Characteristics, outcomes and health care utilization of patients with acute myeloid leukemia aged 70 years or older: A single-center retrospective analysis.

The overall prognosis of older patients with acute myeloid leukemia (AML) is dismal. Only a small subgroup experiences long-term survival. The discrimination between patients who are candidates for potentially curative approaches and those who are not is crucial since - in addition to differences in terms of AML-directed treatment - different policies concerning intensive care unit (ICU) admission and involvement of specialized palliative care (SPC) seem obvious. To shed more light on characteristics, outcomes and health care utilization of older individuals with AML, we conducted an analysis comprising 107 consecutive patients with newly diagnosed AML aged ≥70 years treated at an academic tertiary care center in Germany between 1 January 2015, and 31 December 2020. Median age was 75 years (range: 70-87 years); 45% of patients were female. The proportion of patients receiving intensive induction chemotherapy was 35%, 55% had low-intensity treatment and 10% did not receive AML-directed treatment or follow-up ended before treatment initiation. At least one ICU admission was documented for 47% of patients; SPC was involved in 43% of cases. Median follow-up was 199 days. The median overall survival (OS) was 2.5 months; the 1-year OS rate was 16%. Among patients who died during observation, the median proportion of time spent in the hospital between AML diagnosis and death was 56%. The most common places of death were normal wards (31%) and the ICU (28%). Patients less frequently died in a palliative care unit (14%) or at home (12%). In summary, results of the present analysis confirm the unfavorable prognosis of older patients with AML despite intensive health care utilization. Future efforts in this patient group should aim at optimizing the balance between appropriate AML-directed treatment on the one hand and health care utilization including ICU stays on the other hand.

Quality of life from childhood to adulthood: Perspectives from adult patients with pediatric-onset intestinal failure.

Adult patients with pediatric onset short bowel syndrome (SBS) or intestinal failure (IF) have been described as a distinct population warranting further research. The aim of this exploratory study aimed was to offer initial insights into this population's navigation of childhood, adolescence, and transition into adulthood. Both quantitative and qualitative data were collected from a convenience sample of adults with pediatric-onset SBS/IF using a disease-specific pilot survey; 14 questionnaires were completed. Responses indicated childhood and adulthood were complex and marked by joys and trials, while adolescence was experienced by many as a particularly challenging time. As adults, numerous patients experienced barriers to accessing the medical care they desired and described difficulties finding experienced and knowledgeable providers who listened and offered individualized care. This study highlights the importance of further studying this unique patient population, suggesting it can offer critical insights to inform the development of interventions and transition programs.

[Overdosing of direct oral anticoagulants]. / Überdosierung von direkten oralen Antikoagulanzien.

BACKGROUND: Direct oral anticoagulants (DOAC) are increasingly used for prophylaxis and treatment of thromboembolic events. Incorrectly dosed DOAC treatment is associated with excess mortality. PURPOSE: This article aims at raising awareness of DOAC overdosing and its causes as well as presenting a diagnostic and therapeutic work-up. MATERIAL AND METHODS: Based on a case presentation, a structured review of the current literature on DOAC overdosing was performed and treatment recommendations were extracted. RESULTS: In addition to wittingly or unwittingly increased DOAC intake, common causes of overdose are inadequate dose adjustment for concomitant medication or comorbidities. Global coagulation testing should be supplemented with DOAC-specific testing. Severe bleeding and the need for invasive diagnostics or urgent surgery represent indications for treating DOAC overdoses. Based on the cause of an DOAC overdose, active charcoal, endoscopic pill rescue, antagonization with idarucizumab or andexanet alfa and the targeted substitution of coagulation factors represent treatment options. CONCLUSION: The sensitization of clinicians is important to ensure a timely diagnosis and adequate treatment of DOAC overdosing. This report provides an overview of current knowledge on diagnostics and treatment; however, further studies are necessary to improve the existing algorithms.

Exploring the impact of pediatric short bowel syndrome on parent well-being using a disease-specific pilot survey.

