provide clues about contributors to immune dysregulation features within families.
Assuntos
Anemia Hemolítica Autoimune , Autoimunidade , Púrpura Trombocitopênica Idiopática , Trombocitopenia , Humanos , Anemia Hemolítica Autoimune/imunologia , Anemia Hemolítica Autoimune/epidemiologia , Anemia Hemolítica Autoimune/genética , Feminino , Masculino , Púrpura Trombocitopênica Idiopática/imunologia , Púrpura Trombocitopênica Idiopática/genética , Púrpura Trombocitopênica Idiopática/epidemiologia , Criança , Pré-Escolar , Trombocitopenia/imunologia , Trombocitopenia/genética , Adolescente , Lactente , Adulto , Adulto Jovem , Fatores de Risco , Pessoa de Meia-IdadeRESUMO
Purpose: Function and cosmesis may be improved by replantation following digital amputation in pediatric patients. However, accurate failure and complication rate estimates may be limited as most pertinent studies reflect single center/surgeon experience and therefore are limited by small sample sizes. The primary aim of this study was to assess the rate of failure (amputation) following pediatric digital replantation. Secondary aims include evaluating the rate of complications and associated resource utilization (intensive care unit stays, readmission rate, and hospital length of stay). Methods: Digital replantation patients were identified from 47 pediatric hospitals using the 2004 to 2020 Pediatric Health Information System nationwide database. Using applicable International Classification of Disease 9/10 and Current Procedural Terminology codes, we identified complications after replantation, including revision amputation, infection, surgical complications, medical complications, admission to intensive care unit (ICU), and length of stay. Results: Of the 348 patients who underwent replantation the mean age was 8.3 ± 5.1 years, and 27% were female. Mean hospital length of stay was 5.8 ± 4.7 (range, 1-28) days. Of the 53% of patients who required ICU admission, the mean ICU length of stay was 2.4 ± 3.3 days. Failure/amputation after replantation occurred in 71 (20.4%) patients, at a mean of 9.7 ± 27.2 days postoperatively. Surgical complications occurred in 58 (17%) patients, 30-day hospital readmissions occurred in 5.7% of patients, and 90-day readmissions occurred in 6.3% patients. Conclusion: The estimated rate of failure following pediatric digit replantation was 20%. Our data on failure and complication rates and associated resource utilization may be useful in counseling pediatric replantation patients and their families and provide an update on prior literature. Level of Evidence: IV, Prognosis.
RESUMO
Unfractionated heparin (UFH) is a commonly used anticoagulant for pediatric patients undergoing extracorporeal membrane oxygenation (ECMO), but evidence is lacking on the ideal dosing. We aimed to (1) develop a population pharmacokinetic (PK) model for UFH, measured through anti-factor Xa assay; (2) optimize UFH starting infusions and dose titrations through simulations; and (3) explore UFH exposure-clinical outcomes relationship. Data from 218 patients admitted to Utah's Primary Children's Hospital were retrospectively collected. A 1-compartment PK model with time-varying clearance (CL) adequately described UFH PK. Weight on CL and volume of distribution and ECMO circuit change on CL were significant covariates. The typical estimates for initial CL and first-order rate constant to reach steady-state CL were 0.57 L/(h·10 kg) and 0.02/h. Comparable to non-ECMO patients, the typical steady-state CL was 0.81 L/(h·10 kg). Simulations showed that a 75 IU/kg UFH bolus dose followed by starting infusions of 25 and 20 IU/h/kg for patients aged younger than 6 years and 6 years or older, respectively, achieved the therapeutic target in 56.6% of all patients, whereas only 3.1% exceeded the target. The proposed UFH titration schemes achieved the target in more than 90% of patients while less than 0.63% were above the target after 24 and 48 hours of treatment. The median intensive care unit survival time in patients within and below the target at 24 hours was 136 and 66 hours, respectively. In conclusion, PK model of UFH was developed for pediatric patients on ECMO. The proposed UFH dosing scheme attained the anti-factor Xa target rapidly and safely.
