Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 82
Filter
1.
Front Cardiovasc Med ; 11: 1356010, 2024.
Article in English | MEDLINE | ID: mdl-38725831

ABSTRACT

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare genetic premature aging disease that is historically fatal in teenage years, secondary to severe accelerated atherosclerosis. The only approved treatment is the farnesyltransferase inhibitor lonafarnib, which improves vascular structure and function, extending average untreated lifespan of 14.5 years by 4.3 years (30%). With this longer lifespan, calcific aortic stenosis (AS) was identified as an emerging critical risk factor for cardiac death in older patients. Intervention to relieve critical AS has the potential for immediate improvement in healthspan and lifespan. However, HGPS patient-device size mismatch, pervasive peripheral arterial disease, skin and bone abnormalities, and lifelong failure to thrive present unique challenges to intervention. An international group of experts in HGPS, pediatric and adult cardiology, cardiac surgery, and pediatric critical care convened to identify strategies for successful treatment. Candidate procedures were evaluated by in-depth examination of 4 cases that typify HGPS clinical pathology. Modified transcatheter aortic valve replacement (TAVR) and left ventricular Apico-Aortic Conduit (AAC) placement were deemed high risk but viable options. Two cases received TAVR and 2 received AAC post-summit. Three were successful and 1 patient died perioperatively due to cardiovascular disease severity, highlighting the importance of intervention timing and comparative risk stratification. These breakthrough interventions for treating critical aortic stenosis in HGPS patients could rewrite the current clinical perspective on disease course by greatly improving late-stage quality of life and increasing lifespan. Expanding worldwide medical and surgical competency for this ultra-rare disease through expert information-sharing could have high impact on treatment success.

3.
Circulation ; 149(19): 1493-1500, 2024 May 07.
Article in English | MEDLINE | ID: mdl-38563137

ABSTRACT

BACKGROUND: The association between chest compression (CC) pause duration and pediatric in-hospital cardiac arrest survival outcomes is unknown. The American Heart Association has recommended minimizing pauses in CC in children to <10 seconds, without supportive evidence. We hypothesized that longer maximum CC pause durations are associated with worse survival and neurological outcomes. METHODS: In this cohort study of index pediatric in-hospital cardiac arrests reported in pediRES-Q (Quality of Pediatric Resuscitation in a Multicenter Collaborative) from July of 2015 through December of 2021, we analyzed the association in 5-second increments of the longest CC pause duration for each event with survival and favorable neurological outcome (Pediatric Cerebral Performance Category ≤3 or no change from baseline). Secondary exposures included having any pause >10 seconds or >20 seconds and number of pauses >10 seconds and >20 seconds per 2 minutes. RESULTS: We identified 562 index in-hospital cardiac arrests (median [Q1, Q3] age 2.9 years [0.6, 10.0], 43% female, 13% shockable rhythm). Median length of the longest CC pause for each event was 29.8 seconds (11.5, 63.1). After adjustment for confounders, each 5-second increment in the longest CC pause duration was associated with a 3% lower relative risk of survival with favorable neurological outcome (adjusted risk ratio, 0.97 [95% CI, 0.95-0.99]; P=0.02). Longest CC pause duration was also associated with survival to hospital discharge (adjusted risk ratio, 0.98 [95% CI, 0.96-0.99]; P=0.01) and return of spontaneous circulation (adjusted risk ratio, 0.93 [95% CI, 0.91-0.94]; P<0.001). Secondary outcomes of any pause >10 seconds or >20 seconds and number of CC pauses >10 seconds and >20 seconds were each significantly associated with adjusted risk ratio of return of spontaneous circulation, but not survival or neurological outcomes. CONCLUSIONS: Each 5-second increment in longest CC pause duration during pediatric in-hospital cardiac arrest was associated with lower chance of survival with favorable neurological outcome, survival to hospital discharge, and return of spontaneous circulation. Any CC pause >10 seconds or >20 seconds and number of pauses >10 seconds and >20 seconds were significantly associated with lower adjusted probability of return of spontaneous circulation, but not survival or neurological outcomes.


