A second look at secondary hypogammaglobulinemia.

Hypogammaglobulinemia is defined as a reduced immunoglobulin level, which can be either primary due to inborn errors of immunity or acquired in the setting of poor antibody production or increased antibody loss. Secondary hypogammaglobulinemia (SHG) should be considered in patients with a history of immunosuppressive therapy, transplant, protein loss syndromes, certain autoimmune conditions, and malignancies, as it can be associated with increased infectious risk. Appropriate history and lab-based screening in these populations can identify SHG allowing treatment and close monitoring as appropriate.

Autoimmunity in people with cystic fibrosis.

Cystic fibrosis (CF) clinicians may see patients who have difficult-to-manage symptoms that do not have a clear CF-related etiology, such as unusual gastrointestinal (GI) complaints, vasculitis, or arthritis. Alterations in immunity, inflammation and intraluminal dysbiosis create a milieu that may lead to autoimmunity, and the CF transmembrane regulator protein may have a direct role as well. While autoantibodies and other autoimmune markers may develop, these may or may not lead to organ involvement, therefore they are helpful but not sufficient to establish an autoimmune diagnosis.

Frequency of Infant Egg Consumption and Risk of Maternal-Reported Egg Allergy at 6 Years.

Background: Recent research suggests that early egg introduction during infancy may help to prevent egg allergy development. However, the infant egg consumption frequency that is sufficient to induce this immune tolerance remains uncertain.

Objectives: We examined the associations between the infant egg consumption frequency and maternal-reported child egg allergy at 6 y.

Egg Introduction during Infancy is Associated with Lower Fat Mass Index in Females at Early Adolescence.

Background: Egg consumption may play an important role in early-life growth given their high-quality protein, essential fatty acids, and micronutrients.

Objectives: Study objectives were to examine the longitudinal associations of infant age at egg introduction with obesity outcomes in early childhood, middle childhood (mid-childhood), and early adolescence.

Methods: We used existing data from 1089 mother-child dyads from Project Viva to estimate age at egg introduction through a questionnaire completed by mothers at ∼1 y postpartum (mean ± SD, 13.

PEGylated Recombinant Adenosine Deaminase Maintains Detoxification and Lymphocyte Counts in Patients with ADA-SCID.

Purpose: Metabolic detoxification with enzyme replacement therapy (ERT) promotes immune recovery in patients with adenosine deaminase (ADA)-deficient severe combined immunodeficiency (ADA-SCID). Elapegademase is a PEGylated recombinant bovine ADA ERT developed to replace the now-discontinued bovine-derived pegademase. This study was a 1-way crossover from pegademase to elapegademase in 7 patients with ADA-SCID to assess efficacy and safety outcomes for elapegademase.

The Impact of Caregiver Depression on Child Asthma Outcomes: Pathways and Mechanisms.

Background: Depression is common in caregivers of children with asthma and is associated with poor outcomes in their child. No prior studies have longitudinally examined caregiver depression remission as a predictor of improvement in child asthma control.

Objective: This 2-site study examined whether the proportion of time a caregiver was in depression remission predicted subsequent child asthma control at exit.

Trends and Patterns of Social History Data Collection Within an Electronic Health Record.

There is increased acceptance that social and behavioral determinants of health (SBDH) impact health outcomes, but electronic health records (EHRs) are not always set up to capture the full range of SBDH variables in a systematic manner. The purpose of this study was to explore rates and trends of social history (SH) data collection-1 element of SBDH-in a structured portion of an EHR within a large academic integrated delivery system. EHR data for individuals with at least 1 visit in 2017 were included in this study.

Endovascular Repair of Thoracic Aortic Aneurysm Using Transcaval Aortic Access Technique.

Repair of thoracic aortic aneurysms has evolved in recent history. Open surgical intervention has largely been replaced with endovascular intervention. However, there are anatomic limitations to thoracic endovascular aneurysm repair.

Delayed egg introduction beyond infancy and increased egg allergy risk in childhood.

Aim: Egg is a major food allergen in childhood. Recent studies suggest that early introduction of allergenic foods can decrease the risk of developing egg allergy. The impact of early egg introduction in the general population is unclear.

Infant age at egg introduction and malnutrition-related child growth in the United States.

To evaluate the relationship between infant age of egg introduction and malnutrition-related growth outcomes in the United States, we analysed secondary data of 1716 mother-child dyads in the Infant Feeding Practices Study II and its Year 6 Follow-Up Study. Malnutrition-related growth outcomes included body mass index z-score (BMIZ), obesity (weight-for-height z-score [WHZ] ≥3 or BMIZ ≥ 2), WHZ, wasting (WHZ < -2), height-for-age z-score (HAZ), and stunting (HAZ < -2). Infant age at egg introduction was analysed as a continuous variable.