Assuntos
Oxigenação por Membrana Extracorpórea , Heparina , Humanos , Criança , Idoso , Heparina/efeitos adversos , Estudos Retrospectivos , Anticoagulantes/efeitos adversos , Testes de Coagulação SanguíneaRESUMO
Objectives: Approximately 40% of boys with X-linked adrenoleukodystrophy (ALD) develop inflammatory demyelinating brain lesions (cerebral ALD, cALD) and are at risk for death or severe disability. Risk factors for cALD are poorly understood. Our objective was to evaluate whether vitamin D status, which influences immune function, is associated with risk for cALD. Methods: We used two independent cohorts to assess whether low vitamin D status is correlated with cALD. We used complementary proxies for vitamin D status: plasma 25-hydroxyvitamin D levels and latitude. In our first cohort, we measured 25-hydroxyvitamin D in biobanked plasma samples from ALD boys with initially normal brain MRIs followed at two expert centers. In a second cohort, we measured latitude (using home ZIP code) among ALD boys identified in a national administrative database (PHIS) covering 51 US pediatric hospitals. We used logistic regression models to estimate the odds of developing cALD in each cohort. Results: In the first cohort, we identified 20 ALD boys with a total of 53 plasma sample timepoints who met inclusion criteria; 50% (n = 10) subsequently developed cALD. Average 25-hydroxyvitamin D levels were lower among boys who developed cALD than those who did not (median 28.9 vs 36.6 ng/ml); p = 0.019. For each 10 ng/mL decrease in 25-hydroxyvitamin D, the odds ratio for developing cALD was 6.94; p = 0.044. In the second cohort, we identified 230 ALD boys across 28 states; 57% of boys (n = 132) developed cALD. Each 2° increase in latitude conferred an odds ratio of 1.17 (95% confidence interval, 1.01, 1.35); p = 0.036 for developing cALD. Conclusions: Using independent cohorts, we found that ALD boys with lower pre-morbid plasma levels of 25-hydroxyvitamin D, or more northerly latitude of residence, were more likely to develop cALD. These findings offer complementary lines of evidence that vitamin D and/or ultraviolet light exposure influence cALD risk.
RESUMO
AIM: Previous work has shown that children of Hispanic ethnicity have reduced likelihood to achieve seizure remission, but it was unknown why. The purpose of this study was to evaluate antiseizure medicine (ASM) refill characteristics, comparing Hispanic and non-Hispanic White pediatric patients. METHODS: This was a retrospective population-based study in children between ages 6 months and 15 years. Epilepsy outcome was categorized as seizure free, treatment failure, or undetermined. ASM refill characteristics were determined from an insurance provider. RESULTS: 247 patients were identified: 52 (21 %) were treatment failure; 181 (73 %) were seizure free; and 14 (5.7 %) were undetermined. ASM refill rates were similar in Hispanic and White patients (38.2, 32.1, respectively). Hispanic and White patients had similar numbers of different ASMs prescribed (2.1 and 2.4). There was not a significant difference in proportion of days covered between Hispanic and White patients (0.99 and 0.95). INTERPRETATION: We found no differences between pediatric Hispanic and White epilepsy patients, for number of ASM refills, the number of ASMs prescribed, the choice of ASMs, the proportion of days covered, or the lateness of refills. Our findings suggest that the observation of reduced likelihood to achieve seizure remission in pediatric Hispanic patients is not associated with ASM refill patterns.
Assuntos
Epilepsia , Hispânico ou Latino , Anticonvulsivantes/uso terapêutico , Criança , Epilepsia/tratamento farmacológico , Etnicidade , Humanos , Estudos Retrospectivos , Convulsões/tratamento farmacológicoRESUMO
Objective: To determine differences in long-term health and neurological outcomes following infantile spasms (IS) in patients treated with adrenocorticotropic hormone (ACTH) vs. prednisolone/prednisone (PRED). Methods: A retrospective, case-control study of patients with an International Classification of Diseases, Ninth Revision, Clinical Modifications (ICD-9) diagnosis of IS, identified over a 10-year period from a national administrative database, was conducted. IS patients treated with ACTH or PRED were determined and cohorts established by propensity score matching. Outcomes, defined by hospital discharge ICD codes, were followed for each patient for 5 years. Related ICD codes were analyzed jointly as phenotype codes (phecodes). Analysis of phecodes between cohorts was performed including phenome-wide association analysis. Results: A total of 5,955 IS patients were identified, and analyses were subsequently performed for 493 propensity score matched patients, each in the ACTH and PRED cohorts. Following Bonferroni correction, no phecode was more common in either cohort (p < 0.001). However, assuming an a priori difference, one phecode, abnormal findings on study of brain or nervous system (a category of abnormal neurodiagnostic tests), was more common in the PRED cohort (p <0.05), and was robust to sensitivity analysis. Variability in outcomes was noted between hospitals. Significance: We found that long-term outcomes for IS patients following ACTH or PRED treatment were very similar, including for both neurological and non-neurological outcomes. In the PRED-treated cohort there was a higher incidence of abnormal neurodiagnostic tests, assuming an a priori statistical model. Future studies can evaluate whether variability in outcomes between hospitals may be affected by post-treatment differences in care models.