Subject(s)
Cardiopulmonary Resuscitation , Heart Arrest , Humans , Heart Arrest/mortality , Heart Arrest/therapy , Female , Male , Child , Child, Preschool , Cardiopulmonary Resuscitation/mortality , Time Factors , Infant , Treatment Outcome , Adolescent
7.
Circulation ; 147(23): 1734-1744, 2023 06 06.
Article in English | MEDLINE | ID: mdl-36919608

ABSTRACT

BACKGROUND: Hutchinson-Gilford progeria syndrome (HGPS) is an ultrarare, fatal, premature aging disease caused by a toxic protein called progerin. Circulating progerin has not been previously detected, precluding research using readily available biological samples. This study aimed to develop a plasma progerin assay to evaluate progerin's quantity, response to progerin-targeted therapy, and relationship to patient survival. METHODS: Biological samples were collected by The Progeria Research Foundation Cell and Tissue Bank from a non-HGPS cohort cross-sectionally and a HGPS cohort longitudinally. HGPS donations occurred at baseline and intermittently while treated with farnesylation inhibitors lonafarnib±pravastatin and zoledronate, within 3 sequential open-label clinical trials at Boston Children's Hospital totaling >10 years of treatment. An ultrasensitive single-molecule counting progerin immunoassay was developed with prespecified performance parameters. Intra- and interpatient group statistics were descriptive. The relationship between progerin and survival was assessed by using joint modeling with time-dependent slopes parameterization. RESULTS: The assay's dynamic detection range was 59 to 30 000 pg/mL (R2=0.9987). There was no lamin A cross-reactivity. Mean plasma progerin in non-HGPS participants (n=69; 39 male, 30 female; age, 0.2-71.3 years) was 351±251 pg/mL, and in drug-naive participants with HGPS (n=74; 37 female, 37 male; age, 2.1-17.5 years) was 33 261±12 346 pg/mL, reflecting a 95-fold increase in affected children (P<0.0001). Progerin levels did not differ by sex (P=0.99). Lonafarnib treatment resulted in an average per-visit progerin decrease from baseline of between 35% to 62% (all P<0.005); effects were not augmented by adding pravastatin and zoledronate. Progerin levels fell within 4 months of therapy and remained lower for up to 10 years. The magnitude of progerin decrease positively associated with patient survival (P<0.0001; ie, 15 000 pg/mL decrease yields a 63.9% decreased risk of death). For any given decrease in progerin, life expectancy incrementally increased with longer treatment duration. CONCLUSIONS: A sensitive, quantitative immunoassay for progerin was developed and used to demonstrate high progerin levels in HGPS plasma that decreased with lonafarnib therapy. The extent of improved survival was associated with both the magnitude of progerin decrease and duration at lower levels. Thus, plasma progerin is a biomarker for HGPS whose reduction enables short- and long-term assessment of progerin-targeted treatment efficacy. REGISTRATION: URL: https://www. CLINICALTRIALS: gov. Unique identifiers: NCT00879034 and NCT00916747.


Subject(s)
Progeria , Child , Humans , Male , Female , Infant , Child, Preschool , Adolescent , Young Adult , Adult , Middle Aged , Aged , Progeria/diagnosis , Progeria/drug therapy , Progeria/metabolism , Zoledronic Acid/therapeutic use , Pravastatin/therapeutic use , Piperidines/therapeutic use , Lamin Type A/metabolism
8.
Resusc Plus ; 13: 100355, 2023 Mar.
Article in English | MEDLINE | ID: mdl-36686322

