Infantile systemic hyalinosis (ISH) is a rare disorder characterized by hyaline deposition in the skin as well as visceral organs. Though commonly associated with a protein losing enteropathy (PLE) and loss of immunoglobulins the profile of cellular and humoral branches of the immune system has not previously been described. We describe the immune system deficits in a case of ISH associated with PLE and infections. A history of multiple infections prompted an immunocompetency evaluation in a patient with ISH and PLE. Low immunoglobulin G (IgG), poorly protective pneumococcal titers, and nonresponse to Candida on lymphocyte transformation in the face of increasing infections lead to the diagnosis of a combined immunodeficiency. This is the first case report detailing quantitative and qualitative deficits of the humoral and cellular branches of the immune system in a patient with ISH. The understanding of the different aspects of this disease including the immune deficits impacts not only prognosis but also end-of-life decisions as well.
Varicella zoster virus (VZV) infection, early in life, has been shown to have a protective role against the development of asthma and atopy in children. It is unknown whether VZV infection in late childhood and adolescence also has an asthma protective effect. We studied whether VZV infection in late childhood and adolescence, or administration of the live attenuated varicella vaccine, provides a similar protective effect. In the present study, we use a retrospective chart review of a pediatric practice (N = 62) to analyze the effect of wild-type (WT) VZV infection or recombinant varicella vaccine in children (age 3–21 years) diagnosed with asthma. We found (1) asthma onset occurred 9.4 ± 4.4 years after WT VZV infection (4.4 ± 2.2) by age 13.9 ± 6.5 years, but only 3.0 ± 1.7 years after recombinant vaccine (3.2 ± 3.0 years) at age 5.0 ± 3.6 years (P = 0.05); (2) asthma classifications were 61.5% intermittent, 15.4% mild persistent, and 23.1% moderate persistent after WT VZV infection, but were 30.0% intermittent, 36.7% mild persistent, and 33.3% moderate persistent after recombinant vaccine (α = 0.01); (3) severe asthma flare-ups requiring inhaled or oral corticosteroids either ≤ 1 year or > 1 year after infection occurred in 0.0% and 30.8% patients after WT infection, and 21.4% and 48.3% after recombinant vaccine (α = 0.01); and (4) history of allergies, necessity for allergy referral, and elevated serum IgE levels occurred in 15.4%, 7.7%, and 15.4% of patients who had WT VZV infection before asthma onset, and 36.7%, 33.3%, and 26.7% of patients who received recombinant vaccine (α = 0.01). These studies demonstrate that a single episode of varicella infection, even in late childhood and adolescence, is sufficient to delay the onset of asthma symptoms and decrease asthmatic symptoms and flare-ups.
Pediatric Asthma, Allergy & ImmunologyVol. 22, No. 1 Pharmacotherapy UpdateEpisodic Viral Wheezing in Preschool Children: What Works?H. William KellyPharm.D.H. William KellyPharm.D.Search for more papers by this authorPublished Online:1 May 2009https://doi.org/10.1089/pai.2009.2201.phAboutSectionsPDF/EPUB Permissions & CitationsPermissionsDownload CitationsTrack CitationsAdd to favorites Back To Publication ShareShare onFacebookTwitterLinked InRedditEmail FiguresReferencesRelatedDetails Volume 22Issue 1Mar 2009 Information© Mary Ann Liebert, Inc.To cite this article:H. William KellyPharm.D..Episodic Viral Wheezing in Preschool Children: What Works?.Pediatric Asthma, Allergy & Immunology.Mar 2009.39-42.http://doi.org/10.1089/pai.2009.2201.phPublished in Volume: 22 Issue 1: May 1, 2009Online Ahead of Print:April 8, 2009PDF download