RESUMO
OBJECTIVES: Cerebral X-linked adrenoleukodystrophy (cALD) is an inflammatory demyelination of the brain that can lead to death unless treated by hematopoietic stem cell transplantation. Survival and improved outcomes for cerebral adrenoleukodystrophy are associated with hematopoietic stem cell transplantation at earliest evidence of disease on magnetic resonance imaging (MRI). Our goal was to determine average duration between diagnosis of cALD and hematopoietic stem cell transplantation. METHODS: This was a retrospective review of data of patients aged 18 years or younger, using a nationwide administrative health care database (Pediatric Health Information System), with an International Classification of Diseases, Tenth Revision (ICD-10) diagnosis of adrenoleukodystrophy. Time range was October 1, 2015, through June 30, 2021. We determined time to hematopoietic stem cell transplantation by duration between index brain MRI and a code for hematopoietic stem cell transplantation. RESULTS: We identified 27 patients with cerebral adrenoleukodystrophy. Total charges for the cohort was $53 million. Time to transplant averaged 97 days. For Hispanic patients, time to transplant was 117 days, compared with 80 days for White, non-Hispanic patients. Comparison of different hospitals showed significant variability in time to hematopoietic stem cell transplantation. DISCUSSION: We found that time to hematopoietic stem cell transplantation was >3 months for patients with cerebral adrenoleukodystrophy in the hospitals we evaluated. We noted differences in average time by race/ethnicity and by hospital. Our findings suggest opportunity to reduce time to transplant in cerebral adrenoleukodystrophy.
Assuntos
Adrenoleucodistrofia , Transplante de Células-Tronco Hematopoéticas , Adrenoleucodistrofia/complicações , Adrenoleucodistrofia/patologia , Adrenoleucodistrofia/terapia , Criança , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Imageamento por Ressonância Magnética , Estudos RetrospectivosRESUMO
OBJECTIVE: The purpose of our study was to understand the healthcare burden and incidence of Krabbe disease (Krabbe). METHODS: Retrospective analysis of Krabbe patients identified October 1, 2015 through December 31, 2020, ages birth through age 3, evaluated in two national databases. We estimated point prevalence and incidence from year 2016 data. RESULTS: We identified 98 unique Krabbe patients with 736 visits including 260 were inpatient admissions. Total healthcare charges were $51.5 million dollars. We determined a point prevalence of 34 68 Krabbe patients in 2016 ages 0 3 years. This estimates a birth incidence of ~1 in 310,000 live births. Significance: Krabbe disease patients had over $51 million in health care charges and hundreds of hospitalizations. Estimated prevalence and birth incidence is similar to rates observed from newborn screening. Our findings show the tremendous health impacts of Krabbe disease, and provide guidance for efforts in screening and treatment planning.
Assuntos
Efeitos Psicossociais da Doença , Hospitalização/estatística & dados numéricos , Leucodistrofia de Células Globoides/epidemiologia , Leucodistrofia de Células Globoides/terapia , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Estados Unidos/epidemiologiaRESUMO
Krabbe disease (KD) is a leukodystrophy caused by mutations in the galactosylceramidase gene. Presymptomatic hematopoietic stem cell transplantation (HSCT) is associated with improved outcomes, but most data are from single-center studies. Our objective was to characterize national patterns of HSCT for KD including whether there were disparities in HSCT utilization and outcomes. We conducted a retrospective study of KD patients ≤ age 18 years from November 1, 2015, through December 31, 2019, using the U.S. Children's Hospital Association's Pediatric Health Information System database. We evaluated outcomes for HSCT, intensive care unit days, and mortality, comparing age, sex, race/ethnicity, rural/urban location, and median household income. We identified 91 KD patients. HSCT, performed in 32% of patients, was associated with reduced mortality, 31 vs. 68% without HSCT (p < 0.003). Trends included the fact that more males than females had HSCT (39 vs. 23%); more Asian and White patients had HSCT compared to Black or Hispanic patients (75, 33, 25, and 17%, respectively); and patients from households with the lowest-income quartile (< $25,000) had more HSCT compared to higher-income quartiles (44 vs. 33, 30, and 0%). Overall, receiving HSCT was associated with reduced mortality. We noted trends in patient groups who received HSCT. Our findings suggest that disparities in receiving HSCT could affect outcomes for KD patients.
