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  1. Article ; Online: Implementation of the 'TAKE STOCK' Hot Debrief Tool in the ED: a quality improvement project.

    Sugarman, Max / Graham, Blair / Langston, Sarah / Nelmes, Pam / Matthews, John

    Emergency medicine journal : EMJ

    2021  Volume 38, Issue 8, Page(s) 579–584

    Abstract: Hot debriefing (HoD) describes a structured team-based discussion which may be initiated following a significant event. Benefits may include improved teamwork, staff well-being and identification of learning opportunities. Existing literature indicates ... ...

    Abstract Hot debriefing (HoD) describes a structured team-based discussion which may be initiated following a significant event. Benefits may include improved teamwork, staff well-being and identification of learning opportunities. Existing literature indicates that while staff value HoD following significant events, it is infrequently undertaken in practice. Internationally, several frameworks for HoD have been developed, although none are widely adopted for use in the ED. A quality improvement project was conducted to introduce HoD into a single UK ED in North West England, between January and March 2019. Following stakeholder consultation, the 9-item 'TAKE STOCK' tool was developed. Implementation of the tool increased the number of HoD (0-2.2 HoD episodes/week). Findings from the first plan-do-study-act (PDSA) cycle are presented, which revealed the key strengths and limitations of this model. Staff perceptions of the tool were evaluated using a self-administered short questionnaire designed by the authors. Satisfaction with TAKE STOCK was assessed using 10-point numerical scales. Across respondents (n-15), average satisfaction scores exceeded 9 out of 10 concerning patient care, staff self-care, decision-making, education, teamwork and identification of equipment issues. Implementation of HoD into the ED is feasible and viewed as beneficial by staff. Implementation toolkits for TAKE STOCK have been requested by 42 additional UK hospitals and ambulance trusts, demonstrating significant interest in its use. Research is now required to formally validate HoD frameworks for use in the ED, and assess whether HoD results in sustained improvements to staff and patient outcomes.
    MeSH term(s) Emergency Service, Hospital/organization & administration ; England ; Feedback ; Humans ; Patient Care Team/organization & administration ; Quality Improvement
    Language English
    Publishing date 2021-01-13
    Publishing country England
    Document type Journal Article
    ZDB-ID 2040124-3
    ISSN 1472-0213 ; 1472-0205
    ISSN (online) 1472-0213
    ISSN 1472-0205
    DOI 10.1136/emermed-2019-208830
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: An atypical p.N215S variant of Fabry disease with end-stage renal failure.

    Sugarman, Max / Choudhury, Jamil / Jovanovic, Ana

    Molecular genetics and metabolism reports

    2018  Volume 15, Page(s) 43–45

    Abstract: Fabry disease is an X-linked metabolic disorder resulting in widespread deposition of Globotriaosylceramide within a variety of human tissues. The classical Fabry phenotype is one of early onset disease, with extensive tissue involvement resulting in ... ...

    Abstract Fabry disease is an X-linked metabolic disorder resulting in widespread deposition of Globotriaosylceramide within a variety of human tissues. The classical Fabry phenotype is one of early onset disease, with extensive tissue involvement resulting in acroparaesthesia, gastrointestinal disturbances, angiokeratoma, cornea verticillata renal failure, and cardiovascular disease. We describe two brothers exhibiting the GLA p.N215S mutation, a variant most often conferring a late-onset disease confined to the myocardium. The proband was diagnosed aged 34, following investigation into proteinuria. Despite Enzyme Replacement Therapy, he progressed to end-stage renal failure, and subsequently received a renal transplant. He also developed hypertrophic cardiomyopathy. His sibling however, whose disease was detected aged 32 following screening, exhibits mild left ventricular hypertrophy, and no evidence of renal disease. He remains clinically asymptomatic. This case report details a discordant phenotype in brothers with Fabry disease and p.N215S mutation. Despite the fact that in the majority of patients this mutation is associated with a late onset presentation with hypertrophic cardiomyopathy, we have clearly demonstrated that patients with GLA p.N215S mutation can present with the classical phenotype. Further studies are required to elucidate the underlying modifying factors that influence clinical presentation with a more severe phenotype.
    Language English
    Publishing date 2018-02-06
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2821908-9
    ISSN 2214-4269
    ISSN 2214-4269
    DOI 10.1016/j.ymgmr.2018.01.006
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: An atypical p.N215S variant of Fabry disease with end-stage renal failure

    Max Sugarman / Jamil Choudhury / Ana Jovanovic

    Molecular Genetics and Metabolism Reports, Vol 15, Iss , Pp 43-

    2018  Volume 45

    Abstract: Fabry disease is an X-linked metabolic disorder resulting in widespread deposition of Globotriaosylceramide within a variety of human tissues. The classical Fabry phenotype is one of early onset disease, with extensive tissue involvement resulting in ... ...

