Feasibility and Acceptability of a Reiki Intervention With Very Young Children Receiving Palliative Care.
BACKGROUND: Very little research has been reported examining nonpharmacologic symptom management strategies for very young, hospitalized children receiving palliative care, and none has involved Reiki-a light touch therapy. OBJECTIVES: To determine if completing a Reiki intervention with hospitalized 1- to 5-year-old children with chronic, life-limiting conditions receiving palliative care was feasible and acceptable. METHODS: Children ages 1 to 5 years receiving palliative care who were expected to be hospitalized for at least 3 weeks were recruited for a single-arm, mixed-methods, quasi-experimental pre/poststudy. Six protocolized Reiki sessions were conducted over 3 weeks. We calculated feasibility by the percentage of families enrolled in the study and acceptability by the percentage of families who completed all measures and 5 out of 6 Reiki sessions. Measures were collected at baseline, the end of the intervention period, and three weeks later. At the final follow-up visit, parents were verbally asked questions relating to the acceptability of the intervention in a short structured interview. RESULTS: We screened 90 families, approached 31 families, and recruited 16 families while 15 families declined. Reasons for not participating included that the child had "a lot going on," would be discharged soon, and families were overwhelmed. Of those enrolled, most completed all measures at three time points and 5 out of 6 Reiki sessions. We completed nearly all scheduled Reiki sessions for families that finished the study. All parents reported that they would continue the Reiki if they could, and almost all said they would participate in the study again; only one parent was unsure. DISCUSSION: Young children and their parents found Reiki acceptable; these results are comparable to an earlier study of children 7-16 years receiving palliative care at home and a study of massage for symptom management for hospitalized children with cancer. These findings add to the literature and support further investigation of Reiki's efficacy as a nonpharmacologic symptom management intervention.
Thrane SE; Grossoehme DH; Tan A; Shaner V; Friebert S
Nursing Research
2021
2021-07-12
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1097/NNR.0000000000000540" target="_blank" rel="noreferrer noopener">10.1097/NNR.0000000000000540</a>
End of Life Outside of "Business Hours": A Retrospective Review Evaluating Time of Death and Provider Time at End of Life.
palliative care; pediatric; physician; compassion fatigue; advance practice nurse; OF-LIFE; PEDIATRIC PALLIATIVE CARE
Introduction: Pediatric palliative care (PPC) benefits patients and families, while potentially creating emotional and resource-management burdens for providers. This study's purpose was to characterize the occurrence of deaths attended by PPC providers outside of "business hours." Methods: N = 786 PPC patients at a single center died between 2008 and 2015. Descriptive statistics were prepared for all variables (Wilcoxon rank-sum test for continuous; chi-square or Fisher's exact test for categorical). Results: N = 434 (55%) of deaths occurred outside of business hours; n = 332 (70%) were attended by PPC. Time spent attending a death was not significantly longer when other PPC providers were present but was when certain tasks were performed (coordination with medical examiner and memory making). Conclusion: The occurrence of the majority of deaths outside of business hours has significant implications for service delivery models, provider emotional health, and health care value.
Hardy-Gomez M; Grossoehme DH; Strasshofer D; Brown M; Friebert S
Journal Of Palliative Medicine
2021
2021-07-01
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journalArticle
<a href="http://doi.org/10.1089/jpm.2021.0127" target="_blank" rel="noreferrer noopener">10.1089/jpm.2021.0127</a>
Acute Tramadol Ingestion With Transient Acute Kidney Injury in an Adolescent Female.
acute kidney injury; DRUG side effects; adolescent; tramadol; ACUTE kidney failure; case report; adverse drug effect; analgesics; drug overdose; RHABDOMYOLYSIS; STRIATED muscle necrosis; TRAMADOL
Renal toxicity has been described with tramadol overdoses; however, it is typically associated with rhabdomyolysis, multiorgan failure and/or mortality. Our patient was a 16-year-old female who was evaluated following an intentional tramadol ingestion, estimated 27.8 to 37 mg/kg, and had a seizure prior to arriving at our health care facility. Her symptoms were consistent with a tramadol ingestion; however, she developed transient acute renal impairment (peak serum creatinine, 4.04 mg/dL), which improved over 6 days with minimal intervention. No other causes were identified to explain her acute renal impairment thus it was attributed to the tramadol overdose. Providers should be aware that transient acute renal impairment could occur with an intentional tramadol ingestion and may not require aggressive intervention. [ABSTRACT FROM AUTHOR]
Mike TB; DeVault H; Blackford MG
Journal Of Pediatric Pharmacology & Therapeutics
2021
2021-05
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.5863/1551-6776-26.4.411" target="_blank" rel="noreferrer noopener">10.5863/1551-6776-26.4.411</a>
Providers' Ability to Identify Sentinel Injuries Concerning for Physical Abuse in Infants.
Eismann EA; Shapiro RA; Thackeray J; Makoroff K; Bressler CJ; Kim GJ; Vavul-Roediger L; McPherson P; Izsak E; Spencer SP
Pediatric Emergency Care
2021
2021-05
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1097/PEC.0000000000001574" target="_blank" rel="noreferrer noopener">10.1097/PEC.0000000000001574</a>
A Novel Exercise Testing Algorithm to Diagnose Mitochondrial Myopathy.
Cardiopulmonary exercise testing; Mitochondrial myopathy; Oxygen uptake (V&c.dotab;O2); Oxygen Uptake Efficiency Slope (OUES); V&c.dotab;O2 /Work slope
INTRODUCTION: Oxygen uptake efficiency slope (OUES) is a non-invasive cardiopulmonary exercise testing (CPET) measurement based on oxygen uptake (V&c.dotab;O(2) ) and minute ventilation (V&c.dotab;(E) ) and is a marker of the efficiency of oxygen utilization by the body. However, it has not been studied in mitochondrial disorders. We explored noninvasive CPET parameters including OUES as a way to reliably diagnose mitochondrial myopathy. METHODS: We performed cycle ergometer maximal exercise testing on definite and suspected mitochondrial myopathy subjects (MM-D & MM-S) and their age and sex matched controls. OUES was corrected for body surface area (OUES/BSA) to eliminate the effect of body size. RESULTS: 40 participants [20 mitochondrial myopathy subjects {13 MM-D (6 males; aged 14-64 years) / 7 MM-S (5 males, aged 11-30 years} and 20 controls] completed the study. MM-D subjects showed lower aerobic fitness than controls. OUES/BSA was lower in MM-D subjects suggesting inefficient oxygen utilization. Area under the curve (AUC) and 95% Confidence Interval (CI) for OUES/BSA [0.91 (0.80, 1.00)], Peak V&c.dotab;O(2) % predicted [0.95 (0.86, 1.00)] and V&c.dotab;O(2) /Work slope [0.94 (0.85, 1.00)] showed excellent ability to diagnose mitochondrial myopathy in MM-D subjects. We applied a diagnostic approach based on the above parameters to MM-S subjects and their controls and were able to support or disprove the diagnosis of mitochondrial myopathy. DISCUSSION: We proposed and applied an approach based on the above three CPET parameters to reliably diagnose mitochondrial myopathy and found it to be clinically useful. This article is protected by copyright. All rights reserved.