BACKGROUND: Children with short bowel syndrome (SBS) have complex care needs, most of which are met in the home by family caregivers who may experience a range of stressors unique to this experience. Prior research suggests that parents of children with SBS have poorer health-related quality of life than peers parenting children without health needs, but the mechanisms shaping parent outcomes are understudied. METHODS: A pilot survey was developed using a community-driven research design to measure the impact of disease-specific items on parent-perceived well-being. The cross-sectional survey, which included both closed-ended and open-ended items, was distributed to a convenience sample of parents of children with SBS. Quantitative and qualitative data were integrated for a mixed-methods analysis of how individual items impacted parent well-being. RESULTS: Twenty parents completed the survey. Sleep interruptions, lack of support and resources, and psychological stressors and their mental health implications were more frequently reported as stressors than logistics related to caregiving (e.g., managing therapies and preparing specialized meals). CONCLUSION: The impact of a child's SBS on parent well-being may stem mainly from three interconnected domains: poor sleep and its consequences, lack of access to support and resources, and a range of psychological stressors that affect parent mental health. Understanding the mechanisms through which SBS shapes parent well-being is a necessary first step for developing targeted interventions to support parents and provide family-centered care.

A diagnostic challenge-First case of chronic lymphatic leukemia-associated necrotizing sweet syndrome.

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare disorder typically characterized by the clinical triad including a sudden onset of fever, painful skin lesions, and neutrophilia. The histopathological findings are a dense neutrophilic infiltrate and oedema of the dermis and epidermis without evidence of a vasculitis. Besides treatment of the underlying cause, sweet syndrome is typically treated with high-dose corticosteroids leading to a relapse-free response in 70% of patients. However, if left unrecognized or untreated, the condition may lead to serious complications. Here, we report on the case of a 38-year-old patient in whom, under the assumption of the presence of necrotizing fasciitis, exarticulation of the right arm was performed. In the absence of pathogen detection and insufficient response to anti-infective therapies, the diagnosis of a sweet syndrome was assumed and, later, confirmed by an excellent response to high-dose administration of systematic glucocorticoids. The case emphasizes the need to be aware of this rare syndrome, which can be easily misdiagnosed due to its close resemblance to infection and stresses the need of further research to define distinct diagnostic tools.

Advocating for a patient- and family centered care approach to management of short bowel syndrome.

Patient- and family centered care (PFCC) is a model of providing healthcare that incorporates the preferences, needs, and values of the patient and their family and is built on a solid partnership between the healthcare team and patient/family. This partnership is critical in short bowel syndrome (SBS) management since the condition is rare, chronic, involves a heterogenous population, and calls for a personalized approach to care. Institutions can facilitate the practice of PFCC by supporting a teamwork approach to care, which, in the case of SBS, ideally involves a comprehensive intestinal rehabilitation program consisting of qualified healthcare practitioners who are supported with the necessary resources and budget. Clinicians can engage in a range of processes to center patients and families in the management of SBS, including fostering whole-person care, building partnerships with patients and families, cultivating communication, and providing information effectively. Empowering patients to self-manage important aspects of their condition is an important component of PFCC and can enhance coping to chronic disease. Therapy nonadherence represents a breakdown in the PFCC approach to care, especially when nonadherence is sustained, and the healthcare provider is intentionally misled. An individualized approach to care that incorporates patient/family priorities should ultimately enhance therapy adherence. Lastly, patients/families should play a central role in determining meaningful outcomes as it relates to PFCC and shaping the research that affects them. This review highlights needs and priorities of patients with SBS and their families and suggests ways to address gaps in existing care to improve outcomes.

[Non-utilisation of medical services during the COVID-19 pandemic among persons with chronic diseases]. / Nichtinanspruchnahme medizinischer Leistungen in der COVID-19-Pandemie bei Personen mit chronischen Erkrankungen.

INTRODUCTION: The COVID-19 pandemic is also impacting the medical care for other diseases. The extent to which people with chronic diseases are affected by the suspension of medical services is investigated-differentiating between patient and provider perspectives. METHOD: A cross-sectional study was conducted based on data from the longitudinal Hamburg City Health Study (HCHS). The study population was all HCHS participants (a sample of the population of Hamburg, 45-74 years) between April 2020 and November 2021. Utilisation of medical services was collected via the "COVID-19-module" of the HCHS. The chronic conditions included cardiovascular disease, kidney and lung disease, cancer and diabetes mellitus; analyses were descriptive and multivariate. RESULTS: Of the 2047 participants, 47.9% had at least one previous illness. Of those with pre-existing conditions, 21.4% had at least one healthcare service suspended or an appointment cancelled. In addition, 15.4% stated that they decided by themselves not to attend a doctor's appointment. Specialist care services (43.8% of all cancellations) were cancelled more frequently than general practitioner care (16.6%). After adjustment for age, gender and education, lung disease (OR 1.80; p < 0.008) and cancer (OR 2.33; p < 0.001) were found to be independent risk factors for appointment cancellations by healthcare providers. Of cancellations by patients, 42.2% were due to their fear of an infection with the coronavirus SARS-CoV­2. DISCUSSION: Health policy and the media are faced with the challenge of dealing with fears of infection in the population in such a way that they do not lead to an avoidance of necessary care services.

