Pediatric Allergy Immunology and Pulmonology最新文献

{"title":"Annual Respiratory Evaluations in Congenital Central Hypoventilation Syndrome and Changes in Ventilatory Management.","authors":"Amit S Shah,&nbsp;Roberta M Leu,&nbsp;Thomas G Keens,&nbsp;Ajay S Kasi","doi":"10.1089/ped.2021.0072","DOIUrl":"https://doi.org/10.1089/ped.2021.0072","url":null,"abstract":"<p><p><b><i>Background:</i></b> Annual in-hospital respiratory evaluations (AREs) during wakefulness and sleep are recommended to assess ventilatory requirements in patients with congenital central hypoventilation syndrome (CCHS) aged ≥2-3 years based on expert consensus. This study aimed to determine if AREs in patients with CCHS led to changes in ventilatory management. <b><i>Methods:</i></b> Retrospective review of patients with CCHS who underwent AREs with or without polysomnography between 2017 and 2019 was conducted. Clinical symptoms, results of AREs, and subsequent changes in ventilatory management were analyzed. <b><i>Results:</i></b> We identified 10 patients with CCHS aged 4-20 years. All patients required assisted ventilation (AV) only during sleep delivered by positive pressure ventilation via tracheostomy (<i>n</i> = 7) or diaphragm pacing (<i>n</i> = 3). In total, 7 (70%) patients had abnormal oxygenation and/or ventilation requiring changes in ventilator settings or duration of AV. Six patients required an increase in settings and/or duration of AV, and only 1 patient required a decrease in ventilator settings. Two patients had awake hypercapnia during a routine outpatient visit that improved following increase in ventilator settings and a period of continuous AV. One patient who was previously ventilator-dependent only during sleep was identified to require 16 h per day of AV. All patients (<i>n</i> = 3) who reported symptoms such as headache or oxygen desaturations during sleep required an increase in ventilator settings. <b><i>Conclusion:</i></b> We report a high prevalence of changes in AV management following an ARE. Our results demonstrate the importance of regular AREs in patients with CCHS to assess their ventilatory requirements and optimize AV.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":"34 3","pages":"97-101"},"PeriodicalIF":0.9,"publicationDate":"2021-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8664117/pdf/ped.2021.0072.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39357495","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Successful Rapid Desensitization to Micafungin in a Pediatric Patient.","authors":"Stephanie L Ward,&nbsp;Michelle C Maciag,&nbsp;Sarah Jones,&nbsp;Joyce Lee,&nbsp;John Lee,&nbsp;Ana Dioun Broyles","doi":"10.1089/ped.2020.1204","DOIUrl":"https://doi.org/10.1089/ped.2020.1204","url":null,"abstract":"<p><p><b><i>Introduction:</i></b> Echinocandin antifungal medications including micafungin are being used more commonly in the treatment of invasive fungal infections in both pediatric and adult patients. Micafungin is also a first-line therapeutic option for candidemia and antifungal prophylaxis in a variety of clinical settings. Hypersensitivity reactions have not been well described; however, isolated cases have been reported. No cases of desensitization to echinocandins have been previously described. <b><i>Case Presentation:</i></b> In this report, we described a 14-year-old female with high-risk pre-B cell acute lymphoblastic leukemia diagnosed with pulmonary aspergillosis. She developed a hypersensitivity reaction to micafungin, which was deemed first-line therapy for the infection. A rapid intravenous desensitization protocol was successfully completed without reactions. The patient completed the remaining 2 months of therapy without reactions. <b><i>Conclusion:</i></b> This report outlines the first report of a successful desensitization to micafungin or any echinocandin. This is a safe method of completing antifungal therapy in a patient with echinocandin hypersensitivity and may be considered for other patients with micafungin hypersensitivities.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":"34 3","pages":"106-108"},"PeriodicalIF":0.9,"publicationDate":"2021-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8558086/pdf/ped.2020.1204.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39396075","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Independent Lung Ventilation in a Pediatric Patient with a Firearm Injury.","authors":"Muhammed Udurgucu,&nbsp;Hatice Albayrak,&nbsp;Hatice Elif Kinik Kaya,&nbsp;Sertaç Hancıoğlu,&nbsp;Nazik Yener","doi":"10.1089/ped.2021.0054","DOIUrl":"https://doi.org/10.1089/ped.2021.0054","url":null,"abstract":"<p><p><b><i>Background:</i></b> Chest trauma is uncommon in pediatric patients, however, it may be a cause of significant morbidity and mortality. The type and extent of the injury may lead to ventilation and perfusion problems, therefore, there may be a need for mechanical ventilation. <b><i>Conclusions:</i></b> \"Independent lung ventilation\" may be an appropriate option in selected cases in which the aim is to protect the healthy lung or ventilation cannot be obtained with known mechanical ventilation methods. <b><i>Case:</i></b> We presented a pediatric patient followed up in the intensive care unit because of a firearm injury, in whom left lung expansion could not be obtained despite repeated interventions, and independent lung ventilation resulted in success.