    Abstract Fabry disease is an X-linked metabolic disorder resulting in widespread deposition of Globotriaosylceramide within a variety of human tissues. The classical Fabry phenotype is one of early onset disease, with extensive tissue involvement resulting in acroparaesthesia, gastrointestinal disturbances, angiokeratoma, cornea verticillata renal failure, and cardiovascular disease.We describe two brothers exhibiting the GLA p.N215S mutation, a variant most often conferring a late-onset disease confined to the myocardium.The proband was diagnosed aged 34, following investigation into proteinuria.Despite Enzyme Replacement Therapy, he progressed to end-stage renal failure, and subsequently received a renal transplant. He also developed hypertrophic cardiomyopathy.His sibling however, whose disease was detected aged 32 following screening, exhibits mild left ventricular hypertrophy, and no evidence of renal disease. He remains clinically asymptomatic.This case report details a discordant phenotype in brothers with Fabry disease and p.N215S mutation. Despite the fact that in the majority of patients this mutation is associated with a late onset presentation with hypertrophic cardiomyopathy, we have clearly demonstrated that patients with GLA p.N215S mutation can present with the classical phenotype. Further studies are required to elucidate the underlying modifying factors that influence clinical presentation with a more severe phenotype. Keywords: Fabry disease, p.N215S, Genetics, Metabolic disease
    Keywords Medicine (General) ; R5-920 ; Biology (General) ; QH301-705.5
    Subject code 610
    Language English
    Publishing date 2018-06-01T00:00:00Z
    Publisher Elsevier
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  4. Article ; Online: RBCK1-related disease: A rare multisystem disorder with polyglucosan storage, auto-inflammation, recurrent infections, skeletal, and cardiac myopathy-Four additional patients and a review of the current literature.

    Phadke, Rahul / Hedberg-Oldfors, Carola / Scalco, Renata S / Lowe, David M / Ashworth, Michael / Novelli, Marco / Vara, Roshni / Merwick, Aine / Amer, Halima / Sofat, Reecha / Sugarman, Max / Jovanovic, Ana / Roberts, Mark / Nakou, Vasiliki / King, Andrew / Bodi, Istvan / Jungbluth, Heinz / Oldfors, Anders / Murphy, Elaine

    Journal of inherited metabolic disease

    2020  Volume 43, Issue 5, Page(s) 1002–1013

    Abstract: In this article, we report four new patients, from three kindreds, with pathogenic variants in RBCK1 and a multisystem disorder characterised by widespread polyglucosan storage. We describe the clinical presentation of progressive skeletal and cardiac ... ...

    Abstract In this article, we report four new patients, from three kindreds, with pathogenic variants in RBCK1 and a multisystem disorder characterised by widespread polyglucosan storage. We describe the clinical presentation of progressive skeletal and cardiac myopathy, combined immunodeficiencies and auto-inflammation, illustrate in detail the histopathological findings in multiple tissue types, and report muscle MRI findings.
    MeSH term(s) Child ; Child, Preschool ; Female ; Glucans/metabolism ; Glycogen Storage Disease/genetics ; Glycogen Storage Disease/metabolism ; Humans ; Inflammation/pathology ; Male ; Muscle, Skeletal/pathology ; Muscular Diseases/pathology ; Reinfection/pathology ; Transcription Factors/genetics ; Ubiquitin-Protein Ligases/genetics
    Chemical Substances Glucans ; Transcription Factors ; polyglucosan (9012-72-0) ; RBCK1 protein, human (EC 2.3.2.27) ; Ubiquitin-Protein Ligases (EC 2.3.2.27)
    Language English
    Publishing date 2020-04-16
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 438341-2
    ISSN 1573-2665 ; 0141-8955
    ISSN (online) 1573-2665
    ISSN 0141-8955
    DOI 10.1002/jimd.12234
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article: The Community and Patient Partnered Research Network (CPPRN): Application of Patient-Centered Outcomes Research to Promote Behavioral Health Equity.

    Arevian, Armen C / Springgate, Benjamin / Jones, Felica / Starks, Sarah L / Chung, Bowen / Wennerstrom, Ashley / Jones, Loretta / Kataoka, Sheryl H / Griffith, Krystal / Sugarman, Olivia K / Williams, Pluscedia / Haywood, Catherine / Kirkland, Angela / Meyers, Diana / Pasternak, Ryan / Simmasalam, Rubinee / Tang, Lingqi / Castillo, Enrico G / Mahajan, Anish /
    Stevens, Max / Wells, Kenneth B

    Ethnicity & disease

    2018  Volume 28, Issue Suppl 2, Page(s) 295–302

    Abstract: Objective: We describe the rationale, development, and progress on the Community and Patient Partnered Research Network (CPPRN). The CPPRN builds on more than a decade of partnered work and is designed to promote health equity by developing partnered ... ...