RESUMO
OBJECTIVE: To create neonatal reference intervals for the MicroR and HYPO-He complete blood count (CBC) parameters and to test whether these parameters are sensitive early markers of disease at early stages of microcytic/hypochromic disorders while the CBC indices are still normal. STUDY DESIGN: We retrospectively collected the CBC parameters MicroR and HYPO-He, along with the standard CBC parameters, from infants aged 0-90 days at Intermountain Healthcare hospitals using Sysmex hematology analyzers. We created reference intervals for these parameters by excluding values from neonates with proven microcytic disorders (ie, iron deficiency or alpha thalassemia) from the dataset. RESULT: From >11 000 CBCs analyzed, we created reference intervals for MicroR and HYPO-He in neonates aged 0-90 days. The upper intervals are considerably higher in neonates than in adults, validating increased anisocytosis and polychromasia among neonates. Overall, 52% of neonates with iron deficiency (defined by reticulocyte hemoglobin equivalent <25 pg) had a MicroR >90% upper interval (relative risk, 4.14; 95% CI, 3.80-4.53; P < .001), and 68% had an HYPO-He >90% upper interval (relative risk, 6.64; 95% CI, 6.03-7.32; P < .001). These 2 new parameters were more sensitive than the red blood cell (RBC) indices (P < .001) in identifying 24 neonates with iron deficiency at birth. CONCLUSIONS: We created neonatal reference intervals for MicroR and HYPO-He. Although Sysmex currently designates these as research use only in the US, they can be measured as part of a neonate's CBC with no additional phlebotomy volume or run time and can identify microcytic and hypochromic disorders even when the RBC indices are normal.
Assuntos
Anemia Ferropriva/diagnóstico , Reticulócitos/química , Anemia Ferropriva/sangue , Biomarcadores/sangue , Humanos , Lactente , Recém-Nascido , Valores de Referência , Contagem de Reticulócitos/métodos , Estudos RetrospectivosRESUMO
In order to reduce iron deficiency in neonates at-risk for iron deficiency, we implemented a guideline to increase the consistency of early iron supplementation in infants of diabetic mothers, small for gestational age neonates and very low birthweight premature neonates. Three years following implementation we performed a retrospective analysis in order to assess adherence to the guideline and to compare timing of early iron supplementation and reticulocyte-hemoglobin (RET-He) values at one month of life in at-risk infants. Adherence with early iron supplementation guidelines was 73.4% (399/543) with 51% (275/543) having RET-He values obtained at one month. Despite good adherence, 16% (44/275) had RET-He <25 pg (5th percentile for gestational age). No infants receiving red blood cell transfusion (0/20) had RET-He <25 pg vs. 26.1% (40/153) of those treated with darbepoetin (p < 0.001). There was no evidence of increased feeding intolerance (episodes of emesis/day) with early iron supplementation.
Assuntos
Unidades de Terapia Intensiva Neonatal , Deficiências de Ferro/tratamento farmacológico , Ferro/administração & dosagem , Feminino , Humanos , Recém-Nascido , Ferro/efeitos adversos , Deficiências de Ferro/sangue , Masculino , Estudos RetrospectivosRESUMO
BACKGROUND: The impact of published evidence on clinical practice has been understudied in pediatric cardiology. OBJECTIVE: We sought to assess changes in prescribing behavior for angiotensin-converting enzyme inhibitor (ACEI) and digoxin at discharge after initial palliation of infants with single ventricle (SV) physiology following the publication of two large studies: The Pediatric Heart Network Infant Single Ventricle (PHN-ISV) trial showing no benefit with routine ACEI use and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) analysis showing an association between digoxin and survival. METHODS: ICD-9-10 codes identified SV infants from the Pediatric Health Information System (1/2004 to 1/2018) and charge codes identified medications at discharge. Generalized estimating equations implementing segmented logistic regressions modeled medication use, before and after (with a 3-month washout period) the relevant publication (ACEI 7/1/2010; digoxin 4/1/2016). A subgroup analysis was performed for hypoplastic left heart syndrome (HLHS). RESULTS: ACEI use (37 centers, n = 4700) at discharge did not change over time during the pre-publication period. After publication of the PHN-ISV trial, ACEI use decreased (OR: 0.61, CI 0.44-0.84, p = 0.003). Digoxin use (43 centers, n = 4778) decreased by 1% monthly before publication. After the NPC-QIC publication, digoxin use increased (OR: 2.07, CI 1.05-4.08, p = 0.04) with an ongoing increase of 9% per month. Results were similar for the HLHS subgroup. CONCLUSIONS: Prescribing behavior changed congruently after the publication of evidence-based studies, with decreased ACEI use and increased digoxin use at discharge following initial palliation of SV infants. Our findings suggest scientific findings were rapidly implemented into clinical practice.