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":"34 3","pages":"112-114"},"PeriodicalIF":0.9,"publicationDate":"2021-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8664120/pdf/ped.2021.0054.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39396074","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"X-Linked Agammaglobulinemia and COVID-19: Two Case Reports and Review of Literature.","authors":"Fiji Madona Devassikutty,&nbsp;Abhinav Jain,&nbsp;Athulya Edavazhippurath,&nbsp;Michael Chittettu Joseph,&nbsp;Mohammed Manakkattu Thekke Peedikayil,&nbsp;Vinod Scaria,&nbsp;Pulukool Sandhya,&nbsp;Geeta Madathil Govindaraj","doi":"10.1089/ped.2021.0002","DOIUrl":"https://doi.org/10.1089/ped.2021.0002","url":null,"abstract":"<p><p><b><i>Introduction:</i></b> The Centers for Disease Control and Prevention (CDC) has listed primary immunodeficiency disorders as being predisposed to severe coronavirus disease 2019 (COVID-19). However, patients affected with X-linked agammaglobulinemia (XLA) have shown contrary results. In this study, we present 2 boys in late adolescence from south India with XLA who were infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), as well as a review of cases reported in the literature. <b><i>Case Presentation:</i></b> Two patients with XLA had been diagnosed late and were started on regular immunoglobulin prophylaxis only during adolescence. Both of them had developed bronchiectasis, an irreversible suppurative lung disease. However, both patients made an uneventful recovery without the need for artificial ventilation or convalescent plasma. <b><i>Conclusion:</i></b> Successful outcomes of patients with XLA and COVID-19, except for delayed recovery, from our experience and from global reports are intriguing and the role of B cell depletion is being studied as well. Further research and clinical experience are necessary to fully elucidate the reasons for these observations.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":"34 3","pages":"115-118"},"PeriodicalIF":0.9,"publicationDate":"2021-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8664121/pdf/ped.2021.0002.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39396077","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome Manifesting as Lymphocytic Interstitial Pneumonia and Treatment-Resistant Bullous Pemphigoid.","authors":"Stine Maria Andersen,&nbsp;Simon Fage,&nbsp;Sune Leisgaard Rubak,&nbsp;Mette Holm,&nbsp;Jens Magnus Bernth Jensen,&nbsp;Trine Mogensen,&nbsp;Mette Deleuran","doi":"10.1089/ped.2020.1307","DOIUrl":"https://doi.org/10.1089/ped.2020.1307","url":null,"abstract":"<p><p>Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare immune deficiency with a broad clinical presentation. IPEX syndrome causes dysfunctional regulatory T cells, increasing the risk of autoimmune diseases. In this case report, we describe a 7-year-old boy with lymphocytic interstitial pneumonia and bullous pemphigoid who was recently diagnosed with IPEX syndrome.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":"34 2","pages":"76-79"},"PeriodicalIF":0.9,"publicationDate":"2021-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8329723/pdf/ped.2020.1307.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39244579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Multilevel Airway Stenosis Being Bypassed by a Customized Tracheostomy Tube in an Infant with Myhre Syndrome.","authors":"Min Jin Jeon,&nbsp;Min Jung Kim,&nbsp;Ji Hye Kim,&nbsp;Ji Soo Park,&nbsp;Jisook Yim,&nbsp;Myungshin Kim,&nbsp;Seong Keun Kwon,&nbsp;Soyoung Lee,&nbsp;Jung Min Ko,&nbsp;Jong-Hee Chae,&nbsp;Dong In Suh","doi":"10.1089/ped.2021.0029","DOIUrl":"https://doi.org/10.1089/ped.2021.0029","url":null,"abstract":"<p><p><b><i>Background:</i></b> Myhre syndrome is a rare connective tissue disorder caused by heterozygous pathogenic variants in the <i>SMAD4</i> gene. Although recognizing Myhre syndrome in early childhood is challenging, it is important to manage airway stenosis in patients with Myhre syndrome. <b><i>Case Presentation:</i></b> We report the case of a 2-month-old boy who initially presented with severe multilevel airway stenosis, dysmorphic face, and multiple abnormalities. Lung fibrosis and mild aortic valve stenosis were additionally observed on follow-up examinations. A heterozygous missense variant, c.1499T>C (p.Ile500Thr), in <i>SMAD4</i> was identified through exome sequencing. Tracheostomy was performed, and the patient has maintained stable respiration through a customized tracheostomy tube with a home ventilator. <b><i>Conclusions:</i></b> Patients who have dysmorphic face, airway stenosis, and cardiovascular anomalies that do not fit the diagnosis of common syndromes should be evaluated for rare diseases, including Myhre syndrome. Since respiratory complications can be life threatening, early diagnosis and suitable intervention are necessary.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":"34 2","pages":"83-87"},"PeriodicalIF":0.9,"publicationDate":"2021-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8329727/pdf/ped.2021.0029.