Eosinophilic Esophagitis: A Review for the Primary Care Practitioner.

This chapter presents an overview of eosinophilic esophagitis (EoE) for the Primary Care Practitioner (PCP). The focus is on helping PCPs keep it in their differential diagnosis by discussing the spectrum of clinical presentations, how to screen for EoE in at-risk populations and subsequently manage the patient with this condition. The authors review epidemiology, risk factors and associated conditions, pathology, clinical presentation, diagnosis, and management options.

Eosinophilic Esophagitis: A Review for the Primary Care Practitioner.

This chapter presents an overview of eosinophilic esophagitis (EoE) for the Primary Care Practitioner (PCP). The focus is on helping PCPs keep it in their differential diagnosis by discussing the spectrum of clinical presentations, how to screen for EoE in at-risk populations and subsequently manage the patient with this condition. The authors review epidemiology, risk factors and associated conditions, pathology, clinical presentation, diagnosis, and management options.

Impact of Penicillin Allergy Label on Clinical Outcomes of Pneumonia in Children.

BACKGROUND: Penicillin (PCN) allergy label, reported in approximately 5% of children, influences antibiotic choice and prolongs hospital stay. To our knowledge, the impact of PCN allergy label on clinical outcomes of pneumonia in children is not well characterized. OBJECTIVES: To investigate the impact of PCN allergy label on clinical outcomes of pneumonia in children. METHODS: In this propensity score-matched cohort study, we used the TriNetX research network, a population-based database, to compare the 30-day risk of hospitalization, need for intensive level of care, and acute respiratory failure from pneumonia between pediatric patients (aged 1-17 years) with and without a PCN allergy label after matching the 2 cohorts for demographic and medical comorbidities. Antibiotic prescription patterns were also contrasted. RESULTS: When comparing 3793 pediatric patients with pneumonia labeled with a PCN allergy with matched children without a PCN allergy label, PCN allergy label was associated with a higher risk of hospitalization (relative risk [RR], 1.15; 95% confidence interval [CI], 1.07-1.23), acute respiratory failure (RR, 1.27; 95% CI, 1.17-1.39), and need for intensive level of care (RR, 1.46; 95% CI, 1.15-1.84). PCN allergy label resulted in overutilization of broader-spectrum antibiotics and increased complications including cutaneous drug reactions (RR, 2.43; 95% CI, 1.31-4.52) and Clostridioides difficile infection (RR, 2.25; 95% CI, 1.14-4.44). CONCLUSION: Children with a PCN allergy label are more likely to be hospitalized, receive broader-spectrum antibiotics, and develop acute respiratory failure from pneumonia. Delabeling may offer a way to lessen morbidity from pneumonia in children.

Novel Mutation of Interferon-γ Receptor 1 Gene Presenting as Early Life Mycobacterial Bronchial Disease.

Mendelian susceptibility to mycobacterial diseases (MSMD) are a spectrum of inherited disorders characterized by localized or disseminated infections caused by atypical mycobacteria. Interferon-γ receptor 1 (IFNGR1) deficiency was the first identified genetic disorder recognized as MSMD. Mutations in the genes encoding IFNGR1 can be recessive or dominant and cause complete or partial receptor deficiency. We present the case of a 2½-year-old boy with a history of recurrent wheezing, diagnosed with endobronchial mycobacterial infection. Immunological workup revealed a homozygous nonsense mutation in the IFNGR1 gene, a novel mutation predicted in silico to cause complete IFNGR1 deficiency. This case demonstrates that (a) Interferon-γ receptor deficiency can present resembling common disorders of the lung; (b) mycobacterial infections should be suspected when parenchymal lung disease, hilar lymphadenopathy, and endobronchial disease are present; and (c) high index of suspicion for immunodeficiency should be maintained in patients with disseminated nontubercular mycobacterial infection.

Food allergies are rarely a concern when considering vaccines for adolescents.

Routine immunization provides protection from numerous infectious diseases and substantially reduces morbidity mortality from these diseases. In the United States, vaccination programs focused on infants and children have successfully decreased the incidence of many childhood vaccine-preventable diseases. However, vaccination coverage among adolescents has remained stagnant. Contributing to this lack of coverage is that patients with food allergies might be advised unnecessarily to avoid certain vaccinations, thus potentially causing adverse personal and community health. Studies have shown that food allergies are rarely contraindications to vaccine administration. Most adolescents who avoid vaccination because of food allergy concerns are actually able to receive their appropriate vaccinations. However, there are situations when evaluation by an allergist is recommended. In the present article, the authors provide guidance for physicians when administering vaccines to patients with food allergies to prevent adverse events and improve disease protection.