Pediatric Asthma, Allergy & ImmunologyVol. 22, No. 3 PHARMACOTHERAPY UPDATEBronchodilators in Bronchiolitis: Do They Work?H. William KellyPharmDH. William KellyPharmDAddress correspondence to:H. William Kelly, PharmDDepartment of PediatricsUniversity of New MexicoMSC10-55901Albuquerque, NM 87131-0001E-mail:hwkelly@salud.unm.eduSearch for more papers by this authorPublished Online:14 Dec 2009https://doi.org/10.1089/pai.2009.2203.phAboutSectionsView articleView Full TextPDF/EPUB ToolsPermissionsDownload CitationsTrack CitationsAdd to favorites Back To Publication ShareShare onFacebookTwitterLinked InRedditEmail View articleAbstractFiguresReferencesRelatedDetails Volume 22Issue 3Sep 2009 Information© Mary Ann Liebert, Inc.To cite this article:H. William KellyPharmD.Bronchodilators in Bronchiolitis: Do They Work?.Pediatric Asthma, Allergy & Immunology.Sep 2009.131-132.http://doi.org/10.1089/pai.2009.2203.phPublished in Volume: 22 Issue 3: December 14, 2009Online Ahead of Print:November 29, 2009PDF download
We report a previously healthy infant presenting with cytomegalovirus (CMV) pneumonia and Pneumoncystis jiroveci (PJP) pneumonia. Immune suppression was primarily caused by perinatal CMV infection with a significant contribution by systemic steroid therapy. The potential role of CMV infection in the development of (usually transient) immunodeficiency in young children is reviewed. The complex interaction of CMV infection and the immune effects of steroids with host defense in young children are reviewed. Other factors that may contribute to immune suppression or affect outcome in dual infection with CMV and PJP are discussed.
Pediatric Asthma, Allergy & ImmunologyVol. 22, No. 1 Letter to the EditorType I Hypersensitivity Reaction to Ingestion of Mycoprotein (Quorn) in a Patient With Mold AllergyManbir Singh SandhuM.D. and Russell J. HoppD.O., FAAAAI, FAAPManbir Singh SandhuM.D. and Russell J. HoppD.O., FAAAAI, FAAPPublished Online:1 May 2009https://doi.org/10.1089/pai.2008.0518AboutSectionsPDF/EPUB Permissions & CitationsPermissionsDownload CitationsTrack CitationsAdd to favorites Back To Publication ShareShare onFacebookXLinked InRedditEmail "Type I Hypersensitivity Reaction to Ingestion of Mycoprotein (Quorn) in a Patient With Mold Allergy." , 22(1), pp. 5–6FiguresReferencesRelatedDetailsCited byFungi as a Source of Edible Proteins and Animal Feed3 January 2023 | Journal of Fungi, Vol. 9, No. 1Safety of Alternative Proteins: Technological, Environmental and Regulatory Aspects of Cultured Meat, Plant-Based Meat, Insect Protein and Single-Cell Protein28 May 2021 | Foods, Vol. 10, No. 6Self-reported adverse reactions associated with mycoprotein (Quorn-brand) containing foods Michael F. Jacobson, PhD, Center for Science in the Public InterestAnnals of Allergy, Asthma & ImmunologyFood allergy: A practice parameter update—2014Journal of Allergy and Clinical Immunology, Vol. 134, No. 5 Volume 22Issue 1Mar 2009 Information© Mary Ann Liebert, Inc.To cite this article:Manbir Singh SandhuM.D. and Russell J. HoppD.O., FAAAAI, FAAP.Type I Hypersensitivity Reaction to Ingestion of Mycoprotein (Quorn) in a Patient With Mold Allergy.Pediatric Asthma, Allergy & Immunology.Mar 2009.5-6.http://doi.org/10.1089/pai.2008.0518Published in Volume: 22 Issue 1: May 1, 2009Online Ahead of Print:April 8, 2009 TopicsAllergy and hypersensitivity PDF download
Asthma is the most common chronic illness of childhood and leading cause of pediatric hospitalization. Patient/parent adherence rates to medications and home-management recommendations are low. The provision of incentives for adherence to parents has had limited consideration. The objective of this study was to assess physician views regarding appropriateness and effectiveness of incentives for adherence in the management of childhood asthma. We performed a cross-sectional, web-based anonymous survey of Pennsylvania pediatricians and specialists caring for children with asthma. Three hundred and twenty-nine physicians responded, reflecting demographic characteristics of the state’s physicians. Overall, 61% agreed incentives for adherence would be appropriate, 77% agreed they would be effective, and 50% agreed they would be both appropriate and effective. Among 287 respondents supporting incentives as appropriate and/or effective, 4 activities were endorsed by a majority for