Small but mighty: Case report and practical guidance for peripheral blood stem cell collection in small infants.

Modern apheresis devices, with increased procedural precision, automation, and monitoring, have been shown to allow for safe delivery of apheresis therapies in young children. Medical advances are increasing demand for apheresis procedures like mononuclear cell collection in infants <10 kg, including stem-cell supported chemotherapy, cell collection for chimeric antigen receptor T cell development, and now ex vivo gene therapies for rare genetic diseases. Nevertheless, safe delivery in small infants involves a range of unique considerations and challenges, beyond just size, and experience will vary between centers.

Rapid Critical Care Training for Nurses Deployed to Intensive Care Units During the COVID-19 Surge.

Background: During the first COVID-19 pandemic wave, non-intensive care unit (non-ICU) nurses were deployed to temporary ICUs to provide critical care for the patient surge. A rapid critical care training program was designed to prepare them to care for patients in either temporary or permanent ICUs.

Objective: To evaluate the effectiveness of this training program in preparing non-ICU nurses to provide critical care for COVID-19 patients in temporary ICUs.

Guiding warfarin self-management in children: development of a warfarin nomogram.

Background: Warfarin therapy in children is impacted by many variables. To support the implementation of a self-management program within a pediatric anticoagulation service, a pediatric-specific warfarin nomogram was needed. A literature review revealed no published pediatric nomograms; therefore, a nomogram was developed drawing upon an evidence-based "Warfarin Information for Clinicians" hospital guideline.

Sickle cell disease in Australia: a snapshot from the Australian Haemoglobinopathy Registry.

Background: Sickle cell disease (SCD) is the most common monogenic disorder worldwide. In deoxygenated conditions, the altered beta chain (haemoglobin S [HbS]) polymerises and distorts the erythrocyte, resulting in pain crises, vasculopathy and end-organ damage. Clinical complications of SCD cause substantial morbidity, and therapy demands expertise and resources.

Common pathway coagulopathy and hemorrhagic edema of infancy.

When screening tests of haemostasis are abnormal, it is important to identify at which point in the coagulation cascade dysfunction may be occurring. This may assist to identify a specific deficiency/dysfunction, the type of bleeding to be anticipated, and replacement therapy if required. Unmasking of an inherited coagulopathy or the development of an acquired coagulopathy may occur in the setting of a second (febrile) illness.

Defining global strategies to improve outcomes in sickle cell disease: a Lancet Haematology Commission.

All over the world, people with sickle cell disease (an inherited condition) have premature deaths and preventable severe chronic complications, which considerably affect their quality of life, career progression, and financial status. In addition, these people are often affected by stigmatisation or structural racism, which can contribute to stress and poor mental health. Inequalities affecting people with sickle cell disease are also reflected in the distribution of the disease—mainly in sub-Saharan Africa, India, and the Caribbean—whereas interventions, clinical trials, and funding are mostly available in North America, Europe, and the Middle East.

Using an electronic medical record patient portal for warfarin self-management: Empowering children and parents.

Background: Many children taking warfarin perform their international normalized ratio (INR) at home, with results phoned to a clinician who instructs warfarin dosing. Data suggest that parents can be supported to make warfarin dosing decisions themselves, a process known as patient self-management (PSM).

Objectives: This study aimed to determine the suitability and acceptability of warfarin PSM in children using the Epic Patient Portal.

Stimulation of CGRP-expressing neurons in the medial cerebellar nucleus induces light and touch sensitivity in mice.

Calcitonin gene-related peptide (CGRP) is considered a major player in migraine pathophysiology. However, the location and mechanisms of CGRP actions in migraine are not clearly elucidated. One important question yet to be answered is: Does central CGRP signaling play a role in migraine? One candidate site is the cerebellum, which serves as a sensory and motor integration center and is activated in migraine patients.

Geriatric Burn Injuries Presenting to the Emergency Department of a Major Burn Center: Clinical Characteristics and Outcomes.

Background: Burn injuries in geriatric patients are common and may have significant associated morbidity and mortality. Most research has focused on the care of hospitalized patients after admission to burn units. Little is known about the clinical characteristics of geriatric burn victims who present to the emergency department (ED) and their ED assessment and management.

Effects of dual chelation therapy with deferasirox and deferoxamine in patients with beta thalassaemia major.

Background And Objectives: Patients with thalassaemia experience complications related to iron overload. In Australia currently, the two main options for iron chelation are deferasirox and deferoxamine. Optimal iron chelation using monotherapy can be limited due to toxicity or tolerability.

Skin Failure Among Critically Ill Patients Afflicted with Coronavirus Disease 2019 (COVID-19).

To characterize skin integrity among coronavirus disease 2019 (COVID-19) patients treated in the intensive care unit (ICU), and identify risk factors for skin failure (SF) in these patients. The characteristic, profound pro-inflammatory, hypercoagulable state of COVID-19 is manifested by the high severity of illness and extensive organ dysfunction observed in these patients. SF in critically ill patients, although described previously, exhibits a uniquely complex pathogenesis in this population.