Bhatia Rajeev;Cohen BH;McNinch N
Muscle & Nerve
2021
2021-02-03
journalArticle
<a href="http://doi.org/10.1002/mus.27191" target="_blank" rel="noreferrer noopener">10.1002/mus.27191</a>
Prioritization of Pediatric Palliative Care Field-Advancement Activities in the United States: Results of a National Survey.
curriculum; financing; funding; palliative care; pediatric; quality of health care
BACKGROUND: The field of pediatric palliative care (PPC) continues to encounter challenges and opportunities to improving access to high-quality PPC services. In early 2019, a workshop identified eleven potential "next step" actions, and subsequently a national survey-based poll of members of the PPC community was conducted to prioritize these potential actions in terms of their "actionable importance." METHODS: Invitations to the survey were distributed in October 2019 to interdisciplinary PPC health care professionals via email to two major listservs, one hosted by the Section of Hospice and Palliative Medicine of the American Academy of Pediatrics, the other by the Center to Advance Palliative Care. Respondents rated the "actionable importance" of items relative to each other via a discrete choice experiment. Median importance scores are reported for each item. RESULTS: 177 individuals responded to the survey. The majority (62.2%) were physicians, with nurses (16.4%), advanced practice nurses (7.9%), and social workers (7.3%) being the other most common responders. The top 5 potential actions, in descending rank order, were: Determine what parents value regarding PPC (median score of 17.8, out of a total score of all items of 100); Define and disseminate core primary PPC curriculum (median, 15.3); Develop PPC national representation strategy and tactics (median, 12.3); Create PPC-specific program development toolkit (median, 10.9); and, Analyze payment and financing ratios (median, 9.6). CONCLUSIONS: Those seeking to advance the field of PPC should take into account the findings from this study, which suggest that certain actions are more likely to have a beneficial impact on moving the field forward.
Feudtner Chris;Faerber JA;Rosenberg AR;Kobler K;Baker JN;Bowman BA;Wolfe J;Friebert S
Journal Of Pain And Symptom Management
2021
2021-01-21
journalArticle
<a href="http://doi.org/10.1016/j.jpainsymman.2021.01.007" target="_blank" rel="noreferrer noopener">10.1016/j.jpainsymman.2021.01.007</a>
Acute kidney injury associated with urinary stone disease in children and young adults presenting to a pediatric emergency department.
AKI; kidney stones; pediatric; urinary stone disease (USD); urolithiasis
Background: Acute kidney injury (AKI) due to urinary stone disease (USD) is rare in adults; AKI rates in children with USD may be higher, and emerging data links stones to chronic kidney disease (CKD) development in adults. Methods: This study is a retrospective analysis of USD patients at a single pediatric hospital system's emergency department (ED). Patients were initially identified by USD ICD codes; USD was then confirmed by imaging or physician documentation; patients had to have baseline creatinine (Cr) and Cr in the ED for comparison to be included. AKI was defined by Kidney Disease: Improving Global Outcomes (KDIGO), Acute Kidney Injury Network (AKIN), and Pediatric Risk, Injury, Failure, Loss, End Stage (pRIFLE). Results: Of the 589 total visits, 264/589 (45%) had data to evaluate for AKI, 23% were AKI(+) and 77% were AKI(-). pRIFLE was most common (82%) and 18% were only positive by AKIN/KDIGO. AKI(+) were more likely to be younger (16.7 vs. 17.4 years, p = 0.046) and more likely to present with vomiting {odds ratio [OR] [95% confidence interval (CI)]: 2.4 [1.4-4.3], p = 0.002}; also, the proportion of AKI(+) was significantly higher in <18 vs. ≥18 years [26.9 vs. 15.5%, p = 0.032, OR (95% CI): 2.0 (1.1-3.9)]. Urinary tract infection (UTI) and obstruction rates were similar between groups. AKI(+) patients had a significant OR <1 suggesting less risk of receiving non-steroidal anti-inflammatory drugs (NSAIDs); however, 51% of them did receive NSAIDs during their ED encounter. AKI(+) patients were more likely to require admission to the hospital (53 vs. 32%, p = 0.001). Conclusion: We have demonstrated a novel association between USD-induced renal colic and AKI in a group of young adults and children. AKI(+) patients were younger and were more likely to present with vomiting. AKI(+) patients did not have higher rates of obstruction or UTI, and 51% of AKI(+) received NSAIDs.
Farris N; Raina R; Tibrewal A; Brown M; Colvis M; Schwaderer A; Kusumi K
Frontiers in Pediatrics
2020
1905-07
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journalArticle
<a href="http://doi.org/10.3389/fped.2020.591520" target="_blank" rel="noreferrer noopener">10.3389/fped.2020.591520</a>
Promoting bone health in children and adolescents following solid organ transplantation.
bisphosphonates; bone; calcium; magnesium; metabolic bone disease; phosphorous; physical activity; solid organ transplant; vitamin D
Solid organ transplantation in children and adolescents provides many benefits through improving critical organ function, including better growth, development, cardiovascular status, and quality of life. Unfortunately, bone status may be adversely affected even when overall status is improving, due to issues with pre-existing bone disease as well as medications and nutritional challenges inherent post-transplantation. For all children and adolescents, bone status entering adulthood is a critical determinant of bone health through adulthood. The overall health and bone status of transplant recipients benefits from attention to regular physical activity, good nutrition, adequate calcium, phosphorous, magnesium and vitamin D intake and avoidance/minimization of soda, extra sodium, and obesity. Many immunosuppressive agents, especially glucocorticoids, can adversely affect bone function and development. Minimizing exposure to "bone-toxic" medications is an important part of promoting bone health in children post-transplantation. Existing guidelines detail how regular monitoring of bone status and biochemical markers can help detect bone abnormalities early and facilitate valuable bone-directed interventions. Attention to calcium and vitamin D supplementation, as well as tapering and withdrawing glucocorticoids as early as possible after transplant, can provide best bone outcomes for these children. Dual-energy X-ray absorptiometry can be useful to detect abnormal bone mass and fracture risk in this population and newer bone assessment methods are being evaluated in children at risk for poor bone outcomes. Newer bone therapies being explored in adults with transplants, particularly bisphosphonates and the RANKL inhibitor denosumab, may offer promise for children with low bone mass post-transplantation.
Kusumi K; Shaikhkhalil A; Patel HP; Mahan John D
Pediatric Transplantation
2020
2020-12-19
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1111/petr.13940" target="_blank" rel="noreferrer noopener">10.1111/petr.13940</a>
Epidemiology of brainstem high-grade gliomas in children and adolescents in the united states, 2000-2017.
Brainstem; CBTRUS; DIPG; Glioma; High-Grade Glioma
BACKGROUND: Limited population-based data exists for the brainstem gliomas for children ages ≤19 years, which includes high-grade aggressively-growing tumors such as diffuse intrinsic pontine glioma (DIPG). We examined the overall incidence and survival patterns in children with brainstem High-Grade glioma (HGG) by age, sex, and race and ethnicity. METHODS: We used data from Central Brain Tumor Registry of the United States (CBTRUS), obtained through data use agreements with the Centers for Disease Control (CDC) and the National Cancer Institute (NCI) from 2000 - 2017, and survival data from the CDC's National Program of Cancer Registries (NPCR), from 2001 - 2016 for malignant brainstem HGG for ages ≤19 years (per WHO ICD-O-3 codes). HGG was determined by established histologic and/or imaging criteria. Age-adjusted incidence rates and survival data were used to assess differences overall and by age, sex race, and ethnicity. RESULTS: The incidence of brainstem HGG was higher among the female and Non-Hispanic population. Majority (69.8%) of these tumors were diagnosed radiographically. Incidence was higher in children aged 01-09 years compared to older children. Whites had a higher incidence compared to Blacks. However, the risk of death was higher among Blacks and Other race compared to Whites. There was no difference in survival by sex. CONCLUSIONS: We report the most comprehensive incidence and survival data on these lethal brainstem HGGs. Incidence and survival among patients with brainstem HGGs differed significantly by race, ethnicity, age-groups and grade.
Patil N; Kelly ME; Yeboa DN; Buerki RA; Cioffi G; Balaji S; Ostrom QT; Kruchko C; Barnholtz-Sloan J
Neuro-oncology
2020
2020-12-21
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1093/neuonc/noaa295" target="_blank" rel="noreferrer noopener">10.1093/neuonc/noaa295</a>
Use of telehealth in fellowship-affiliated developmental behavioral pediatric practices during the COVID-19 pandemic.