linkage to incentives: scheduled asthma checkups (85%), annual influenza shot (78%), refill of controller medicines (71%), and proven adherence to controllers with electronic monitoring devices (58%). Refund of co-payments was the most supported method to deliver incentives. A minority of all respondents agreed with statements that incentives would threaten patient/parent autonomy (15%), undercut social fairness (20%), or interfere with patient–doctor relationships (20%). Bivariate analysis did not reveal significant differences in reported attitudes by physician demographic or practice characteristics. Comments from respondents revealed a wide range of opinions about the role incentives in childhood asthma. Respondents were mostly open-minded to incentives for adherence in childhood asthma, though some respondents had concerns about the specific program design and implementation. These findings should be linked with surveys of other clinicians and parents in helping with a pilot to evaluate the real-world ability of incentives to improve adherence and outcomes in childhood asthma.
Allergic rhinitis (AR) is a common chronic condition. Recent studies raise the possible relationship between allergic conditions and sensory modulation disorders, mainly expressed in sensory hypersensitivity. This study aimed to evaluate sensory modulation abilities of children with AR and to assess whether these abilities have stronger expression in specific sensory systems, compared to healthy peers. Twenty-eight children with moderate/severe persistent AR and 28 healthy children, aged 4–11 years, participated in this study. Sensory modulation abilities were assessed using the Short Sensory Profile (SSP). Children with rhinitis showed significantly worse sensory modulation abilities than did healthy controls, in most SSP sections. The highest percentage of children with rhinitis showed deficiencies in the Taste/Smell Sensitivity and Low Energy/Weak SSP sections. Among the AR group, these sections were found to be impacted by age and gender. Children with AR may suffer from sensory modulation deficiencies. Thus, multidisciplinary intervention programs for children with rhinitis are recommended focusing on treating the symptoms together with consideration of additional possible factors that may influence the child’s daily function and well-being, as the ability to modulate sensations in daily environments.
Previous reports on spinal muscular atrophy type 1 (SMA-1) underestimate potential survival because of failure to optimally use noninvasive respiratory muscle aids including mechanically assisted coughing and noninvasive ventilation (NIV) at full support settings. We report our center's experience in prolonging survival for these patients. We focus on early initiation of nasal noninvasive ventilation, mechanically assisted coughing, and pulse oximetry monitoring during acute respiratory tract infections to guide use of assistive technologies. Seventeen SMA-1 patients with ventilation via tracheostomy are living, with a mean age of 78.2 (range 65–179) months. Ten died at a mean age of 61.6 (range 16–270) months of age. Twenty five of 27 were not able to regain autonomous breathing ability after the tracheostomy. None of the 21 who had not developed the ability to verbalize before undergoing tracheotomy did so after tracheotomy. Six patients had comprehendible speech at the time of tracheotomy and retained some ability to vocalize subsequently. Seventy-two SMA-1 patients using noninvasive ventilation are alive at mean age 86.1 (range 13–196) months; 13 died at 52.3 (range 13–111) months. Sixty seven of the 75 could communicate verbally. The noninvasive ventilation patients had significantly more hospitalizations than tracheostomy patients until age 3 (P < 0.001) but not thereafter. SMA-1 survival past adolescence is possible using both noninvasive ventilation and tracheostomy ventilation. The tracheostomy-ventilated patients had greater levels of ventilator dependence and reduced verbal abilities.
