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
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/10.1089/jpm.2021.0127" target="_blank" rel="noreferrer noopener">10.1089/jpm.2021.0127</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
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/10.3389/fped.2020.591520" target="_blank" rel="noreferrer noopener">10.3389/fped.2020.591520</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>
Guidelines Of Care For The Management Of Basal Cell Carcinoma
5-year follow-up; aminolevulinate photodynamic; basal cell carcinoma; biopsy; curettage; Dermatology; expectancy; fresh tissue technique; hedgehog pathway inhibitor; imiquimod 5-percent cream; limited life; metastasis; mohs micrographic surgery; nonmelanoma; open-label trial; phototherapy; radiotherapy; randomized controlled trial; skin-cancer; staging; Surgery; surveillance; therapy; topical therapy
Basal cell carcinoma (BCC) is the most common form of human cancer, with a continually increasing annual incidence in the United States. When diagnosed early, the majority of BCCs are readily treated with office-based therapy, which is highly curative. In these evidence-based guidelines of care, we provide recommendations for the management of patients with BCC, as well as an in-depth review of the best available literature in support of these recommendations. We discuss biopsy techniques for a clinically suspicious lesion and offer recommendations for the histopathologic interpretation of BCC. In the absence of a formal staging system, the best available stratification based on risk for recurrence is reviewed. With regard to treatment, we provide recommendations on treatment modalities along a broad therapeutic spectrum, ranging from topical agents and superficially destructive modalities to surgical techniques and systemic therapy. Finally, we review the available literature and provide recommendations on prevention and the most appropriate follow-up for patients in whom BCC has been diagnosed.
Bichakjian C; Armstrong A; Baum C; Bordeaux J S; Brown M; Busam K J; Eisen D B; Iyengar V; Lober C; Margolis D J; Messina J; Miller A; Miller S; Mostow E; Mowad C; Nehal K; Schmitt-Burr K; Sekulic A; Storrs P; Teng J; Yu S; Huang C; Boyer K; Begolka W S; Alam M; Kim J Y S; Kozlow J H; Mittal B; Moyer J; Olencki T; Rodgers P
Journal of the American Academy of Dermatology
2018
2018-03
Journal Article or Conference Abstract Publication
<a href="http://doi.org/10.1016/j.jaad.2017.10.006" target="_blank" rel="noreferrer noopener">10.1016/j.jaad.2017.10.006</a>
Guidelines Of Care For The Management Of Basal Cell Carcinoma
5-year follow-up; aminolevulinate photodynamic; basal cell carcinoma; biopsy; curettage; Dermatology; expectancy; fresh tissue technique; hedgehog pathway inhibitor; imiquimod 5-percent cream; limited life; metastasis; mohs micrographic surgery; nonmelanoma; open-label trial; phototherapy; radiotherapy; randomized controlled-trial; skin-cancer; staging; Surgery; surveillance; therapy; topical therapy
Bichakjian C; Armstrong A; Baum C; Bordeaux J S; Brown M; Busam K J; Eisen D B; Iyengar V; Lober C; Margolis D J; Messina J; Miller A; Miller S; Mostow E; Mowad C; Nehal K; Schmitt-Burr K; Sekulic A; Storrs P; Teng J; Yu S; Huang C; Boyer K; Begolka W S; Alam M; Kim J Y S; Kozlow J H; Mittal B; Moyer J; Olencki T; Rodgers P
Journal of the American Academy of Dermatology
2018
2018-03
Journal Article or Conference Abstract Publication
<a href="http://doi.org/10.1016/j.jaad.2017.10.006" target="_blank" rel="noreferrer noopener">10.1016/j.jaad.2017.10.006</a>
Guidelines of care for the management of cutaneous squamous cell carcinoma
5-year follow-up; american joint committee; biopsy; carcinoma; curettage; Dermatology; fresh tissue technique; limited life expectancy; lymph-node biopsy; metastasis; mohs micrographic surgery; nonmelanoma skin-cancer; organ transplant; phototherapy; radiotherapy; randomized controlled-trial; recipients; squamous cell; staging; Surgery; surveillance; topical photodynamic therapy; topical therapy
Cutaneous squamous cell carcinoma (cSCC) is the second most common form of human cancer and has an increasing annual incidence. Although most cSCC is cured with office-based therapy, advanced cSCC poses a significant risk for morbidity, impact on quality of life, and death. This document provides evidence-based recommendations for the management of patients with cSCC. Topics addressed include biopsy techniques and histopathologic assessment, tumor staging, surgical and nonsurgical management, follow-up and prevention of recurrence, and management of advanced disease. The primary focus of these recommendations is on evaluation and management of primary cSCC and localized disease, but where relevant, applicability to recurrent cSCC is noted, as is general information on the management of patients with metastatic disease.
Alam M; Armstrong A; Baum C; Bordeaux J S; Brown M; Busam K J; Eisen D B; Iyengar V; Lober C; Margolis D J; Messina J; Miller A; Miller S; Mostow E; Mowad C; Nehal K; Schmitt-Burr K; Sekulic A; Storrs P; Teng J; Yu S; Huang C; Boyer K; Begolka W S; Bichakjian C; Kim J Y S; Kozlow J H; Mittal B; Moyer J; Olenecki T; Rodgers P
Journal of the American Academy of Dermatology
2018
2018-03
Journal Article or Conference Abstract Publication
<a href="http://doi.org/10.1016/j.jaad.2017.10.007" target="_blank" rel="noreferrer noopener">10.1016/j.jaad.2017.10.007</a>