Clinical Manifestation of Hyper IgE Syndrome Including Otitis Media
AbstractPurpose of ReviewThe hyper IgE syndromes (HIES) comprise a group of rare primary immunodeficiency disorders (PIDDs), which are characterized by extremely high serum IgE levels, eczema, recurrent skin and pulmonary infections. Both autosomal dominant (AD) HIES due to STAT3 mutations and autosomal recessive (AR) HIES due to PGM3, SPINK5, DOCK8 and TKY2 mutations have been reported. Here, we aim to summarize and compare the major clinical manifestations of different subtypes of HIES. We will also discuss otitis media, which usually do not get enough attention in HIES. Update and familiarity with these clinical features will help to make a better diagnose, assessment and treatment of HIES.Recent FindingsAlthough hyper serum IgE levels have been identified in PGM3 deficiency and Comel –Netherton syndrome,PGM3 andSPINK5 genes were not included in the list of genetic etiologies of AR-HIES by the Expert Committee of the International Union of Immunological Societies until 2015. The identification of these HIES-causing genes greatly promoted the pathogenic mechanism studies of HIES. Also, in recent years, more clinical manifestations, which were often not of concern in HIES patients, have been shown to be highly related to HIES. For example, a significantly high frequency of vascular and gastrointestinal abnormities has been reported in STAT3-deficient AD-HIES patients. These new findings might help to provide new clues to the functional study of these HIES-related genes...
Publication date: Available online 19 September 2019Source: The LancetAuthor(s): Claus Bachert, Joseph K Han, Martin Desrosiers, Peter W Hellings, Nikhil Amin, Stella E Lee, Joaquim Mullol, Leon S Greos, John V Bosso, Tanya M Laidlaw, Anders U Cervin, Jorge F Maspero, Claire Hopkins, Heidi Olze, G Walter Canonica, Pierluigi Paggiaro, Seong H Cho, Wytske J Fokkens, Shigeharu Fujieda, Mei ZhangSummaryBackgroundPatients with chronic rhinosinusitis with nasal polyps (CRSwNP) generally have a high symptom burden and poor health-related quality of life, often requiring recurring systemic corticosteroid use and repeated sinus sur...
Conclusions: The PHiD-CV vaccination program in Brazil has resulted in important reductions of pneumococcal disease and substantial cost savings. Instead of switching PCVs, expanding vaccine coverage or investing in other health care interventions would be a more efficient use of resources to improve the health of the population in Brazil.
This study retrospectively analyzed the case records of 940 formula-fed cow's milk allergic infants in the UK extracted from The Health Improvement Network database. Results: At 24 months after starting a formula, 77% of infants fed an extensively hydrolyzed casein formula supplemented with Lactobacillus rhamnosus GG (eHCF LGG; n = 470) were estimated to have been successfully managed compared with 63% of matched infants who were fed an extensively hydrolyzed whey formula (eHWF; n = 470; p
Date: Wednesday, 10 30, 2019; Speaker: Pamela A. Guerrerio, MD, PhD, Chief, Food Allergy Research Unit, , Laboratory of Allergic Diseases, NIAID; Building: Building 10 (Clinical Center); Lipsett Amphitheater; CME Credit; Videocast Event
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Conclusion: Behavioral interventions for asthma management in SAs are effective. Educational interventions that aim to optimize asthma knowledge, control, and inhaler technique, and yoga/breathing exercises are most effective for improved long-term outcomes in adults and children across LICs and MICs. Further research is needed to evaluate the effectiveness of all behavioral interventions for SAs in HICs to better inform current guidance by policy makers and health care providers. PMID: 31532264 [PubMed - as supplied by publisher]
This study investigated the clinical utility and efficiency of MCT at a tertiary center in evaluating pediatric asthma. Methods: Retrospective chart review was done for all MCT done at a tertiary center over a six year period (2011-2017). Demographics, referring provider, referral diagnosis, current symptoms with and without exercise, and baseline spirometry were collected. Pretest probability of asthma was assigned by author (RB) who was blinded to MCT results and PC20. Post-test probability of asthma was assigned based on pretest probability, MCT result (+/-), and PC20. Three assigned asthma probability categories were &...