In 2008, the American Lung Association convened a conference of policy leaders and asthma experts to identify policies that, if implemented at the local, state, and federal levels, would reduce asthma morbidity and mortality. The consensus process resulted in 19 policy recommendations grouped in 6 major categories—public health infrastructure and surveillance, outdoor air pollution, health-care systems and financing, homes, schools, and workplaces—that were later developed into the National Asthma Public Policy Agenda. The project establishes a blueprint for national asthma policy that advocates and organizations around the nation can embrace. A successful impact in the fight against asthma requires intervention at different levels simultaneously and tools beyond those used in the medical care of the disease. It also requires the participation of diverse stakeholders, including physicians and other health professionals. By using their expertise to influence public policy, physicians can improve health outcomes and reduce the suffering and death from asthma. Involvement can be at an individual or collective level. Health-care providers remain crucial players in asthma policy change.
Although sublingual immunotherapy (SLIT) is a common adjuvant treatment for allergic rhinitis and allergic asthma in Europe, it is used minimally in the United States because of lack of approval by the US Food and Drug Administration (FDA) of allergen extracts for sublingual treatment and inconsistent study findings on extract potency, dosing levels, and dosing schedules. Data are particularly lacking on use of SLIT in pediatric patients, who may represent a potential patient population for this type of immunotherapy delivery because of its favorable safety and acceptability to children compared to subcutaneous immunotherapy (SCIT). Differences in the mechanism of action of SLIT compared to SCIT may also confer some treatment and safety advantages. Clinical trials on SLIT in pediatric patients have provided inconsistent results, but this inconsistency may be due in part to less than optimum dosing and durations of treatment. The appeal of SLIT as a treatment in children is further enhanced by its potential for possibly preventing progression to asthma if initiated at a young age. This article reviews the clinical evidence on studies in pediatric populations regarding efficacy and safety, and its implications in the treatment of allergic rhinitis and allergic asthma in children.
There is emerging evidence that rhinitis frequently precedes the onset of asthmatic symptoms. The frequent association of rhinitis and asthma has led to the concept that these conditions are likely manifestations of the same disease. This hypothesis has important implications for both the diagnosis and the management of these common conditions. Unfortunately, there is little known regarding the risk factors responsible for the progression of rhinitis to asthma. The current treatment options can control symptoms but do not prevent or cure the disease. However, there is recent data supporting the notion that it is possible to prevent new asthma cases by modifying the immune response and clinical outcome with allergen immunotherapy. This review article evaluates the impact of rhinitis on the development of asthma, examines putative predictors for the progression of rhinitis to asthma, and reviews recent, promising literature suggesting that early treatment of allergic individuals with immunotherapy may aid in asthma prevention.
Pediatric Asthma, Allergy & ImmunologyVol. 22, No. 1 ReviewsSublingual Immunotherapy: An Argument Against Its Current Use in the United StatesRussell J. HoppD.O., FAAAI, FAAPRussell J. HoppD.O., FAAAI, FAAPSearch for more papers by this authorPublished Online:1 May 2009https://doi.org/10.1089/pai.2008.0519AboutSectionsPDF/EPUB Permissions & CitationsPermissionsDownload CitationsTrack CitationsAdd to favorites Back To Publication ShareShare onFacebookTwitterLinked InRedditEmail "Sublingual Immunotherapy: An Argument Against Its Current Use in the United States." , 22(1), pp. 37–38FiguresReferencesRelatedDetails Volume 22Issue 1Mar 2009 Information© Mary Ann Liebert, Inc.To cite this article:Russell J. HoppD.O., FAAAI, FAAP.Sublingual Immunotherapy: An Argument Against Its Current Use in the United States.Pediatric Asthma, Allergy & Immunology.Mar 2009.37-38.http://doi.org/10.1089/pai.2008.0519Published in Volume: 22 Issue 1: May 1, 2009Online Ahead of Print:April 15, 2009PDF download
We present a retrospective epidemiologic population-based study detailing the incidence rate, point prevalence, clinical history, laboratory findings, and inheritance pattern of patients with chronic granulomatous disease (CGD) in Eastern Province of Saudi Arabia. The incidence rate of CGD is 5.2 cases per 100,000 person-year in our population, which is the highest reported in the literature and is exclusively the autosomal recessive inherited form, rather than the X-linked inherited form. The 11 patients in five families in our study are from consanguineous marriages. The point prevalence of CGD in our population at the end of this study is 6.4 per 100,000. Genotyping studies on six of these patients demonstrate that only two are homozygous for NCF1 c.75_76delGT mutation while the remaining four patients are heterozygous for NCF1 c.75_76delGT mutation. Screening for mutations in CYBB, CYBA, and NCF2 genes are negative.
Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) patients both have chronic lung infection and neutrophilic inflammation, but there is a milder clinical phenotype in PCD. To understand the detailed inflammatory mechanisms in these two conditions in order to identify potential therapeutic targets and useful biomarkers of disease severity, spontaneously expectorated sputum was collected from CF and PCD patients with similar spirometry. Sputum was homogenized in phosphate-buffered saline, centrifuged, and the supernatants analyzed. Surprisingly, the level of neutrophil-stimulating activity was similar in CF and PCD patients, with a trend for lower amounts of CXC chemokines in CF patients. In an exploratory, hypothesis generating study, we found that levels of neutrophil elastase, cathepsin G, and elastin degradation products were significantly elevated in CF patients in exacerbation compared to stable PCD patients, despite similar spirometry in both groups. Neutrophil chemoattractants are similar or even reduced in CF compared with PCD, suggesting that they may not be useful therapeutic targets or biomarkers in CF. However, matrix-degrading enzymes were markedly higher in CF. Further research is needed to explore the reasons for the differences between PCD and CF, but if these findings are confirmed, we speculate that inhibition of neutrophil-derived proteolytic enzymes in CF patients may be a more useful therapeutic target than inhibiting neutrophil chemoattraction.
Hypertrophied adenoid glands are a common finding in pediatric patients with obstructive nasal symptoms. Recurrence of the symptoms following adenoidectomy is reported. One possible etiology for recurrent symptoms is regrowth of the adenoid tissue; however, prevalence of and risk factors for regrowth are not well delineated. The study was undertaken to determine the frequency of regrowth of adenoid tissue in a population of post-adenoidectomy patients presenting to a pediatric allergy office with obstructive nasal complaints, as well as to identify potential contributing risk factors in this population. Patients presenting with obstructive nasal symptoms, status post-adenoidectomy were retrospectively identified. Charts were reviewed for evidence of adenoid regrowth on imaging studies. A retrospective analysis was then performed comparing the presence and absence of adenoid regrowth with aeroallergen sensitivity, sinusitis, gastroesophageal reflux disease (GERD), tobacco exposure, asthma, and immunodefici...
We aimed to determine the variability of response to inhaled corticosteroids (ICS) and leukotriene receptor antagonist (LTRA) in asthmatic children according to the patient phenotype. The present study comprised 56 children (mean age, 9.54 [SD, 2.3] years) with moderate persistent asthma and 18 healthy controls of matched age and sex. Asthmatics were allocated randomly to receive either montelukast (5 mg at bed time) or fluticasone propionate (100 μg twice daily) for 28 days. Serum concentrations of inflammatory mediators as soluble interleukin-2 receptor (sIL-2R), soluble intracellular adhesion molecules-1 (sICAM-1), soluble vascular cell adhesion molecules-1 (sVICAM-1), total serum IgE, peripheral blood eosinophils, and forced expiratory volume in 1 second (FEV1) were done before and after treatment to patients, and done once to controls. Significant increase in all inflammatory mediators, with significant decrease in FEV1, was detected in asthmatics before treatment compared to controls. Asthmatics presented with cough and wheeze showed significant increase of FEV1 and significant decrease in eosinophilic percentage in both treated groups, while the differential response between the two medications was found to be insignificant. Those presented with cough and shortness of breath showed significant increase of FEV1 and significant decrease in eosinophilic percentage in fluticasone-treated group only. Other inflammatory mediators showed insignificant changes between studied groups after intervention. Response to montelukast and fluticasone vary considerably according to the clinical phenotypes of asthma. Response to montelukast may be more effective in asthmatics presented with wheeze compared to those with shortness of breath. Whereas ICS provide clinical benefit in both asthmatic phenotypes.