Assuntos
Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Digoxina/uso terapêutico , Padrões de Prática Médica , Coração Univentricular/tratamento farmacológico , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Lactente , Masculino , Procedimentos de Norwood/normas , Cuidados Paliativos/métodos , Melhoria de Qualidade , Ensaios Clínicos Controlados Aleatórios como Assunto , Estudos RetrospectivosRESUMO
BACKGROUND: Neurological manifestations are commonly reported in patients with celiac disease (CD). We aimed to characterize epilepsy features in a pediatric population with CD and the effect of a gluten-free diet (GFD) on seizure burden. METHODS: A retrospective chart review was performed on pediatric patients treated at the University of Utah and Primary Children's Hospital in Salt Lake City, Utah, with both epilepsy and CD and compared with a control group with epilepsy only. RESULTS: We identified 56 patients with epilepsy and biopsy-confirmed CD (n = 36, 64%) or elevated tissue transglutaminase antibodies (tTG-Ab) without biopsy-confirmed CD (n = 20, 36%). Age- and gender-matched controls were selected from patients with epilepsy only (n = 168). Patients with biopsy-proven CD or positive tTG-Ab had high percentage of drug-resistant epilepsy (DRE) compared with the control group (P < 0.05). Age at seizure onset preceded the diagnosis of CD on average by 5.9 years for patients with DRE (P < 0.01) compared with 2.2 years for those with drug-responsive epilepsy. Adhering to a GFD reduced seizure frequency or resulted in weaning dosage or weaning off of one or more antiseizure medications in a majority of patients with DRE. CONCLUSIONS: DRE was more prevalent in pediatric patients with biopsy-confirmed CD and positive tTG-Ab compared with the control group (which included childhood epilepsy syndromes), but comparable with the prevalence of DRE in the general population. Adherence to a GFD in combination with antiseizure medications appears to reduce seizure burden for those with CD and DRE.
Assuntos
Anticonvulsivantes/administração & dosagem , Doença Celíaca , Dieta Livre de Glúten , Epilepsia Resistente a Medicamentos , Síndromes Epilépticas , Adolescente , Doença Celíaca/complicações , Doença Celíaca/diagnóstico , Doença Celíaca/dietoterapia , Criança , Pré-Escolar , Epilepsia Resistente a Medicamentos/dietoterapia , Epilepsia Resistente a Medicamentos/tratamento farmacológico , Epilepsia Resistente a Medicamentos/etiologia , Síndromes Epilépticas/dietoterapia , Síndromes Epilépticas/tratamento farmacológico , Síndromes Epilépticas/etiologia , Feminino , Humanos , Lactente , Masculino , Estudos RetrospectivosRESUMO
OBJECTIVE: To assess the impact of geographic access to surgical center on readmission risk and burden in children after congenital heart surgery. STUDY DESIGN: Children <6 years old at discharge after congenital heart surgery (Risk Adjustment for Congenital Heart Surgery-1 score 2-6) were identified using Pediatric Health Information System data (46 hospitals, 2004-2015). Residential distance from the surgery center, calculated using ZIP code centroids, was categorized as <15, 15-29, 30-59, 60-119, and ≥120 miles. Rurality was defined using rural-urban commuting area codes. Geographic risk factors for unplanned readmissions to the surgical center and associated burden (total hospital length of stay [LOS], costs, and complications) were analyzed using multivariable regression. RESULTS: Among 59 696 eligible children, 19 355 (32%) had ≥1 unplanned readmission. The median LOS was 9 days (IQR 22) across the entire cohort. In those readmitted, median total costs were $31 559 (IQR $90 176). Distance from the center was inversely related but rurality was positively related to readmission risk. Among those readmitted, increased distance was associated with longer LOS, more complications, and greater costs. Compared with urban patients, highly rural patients were more likely to have an unplanned readmission but had fewer average readmission days. CONCLUSIONS: Geographic measures of access differentially affect readmission to the surgery center. Increased distance from the center was associated with fewer unplanned readmissions but more complications. Among those readmitted, the most isolated patients had the greatest readmission costs. Understanding the contribution of geographic access will aid in developing strategies to improve care delivery to this population.