OBJECTIVE: This study aims to describe the use of telehealth in developmental behavioral pediatric (DBP) fellowship-affiliated practices during the coronavirus disease 2019 (COVID-19) global pandemic. METHODS: An electronic survey was disseminated to all DBP fellowship-associated practice locations to determine the use of telehealth in DBP care provision, before and since the beginning of the COVID-19 pandemic. We analyzed responses using descriptive statistics. RESULTS: A total of 35 of 42 eligible practice sites responded (83% response rate). Most sites (51.4%) reported using telehealth less than once per month before the COVID-19 pandemic. Since the onset of COVID-19, 100% of programs reported conducting video-based telehealth visits multiple days per week. Most sites reported conducting evaluations and follow-up visits for attention-deficit/hyperactivity disorder, autism spectrum disorder, behavioral concerns, developmental delay, genetic disorders, and learning disability. Most sites were able to continue medication management by telehealth (>88%), offer interpreter services for families with limited English proficiency participating in telehealth visits (>90%), and incorporate trainees and interdisciplinary team members in telehealth visits (>90%). Greater variability was observed in sites' ability to collect telehealth practice evaluation measures. CONCLUSION: Most sites are providing evaluations and ongoing care for DBP conditions through telehealth. The rapid adoption of telehealth can have ramifications for the way that DBP care is delivered in the future; therefore, it is imperative to understand current practice patterns and variations to determine the best use of telehealth.
Wallis KE; Mulé C; Mittal S; Cerda N; Shaffer R; Scott A; Langkamp D; Augustyn M; Perrin E; Soares N; Blum NJ
Journal of Developmental and Behavioral Pediatrics
2020
2020-12-21
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1097/DBP.0000000000000897" target="_blank" rel="noreferrer noopener">10.1097/DBP.0000000000000897</a>
Practical Challenges and Considerations for Early Introduction of Potential Food Allergens for Prevention of Food Allergy.
Guidelines; Prevention; Complementary feeding; Early introduction; Food allergy; Infant nutrition
Recent randomized controlled trials aimed at the prevention of food allergy have led to sweeping changes in food allergy prevention guidelines. Emphasis is now on the introduction of potential food allergens, particularly peanut and egg, rather than avoidance. Although guidelines recommend against delaying the introduction of other potential allergens, there remains little or no evidence of the benefit of their early introduction. Parents and physicians alike report a need for greater guidance and resources on early potential allergen introduction in the complementary feeding period. A thorough understanding of early introduction literature, current prevention guidelines, and infant nutrition will empower physicians to address patient needs and concerns both when advice is established as effective and where uncertainty remains. We discuss the state of the science, compare recommendations between guidelines, and provide practical options to introduce allergenic foods, alongside other complementary foods, within the first year of life. We include a review of the available literature, including review and suggestions of potential doses of food allergens, and the first published comparison of commercially available products and homemade early introduction foods to help clinicians support their patients. We address the nutritional, dietary, and practical considerations of introducing food allergens in the first year of life while adhering to infant feeding guidelines. Finally, given the limitations of existing guidelines, we review the need for shared decision-making between physicians and parents regarding early allergen introduction.
Schroer B; Groetch M; Mack DP; Venter C
The Journal Of Allergy And Clinical Immunology. In Practice
2020
2020-10-27
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journalArticle
<a href="http://doi.org/10.1016/j.jaip.2020.10.031" target="_blank" rel="noreferrer noopener">10.1016/j.jaip.2020.10.031</a>
Utility of specific amino acid ratios in screening for pyruvate dehydrogenase complex deficiencies and other mitochondrial disorders associated with congenital lactic acidosis and newborn screening prospects.
ketogenic diet; alanine; ketogenic amino acids; lactic acidosis; mitochondrial disorder; newborn screening; proline; pyruvate dehydrogenase complex deficiency
Pyruvate dehydrogenase complex deficiencies (PDCDs) and other mitochondrial disorders (MtDs) can (a) result in congenital lactic acidosis with elevations of blood alanine (Ala) and proline (Pro), (b) lead to decreased ATP production, and (c) result in high morbidity and mortality. With ~140,000 live births annually in Ohio and ~1 in 9,000 overall prevalence of MtDs, we estimate 2 to 3 newborns will have PDCD and 13 to 14 others likely will have another MtD annually. We compared the sensitivities of plasma amino acids (AA) Alanine (Ala), Alanine:Leucine (Ala:Leu), Alanine:Lysine and the combination of Ala:Leu and Proline:Leucine (Pro:Leu), in subjects with known primary-specific PDCD due to PDHA1 and PDHB mutations vs controls. Furthermore, in collaboration with the Ohio newborn screening (NBS) laboratory, we determined Ala and Pro concentrations in dried blood spot (DBS) specimens using existing NBS analytic approaches and evaluated Ala:Leu and Pro:Leu ratios from DBS specimens of 123,414 Ohio newborns in a 12-month period. We used the combined Ala:Leu ≥4.0 and Pro:Leu ≥3.0 ratio criterion from both DBS and plasma specimens as a screening tool in our retrospective review of newborn data. The screening tool applied on DBS and/or plasma (or serum) AA specimens successfully identified three unrelated females with novel de novo PDHA1 mutations, one male with a novel de novo X-linked HSD17B10 mutation, and a female with VARS2 mutations. This work lays the first step for piloting an NBS protocol in Ohio for identifying newborns at high risk for primary-specific PDCD and other MtDs who might benefit from neonatal diagnosis and early institution of known therapy and/or potential novel therapies for such disorders.
Bedoyan JK; Hage R; Shin HK; Linard S; Ferren E; Ducich N; Wilson K; Lehman A; Schillaci L; Manickam K; Mori Mari; Bartholomew D; DeBrosse S; Cohen B; Parikh S; Kerr D
Jimd Reports
2020
2020-11
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1002/jmd2.12153" target="_blank" rel="noreferrer noopener">10.1002/jmd2.12153</a>
Epidemiology of vestibular schwannoma in the United States, 2004-2016.
epidemiology; brain tumors; CBTRUS; vestibular schwannoma
BACKGROUND: Vestibular schwannomas (VS) are nonmalignant tumors of the eighth cranial nerve and are the most common nonmalignant nerve sheath tumor. This study provides the most comprehensive and current analysis of VS epidemiology in the United States. METHODS: Incidence data were obtained from the Central Brain Tumor Registry of the United States, from 2004 to 2016 for VS. Age-adjusted incidence rates (AAIRs), rate ratios (AAIRRs), and prevalence ratios (AAPRs) per 100 000 were analyzed by age, sex, race and ethnicity, and laterality. Additional analyses were performed to assess differences in treatment, laterality, and diagnostic confirmation. RESULTS: Incidence of VS was highest among adults (aged 65-74 years, AAIR: 3.18, 95% confidence interval [CI]: 3.15-3.25). However, there was a much higher distribution of bilateral tumors compared to unilateral in children aged 0-19 years (28.5% vs 1.0%, P < .001). VS incidence was highest among white non-Hispanics (AAIR:1.30, 95% CI: 1.29-1. 31) and lowest among black non-Hispanics. Incidence of radiographically confirmed VS increased from 2004 to 2016 (annual percent change: 1.64, 95% CI: 0.15-3.16, P = .03). For treatment, 40.1% received surgery, while only 23.7% received radiation. There were an estimated 44 762 prevalent cases of VS in 2016 (AAPR: 12.17, 95% CI: 12.06-12.29). CONCLUSIONS: VS incidence and prevalence are highest among adults and white non-Hispanics. Bilateral VS was more common among children. There was an increase of radiographically confirmed VS over time. A higher proportion of patients received surgical treatment than radiotherapy. Population-based statistics provide healthcare professionals with vital information regarding disease burden and help improve patient care.
Cioffi G; Yeboa DN; Kelly M; Patil N; Manzoor N; Greppin K; Takaoka K; Waite K; Kruchko C; Barnholtz-Sloan JS
Neuro-oncology Advances
2020
2020-12
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1093/noajnl/vdaa135" target="_blank" rel="noreferrer noopener">10.1093/noajnl/vdaa135</a>
Predictors of technology success in patients with cystic fibrosis-related diabetes
Jain A;Kaminski B;Kutney KA
Pediatric Pulmonology
2020
2020-10
journalArticle
<a href="http://doi.org/" target="_blank" rel="noreferrer noopener"></a>
Biochemical phenotype and its relationship to treatment in 16 individuals with PCCB c.1606A > G (p.Asn536Asp) variant propionic acidemia.
