Neonatal Outcomes following 2 Cases of Maternal CAR-T Therapy for High-Grade B-Cell Lymphoma.

Introduction: Chimeric antigen receptor T cells (CAR-Ts) targeting CD19 represent a significant advance in treatment for patients with relapsed/refractory B-cell malignancies. Although a significant minority of recipients are women during their reproductive years, there is a paucity of data regarding pregnancy and neonatal outcomes in women previously treated with CAR-T. This is important as maternal T cells are known to cross the placenta and into breastmilk during pregnancy and breastfeeding, respectively.

Update on hereditary C1q deficiency: pathophysiology, clinical presentation, genotype and management.

Purpose Of Review: C1q deficiency is a rare inborn error of immunity characterized by susceptibility to severe infections and profound immune dysregulation, with a systemic lupus erythematosus-like phenotype. The management of patients with C1q deficiency is challenged by the rarity of this condition and the wide clinical variability. This review aims to emphasize the importance of a thorough immunological and clinical characterization to help guide a personalized and comprehensive approach to patients.

Experience of pediatric to adult transition in immunology services: patient experience questionnaire and micro-costing analysis.

The effective transition from pediatric to adult care for individuals with chronic medical conditions should address the medical, psychosocial and educational needs of the cohort. The views and experiences of service users and their families are an integral component of service development. This study sought to evaluate the current provision of transition services from pediatric immunology services to adult immunology services for patients with a diagnosis of an inborn error of immunity at St.

Using TLC-MALDI-TOF to Interrogate In Vitro Peptidyl Proximal Preferences of PARP14 and Glycohydrolase Specificity.

The transfer of ADP-ribose (ADPr) from nicotinamide adenine dinucleotide (NAD) to target proteins is mediated by a class of human diphtheria toxin-like ADP-ribosyltransferases (ARTDs; previously referred to as poly-ADP-ribose polymerases or PARPs) and the removal of ADPr is catalyzed by a family of glycohydrolases. Although thousands of potential ADPr modification sites have been identified using high-throughput mass-spectrometry, relatively little is known about the sequence specificity encoded near the modification site. Herein, we present a matrix-assisted laser desorption/ionization time-of-flight (MALDI-TOF) method that facilitates the analysis of proximal factors that guide ARTD target selection.

Transition of Resuscitative Endovascular Balloon Occlusion of the Aorta from Zone 3 to Zone 1 to Treat Hemodynamic Collapse during Continued Hemorrhage.

Introduction: Noncompressible torso hemorrhage (NCTH) accounts for most potentially survivable deaths on the battlefield. Treatment of NCTH is challenging, especially in far-forward environments with limited capabilities. Resuscitative endovascular balloon occlusion of the aorta (REBOA) has shown promise in the care of patients with NCTH.

Inhalation Injury Severity Score on Admission Predicts Overall Survival in Burn Patients.

Inhalation injury is diagnosed in up to one-third of burn patients and is associated with increased morbidity and mortality. There are multiple scoring systems to grade inhalation injury, but no study has evaluated the ability of these scoring systems to predict outcomes of interest such as overall survival. We conducted a prospective, observational study of 99 intubated burn patients who underwent fiberoptic bronchoscopy within 24 hr of admission and graded inhalation injury using three scoring systems: abbreviated injury score (AIS), inhalation injury severity score (I-ISS), and mucosal score (MS).

Identification of a Novel PARP14 Site Motif and Glycohydrolase Specificity Using TLC-MALDI-TOF.

Transfer of ADP-ribose (ADPr) from nicotinamide adenine dinucleotide (NAD) to target proteins is mediated by a class of human poly-ADP-ribose polymerases, PARPs, and removal of ADPr is catalyzed by a family of glycohydrolases. Although thousands of potential ADPr modification sites have been identified using high-throughput mass-spectrometry, relatively little is known about sequence specificity encoded near the modification site. Herein, we present a matrix-assisted laser desorption/ionization time-of-flight (MALDI-TOF) method that facilitates the discovery and validation of ADPr site motifs.

Recent advances in graft-versus-host disease.

Acute and chronic graft-versus-host disease (GVHD) continue to present a significant challenge to physicians, accounting for considerable haematopoietic stem cell transplant (HSCT)-related morbidity and mortality, particularly those patients with steroid-refractory disease. In this review, we discuss recent advances in understanding the underlying pathophysiology, prevention and management of acute and chronic GVHD. Barriers to progress include the difficulty in obtaining high-quality evidence with sufficient patient numbers to identify optimal preventative and treatment strategies, with the heterogeneity of multiple patient, donor, graft and transplant-related factors, in addition to limited availability of human tissue to study the underlying pathophysiology, particularly in steroid-refractory disease.

Extracorporeal photopheresis as an immunomodulatory treatment modality for chronic GvHD and the importance of emerging biomarkers.

Haematopoietic stem cell transplantation (HSCT) is the treatment of choice for malignant haematological diseases. Despite continuous improvements in pre- and post-transplantation procedures, the applicability of allo-HSCT is limited by life-threatening complications such as graft-versus-host disease (GvHD), engraftment failure, and opportunistic infections. Extracorporeal photopheresis (ECP) is used to treat steroid resistant GvHD with significant success.