Assuntos
Acessibilidade aos Serviços de Saúde/estatística & dados numéricos , Cardiopatias Congênitas/cirurgia , Hospitais Pediátricos/provisão & distribuição , Readmissão do Paciente/estatística & dados numéricos , Centros de Atenção Terciária/provisão & distribuição , Criança , Pré-Escolar , Feminino , Custos de Cuidados de Saúde/estatística & dados numéricos , Acessibilidade aos Serviços de Saúde/economia , Cardiopatias Congênitas/economia , Hospitais Pediátricos/economia , Humanos , Lactente , Recém-Nascido , Estudos Longitudinais , Masculino , Readmissão do Paciente/economia , Análise de Regressão , Estudos Retrospectivos , Saúde da População Rural/economia , Saúde da População Rural/estatística & dados numéricos , Serviços de Saúde Rural/economia , Serviços de Saúde Rural/provisão & distribuição , Centros de Atenção Terciária/economia , Estados Unidos , Saúde da População Urbana/economia , Saúde da População Urbana/estatística & dados numéricos , Serviços Urbanos de Saúde/economia , Serviços Urbanos de Saúde/provisão & distribuiçãoRESUMO
OBJECTIVE: The aim was to analyze the impact of decreased head computed tomography (CT) imaging on detection of abnormalities and outcomes for children with isolated head trauma. METHODS: The study involves a multicenter retrospective cohort of patients younger than 19 years presenting for isolated head trauma to emergency departments in the Pediatric Health Information System database from 2003 to 2015. Patients directly admitted or transferred to another facility and those with a discharge diagnosis code for child maltreatment were excluded. Outcomes were ascertained from administrative and billing data. Trends were tested using mixed effects logistic regression, accounting for clustering within hospitals and adjusted for age, sex, insurance type, race, presence of a complex chronic condition, and hospital-level case mix index. RESULTS: Between 2003 and 2015, 306,041 children presented for isolated head trauma. The proportion of children receiving head CT imaging was increasing until 2008, peaking at just under 40%, before declining to 25% by 2015. During the recent period of decreased head CT imaging, the detection of skull fractures (odds ratio [OR]/year, 0.96; 95% confidence interval [CI], 0.95-0.97) and intracranial bleeds (OR/year, 0.96; 95% CI, 0.94-0.97), hospitalization (OR/year, 0.96; 95% CI, 0.95-0.96), neurosurgery (OR/year, 0.91; 95% CI, 0.87-0.95), and revisit (OR/year, 0.98; 95% CI, 0.96-1.00) also decreased, without significant changes in mortality (OR/year, 0.93; 95% CI, 0.84-1.04) or persistent neurologic impairment (OR/year, 1.03; 95% CI, 0.92-1.15). CONCLUSIONS: The recent decline in CT scanning in children with isolated head trauma was associated with a reduction in detection of intracranial abnormalities, and a concomitant decrease in interventions, without measurable patient harm.
Assuntos
Traumatismos Craniocerebrais , Fraturas Cranianas , Criança , Serviço Hospitalar de Emergência , Hospitalização , Humanos , Lactente , Estudos RetrospectivosRESUMO
Purpose: The therapeutic utility of Cannabis in cancer is a topic of intense interest. Dronabinol is synthetic Δ9-tetrahydrocannabinol (THC), the primary psychoactive component of Cannabis sativa, and is approved for treating refractory chemotherapy-induced nausea and vomiting. Little is known about dronabinol prescribing in children and young adults, and no published concentration data are available. This study evaluated national level dronabinol use and assessed concentrations of THC and its primary metabolites in patients with cancer <27 years of age prescribed dronabinol. Methods: Observational review of records from the Pediatric Health Information System (PHIS) and a regional network of hospitals in the Intermountain West, including a tertiary care children's hospital, Primary Children's Hospital (PCH), for inpatients <27 years of age prescribed dronabinol. Prospective blood samples were collected from children with cancer at PCH. Results: Across PHIS institutions, overall dronabinol prescribing aligned with the pharmacy records for those with cancer (p < 0.0001), and of these, 10.4% received dronabinol as inpatients. Blood collected within 72 hours of dronabinol administration was available from 10 children with a median age of 12.5 (range 6-17) years. Quantifiable concentrations were found in 4 (13%), 6 (20%), and 1 (3%) samples assayed for THC, 11-nor-9-carboxy-Δ9-tetrahydrocannabinol (COOH-THC), and 11-hydroxy-Δ9-tetrahydrocannabinol (OH-THC), respectively. THC concentrations ranged between 0.100 and 0.128 ng/mL and were not associated with dose. Conclusion: Dronabinol prescribing appears exclusive to patients diagnosed with cancer, and its use has increased steadily in the past decade. In a small sample of children administered dronabinol, THC and metabolite concentrations were consistently low or undetectable.