Treatment; Phenotype; Biomarker; Propionic acidemia
Propionic acidemia (PA) is caused by inherited deficiency of mitochondrial propionyl-CoA carboxylase (PCC) and results in significant neurodevelopmental and cardiac morbidity. However, relationships among therapeutic intervention, biochemical markers, and disease progression are poorly understood. Sixteen individuals homozygous for PCCB c.1606A > G (p.Asn536Asp) variant PA participated in a two-week suspension of therapy. Standard metabolic markers (plasma amino acids, blood spot methylcitrate, plasma/urine acylcarnitines, urine organic acids) were obtained before and after stopping treatment. These same markers were obtained in sixteen unaffected siblings. Echocardiography and electrocardiography were obtained from all subjects. We characterized the baseline biochemical phenotype of untreated PCCB c.1606A > G homozygotes and impact of treatment on PCC deficiency biomarkers. Therapeutic regimens varied widely. Suspension of therapy did not significantly alter branched chain amino acid levels, their alpha-ketoacid derivatives, or urine ketones. Carnitine supplementation significantly increased urine propionylcarnitine and its ratio to total carnitine. Methylcitrate blood spot and urine levels did not correlate with other biochemical measures or cardiac outcomes. Treatment of PCCB c.1606A > G homozygotes with protein restriction, prescription formula, and/or various dietary supplements has a limited effect on core biomarkers of PCC deficiency. These patients require further longitudinal study with standardized approaches to better understand the relationship between biomarkers and disease burden.
Wenger O;Brown M;Smith B;Chowdhury D;Crosby AH;Baple EL;Yoder M;Laxen W;Tortorelli S;Strauss KA
Molecular Genetics and Metabolism
2020
2020-10-03
journalArticle
<a href="http://doi.org/10.1016/j.ymgme.2020.09.006" target="_blank" rel="noreferrer noopener">10.1016/j.ymgme.2020.09.006</a>
Improving pediatric readiness in general emergency departments: A prospective interventional study.
emergency medicine; EMSC; facility recognition; Pediatric readiness
OBJECTIVE: To describe the impact of a national interventional collaborative on pediatric readiness within General Emergency Departments. STUDY DESIGN: A prospective, multicenter, interventional study measured pediatric readiness in general emergency departments before and after participation in a pediatric readiness improvement intervention. Pediatric readiness was assessed using the weighted pediatric readiness score (WPRS) on a 100-point scale. The study protocol extended over six months and involved three phases: 1) a baseline on-site assessment of pediatric readiness and simulated quality of care, 2) pediatric readiness interventions, and 3) a follow-up on-site assessment of WPRS. The intervention phase included a benchmarking performance report, resources toolkits, and ongoing interactions between general emergency departments and academic medical centers. RESULTS: Thirty-six general emergency departments were enrolled, and 34 (94%) completed the study. Four EDs (11%) were located in Canada, and the rest were in the U.S. The mean improvement in WPRS was 16.3 (p<0.001) from a baseline of 62.4 (SEM=2.2) to 78.7 (SEM=2.1), with significant improvement in the domains of administration/coordination of care; policies, protocol and procedures; and quality improvement. Six EDs (17%) were fully adherent to the protocol timeline. CONCLUSION: Implementing a collaborative intervention model including simulation and quality improvement initiatives is associated with improvement in weighted pediatric readiness scores (WPRS) when disseminated to a diverse group of general emergency departments partnering with their regional pediatric academic medical centers. This work provides evidence that innovative collaboration facilitated by academic medical centers can serve as an effective strategy to improve pediatric readiness and processes of care.
Abulebda K;Whitfill T;Montgomery EE;Thomas A;Dudas RA;Leung JS;Scherzer DJ;Aberesold M;Van IWL;Kant S;Walls TA;Sessa AK;Janofsky S;Fenster DB;Kessler DO;Chatfield J;Okada P;Arteaga G M;Berg MD;Knight LJ;Keilman A;Makharashvili A;Good G;Bingham L;Mathias EJ;Nagy K;Hamilton MF;Vora S;Mathias K;Auerbach M A
The Journal of Pediatrics
2020
2020-10-30
journalArticle
<a href="http://doi.org/10.1016/j.jpeds.2020.10.040" target="_blank" rel="noreferrer noopener">10.1016/j.jpeds.2020.10.040</a>
Using and interpreting electrodiagnostic tests.
Electrodiagnostic testing, consisting of nerve conduction studies and needle electrode examination, serves as an extension of a neurologic examination for evaluating a variety of focal and generalized neuromuscular conditions. By providing important clues on location, chronicity, severity, and pathophysiology, it can help to establish a diagnosis, evaluate the need for surgery, and assess patients who do not improve as expected after surgery.
Ginsberg MR;Morren JA;Levin K
Cleveland Clinic Journal of Medicine
2020
2020-11-02
journalArticle
<a href="http://doi.org/10.3949/ccjm.87a.19154" target="_blank" rel="noreferrer noopener">10.3949/ccjm.87a.19154</a>
Quality improvement in neurology: neurology outcomes quality measurement set.
Humans; Physician-Patient Relations; Quality of Life/psychology; Quality Improvement/standards; Nervous System Diseases/diagnosis/psychology/therapy; Neurology/methods/standards; Outcome Assessment Health Care/methods/standards; Quality Indicators Health Care/standards
Sico JJ;Sarwal A;Benish SM;Busis NA;Cohen BH;Das RR;Finsilver S;Halperin JJ;Kelly AG;Meunier L;Phipps MS;Thirumala PD;Villanueva R;von Gaudecker J;Bennett A;Shenoy AM
Neurology
2020
2020-06-02
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1212/wnl.0000000000009525" target="_blank" rel="noreferrer noopener">10.1212/wnl.0000000000009525</a>
"Living life as if i never had cancer": A study of the meaning of living well in adolescents and young adults who have experienced cancer
childhood; definition; quality of life; advance care planning; cancer; adolescent; advance care
Background Cancer diagnoses pose challenges to adolescents' and young adults' (AYA) physical, mental, and emotional health, and developmental tasks. In order for AYAs, caregivers, clinicians, and other collaborators to optimize health outcomes (coproduction of health), understanding what living well means for AYAs who have experienced cancer is necessary. The objective was to develop an empirical definition of "living well" for AYAs who have experienced cancer to broadly understand AYA values and priorities. This definition may ultimately guide future conversations between caregivers and AYAs, eliciting thorough, personal definitions of living well from individual AYAs. Such conversations may enhance AYA participation in coproducing their health. Procedure Qualitative analysis using a phenomenological approach of N = 30 structuredRespecting Choicesinterviews conducted with AYAs (14-21 years; mean 84.2 [SD 69] months postcancer diagnosis with 21% on active treatment) from four tertiary pediatric hospitals in the context of a primary study of a pediatric advance care planning intervention trial. Results AYAs who have experienced cancer conceptualized "living well" as maintaining physical, mental, and emotional health, as well as engaging in purposeful, age-appropriate activities with people important to them. Living well had three components: living mindfully, living an identity as a healthy AYA, and spending time with friends and family. Conclusions Conversations with AYAs who have experienced cancer elicited rich, complex concepts of "living well." Provider initiation of discussions about living well may facilitate personalized goals of care conversations. This study may serve as the basis to design and prototype future clinical interventions to enhance AYA engagement.
Schreiner K; Grossoehme DH; Friebert S; Baker JN; Needle J; Lyon ME
Pediatric Blood & Cancer
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journalArticle
<a href="http://doi.org/10.1002/pbc.28599" target="_blank" rel="noreferrer noopener">10.1002/pbc.28599</a>
Aberrant epigenetic silencing of neuronatin is a frequent event in human osteosarcoma.