ABSTRACT

Aim: To explore perspectives of families in the pediatric intensive care unit (PICU) about an emergency interventional trial on peri-arrest bolus epinephrine for acute hypotension using Exception From Informed Consent (EFIC). Methods: We performed face-to-face interviews with families whose children were hospitalized in the PICU. A research team member provided an educational presentation about the planned trial and administered a survey with open- and closed-ended items. Analyses included descriptive statistics for quantitative data and thematic analysis for qualitative data. Results: Sixty-seven participants contributed to 60 survey responses (53 individuals and 7 families for whom 2 family members participated). Most participants answered favorably toward the planned trial: 55/58 (95%) reported that the trial seemed "somewhat" or "very important"; 52/57 (91%) felt the use of EFIC was "somewhat" or "completely acceptable"; and 43/58 (74%) said they would be "somewhat" or "very likely" to allow their child to participate. Five themes emerged supporting participation in the planned trial: 1) trust in the clinical team; 2) familiarity with the study intervention (epinephrine); 3) study protocol being similar to standard care; 4) informed consent during an emergency was not feasible; and 5) importance of research. Barriers to potential participation included requests for additional time to decide about participating and misconceptions about study elements, especially eligibility. Conclusions: Families of PICU patients generally supported plans for an emergency interventional trial using EFIC. Future inpatient EFIC studies may benefit from highlighting the themes identified here in their educational materials.

9.
Phys Occup Ther Pediatr ; 43(4): 482-501, 2023.
Article in English | MEDLINE | ID: mdl-36628480

ABSTRACT

AIMS: Limited information is available on impairments, activity limitations and participation restrictions in youth with Hutchinson-Gilford progeria syndrome (HGPS), a rare genetic premature aging disease. The purposes were to: (1) describe range of motion (ROM), grip, pinch and quadriceps strength, functional balance, walking endurance, and gross motor limitations and participation restrictions; (2) evaluate the association between ROM impairments and age; and (3) evaluate the association between the Gross Motor Function Measure-88 (GMFM) scores and lower extremity (LE) ROM, quadriceps strength, and age. METHODS: Upper and LE ROM, grip, pinch and quadriceps strength, Timed Up and Go (TUG), Six Minute Walk Test, GMFM-88, and Canadian Occupational Performance Measure data were recorded for 38 participants with HGPS. RESULTS: All youth exhibited ROM impairments and most displayed decreased grip and pinch strength, walking endurance, and gross motor skills when compared to same-aged peers. However, the majority had good functional balance with TUG scores in the normal range. Participation restrictions included difficulty keeping up with peers when walking and difficulty completing activities of daily living. Some ROM measurements were negatively associated with age indicating that older participants had more extensive ROM limitation than younger participants. CONCLUSIONS: Physical and occupational therapists can use this information when evaluating youth with HGPS, designing a plan of care, and providing treatment interventions.


Subject(s)
Progeria , Humans , Adolescent , Progeria/genetics , Activities of Daily Living , Canada , Walking , Range of Motion, Articular
10.
Geroscience ; 45(2): 1231-1236, 2023 04.
Article in English | MEDLINE | ID: mdl-35752705

ABSTRACT

Clonal hematopoiesis of indeterminate potential (CHIP), defined as the presence of somatic mutations in cancer-related genes in blood cells in the absence of hematological cancer, has recently emerged as an important risk factor for several age-related conditions, especially cardiovascular disease. CHIP is strongly associated with normal aging, but its role in premature aging syndromes is unknown. Hutchinson-Gilford progeria syndrome (HGPS) is an ultra-rare genetic condition driven by the accumulation of a truncated form of the lamin A protein called progerin. HGPS patients exhibit several features of accelerated aging and typically die from cardiovascular complications in their early teens. Previous studies have shown normal hematological parameters in HGPS patients, except for elevated platelets, and low levels of lamin A expression in hematopoietic cells relative to other cell types in solid tissues, but the prevalence of CHIP in HGPS remains unexplored. To investigate the potential role of CHIP in HGPS, we performed high-sensitivity targeted sequencing of CHIP-related genes in blood DNA samples from a cohort of 47 HGPS patients. As a control, the same sequencing strategy was applied to blood DNA samples from middle-aged and elderly individuals, expected to exhibit a biological age and cardiovascular risk profile similar to HGPS patients. We found that CHIP is not prevalent in HGPS patients, in marked contrast to our observations in individuals who age normally. Thus, our study unveils a major difference between HGPS and normal aging and provides conclusive evidence that CHIP is not frequent in HGPS and, therefore, is unlikely to contribute to the pathophysiology of this accelerated aging syndrome.