Residual NADPH oxidase activity and isolated lung involvement in x-linked chronic granulomatous disease.

Chronic granulomatous disease (CGD) is characterized by inherited immune defects resulting from mutations in the NADPH oxidase complex genes. The X-linked type of CGD is caused by defects in the CYBB gene that encodes gp91-phox, a fundamental component of the NADPH oxidase complex. This mutation originates the most common and severe form of CGD, which typically has absence of NADPH oxidase function and aggressive multisystemic infections. We present the case of a 9-year-old child with a rare CYBB mutation that preserves some NADPH oxidase activity, resulting in an atypical mild form of X-linked CGD with isolated lung involvement. Although the clinical picture and partially preserved oxidase function suggested an autosomal recessive form of CGD, genetic testing demonstrated a mutation in the exon 3 of CYBB gene (c.252 G>A, p.Ala84Ala), an uncommon X-linked CGD variant that affects splicing. Atypical presentation and diagnostic difficulties are discussed. This case highlights that the diagnosis of mild forms of X-linked CGD caused by rare CYBB mutations and partially preserved NADPH function should be considered early in the evaluation of atypical and recurrent lung infections.

Infants with low immunoglobulin levels: Isolated low IgA level vs other immunoglobulin abnormalities.

BACKGROUND: The International Union of Immunological Societies defined transient hypogammaglobulinemia of infancy as decreased IgG and IgA levels. Some others, however, include decreased IgA level alone. We compared infants with decreased levels of IgG and IgA, all isotypes, and IgA alone. OBJECTIVE: To determine whether infants presenting with diminished IgA only differ clinically and in time of immunoglobulin recovery, from those with decreased levels of IgG and IgA, or of all major isotypes. METHODS: Eighty-seven term infants found to have immunoglobulin isotype(s) 2 or more SDs below mean, normal antibody response, intact cellular immunity, and absence of other immunodeficiency syndrome features were evaluated between January 1, 1977 and December 31, 2008. Infants had decreased IgA level (group 1, n = 43), decreased IgA and IgG levels (group 2, n = 39), or low IgA, IgG, and IgM levels (group 3, n = 5). RESULTS: Groups had similar histories. Immunoglobulins normalized in a similar percentage of all groups during infancy but earlier for group 1 (P = .005). CONCLUSION: Little reason exists to separate infants with isolated decreased IgA levels from those with decreased levels of IgA and IgG or all isotypes.

Correlates of outcome for atopic dermatitis.

BACKGROUND: The worldwide incidence and prevalence of atopic dermatitis (AD) are increasing. Few good studies have addressed AD in terms of the factors affecting disease prognosis. OBJECTIVE: To identify significant correlates of persistent AD because this would be clinically valuable information. METHODS: Potential correlates of AD, including race, onset age, age of solid food introduction, breastfeeding, sinopulmonary infections, other atopic diseases, peripheral eosinophilia, total IgE level, and eosinophilic cationic protein levels, were investigated in 177 patients aged 5 to 18 years. Correlates were compared with AD remission vs nonremission status. RESULTS: A total of 133 patients (75.1%) were not in remission at the age of 5 years or older and were, thus, classified as having persistent AD. Patients with histories of peanut allergy (odds ratio [OR], 2.92; 95% confidence interval [CI], 1.30-6.55), egg allergy (OR, 2.71; 95% CI, 1.17-6.30), or dust mite allergy (OR, 4.02; 95% CI, 1.84-8.82) were significantly more likely to have persistent AD than those without these factors. There was a trend toward increased odds of persistence in those with peripheral eosinophilia (P = .06) and decreased odds of persistence in those with frequent sinopulmonary infections (OR, 0.51; 95% CI, 0.25-1.03). CONCLUSIONS: Egg, peanut, and dust mite allergies are significant correlates of AD persisting beyond school age. There may also be increased odds in those with peripheral eosinophilia and decreased odds in those with frequent sinopulmonary infections. This highlights the importance of assessing these correlates in patients with AD and modifying the correlates that can be modified. Further studies on whether modification of these correlates and/or early aggressive AD management improves outcome are needed.