DNA methylation; neuronatin; osteosarcoma; tumor suppressor genes
The paternally imprinted neuronatin (NNAT) gene has been identified as a target of aberrant epigenetic silencing in diverse cancers, but no association with pediatric bone cancers has been reported to date. In screening childhood cancers, we identified aberrant CpG island hypermethylation in a majority of osteosarcoma (OS) samples and in 5 of 6 human OS cell lines studied but not in normal bone-derived tissue samples. CpG island hypermethylation was associated with transcriptional silencing in human OS cells, and silencing was reversible upon treatment with
Saeed H; Sinha S; Mella C; Kuerbitz JS; Cales ML; Steele MA; Stanke J; Damron D; Safadi F; Kuerbitz SJ
Oncotarget
2020
2020-05
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.18632/oncotarget.27583" target="_blank" rel="noreferrer noopener">10.18632/oncotarget.27583</a>
Quality improvement in neurology: Neurology Outcomes Quality Measurement Set.
Sico Jason J; Sarwal Aarti; Benish Sarah M; Busis Neil A; Cohen Bruce H; Das Rohit R; Finsilver Shari; Halperin John J; Kelly Adam G; Meunier Lisa; Phipps Michael S; Thirumala Parthasarathy D; Villanueva Raissa; von Gaudecker Jane; Bennett Amy; Shenoy Anant M
Neurology
2020
2020-05-12
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1212/WNL.0000000000009525" target="_blank" rel="noreferrer noopener">10.1212/WNL.0000000000009525</a>
Mitochondrial diseases in North America: An analysis of the NAMDC Registry.
criteria; dna; melas; merrf; natural-history; nuclear; prevalence
Objective: To describe clinical, biochemical, and genetic features of participants with mitochondrial diseases (MtDs) enrolled in the North American Mitochondrial Disease Consortium (NAMDC) Registry. Methods: This cross-sectional, multicenter, retrospective database analysis evaluates the phenotypic and molecular characteristics of participants enrolled in the NAMDC Registry from September 2011 to December 2018. The NAMDC is a network of 17 centers with expertise in MtDs and includes both adult and pediatric specialists. Results: One thousand four hundred ten of 1,553 participants had sufficient clinical data for analysis. For this study, we included only participants with molecular genetic diagnoses (n = 666). Age at onset ranged from infancy to adulthood. The most common diagnosis was multisystemic disorder (113 participants), and only a minority of participants were diagnosed with a classical mitochondrial syndrome. The most frequent classical syndromes were Leigh syndrome (97 individuals) and mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (71 individuals). Pathogenic variants in the mitochondrial DNA were more frequently observed (414 participants) than pathogenic nuclear gene variants (252 participants). Pathogenic variants in 65 nuclear genes were identified, with POLG1 and PDHA1 being the most commonly affected. Pathogenic variants in 38 genes were reported only in single participants. Conclusions: The NAMDC Registry data confirm the high variability of clinical, biochemical, and genetic features of participants with MtDs. This study serves as an important resource for future enhancement of MtD research and clinical care by providing the first comprehensive description of participant with MtD in North America.
Barca Emanuele; Long Yuelin; Cooley Victoria; Schoenaker Robert; Emmanuele Valentina; DiMauro Salvatore; Cohen Bruce H; Karaa Amel; Vladutiu Georgirene D; Haas Richard; Van Hove Johan L K; Scaglia Fernando; Parikh Sumit; Bedoyan Jirair K; DeBrosse Susanne D; Gavrilova Ralitza H; Saneto Russell P; Enns Gregory M; Stacpoole Peter W; Ganesh Jaya; Larson Austin; Zolkipli-Cunningham Zarazuela; Falk Marni J; Goldstein Amy C; Tarnopolsky Mark; Gropman Andrea; Camp Kathryn; Krotoski Danuta; Engelstad Kristin; Rosales Xiomara Q; Kriger Joshua; Grier Johnston; Buchsbaum Richard; Thompson John L P; Hirano Michio
Neurology. Genetics
2020
2020-04
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1212/NXG.0000000000000402" target="_blank" rel="noreferrer noopener">10.1212/NXG.0000000000000402</a>
Bone mineral density in adolescent urinary stone formers: is sex important?
Bone; children; disease; fracture; health; inflammation; kidney-stones; nephrolithiasis; osteoporosis; Pediatrics; risk; Sex; Urolithiasis; Urolithiasis
Urinary stone disease (USD) is affecting a greater number of children and low bone mineral density (BMD) and increased skeletal fractures have been demonstrated in stone patients; however, the mechanism(s) driving bone disease remain unclear. This pilot study was undertaken to assess an adolescent kidney stone cohort's BMD and evaluate for an inverse correlation between BMD and urine concentration of lithogenic minerals and/or inflammatory levels. Prospective case-control study was carried out at a large pediatric center. 15 participants with USD (12-18 years of age, 8 female) were matched by age, sex, and body mass index to 15 controls. Lumbar and total body BMD z-score did not differ between groups. When stone formers were separated by sex, there was a significant difference between male stone formers vs. controls total body BMD z-score (Fig. 1). BMD z-score did not significantly correlate with urine calcium, oxalate, citrate or magnesium. Higher urine IL-13 did significantly correlate with higher total body BMD z-score (r = 0.677, p = 0.018). Total body BMD z-score did significantly correlate with body mass index (BMI) as expected for the control group (r = 0.6321, p = 0.0133). However, this relationship was not present in the USD group (r = - 0.1629, p = 0.5619). This is a small but hypothesis-generating study which demonstrates novel evidence of male-specific low BMD in adolescent stone formers. Furthermore, we demonstrated a positive association between urine
Kusumi Kirsten; Schwaderer Andrew L; Clark Curtis; Budge Kevin; Hussein Nazar; Raina Rupesh; Denburg Michelle; Safadi Fayez F
Urolithiasis
2020
2020-03-31
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1007/s00240-020-01183-w" target="_blank" rel="noreferrer noopener">10.1007/s00240-020-01183-w</a>
What Is That? Innumerable Mysterious Densities Identified on Abdominal Imaging.
Radiopaque densities can be observed on imaging after the ingestion of either foreign bodies or some medications. Our case report discusses an 11-year-old boy with autism spectrum disorder and attention deficient disorder who presented to the emergency department because of concerns for constipation and dehydration. Incidentally, an abdominal x-ray showed numerous radiopaque densities throughout his intestines in addition to his constipation. He was admitted, and his home regimen was reviewed to attempt to identify a potential source for these radiopaque densities. This case presented an interesting teaching opportunity in the identification of the radiopaque densities and review of pharmacokinetics.
Kaiser Nicole; Blackford Martha G
Pediatric emergency care
2020
2020-02
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1097/PEC.0000000000002046" target="_blank" rel="noreferrer noopener">10.1097/PEC.0000000000002046</a>
Chemotherapy Strategies for Young Children Newly-Diagnosed with Medulloblastoma up to the Era of Molecular Profiling - A Comparative Outcomes Analysis
Finlay J; Mynarek M; Dhall G; Lafay-Cousin L; Mazewski C M; Ashley D; Cohen B H; von Bueren A O; Gerber N; Leary S; Geyer J R; Tait D; Gajjar A; Rutkowski S
Pediatric Blood & Cancer
2019
2019-12
Journal Article
<a href="http://doi.org/10.1093/neuonc/noz175.767" target="_blank" rel="noreferrer noopener">10.1093/neuonc/noz175.767</a>
Ugh
Pediatrics; Mental health; Medicine; Social Work; Medicine & Public Health; Health Psychology; Medical Sociology; Premedical Education; Family support
Ugh. I am ashamed to say that is the first word that comes to my mind when I see Jake’s name on my schedule for the day. He is 10 and he has been my patient since he was born. He has an 8-year-old sister Annie and a 5-year-old brother Jimmy. They usually all come in together with their mother even if only one of them has an appointment that day.