Subject(s)
Cardiovascular Diseases , Progeria , Humans , Middle Aged , Aged , Adolescent , Progeria/genetics , Clonal Hematopoiesis , Lamin Type A/genetics , Aging/genetics , Aging/metabolism
11.
Pediatr Crit Care Med ; 24(1): 17-24, 2023 01 01.
Article in English | MEDLINE | ID: mdl-36516345

ABSTRACT

OBJECTIVES: Differences between adult and pediatric in-hospital cardiac arrest (IHCA) are well-described. Although most adults are cared for on adult services, pediatric services often admit adults, particularly those with chronic conditions. The objective of this study is to describe IHCA in adults admitted to pediatric services. DESIGN: Retrospective cohort analysis from the American Heart Association's Get With The Guidelines-Resuscitation registry of a subpopulation of adults with IHCA while admitted to pediatric services. Multivariable logistic regression was used to evaluate adjusted survival outcomes and compare outcomes between age groups (18-21, 22-25, and ≥26 yr old). SETTING: Hospitals contributing to the Get With The Guidelines-Resuscitation registry. PATIENTS: Adult-aged patients (≥ 18 yr) with an index pulseless IHCA while admitted to a pediatric service from 2000 to 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 491 adult IHCAs were recorded on pediatric services at 17 sites, during the 19 years of review, and these events represented 0.1% of all adult IHCAs. In total, 221 cases met inclusion criteria with 139 events excluded due to an initial rhythm of bradycardia with poor perfusion. Median patient age was 22 years (interquartile range, 19-28 yr). Ninety-eight percent of patients had at least one pre-existing condition. Return of spontaneous circulation occurred in 63% of events and 30% of the patients survived to discharge. All age groups had similar rates of survival to discharge (range 26-37%; p = 0.37), and survival did not change over the study period (range 26-37%; p = 0.23 for adjusted survival to discharge). CONCLUSIONS: In this cohort of adults with IHCA while admitted to a pediatric service, we failed to find an association between survival outcomes and age. Additional research is needed to better understand resuscitation in this population.


Subject(s)
Cardiopulmonary Resuscitation , Heart Arrest , Child , Humans , Adult , United States/epidemiology , Aged , Young Adult , Retrospective Studies , American Heart Association , Resuscitation , Registries , Hospitals, Pediatric
12.
Pediatr Emerg Care ; 39(1): e15-e19, 2023 Jan 01.
Article in English | MEDLINE | ID: mdl-35470292

ABSTRACT

OBJECTIVE: This study aimed to describe baseline and event characteristics and outcomes for adult patients who experience in-hospital cardiac arrest (IHCA) in a quaternary children's hospital and compare IHCA outcomes in younger (18-24 years) versus older (≥25 years) adults. We hypothesized that the rate of survival to hospital discharge would be lower in the older adult group. METHODS: We performed a retrospective single-center cohort study of inpatient areas of a quaternary children's center. Adult patients (≥18 years of age) with an index pulseless IHCA requiring at least 1 minute of cardiopulmonary resuscitation or defibrillation were included. RESULTS: Thirty-three events met the inclusion criteria with a median patient age of 23.9 years (interquartile range, 20.2-33.3 years). Twenty-one (64%) patients had congenital heart disease, and 25 (76%) patients had comorbidities involving ≥2 organ systems. The most common prearrest interventions were invasive mechanical ventilation (76%) and vasoactive infusions (55%). Seventeen patients (52%) survived to hospital discharge.Survival to discharge was lower in patients 25 years or older compared with patients aged 18 to 24 years old (3 of 15 [20%] vs 14 of 18 [78%], respectively; P = 0.002). CONCLUSIONS: The majority of adult patients with IHCA in our pediatric hospital had preexisting multisystem comorbidities, the most common of which was congenital heart disease. Overall survival to discharge after IHCA was 52%, similar to that reported for the general pediatric population. Survival to discharge was significantly lower in the subgroup of patients 25 years or older when compared with those between the ages of 18 and 24 years.