Systematic review of arts and culture-based interventions for people living with dementia and their caregivers.

Aims: To explore and summarize studies investigating the effect of arts and culture interventions for people living with dementia and their caregivers on the well-being and cognition of the person living with dementia and, caregiver strain.

Methods: We carried out a systematic search of five electronic databases (PubMed, PsychINFO, Embase, CINAHL, and Cochrane Library). We included original research published in peer-reviewed journals including both qualitative and quantitative studies.

Haploidentical CD3 TCR αβ/CD19-depleted HSCT for MHC class II deficiency and persistent SARS-CoV-2 pneumonitis.

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection leads to coronavirus disease 2019 (COVID-19), which can range from a mild illness to a severe phenotype characterized by acute respiratory distress needing mechanical ventilation. Children with combined immunodeficiencies might be unable to mount a sufficient cellular and humoral immune response against COVID-19 and have persistent disease.

Objective: Our aim was to describe a child with combined immunodeficiency and a favorable post-hematopoietic stem cell transplant (HSCT) course following a haploidentical HSCT in the presence of persistent SARS-CoV-2 infection.

Hematopoietic Cell Transplantation for Adenosine Deaminase Severe Combined Immunodeficiency-Improved Outcomes in the Modern Era.

Current treatment for adenosine deaminase (ADA)-deficient severe combined immunodeficiency (SCID) includes enzyme replacement therapy (ERT), allogeneic hematopoietic stem cell transplant (HSCT), or ex vivo corrected autologous hematopoietic stem cell gene therapy. Historic data show HSCT survival is superior using unconditioned matched sibling and family compared to matched unrelated and haploidentical donors. Recent improvement in HSCT outcomes prompted us to retrospectively examine HSCT survival and long-term graft function in ADA-SCID transplanted at our center.

Primary immune regulatory disorders: Undiagnosed needles in the haystack?

Primary Immune Regulatory Disorders (PIRD) describe a group of conditions characterized by loss of normal inflammatory control and immune tolerance mechanisms, with autoimmunity as a predominant clinical feature. PIRD can arise due to defects in the number or function of regulatory T-lymphocytes, defects in the immune mechanisms required to 'turn off' inflammation such as in perforin-dependent cytotoxicity or alterations in cytokine signalling pathways. Diagnosis of PIRD is a significant challenge to physicians due to their rarity, complexity, and diversity in clinical manifestations.

Thymopoiesis, Alterations in Dendritic Cells and Tregs, and Reduced T Cell Activation in Successful Extracorporeal Photopheresis Treatment of GVHD.

Acute graft-versus-host disease (aGVHD) is a significant complication of allogeneic hematopoietic stem cell transplant (HSCT) and negatively affects T cell reconstitution. Extracorporeal photopheresis (ECP) reduces aGVHD, but the mechanisms remain incompletely understood. Our objective was to examine the impact of ECP on thymopoiesis in pediatric aGVHD and the mechanisms at a cellular and transcriptional level.

A survey of extracorporeal photopheresis treatment in pediatric patients in the United Kingdom.

Extracorporeal photopheresis (ECP) is a second-line therapy in acute and chronic GVHD and solid organ transplant rejection. We report ECP use in 98 pediatric patients in seven UK centers from 2010 to 2017, the majority treated for aGVHD (73.5%).

Neonatal thymectomy in children-accelerating the immunologic clock?

The thymus is critical for central tolerance and diverse T-lymphocyte repertoire development, to provide lifelong defense against pathogens while maintaining self-tolerance. Peak thymic output occurs in utero, during infancy, and in early childhood, diminishing throughout life. Infants with congenital heart disease requiring sternotomy often undergo thymectomy to clear the surgical field.

Improved transplant survival and long-term disease outcome in children with MHC class II deficiency.

MHC class II deficiency is a rare, but life-threatening, primary combined immunodeficiency. Hematopoietic cell transplantation (HCT) remains the only curative treatment for this condition, but transplant survival in the previously published result was poor. We analyzed the outcome of 25 patients with MHC class II deficiency undergoing first HCT at Great North Children's Hospital between 1995 and 2018.

A pilot study assessing the spiritual, emotional, physical/environmental, and physiological needs of mechanically ventilated surgical intensive care unit patients via eye tracking devices, head nodding, and communication boards.

Background: Mechanically ventilated patients in the intensive care unit (ICU) are unable to communicate verbally. We sought to evaluate their needs via a communication board (CB) and a novel eye tracking device (ETD) that verbalizes selections made by gazing.

Methods: This was a pilot prospective study conducted in a tertiary care surgical ICU.

Kawasaki Disease - A Review of Treatment and Outcomes in an Irish Paediatric Cohort 2010-14.

Diagnosis of Kawasaki Disease (KD) can be challenging due to lack of a diagnostic test, and some children present with 'incomplete' KD when not all diagnostic criteria are met. Treatment with intravenous immunoglobulin (IVIG) and aspirin reduces the risk of coronary artery complications. There is sub-group of patients who are resistant to IVIG/aspirin therapy and are at increased risk of complications.