Sladjana Courson
Health Disparities : Weaving A New Understanding Through Case Narratives
2019
2019
Journal Article
<a href="http://doi.org/10.1007/978-3-030-12771-8_46" target="_blank" rel="noreferrer noopener">10.1007/978-3-030-12771-8_46</a>
Pediatric Migraine Action Plan (PedMAP)
Turner Scott B; Rende Elizabeth K; Pezzuto Tara; Weaver Samantha; Henderlong-Kropp Annmarie; Greene Kaitlin A; Bicknese Alma R; Dilts Jennifer J; Gautreaux Jennifer; Victorio M Cristina C; Strauss Lauren D; Lagman-Bartolome Ana Marissa; Szperka Christina L; Yonker Marcy; Hershey Andrew D; Gelfand Amy A
Headache
2019
2019-11
Journal Article
<a href="http://doi.org/10.1111/head.13681" target="_blank" rel="noreferrer noopener">10.1111/head.13681</a>
PMID: 31710105
Emergent high fatality lung disease in systemic juvenile arthritis
treatment; inflammation; adult onset still's disease; DMARDs (biologic); juvenile idiopathic arthritis
OBJECTIVE: To investigate the characteristics and risk factors of a novel parenchymal lung disease (LD), increasingly detected in systemic juvenile idiopathic arthritis (sJIA). METHODS: In a multicentre retrospective study, 61 cases were investigated using physician-reported clinical information and centralised analyses of radiological, pathological and genetic data. RESULTS: LD was associated with distinctive features, including acute erythematous clubbing and a high frequency of anaphylactic reactions to the interleukin (IL)-6 inhibitor, tocilizumab. Serum ferritin elevation and/or significant lymphopaenia preceded LD detection. The most prevalent chest CT pattern was septal thickening, involving the periphery of multiple lobes ± ground-glass opacities. The predominant pathology (23 of 36) was pulmonary alveolar proteinosis and/or endogenous lipoid pneumonia (PAP/ELP), with atypical features including regional involvement and concomitant vascular changes. Apparent severe delayed drug hypersensitivity occurred in some cases. The 5-year survival was 42%. Whole exome sequencing (20 of 61) did not identify a novel monogenic defect or likely causal PAP-related or macrophage activation syndrome (MAS)-related mutations. Trisomy 21 and young sJIA onset increased LD risk. Exposure to IL-1 and IL-6 inhibitors (46 of 61) was associated with multiple LD features. By several indicators, severity of sJIA was comparable in drug-exposed subjects and published sJIA cohorts. MAS at sJIA onset was increased in the drug-exposed, but was not associated with LD features. CONCLUSIONS: A rare, life-threatening lung disease in sJIA is defined by a constellation of unusual clinical characteristics. The pathology, a PAP/ELP variant, suggests macrophage dysfunction. Inhibitor exposure may promote LD, independent of sJIA severity, in a small subset of treated patients. Treatment/prevention strategies are needed.
Saper Vivian E; Chen Guangbo; Deutsch Gail H; Guillerman R Paul; Birgmeier Johannes; Jagadeesh Karthik; Canna Scott; Schulert Grant; Deterding Robin; Xu Jianpeng; Leung Ann N; Bouzoubaa Layla; Abulaban Khalid; Baszis Kevin; Behrens Edward M; Birmingham James; Casey Alicia; Cidon Michal; Cron Randy Q; De Aliva; De Benedetti Fabrizio; Ferguson Ian; Fishman Martha P; Goodman Steven I; Graham T Brent; Grom Alexei A; Haines Kathleen; Hazen Melissa; Henderson Lauren A; Ho Assunta; Ibarra Maria; Inman Christi J; Jerath Rita; Khawaja Khulood; Kingsbury Daniel J; Klein-Gitelman Marisa; Lai Khanh; Lapidus Sivia; Lin Clara; Lin Jenny; Liptzin Deborah R; Milojevic Diana; Mombourquette Joy; Onel Karen; Ozen Seza; Perez Maria; Phillippi Kathryn; Prahalad Sampath; Radhakrishna Suhas; Reinhardt Adam; Riskalla Mona; Rosenwasser Natalie; Roth Johannes; Schneider Rayfel; Schonenberg-Meinema Dieneke; Shenoi Susan; Smith Judith A; Sönmez Hafize Emine; Stoll Matthew L; Towe Christopher; Vargas Sara O; Vehe Richard K; Young Lisa R; Yang Jacqueline; Desai Tushar; Balise Raymond; Lu Ying; Tian Lu; Bejerano Gill; Davis Mark M; Khatri Purvesh; Mellins Elizabeth D; Childhood Arthritis and Rheumatology Research Alliance Registry Investigators
Annals Of The Rheumatic Diseases
2019
2019-12
Journal Article
<a href="http://doi.org/10.1136/annrheumdis-2019-216040" target="_blank" rel="noreferrer noopener">10.1136/annrheumdis-2019-216040</a>
PMID: 31562126
Exercise-Induced Dyspnea in Children and Adolescents: Differential Diagnosis
Pediatrics; adults; asthma; induced bronchoconstriction; hyperventilation; vocal-cord dysfunction
Exercise-induced dyspnea in children and adolescents can occur for many reasons. Although asthma is the common cause, failure to prevent exercise-induced asthma by pretreatment with a bronchodilator, such as albuterol, indicates that other etiologies should be considered. Other causes of exercise-induced dyspnea include exercise-induced vocal cord dysfunction, exercise-induced laryngomalacia, exercise-induced hyperventilation, chest wall restrictive abnormalities, cardiac causes, and normal physiologic limitation. When exercise-induced dyspnea is not from asthma, cardiopulmonary exercise testing with reproduction of the patient's dyspnea is the means to identify the other causes. Cardiopulmonary exercise testing monitors oxygen use, carbon-dioxide production, end-tidal pCO(2) (partial pressure of carbon dioxide), and electrocardiogram. Additional components to testing are measurement of blood pH and pCO(2) when symptoms are reproduced, and selective flexible laryngoscopy when upper airway obstruction is observed to specifically identify vocal cord dysfunction or laryngomalacia. This approach is a highly effective means to identify exercise-induced dyspnea that is not caused by asthma.
Bhatia R; Abu-Hasan M; Weinberger M
Pediatric Annals
2019
2019-03
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<a href="http://doi.org/10.3928/19382359-20190219-02" target="_blank" rel="noreferrer noopener">10.3928/19382359-20190219-02</a>
Adolescents with urinary stones have elevated urine levels of inflammatory mediators
Biomarker; Cytokine; Innate immunity; Interleukin
Urinary stones are increasing in children, primarily during adolescence. Although urinary stones are often viewed in the context of intermittent stone events, increasing evidence indicates that stones are a metabolic process associated with chronic kidney disease and cardiovascular disease. These aforementioned stone-associated conditions may have pediatric origins. To compare urine inflammatory markers in otherwise healthy stone forming children versus matched controls. Urine samples were collected from 12 adolescents with urinary stones along with 15 controls. The levels of 30 urine cytokines were measured using a Mesoscale 30-Plex Human Cytokine panel and normalized to urine creatinine levels. Macrophage inflammatory protein 1β and interleukin 13 levels were significantly elevated in the urine of the stone forming adolescents compared to controls. Interleukin 17A was elevated in the urine of controls. This study indicates that urine levels of cytokines involved in chronic inflammation and fibrosis are elevated in urinary stone formers as early as adolescence. Because stone formers are at risk for chronic kidney disease, macrophage inflammatory protein 1β and interleukin 13 represent investigative targets.