Subject(s)
Cardiopulmonary Resuscitation , Heart Arrest , Humans , Child , Aged , Adolescent , Young Adult , Adult , Cohort Studies , Retrospective Studies , Heart Arrest/epidemiology , Heart Arrest/therapy , Hospitals
13.
Pediatr Crit Care Med ; 23(11): 860-871, 2022 11 01.
Article in English | MEDLINE | ID: mdl-35894607

ABSTRACT

OBJECTIVES: IV calcium administration during cardiopulmonary resuscitation (CPR) for pediatric in-hospital cardiac arrest (IHCA) is associated with worse survival. We evaluated survival to hospital discharge in children with heart disease (HD), where calcium is more frequently administered during CPR. DESIGN: Retrospective study of a multicenter registry database. SETTING: Data reported to the American Heart Association's (AHA) Get With The Guidelines-Resuscitation registry. PATIENTS: Children younger than 18 years with HD experiencing an index IHCA event requiring CPR between January 2000 and January 2019. Using propensity score matching (PSM), we selected matched cohorts of children receiving and not receiving IV calcium during CPR and compared the primary outcome of survival to hospital discharge. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We included 4,556 children with HD experiencing IHCA. Calcium was administered in 1,986 (44%), more frequently in children younger than 1 year old (65% vs 35%; p < 0.001) and surgical cardiac (SC) compared with medical cardiac patients (51% vs 36%; p < 0.001). Calcium administration during CPR was associated with longer duration CPR (median 27 min [interquartile range (IQR): 10-50 min] vs 5 min [IQR, 2-16 min]; p < 0.001) and more frequent extracorporeal-CPR deployment (25% vs 8%; p < 0.001). In the PSM cohort, those receiving calcium had decreased survival to hospital discharge (39% vs 46%; p = 0.02) compared with those not receiving calcium. In a subgroup analysis, decreased discharge survival was only seen in SC cohorts. CONCLUSIONS: Calcium administration during CPR for children with HD experiencing IHCA is common and is associated with worse survival. Administration of calcium during CPR in children with HD should be restricted to specific indications as recommended by the AHA CPR guidelines.


Subject(s)
Cardiopulmonary Resuscitation , Heart Arrest , Heart Diseases , Infant , Child , Humans , Calcium , Retrospective Studies , American Heart Association , Heart Arrest/therapy , Registries , Hospitals
14.
Hosp Pediatr ; 12(4): 346-352, 2022 04 01.
Article in English | MEDLINE | ID: mdl-35291016

ABSTRACT

OBJECTIVES: Hospital-based code blue (CB) teams are designed for hospitalized patients (HP) with unanticipated medical emergencies outside of an ICU. At our freestanding pediatric institution, the same team responds to CB calls involving nonhospitalized persons (NHP) throughout the hospital campus. We hypothesized there are significant differences between the characteristics of NHP and HP requiring emergency medical response, and most responses for NHP do not require advanced critical care. METHODS: We analyzed a retrospective cohort of CB responses at our large, urban, academic children's medical center from January to December 2017. We evaluated the demographic and clinical characteristics of these HP compared with NHP events. RESULTS: There were 168 CB activations during the study, of which 135 (80.4%) were for NHP. Ninety-one (67.4%) of the NHP responses involved adults (age >18 years) compared with 6 (18.2%) of the HP. Triggers for CB team activation for NHP were most frequently syncope (42.2%), seizure (10.3%), or fall (9.6%) compared with seizure (30.3%), hypoxia (27.3%), or anaphylaxis (12.1%) for HP. Critical interventions such as bag-mask ventilation and cardiopulmonary resuscitation were infrequently performed for either cohort. CONCLUSIONS: CB activations in our pediatric institution more often involve NHP than HP. NHP responses are more likely to involve adults and infrequently require advanced interventions. Use of a pediatric CB team for NHP events may be an unnecessary use of pediatric critical care resources. Future studies are warranted to evaluate the most effective team composition, training, and response system for NHP in a freestanding children's hospital.