Assuntos
Neoplasias , Adolescente , Cannabis , Criança , Dronabinol/análise , Cromatografia Gasosa-Espectrometria de Massas , Humanos , Neoplasias/tratamento farmacológico , Estudos Prospectivos , Adulto JovemRESUMO
OBJECTIVE: To determine shared comorbidities and to identify underrecognized or unexpected morbidities in children with leukodystrophies using an unbiased phenome-wide association study (PheWAS) analysis of a nationwide pediatric clinical and financial database. METHODS: Data were extracted from the Pediatric Health Information System database. Patients with leukodystrophy were identified with International Classification of Diseases, 10th revision, clinical modification, diagnostic codes for any of 4 specific leukodystrophies (X-linked adrenoleukodystrophy (E71.52x), Hurler disease (E76.01), Krabbe disease (E75.23), and metachromatic leukodystrophy (E75.25)) over a 3-year time period. Confirmed leukodystrophy cases (n = 553) were matched with 1659 controls. A PheWAS analysis was performed on all available ICD diagnostic codes for cases and controls. Comparisons were performed for all 4 leukodystrophies as a group and individually. RESULTS: We found 174 phecodes (grouped ICD codes) associated with leukodystrophies, including 28 codes with a rate difference (RD) > 20%. Known comorbidities of leukodystrophies including developmental delay, epilepsy, and adrenal insufficiency were identified. Unexpected associations identified included hypertension (RD 30%, OR 25), hearing loss (RD 28%, OR 15), and cardiac dysrhythmias (RD 27%, OR 9). Hurler disease had a greater number of unique disease conditions. CONCLUSIONS: PheWAS analysis from a national database demonstrates shared and unique features of leukodystrophies. Developmental delay, cardiac dysrhythmias, fluid and electrolyte disturbances, and respiratory issues were common to all 4 leukodystrophy diseases. Use of a PheWAS in leukodystrophies and other pediatric neurologic diseases offers a method for targeting improved care for patients by identification of morbidities.
RESUMO
Children with complicated appendicitis, osteomyelitis, and complicated pneumonia have historically been treated with postdischarge intravenous antibiotics (PD-IV) using peripherally inserted central catheters (PICCs). Recent studies have shown no advantage and increased complications of PD-IV, compared with oral therapy, and the extent to which use of PD-IV has since changed for these conditions is not known. We used a national children's hospital database to evaluate trends in PD-IV during 2000-2018 for each of these three conditions. PD-IV decreased from 13% to 2% (risk ratio [RR], 0.15; 95% CI, 0.14-0.16) for complicated appendicitis, 61% to 22% (RR, 0.41; 95% CI, 0.39-0.43) for osteomyelitis, and 29% to 19% (RR, 0.63; 95% CI, 0.58-0.69) for complicated pneumonia. Despite these overall reductions, substantial variation in PD-IV use by hospital remains in 2018.
Assuntos
Apendicite , Cateterismo Periférico , Assistência ao Convalescente , Antibacterianos/uso terapêutico , Apendicite/tratamento farmacológico , Criança , Humanos , Alta do PacienteRESUMO
Children with medical complexity (CMC) have chronic, multisystem health conditions, substantial health care needs, major functional limitations, and high resource use. They represent <1% of US children yet account for more than one-third of total pediatric health care costs. Health care systems designed for typical children do not meet the unique needs of CMC. In this special article, we describe the experience of our Comprehensive Care Program for CMC in a pediatric tertiary care center, from its launch in 2007 to its present model. We review the literature, describe our collective lessons learned, and offer suggestions for future directions.