Kusumi Kirsten; Ketz John; Saxena Vijay; Spencer John David; Safadi Fayez; Schwaderer Andrew
Urolithiasis
2019
2019-04
<a href="http://doi.org/10.1007/s00240-019-01133-1" target="_blank" rel="noreferrer noopener">10.1007/s00240-019-01133-1</a>
Diagnosis of 'possible' mitochondrial disease: an existential crisis.
clinical genetics; diagnosis; evidence based practice; metabolic disorders
Primary genetic mitochondrial diseases are often difficult to diagnose, and the term 'possible' mitochondrial disease is used frequently by clinicians when such a diagnosis is suspected. There are now many known phenocopies of mitochondrial disease. Advances in genomic testing have shown that some patients with a clinical phenotype and biochemical abnormalities suggesting mitochondrial disease may have other genetic disorders. In instances when a genetic diagnosis cannot be confirmed, a diagnosis of 'possible' mitochondrial disease may result in harm to patients and their families, creating anxiety, delaying appropriate diagnosis and leading to inappropriate management or care. A categorisation of 'diagnosis uncertain', together with a specific description of the metabolic or genetic abnormalities identified, is preferred when a mitochondrial disease cannot be genetically confirmed.
Parikh Sumit; Karaa Amel; Goldstein Amy; Bertini Enrico Silvio; Chinnery Patrick F; Christodoulou John; Cohen Bruce H; Davis Ryan L; Falk Marni J; Fratter Carl; Horvath Rita; Koenig Mary Kay; Mancuso Michaelangelo; McCormack Shana; McCormick Elizabeth M; McFarland Robert; Nesbitt Victoria; Schiff Manuel; Steele Hannah; Stockler Silvia; Sue Carolyn; Tarnopolsky Mark; Thorburn David R; Vockley Jerry; Rahman Shamima
Journal of medical genetics
2019
2019-03
<a href="http://doi.org/10.1136/jmedgenet-2018-105800" target="_blank" rel="noreferrer noopener">10.1136/jmedgenet-2018-105800</a>
The influence of family environment on dissociation in pediatric injury patients.
Female; Male; Ohio; Socioeconomic Factors; Child; Prospective Studies; Income; Self Report; Confidence Intervals; Family; Social Environment; Human; Semi-Structured Interview; Questionnaires; Chi Square Test; Descriptive Research; Descriptive Statistics; Funding Source; Scales; Correlational Studies; Data Analysis Software; Pretest-Posttest Design; Checklists; Adolescence; Pearson's Correlation Coefficient; Retrospective Design; Analysis of Covariance; Child Development; Bivariate Statistics; Severity of Illness Indices; Family Relations; Family Coping; Interview Guides; Parametric Statistics; Parenting Education; Patient-Family Relations; Dissociative Disorders – Risk Factors; Accidents – Adverse Effects; Wounds and Injuries – Complications
Nugent Nicole R; Sledjeski Eve M; Christopher Norman C; Delahanty Douglas L
Clinical Child Psychology & Psychiatry
2011
2011-10
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1177/1359104511406487" target="_blank" rel="noreferrer noopener">10.1177/1359104511406487</a>
Evaluation of Nonpharmacologic Methods of Pain and Anxiety Management for Laceration Repair in the Pediatric Emergency Department.
PAIN management; CHILDREN; LETTERS to the editor; ANXIETY in children; DISTRACTION (Psychology); PAIN in children; PEDIATRIC emergency services
BACKGROUND. Nonpharmacologic interventions, such as distraction, have been shown to be powerful adjuncts in reducing pain and anxiety in children with both acute and chronic painful conditions. There are no controlled studies evaluating these interventions as adjuncts to facilitate completion of painful procedures in the pediatric emergency department (ED). OBJECTIVE. We assessed the effectiveness of distraction techniques in reducing the sensory and affective components of pain among pediatric patients undergoing laceration repair in the ED. METHODS. Eligible children between 6 and 18 years of age (N = 240) presenting to the ED for laceration repair were randomly assigned to an intervention or control arm. Those assigned to the intervention arm were given a choice of age-appropriate distracters during laceration repair. Quantitative measures of pain intensity, situational anxiety, and pain distress (as perceived by the parent) were assessed by using the 7-point Facial Pain Scale, State Trait Anxiety Inventory for Children, and a visual analog scale, respectively, before and after laceration repair. The State Trait Anxiety Inventory for Children was performed in children ≥10 years of age. RESULTS. There was no difference in mean change in Facial Pain Scale scores between the control and the intervention groups in children \textless10 years of age. Multivariate analysis in this same age group showed that the intervention was independently associated with a reduction in pain distress as perceived by parents based on the mean change in visual analog scale scores. In older children, the intervention was independently associated with reduction in situational anxiety but not in pain intensity or in parental perception of pain distress. CONCLUSIONS. The use of distraction techniques is effective in reducing situational anxiety in older children and lowering parental perception of pain distress in younger children. This technique may have a role in improving the quality of management of procedural pain in a pediatric ED setting. [ABSTRACT FROM AUTHOR]
Sinha Madhumita; Christopher Norman C; Fenn Robin; Reeves Laurie
Pediatrics
2006
2006-04
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1542/peds.2005-1100" target="_blank" rel="noreferrer noopener">10.1542/peds.2005-1100</a>
Initial physiological responses and perceived hyperarousal predict subsequent emotional numbing in pediatric injury patients.
AVOIDANCE (Psychology); POST-traumatic stress disorder; HOSPITAL emergency services; CHILDREN'S injuries; URINE; HEART beat; CHILDREN'S accidents; DEPRESSION in children; EMOTIONS (Psychology)
The present study tested the hypothesis that acute posttraumatic hyperarousal would lead to the development of emotional numbing (EN) symptoms in a pediatric injury population. Eighty-two youths aged 8–18 years were recruited from the emergency department of a Midwestern children's hospital. Heart rate was recorded from emergency medical services reports and a 12-hour urine collection was initiated upon admission. Six weeks and 6 months later, depression and PTSD symptoms were assessed. Initial heart rate and urinary cortisol levels predicted 6-week and 6-month EN after controlling for concurrent depression, avoidance, and reexperiencing symptoms and 6-week hyperarousal symptoms. These findings provide empirical support for prior hypotheses concerning the development of PTSD symptoms over time. [ABSTRACT FROM AUTHOR]
Nugent Nicole R; Christopher Norman C; Delahanty Douglas L
Journal of Traumatic Stress
2006
2006-06
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/jts.20130" target="_blank" rel="noreferrer noopener">10.1002/jts.20130</a>
Emergency medical service and in-hospital vital signs as predictors of subsequent PTSD symptom severity in pediatric injury patients.
POST-traumatic stress disorder; CARDIOVASCULAR diseases; HEART beat; JUVENILE diseases; MEDICAL emergencies; PEDIATRIC trauma centers
Background: The present study investigated the extent to which heart rate (HR) levels soon after a traumatic event predicted posttraumatic stress disorder (PTSD) symptom severity assessed 6 weeks and 6 months later in child trauma victims. Methods: Participants consisted of 82 children (56 boys, 26 girls) aged 8–18 who were admitted to a Midwestern trauma center. HR data were recorded from emergency medical services (EMS) records, upon admission to the emergency department (ED), for the first 20 minutes following admission, and upon discharge. Subsequent PTSD and depressive symptoms were assessed 6-weeks and 6-months post-trauma. Results: HR recorded during EMS transport was significantly correlated with PTSD symptoms at 6 weeks ( r = .42) and at 6 months ( r = .35). After removing the variance associated with demographic variables and depressive symptoms, HR during EMS transport and averaged over the first 20 minutes following admission significantly predicted 6-week PTSD symptoms. The first recorded EMS HR measure significantly predicted 6-month PTSD symptoms. HR averaged over the first 20 minutes of EMS transport and averaged over the first 20 minutes following admission marginally predicted 6-month PTSD symptoms ( ps = .051 and .079, respectively). Conclusions: The present findings suggest that physiological arousal soon after a traumatic event may be associated with increased risk for the development of PTSD symptoms in child trauma victims. These findings provide preliminary support for the use of acute cardiovascular levels as markers of child trauma victims at higher risk of developing symptoms of PTSD. [ABSTRACT FROM AUTHOR]
Nugent Nicole R; Christopher Norman C; Delahanty Douglas L
Journal of Child Psychology & Psychiatry
2006
2006-09
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1469-7610.2006.01648.x" target="_blank" rel="noreferrer noopener">10.1111/j.1469-7610.2006.01648.x</a>
Brief Report: The Impact of Maternal Posttraumatic Stress Disorder Symptoms and Child Gender on Risk for Persistent Posttraumatic Stress Disorder Symptoms in Child Trauma Victims.