Feasibility of an Opioid Sparing Discharge Protocol Following Laparoscopic Bariatric Surgery.

Background: Opioids are commonly prescribed after laparoscopic bariatric surgery but have untoward effects including dependence and diversion. Prior investigation revealed that over three-fourths of discharge opioids prescribed to our patients went unused.

Objectives: To determine the feasibility of an opioid sparing discharge protocol following laparoscopic bariatric surgery.

Practical guidance for the diagnosis and management of secondary hypogammaglobulinemia: A Work Group Report of the AAAAI Primary Immunodeficiency and Altered Immune Response Committees.

Secondary hypogammaglobulinemia (SHG) is characterized by reduced immunoglobulin levels due to acquired causes of decreased antibody production or increased antibody loss. Clarification regarding whether the hypogammaglobulinemia is secondary or primary is important because this has implications for evaluation and management. Prior receipt of immunosuppressive medications and/or presence of conditions associated with SHG development, including protein loss syndromes, are histories that raise suspicion for SHG.

Respiratory Infections in Patients with Primary Immunodeficiency.

Recurrent and life-threatening respiratory infections are nearly universal in patients with primary immunodeficiency diseases (PIDD). Early recognition, aggressive treatment, and prophylaxis with antimicrobials and immunoglobulin replacement have been the mainstays of management and will be reviewed here with an emphasis on respiratory infections. Genetic discoveries have allowed direct translation of research to clinical practice, improving our understanding of clinical patterns of pathogen susceptibilities and guiding prophylaxis.

Lymphadenitis Following Infliximab Therapy for Inflammatory Bowel Disease in a Patient With Chronic Granulomatous Disease: Case Report and Literature Review.

Chronic granulomatous disease (CGD) is an inborn error of immunity caused by inactivating genetic mutations in any one of the components of the phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Phagocytic cell reactive oxygen species generation is impaired in the absence of a functional NADPH oxidase complex. As a result, patients with CGD are at high risk of developing deep-seated infections with certain bacteria and fungi.

Eosinophilic Esophagitis.

Eosinophilic esophagitis is a recently defined condition that has dramatically increased in prevalence in the last several decades. It may occur at any age, but the clinical presentation in young children is often more vague than the classic solid food dysphagia and food impacting that are the major presenting symptoms of eosinophilic esophagitis in adults and adolescents. Successful therapies exist, including medications and dietary modifications, but disease typically recurs when the intervention is discontinued.

Achenbach syndrome as a rare cause of painful, blue finger.

Paroxysmal finger hematoma, also known as Achenbach syndrome, is an underdiagnosed condition that causes apprehension in patients owing to the alarming appearance. It usually presents as a blue-purple discoloration of the volar aspect of one or more digits and can be associated with pain and paresthesia. This condition is benign and is usually self-limiting.

Multivariate Association of Child Depression and Anxiety with Asthma Outcomes.

Background: Pediatric asthma is associated with increased health services utilization, missed school days, and diminished quality of life. Children with asthma also report more frequent depressive and anxiety symptoms than children without asthma, which may further worsen asthma outcomes.

Objective: The current study investigated the relationship between depressive and anxiety symptoms and 4 asthma outcomes (asthma control, asthma severity, lung function, and asthma-related quality of life) in children (N = 205) with moderate to severe persistent asthma.

A pilot school-based health center intervention to improve asthma chronic care in high-poverty schools.

Objective: To test the feasibility and effectiveness of a multifaceted intervention administered through school-based health centers (SBHCs) to improve asthma control for children in high-poverty schools with not well controlled asthma.

Methods: Students 4-14 years old with persistent asthma were enrolled from three SBHCs. The centers' advanced practice providers received training on evidence-based asthma guidelines.

Twist2 is NFkB-responsive when p120-catenin is inactivated and EGFR is overexpressed in esophageal keratinocytes.

Esophageal squamous cell carcinoma (ESCC) is among the most aggressive and fatal cancer types. ESCC classically progresses rapidly and frequently causes mortality in four out of five patients within two years of diagnosis. Yet, little is known about the mechanisms that make ESCC so aggressive.

Loss of p120ctn causes EGFR-targeted therapy resistance and failure.

Epidermal growth factor receptor (EGFR) plays a vital role in cell division and survival signaling pathways. EGFR is activated in nearly every cancer type, and its high expression in tumors is correlated with poor patient outcome. Altogether, EGFR is a prime candidate as a therapeutic target.

North American Propolis Extracts From Upstate New York Decrease () Spore Levels in Honey Bees ().

infections in honey bees () pose a severe threat to colony health. Beekeepers have used dicyclohexylammonium fumagillin to control , although it may be ineffective against . We investigated the ability of various propolis extracts collected from Upstate New York (United States) to decrease infection levels when fed to -infected honey bees.