Background: Ausculation of tidal breathing may not show evidence of wheezing. Forced expiratory maneuvers can bring out wheezing in children; however, it can be difficult to motivate a young child to perform a forced expiratory maneuver. Objectives: This study was performed to determine whether auscultation using a pinwheel to motivate a forced expiratory maneuver would be useful to elicit wheezing in young children with a history of asthma presenting to a general clinic for cough only. Materials and Methods: Subjects. One hundred and forty children aged 3.9 (SD 0.7) years with a history of asthma who had not been symptomatic for ≥3 months, who presented for cough, and who did not have wheeze on normal auscultation. Subjects were randomly assigned to 1 of 2 groups of 70 each: Group A: normal ausculation with no aids to motivate forced expiration. Group B: auscultation during forced expiration elicited by having the child blow out on a pinwheel. Patients in Group B in whom wheezing was detected (Group B-1, n = 47) were given asthma therapy with montelukast and a β-agonist. Patients in Group B in whom wheezing was not detected (Group B-2, n = 23) and all of those in Group A (n = 70) were given treatment for an upper respiratory tract infection. Results. The frequency of returning to clinic with wheezing was significantly higher in Group A (57.1%) than in Group B (27%) (P < 0.001). Conclusions: Having a young child blow out into a pinwheel can allow detection of wheezing that was not present on quiet breathing, and thus can improve confidence in the diagnosis of an asthma exacerbation.
Pediatric Asthma, Allergy & ImmunologyVol. 22, No. 4 PHARMACOTHERAPY UPDATEThe Effect of Smoking on the Efficacy of Pharmacotherapeutic Agents in AsthmaH. William KellyPharmDH. William KellyPharmDSearch for more papers by this authorPublished Online:11 Mar 2010https://doi.org/10.1089/pai.2009.2204.phAboutSectionsView articleView Full TextPDF/EPUB ToolsPermissionsDownload CitationsTrack CitationsAdd to favorites Back To Publication ShareShare onFacebookTwitterLinked InRedditEmail View articleFiguresReferencesRelatedDetails Volume 22Issue 4Dec 2009 Information© Mary Ann Liebert, Inc.To cite this article:H. William KellyPharmD.The Effect of Smoking on the Efficacy of Pharmacotherapeutic Agents in Asthma.Pediatric Asthma, Allergy & Immunology.Dec 2009.215-218.http://doi.org/10.1089/pai.2009.2204.phPublished in Volume: 22 Issue 4: March 11, 2010PDF download
Utilization of hypnosis as part of the care of children with respiratory disorders helps them achieve symptomatic relief, improves the clinician’s ability to diagnose and treat patients with complicated clinical presentations, and saves some patients from undergoing costly investigations or receiving nonessential treatments. Hypnosis can be defined as a state of inward attention during which the mind is focused on ideas of therapeutic value that can potentiate physiologic change. There are few published randomized trials demonstrating the effectiveness of hypnosis in the management of asthma. Published case series suggest that hypnosis is of benefit for children with cystic fibrosis, dyspnea, habit cough, insomnia, or vocal cord dysfunction. Hypnosis helps patients control their response to discomfort, which may allow reduction in the amount of pharmacologic analgesia during medical procedures such as phlebotomy or bronchoscopy. Once children decide to learn hypnosis, initial instruction time can require 5–30 min with older children and even less time with younger children. This training can be incorporated within regular medical office appointments. Clinical hypnosis for use by various staff members at a respiratory specialty center is learned best through active participation in 20-h experiential hypnosis workshops endorsed by medical hypnosis organizations. While acquiring the skills required for incorporating hypnosis into clinical practice may take relatively few hours, it is the clinician’s years of professional training that permits its appropriate use. In conclusion clinical hypnosis is an efficient and effective tool for addressing the mind/body connection for children with respiratory disorders.