Subject(s)
Cardiopulmonary Resuscitation , Hospital Rapid Response Team , Adolescent , Adult , Child , Critical Care , Hospitals, Pediatric , Humans , Retrospective Studies
15.
Pediatrics ; 2022 Mar 15.
Article in English | MEDLINE | ID: mdl-35288738

ABSTRACT

OBJECTIVES: Hospital-based code blue (CB) teams are designed for hospitalized patients (HP) with unanticipated medical emergencies outside of an ICU. At our freestanding pediatric institution, the same team responds to CB calls involving nonhospitalized persons (NHP) throughout the hospital campus. We hypothesized there are significant differences between the characteristics of NHP and HP requiring emergency medical response, and most responses for NHP do not require advanced critical care. METHODS: We analyzed a retrospective cohort of CB responses at our large, urban, academic children's medical center from January to December 2017. We evaluated the demographic and clinical characteristics of these HP compared with NHP events. RESULTS: There were 168 CB activations during the study, of which 135 (80.4%) were for NHP. Ninety-one (67.4%) of the NHP responses involved adults (age >18 years) compared with 6 (18.2%) of the HP. Triggers for CB team activation for NHP were most frequently syncope (42.2%), seizure (10.3%), or fall (9.6%) compared with seizure (30.3%), hypoxia (27.3%), or anaphylaxis (12.1%) for HP. Critical interventions such as bag-mask ventilation and cardiopulmonary resuscitation were infrequently performed for either cohort. CONCLUSIONS: CB activations in our pediatric institution more often involve NHP than HP. NHP responses are more likely to involve adults and infrequently require advanced interventions. Use of a pediatric CB team for NHP events may be an unnecessary use of pediatric critical care resources. Future studies are warranted to evaluate the most effective team composition, training, and response system for NHP in a freestanding children's hospital.

16.
Pediatr Crit Care Med ; 23(5): 353-360, 2022 05 01.
Article in English | MEDLINE | ID: mdl-34991138

ABSTRACT

OBJECTIVES: To develop and implement clinical practice guidelines for safely weaning dexmedetomidine infusions in non-ICU areas. DESIGN: Development, implementation, and analysis of effectiveness of clinical practice guidelines. SETTING: Quaternary care academic free-standing pediatric hospital. PATIENTS: Children, otherwise medically ready for transfer to non-ICU areas, who were undergoing a planned wean of a dexmedetomidine infusion. INTERVENTIONS: Subject matter experts developed evidence-based guidelines for weaning dexmedetomidine in patients whose critical phase of illness had resolved. MEASUREMENTS AND MAIN RESULTS: Searches identified no prospective studies of dexmedetomidine weaning. We identified two retrospective reviews of withdrawal symptoms and one on the use of clonidine. There were case studies on withdrawal symptoms. Guidelines were piloted on a cohort of 24 patients while in the ICU. The guidelines were then implemented in non-ICU areas for patients undergoing dexmedetomidine weaning after ICU transfer. Over a 2-year period (October 1, 2018, to September 30, 2020), 63 patients (1 mo to 18 yr old) successfully weaned dexmedetomidine in non-ICU areas. The median time to discontinuation of dexmedetomidine after transfer to non-ICU areas was 5.8 days (interquartile range, 4.75-15 d). Fifty-eight percent (n = 41) of all patients were considered high risk for dexmedetomidine withdrawal based on the dose, duration of exposure, and the risk of experiencing physiologic detriment with more than mild withdrawal. Twenty-nine patients (46%) exhibited no signs or symptoms of withdrawal while weaning per guidelines. For those with signs and symptoms of withdrawal, the most common were tachycardia (n = 26, 40%), agitation (n = 9, 14%), and hypertension (n = 9, 11%). CONCLUSIONS: Weaning dexmedetomidine in non-ICU areas is feasible and can be accomplished safely even among pediatric patients at high risk for withdrawal using standardized weaning guidelines. At our institution, implementation was associated with reduced ICU length of stay for patients recovering from critical illness.