Multisystem inflammatory syndrome in children: A complication of COVID-19.

Multisystem inflammatory syndrome in children is a previously unrecognized and potentially catastrophic illness that appears in children who have been exposed to or diagnosed with COVID-19. As healthcare agents and members of the community, nurses are positioned to assist in identifying children who may experience previously unrecognized complications of infection from the SARS-CoV-2 virus.

Maternal thiopurine metabolism during pregnancy in inflammatory bowel disease and clearance of thiopurine metabolites and outcomes in exposed neonates.

Background: Azathioprine and mercaptopurine are considered safe during pregnancy. However, the pharmacokinetic effects of pregnancy on thiopurine metabolism are undefined.

Aims: To characterise thiopurine metabolism in pregnancy and measure infant metabolite levels and outcomes.

International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease.

Castleman disease (CD) includes a group of rare and heterogeneous disorders with characteristic lymph node histopathological abnormalities. CD can occur in a single lymph node station, which is referred to as unicentric CD (UCD). CD can also involve multicentric lymphadenopathy and inflammatory symptoms (multicentric CD [MCD]).

Exploring self-management within hospital-based stroke care: current and future opportunities.

Purpose: This study explored stroke self-management within a tertiary hospital setting from the perspectives of health professionals working across the continuum of stroke care.

Materials And Methods: A qualitative descriptive design guided five focus groups in the acute stroke service ( = 2), inpatient rehabilitation ( = 2), and outpatient day hospital service ( = 1). Focus groups were transcribed verbatim and analysed using thematic analysis.

Rapid Critical Care Training of Nurses in the Surge Response to the Coronavirus Pandemic.

Background: In response to the coronavirus pandemic, New York State mandated that all hospitals double the capacity of their adult intensive care units In this facility, resources were mobilized to increase from 104 to 283 beds.

Objective: To create and implement a 3-hour curriculum to prepare several hundred non-critical care staff nurses to manage critically ill patients with coronavirus disease 2019.

Methods: Critical care nursing leaders and staff developed and implemented a flexible critical care nursing curriculum tailored to the diverse experience, expertise, and learning needs of non-critical care nursing staff who were being redeployed to critical care units during the surge response to the pandemic.

Rapid Identification of Biallelic Mutation in a Neonate with Severe Congenital Hemolytic Anemia and Liver Failure.

Heterozygous pathogenic variants in cause autosomal dominant hereditary spherocytosis, an important cause of neonatal nonimmune hemolytic anemia. Biallelic mutations are rarely reported, all with severe neonatal presentation. We describe rapid (68 h) genomic diagnosis of homozygous β-spectrin deficiency in a newborn with severe transfusion-dependent hemolytic anemia, conjugated hyperbilirubinemia, and progressive liver failure.

Utility of clinical comprehensive genomic characterization for diagnostic categorization in patients presenting with hypocellular bone marrow failure syndromes.

Bone marrow failure (BMF) related to hypoplasia of hematopoietic elements in the bone marrow is a heterogeneous clinical entity with a broad differential diagnosis including both inherited and acquired causes. Accurate diagnostic categorization is critical to optimal patient care and detection of genomic variants in these patients may provide this important diagnostic and prognostic information. We performed real-time, accredited (ISO15189) comprehensive genomic characterization including targeted sequencing and whole exome sequencing in 115 patients with BMF syndrome (median age 24 years, range 3 months - 81 years).

New frontiers in antimicrobial leadership: an interview with Joan Butterton.

The Editor of Future Microbiology speaks to Joan R Butterton, M.D., Associate Vice President and Section Head for Antibacterials and CMV for the Infectious Disease therapeutic area at Merck & Co.

The birth of viromics: an interview with Forest Rohwer.

The editor of Future Microbiology, Alice Greenway, speaks to Forest Rohwer from San Diego State University (CA, USA) following his talk entitled 'Counting all the world's phage' at ASM Microbe 7-11 June 2018.

Anti-infectives derived from botanical natural products: an interview with Cassandra Quave.

The editor of Future Microbiology, Alice Greenway, speaks to Cassandra Quave from Emory University (GA, USA) following her talk entitled 'Discovery of Anti-infectives and Virulence Inhibitors from Botanical Natural Products' at American Society for Microbiology (ASM) Microbe 2018 (7-11 June).

International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease.

Castleman disease (CD) describes a group of heterogeneous hematologic disorders with characteristic histopathological features. CD can present with unicentric or multicentric (MCD) regions of lymph node enlargement. Some cases of MCD are caused by human herpesvirus-8 (HHV-8), whereas others are HHV-8-negative/idiopathic (iMCD).

Spore-forming bacteria in the gut: an interview with Hilary Browne.

Hilary Browne speaks to Alice Greenway, Commissioning Editor of Future Microbiology, at the Microbiology Society Annual Conference 2018. Hilary Browne is a Staff Scientist at the Wellcome Sanger Institute in Cambridge (UK). His research focuses on culturing, curating and whole genome sequencing of the human gut microbiota and using these resources to study the human intestinal microbiota.