POST-traumatic stress disorder; STRESS (Psychology); CHILDREN; NEUROSES; TRAUMATIC neuroses
Objective To longitudinally examine the impact of maternal posttraumatic stress disorder symptoms (PTSS) on child adjustment following a child's traumatic injury, focusing on child gender differences. Methods Forty-one child traumatic injury victims aged 8-18 years and their biological mothers were interviewed over two follow-ups (6 weeks and 7 months). Children were administered the Clinician-Administered Posttraumatic Stress Disorder (PTSD) Scale for Children and Adolescents (CAPS-CA), whereas mothers completed the CAPS. Results Six weeks post trauma, maternal PTSS were significantly related to PTSS in boys but not in girls. However, at 7 months, maternal PTSS were strongly related to child PTSS in both boys and girls. Significant 6-week maternal distress-child gender interactions suggested that maternal PTSS, especially avoidance, predicted greater 7-month PTSS but that this was primarily because of a significant relationship in females. Conclusions Maternal distress was found to negatively impact subsequent child adjustment, particularly in females. These results underscore the importance of considering family-centered interventions for child PTSD, especially in girls. [ABSTRACT FROM AUTHOR]
Ostrowski Sarah A; Christopher Norman C; Delahanty Douglas L
Journal of Pediatric Psychology
2007
2007-04
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1093/jpepsy/jsl003" target="_blank" rel="noreferrer noopener">10.1093/jpepsy/jsl003</a>
Parental Posttraumatic Stress Symptoms as a Moderator of Child's Acute Biological Response and Subsequent Posttraumatic Stress Symptoms in Pediatric Injury Patients.
POST-traumatic stress disorder; STRESS (Psychology); CHILDREN; NEUROSES; PARENTS
Objective To examine how parental responses following pediatric injury may influence their child's posttraumatic stress symptoms (PTSS). Methods Heart rate (HR) from 82 pediatric injury patients was measured during emergency medical services (EMSs) transport and following hospital admission. Twelve-hour urinary cortisol levels were assessed upon admission. Child PTSS and parental PTSS and general distress were assessed 6 weeks and 6 months after trauma. Results Six-week parental PTSS predicted 6-month child PTSS even after controlling for demographics and general parent distress (ΔR² = .08, p = .03). Parental PTSS moderated the relationship between (a) child cortisol levels and 6-month child PTSS (ΔR² = .08, p = .03) and between (b) hospital HR and 6-month child PTSS (ΔR² = .09, p = .03). Conclusion The present findings suggest that parental response to trauma may interact with child acute physiological responses to predict persistent child PTSS. [ABSTRACT FROM AUTHOR]
Nugent Nicole R; Ostrowski Sarah; Christopher Norman C; Delahanty Douglas L
Journal of Pediatric Psychology
2007
2007-04
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1093/jpepsy/jsl005" target="_blank" rel="noreferrer noopener">10.1093/jpepsy/jsl005</a>
Acute child and mother psychophysiological responses and subsequent PTSD symptoms following a child's traumatic event.
MENTAL depression; POST-traumatic stress disorder; RESEARCH; STATISTICAL sampling; HYDROCORTISONE; CHILDREN; MOTHERS; TRAUMA centers; URINE
This study examined the relationship between acute cortisol responses to trauma and subsequent PTSD symptoms (PTSS) in children and their biological mothers. Urinary cortisol levels were assessed in 54 children aged 8–18 upon admission to a level-1 trauma center. Six weeks posttrauma, 15-hour urine samples were collected from children and their mothers. Depression and PTSS were assessed at 6 weeks (N = 44) and 7 months (N = 38) posttrauma. Higher child in-hospital cortisol significantly predicted 6-week child PTSS. This was true only for boys at 7 months. In mothers, lower 6-week cortisol levels significantly predicted 7-month PTSS. Results extend findings of differing directions of acute hormonal predictors of PTSS in adults versus children to a sample of genetically related individuals. [ABSTRACT FROM AUTHOR]
Ostrowski Sarah A; Christopher Norman C; van Dulmen Manfred HM; Delahanty Douglas L
Journal of Traumatic Stress
2007
2007-10
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/jts.20286" target="_blank" rel="noreferrer noopener">10.1002/jts.20286</a>
Pediatric Urolithiasis: Clinical Predictors in the Emergency Department.
LOGISTIC regression analysis; CHILDREN'S hospitals; BLADDER stones; DISEASE risk factors; HEMATURIA in children; PEDIATRIC diagnosis; TOMOGRAPHY; URINALYSIS; URINARY calculi – Diagnosis
OBJECTIVE: The objective of this study was to identify factors that predict the presence of urolithiasis detected with unenhanced computed tomography (UCT) in children. METHODS: A retrospective study of all subjects \textless21 years of age who presented to the emergency department at Akron Children's Hospital and underwent UCT of the abdomen between January 2002 and December 2005 was performed. Demographic, clinical, diagnostic, treatment, and disposition data were abstracted by using a standardized form. Univariate and logistic regression analyses of factors associated with urolithiasis were performed. RESULTS: A total of 339 eligible patients were identified, with 110 cases of urolithiasis detected with UCT for 95 individual patients. The mean age of the study patients was 14.4 years; 72 patients (66%) were female. In 17 cases (15%) of urolithiasis, initial urinalysis results were negative for blood. Fifty-seven stones (51.8%) were ureteral, 26(23.6%) were renal, and 4 (3.6%) were in the bladder. Among children who did not have a stone identified through UCT, 23 cases (10%) of potentially significant, alternative diagnoses were identified. A history of urolithiasis, a history of nausea and vomiting, the presence of flank pain on examination, and \textgreater2 red blood cells per high-power field in urine microscopy were positively associated with urolithiasis. A history of fever or dysuria and costovertebral angle tenderness on physical examination were inversely associated with urolithiasis on UCT scans. CONCLUSIONS: UCT plays an important role in the diagnostic evaluation of children with flank pain. Approximately 15% of children with urolithiasis do not have hematuria. [ABSTRACT FROM AUTHOR]
Persaud Andre C; Stevenson Michelle D; McMahon Daniel R; Christopher Norman C
Pediatrics
2009
2009-09
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1542/peds.2008-2427" target="_blank" rel="noreferrer noopener">10.1542/peds.2008-2427</a>
The efficacy of early propranolol administration at reducing PTSD symptoms in pediatric injury patients: A pilot study.
TREATMENT of post-traumatic stress disorder; STRESS (Psychology); GENDER differences (Psychology); CHILDREN'S injuries; BLIND experiment; PHARMACOLOGY; PLACEBOS (Medicine); PROPRANOLOL
Initial research supports the use of propranolol to prevent posttraumatic stress disorder (PTSD); research has not examined pharmacological prevention for children. Twenty-nine injury patients (ages 10–18 years old) at risk for PTSD were randomized to a double-blind 10-day trial of propranolol or placebo initiated within 12 hours postadmission. Six-week PTSD symptoms and heart rate were assessed. Although intent-to-treat analyses revealed no group differences, findings supported a significant interaction between gender and treatment in medication-adherent participants, ΔR2 = .21. Whereas girls receiving propranolol reported more PTSD symptoms relative to girls receiving placebo, ΔR2 = .44, boys receiving propranolol showed a nonsignificant trend toward fewer PTSD symptoms than boys receiving placebo, ΔR2 = .32. Findings inform gender differences regarding pharmacological PTSD prevention in youth. [ABSTRACT FROM AUTHOR]
Nugent Nicole R; Christopher Norman C; Crow John P; Browne Lorin; Ostrowski Sarah; Delahanty Douglas L
Journal of Traumatic Stress
2010
2010-04
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/jts.20517" target="_blank" rel="noreferrer noopener">10.1002/jts.20517</a>