Subject(s)
Dexmedetomidine , Substance Withdrawal Syndrome , Child , Critical Illness , Dexmedetomidine/therapeutic use , Humans , Hypnotics and Sedatives/therapeutic use , Retrospective Studies , Weaning
17.
Resusc Plus ; 9: 100200, 2022 Mar.
Article in English | MEDLINE | ID: mdl-35072126

ABSTRACT

AIM: To describe current practices of peri-arrest bolus epinephrine use amongst pediatric resuscitation experts in a multinational survey. METHODS: A 9-question survey was developed and electronically distributed to pediatric critical care physicians who are site investigators for the Pediatric Resuscitation Quality Collaborative (pediRES-Q) network. Institutional demographics were collected through the American Hospital Association 2018 Annual Survey and linked to responses. Descriptive statistics were used to characterize closed-ended responses, and qualitative content analysis to analyze open-ended responses. RESULTS: Of the 63 collaborative members invited to participate, 49 (78%) responded, representing 35 institutions in 9 countries. Forty-six of the 49 respondents (94%) reported that they would consider using peri-arrest bolus epinephrine during critical situations in patients not requiring cardiopulmonary resuscitation. Initial dosing strategies ranged from 0.1mcg/kg to 10mcg/kg, with the most commonly reported initial dose of 1mcg/kg by 25 of the 37 (68%) respondents who answered this question. Three of the 49 (6%) participants indicated that they would generally avoid using peri-arrest bolus epinephrine, citing lack of evidence to support its use. CONCLUSIONS: In this multinational survey of pediatric resuscitation experts, endorsement of peri-arrest bolus epinephrine use was nearly universal, though a few clinicians cited lack of evidence to support this practice. There was a 100-fold difference in the range of initial weight-based doses reported, as well as a minority of clinicians who reported using non-weight-based dosing. Further research is needed to determine best practices, standardization of initial dosing, clinical factors that may warrant dosing modifications and associations with clinically important outcomes.

18.
Chest ; 161(3): 791-802, 2022 03.
Article in English | MEDLINE | ID: mdl-34587483

ABSTRACT

Severe forms of pulmonary embolism (PE) in children, althought rare, cause significant morbidity and mortality. We review the pathophysiologic features of severe (high-risk and intermediate-risk) PE and suggest novel pediatric-specific risk stratifications and an acute treatment algorithm to expedite emergent decision-making. We defined pediatric high-risk PE as causing cardiopulmonary arrest, sustained hypotension, or normotension with signs or symptoms of shock. Rapid primary reperfusion should be pursued with either surgical embolectomy or systemic thrombolysis in conjunction with a heparin infusion and supportive care as appropriate. We defined pediatric intermediate-risk PE as a lack of systemic hypotension or compensated shock, but with evidence of right ventricular strain by imaging, myocardial necrosis by elevated cardiac troponin levels, or both. The decision to pursue primary reperfusion in this group is complex and should be reserved for patients with more severe disease; anticoagulation alone also may be appropriate in these patients. If primary reperfusion is pursued, catheter-based therapies may be beneficial. Acute management of severe PE in children may include systemic thrombolysis, surgical embolectomy, catheter-based therapies, or anticoagulation alone and may depend on patient and institutional factors. Pediatric emergency and intensive care physicians should be familiar with the risks and benefits of each therapy to expedite care. PE response teams also may have added benefit in streamlining care during these critical events.