Impact of timing and precision of histopathological diagnosis on outcomes of patients with Burkitt lymphoma and high-grade B-cell lymphoma.

BACKGROUND: Burkitt lymphoma (BL) and diffuse large B-cell lymphoma (DLBCL) are aggressive B-cell non-Hodgkin lymphomas (B-NHL) with a generally favorable prognosis after immunochemotherapy. The outcome of BL is superior to DLBCL. In 2016, a distinct group of lymphomas displaying characteristics of both BL and DLBCL (high grade B-cell lymphoma, HGBL) was introduced into the WHO classification. Histopathological discrimination of BL, DLBCL, and HGBL may be challenging. Data on the frequency of histopathological difficulties resulting in revision of the final diagnosis of BL/DLBCL/HGBL and its impact on the prognosis are limited. METHODS: We assessed histopathological features and clinical outcomes of 66 patients with suspected diagnosis of BL at the reporting institution between 2010 and 2020. RESULTS: The median age was 51 years (range 19-82) and final histopathological diagnosis revealed BL (n = 40), DLBCL (n = 12), or HGBL (n = 14). Patients with DLBCL and HGBL were either treated with DLBCL-directed (83.3% and 35.7%) or BL-directed (16.7% and 64.3%) protocols. Patients in whom diagnosis was revised from DLBCL to BL after initiation of DLBCL-directed treatment had a significantly inferior progression-free survival (PFS) than patients initially diagnosed with BL (p = 0.045), thus resembling rather the prognosis of DLBCL/HGBL. There was no difference between patients with DLBCL and HGBL, respectively, regarding PFS and OS (p = 0.38 and p = 0.27). CONCLUSION: These results suggest that timely and precise histopathological diagnosis as well as reference histopathological review of the underlying lymphoma is critical to determine up-front treatment strategies. Consequently, selection of more aggressive treatment protocols in case of difficulties with discrimination between DLBCL/HGBL/BL may be a reasonable approach.

Discipline-Specific Perspectives on Caring for Babies with Trisomy 13 or 18 in the Neonatal Intensive Care Unit.

OBJECTIVE: Care offerings vary across medical settings and between families for babies with trisomy 13 or 18. The purpose of this qualitative descriptive study was to explore nurse, advanced practice practitioner, and neonatologist perspectives on care for babies with trisomy 13 or 18 in the intensive care unit. STUDY DESIGN: Voice-recorded qualitative interviews occurred with 64 participants (41 bedside nurses, 14 advance practice practitioners, and 9 neonatologists) from two neonatal intensive care units (NICU) in the midwestern United States. Consolidated Criteria for Reporting Qualitative Research guidelines were followed. Content analyses occurred utilizing MAXQDA (VERBI Software, 2020). RESULTS: Over half of NICU staff perceived care for babies with trisomy 13 or 18 as different from care for other babies with critical chronic illness. Qualitative themes included internal conflict, variable presentation and prognosis, grappling with uncertainty, family experiences, and provision of meaningful care. Neonatologists emphasized the variability of presentation and prognosis, while nurses emphasized provision of meaningful care. Phrases "hard/difficult" were spoken 31 times; primarily describing the comorbidities, complexities, and prognostic uncertainty. CONCLUSION: Care for babies with these genetic diagnoses reveals need for a shared dialogue not only with families but also across staff disciplines. While perspectives differ, participants depicted striving to offer compassionate, family-centered care while also balancing biomedical uncertainty about interventions for children with trisomy 13 and 18. KEY POINTS: · Care for babies with trisomy 13 or 18 has been recognized as shifting.. · Controversy exists across the diverse and changing range of care models.. · This study describes perspectives of bedside neonatal nurses, advanced practitioners, and neonatologists.. · Differences in perspectives warrant attentiveness to insights and dialogue across disciplines..