    Abstract Objective: We describe the rationale, development, and progress on the Community and Patient Partnered Research Network (CPPRN). The CPPRN builds on more than a decade of partnered work and is designed to promote health equity by developing partnered research on behavioral health and social risk factors in Los Angeles and New Orleans.
    Setting: A community-academic partnership across Los Angeles County and New Orleans.
    Methods: Review of rationale, history, structure, activities and progress in applying community partnered participatory research (CPPR) to CPPRN.
    Findings: Patient and community stakeholders participated in all phases of development, including local and national activities. Key developments include partnered planning efforts, progress on aggregating a large, de-identified dataset across county agencies, and development of an information technology-supported screening approach for behavioral and social determinants in health care, social, and community-based settings.
    Conclusion: The CPPRN represents a promising approach for research data networks, balancing the potential benefit of information technology and data analytic approaches while addressing potential risks and priorities of relevant stakeholders.
    MeSH term(s) Community Networks/organization & administration ; Community Participation/methods ; Community-Based Participatory Research ; Health Equity/organization & administration ; Humans ; Los Angeles ; Mental Health/standards ; New Orleans ; Patient Outcome Assessment ; Quality Improvement ; Social Determinants of Health/standards
    Language English
    Publishing date 2018-09-06
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1274267-3
    ISSN 1945-0826 ; 1049-510X
    ISSN (online) 1945-0826
    ISSN 1049-510X
    DOI 10.18865/ed.28.S2.295
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article: Accelerated pharmacokinetics and glucodynamics of prandial insulins injected with recombinant human hyaluronidase.

    Vaughn, Daniel E / Yocum, Richard C / Muchmore, Douglas B / Sugarman, Barry J / Vick, Andrew M / Bilinsky, Igor P / Frost, Gregory I

    Diabetes technology & therapeutics

    2009  Volume 11, Issue 6, Page(s) 345–352

    Abstract: ... 10 microg/mL). Pharmacokinetic parameters included peak serum insulin concentration (C(max)), time ... to C(max) (t(max)), and area under the curve (AUC) of serum concentration versus time. Glucodynamic ... parameters included time to maximal glucose infusion rate (tGIR(max)) and area under the GIR-versus-time ...

    Abstract Background: This phase 1 study investigated the pharmacokinetics (PK) and glucodynamics of insulin lispro (Humalog; Eli Lilly and Co., Indianapolis, IN) or regular human insulin (Humulin R; Eli Lilly and Co.) administered with or without (+/-) recombinant human hyaluronidase (rHuPH20).
    Methods: Healthy male volunteers (n = 26), 18-55 years old with body mass index 18-28 kg/m(2), weight >70 kg, and normal fasting glucose, were randomized to a crossover sequence of two subcutaneous injections, each followed by a 6-h euglycemic clamp targeting glucose 90-110 mg/dL: Cohort 1 received 20 U of Humalog +/- 300 U of rHuPH20 (11.3 microg/mL), whereas Cohort 2 received 20 U of Humulin R +/- 240 U of rHuPH20 (10 microg/mL). Pharmacokinetic parameters included peak serum insulin concentration (C(max)), time to C(max) (t(max)), and area under the curve (AUC) of serum concentration versus time. Glucodynamic parameters included time to maximal glucose infusion rate (tGIR(max)) and area under the GIR-versus-time curve (G).
    Results: For Humalog and Humulin R, respectively, rHuPH20 co-administration reduced t(max) by 51% (P = 0.0006) and 58% (P = 0.0002), increased C(max) by 90% (P = 0.0003) and 142% (P < 0.0001), increased early exposure (AUC(0-2h)) by 85% (P < 0.0001) and 211% (P < 0.0001), and reduced late exposure (AUC(4-6h)) by 41% (P < 0.0001) and 48% (P < 0.0001). Similarly, rHuPH20 reduced tGIR(max) by 41% (P = 0.006) and 35% (P = 0.01), increased early metabolism (G(0-2h)) by 52% (P = 0.001) and 127% (P < 0.0001), and reduced late metabolism (G(4-6h)) by 29% (P = 0.002) and 26% (P = 0.03) for Humalog and Humulin R, respectively. Injections were well tolerated.
    Conclusions: Co-administration of rHuPH20 accelerated the PK and glucodynamics of both insulin formulations. Additional studies are necessary to evaluate the clinical relevance of these findings in patients with diabetes.
    MeSH term(s) Adolescent ; Adult ; Blood Glucose/drug effects ; Blood Glucose/metabolism ; Cross-Over Studies ; Humans ; Hyaluronoglucosaminidase/pharmacology ; Hypoglycemic Agents/pharmacokinetics ; Hypoglycemic Agents/pharmacology ; Insulin/analogs & derivatives ; Insulin/blood ; Insulin/pharmacokinetics ; Insulin/pharmacology ; Insulin Lispro ; Male ; Middle Aged ; Recombinant Proteins/pharmacology ; Reference Values ; Young Adult
    Chemical Substances Blood Glucose ; Hypoglycemic Agents ; Insulin ; Insulin Lispro ; Recombinant Proteins ; Hyaluronoglucosaminidase (EC 3.2.1.35)
    Language English
    Publishing date 2009-06
    Publishing country United States
    Document type Clinical Trial, Phase I ; Journal Article ; Randomized Controlled Trial ; Research Support, Non-U.S. Gov't
    ZDB-ID 1452816-2
    ISSN 1520-9156
    ISSN 1520-9156
    DOI 10.1089/dia.2009.0013
    Database MEDical Literature Analysis and Retrieval System OnLINE

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