Subject(s)
Pulmonary Embolism , Thrombolytic Therapy , Acute Disease , Child , Embolectomy/methods , Humans , Pulmonary Embolism/diagnosis , Pulmonary Embolism/therapy , Risk Factors , Thrombolytic Therapy/methods , Treatment Outcome
19.
Pediatr Qual Saf ; 6(2): e389, 2021.
Article in English | MEDLINE | ID: mdl-34963999

ABSTRACT

We aimed to describe utilization and indication(s) for long-term central venous catheters (CVCs) in a pediatric intensive care unit (PICU) and identify potential strategies to decrease CVC utilization. METHODS: We conducted a single-center prospective quality improvement initiative at a 30-bed PICU in a large, freestanding, academic children's hospital. We created an electronic report to identify patients with an indwelling CVC for 7 days and older (defined as long term). We discussed the ongoing need for each long-term CVC with PICU clinicians at weekly interdisciplinary structured "CVC stewardship rounds." We then made recommendations around expedited removal of CVCs. We conducted multiple Plan-Do-Study-Act cycles to categorize CVC indications, identify modifiable factors, and educate PICU clinicians. We hypothesized that CVC stewardship rounds would decrease long-term CVC utilization in our PICU. RESULTS: From October 2016 to September 2017, 607 long-term CVCs were eligible for the stewardship intervention. Compared to the preintervention period, we recorded a significant increase in peripherally inserted central catheters and a decrease in nontunneled CVCs (P < 0.001). Most patients had single- or double-lumen CVCs in both the preintervention and intervention periods (86% and 91%, respectively). The utilization of overall long-term CVC devices, and those with modifiable indications, decreased during the intervention period. CONCLUSIONS: A single-center QI intervention focused on PICU CVC stewardship was associated with a decrease in CVC utilization.

20.
Pediatr Crit Care Med ; 22(10): 898-905, 2021 10 01.
Article in English | MEDLINE | ID: mdl-33935271

ABSTRACT

OBJECTIVES: Design, implement, and evaluate a rounding checklist with deeply embedded, dynamic electronic health record integration. DESIGN: Before-after quality-improvement study. SETTING: Quaternary PICU in an academic, free-standing children's hospital. PATIENTS: All patients in the PICU during daily morning rounds. INTERVENTIONS: Implementation of an updated dynamic checklist (eSIMPLER) providing clinical decision support prompts with display of relevant data automatically pulled from the electronic health record. MEASUREMENTS AND MAIN RESULTS: The prior daily rounding checklist, eSIMPLE, was implemented for 49,709 patient-days (7,779 patients) between October 30, 2011, and October 7, 2018. eSIMPLER was implemented for 5,306 patient-days (971 patients) over 6 months. Checklist completion rates were similar (eSIMPLE: 95% [95% CI, 88-98%] vs eSIMPLER: 98% [95% CI, 92-100%] of patient-days; p = 0.40). eSIMPLER required less time per patient (28 ± 1 vs 47 ± 24 s; p < 0.001). Users reported improved satisfaction with eSIMPLER (p = 0.009). Several checklist-driven process measures-discordance between electronic health record orders for stress ulcer prophylaxis and user-recorded indication for stress ulcer prophylaxis, rate of venous thromboembolism prophylaxis prescribing, and recognition of reduced renal function-improved during the eSIMPLER phase. CONCLUSIONS: eSIMPLER, a dynamic, electronic health record-informed checklist, required less time to complete and improved certain care processes compared with a prior, static checklist with limited electronic health record data. By focusing on the "Five Rights" of clinical decision support, we created a well-accepted clinical decision support tool that was integrated efficiently into daily rounds. Generalizability of eSIMPLER's effectiveness and its impact on patient outcomes need to be examined.


Subject(s)
Decision Support Systems, Clinical , Teaching Rounds , Checklist , Child , Electronic Health Records , Humans , Intensive Care Units, Pediatric
SELECTION OF CITATIONS
SEARCH DETAIL
...