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39244195","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Paracetamol Allergy: A Case of a 9-Year-Old Female with a History of Atopy.","authors":"Ariana Teles,&nbsp;Francisco Ribeiro-Mourão,&nbsp;Mariana Branco,&nbsp;Ana Rita Araújo,&nbsp;Teresa Vieira","doi":"10.1089/ped.2021.0027","DOIUrl":"https://doi.org/10.1089/ped.2021.0027","url":null,"abstract":"<p><p><b><i>Background:</i></b> We describe a rare case involving paracetamol, a commonly used drug worldwide. Increased paracetamol consumption increases the risk of adverse drug reactions. <b><i>Case Presentation:</i></b> This is a case of a 9-year-old girl who visited the emergency department due to sudden onset sneezing, nasal itching, urticaria, and angioedema after paracetamol ingestion. The diagnostic and etiologic studies revealed an immunoglobulin E (IgE)-mediated hypersensitivity mechanism to paracetamol. <b><i>Conclusion:</i></b> Few cases of this phenomenon have been reported in previous literature. As confirmed in this study, a negative skin prick test did not exclude hypersensitivity, and conducting intradermal tests (IDTs) increased diagnostic accuracy. The patient had a positive IDT, confirming the underlying IgE-mediated reaction. The follow-up of a confirmed paracetamol hypersensitivity implies patient education about avoidance of any paracetamol-containing formulation, including combination products and clarification of available alternative drugs. This is likely the first publication documenting IgE-mediated paracetamol allergy in pediatric patients. We intend to underline the clinical benefits of diagnostic confirmation toward suspected drug hypersensitivity reactions in children, a particularly useful topic for pediatricians and pediatric allergists.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":"34 2","pages":"80-82"},"PeriodicalIF":0.9,"publicationDate":"2021-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8329691/pdf/ped.2021.0027.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39244581","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Early Peanut Introduction and Testing: A Framework for General Pediatrician Beliefs and Practices.","authors":"Angela Chang, Michael D Cabana, Taylor N LaFlam, Saharsh Patel, Megumi Okumura","doi":"10.1089/ped.2020.1190","DOIUrl":"10.1089/ped.2020.1190","url":null,"abstract":"<p><p><b><i>Background:</i></b> Peanut introduction guidelines have undergone significant reversal since 2001 from recommending delayed introduction to rescinding the recommendations in 2008 to actively recommending early introduction of peanut between 4 and 11 months of age in high-risk infants in 2015. This qualitative study aims to explore pediatrician beliefs, practices, facilitators, and barriers regarding peanut introduction and testing. <b><i>Methods:</i></b> General pediatricians from academic, private, large group, and underserved practices in Northern California underwent individual semi-structured interviews in 2017. We asked about experiences surrounding infant peanut introduction, strategies for staying up-to-date with current recommendations, and barriers and facilitators to the new peanut introduction and testing recommendations. The data were coded, and using grounded theory methodology, a conceptual framework was developed around early peanut introduction and testing in infants. <b><i>Results:</i></b> Eighteen general pediatricians participated. We identified barriers that may contribute to pediatrician reluctance to recommending early peanut introduction or testing including lack of awareness, lack of agreement, lack of resources, and lack of outcome expectancy. A framework was created that suggests that pediatricians need to be knowledgeable about new recommendations, agree with the recommendations, have resources to carry out the counseling and testing, and have buy-in from the parents in order for successful uptake of peanut introduction guidelines. <b><i>Conclusion:</i></b> Recommending early peanut introduction or testing causes significant apprehension in some pediatricians, and there are many barriers to following recent early peanut introduction recommendations. A potential limitation of the study is that it was conducted right after the addendum guidelines were changed, leaving the possibility that attitudes and practices may have evolved since 2017. It is still likely that a multifaceted approach that addresses primary care provider guideline awareness, limited primary care resources for education and testing, and includes support and collaboration from subspecialty practices is more likely to lead to improved early peanut introduction uptake.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":"34 2","pages":"53-59"},"PeriodicalIF":0.9,"publicationDate":"2021-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8329693/pdf/ped.2020.1190.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39244582","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pulmonary Function Testing in the Coronavirus Disease Era: Lessons and Opportunities.","authors":"Scott Bickel,&nbsp;Ronald Morton,&nbsp;Nemr Eid","doi":"10.1089/ped.2021.0059","DOIUrl":"https://doi.org/10.1089/ped.2021.0059","url":null,"abstract":"","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":"34 2","pages":"43-45"},"PeriodicalIF":0.9,"publicationDate":"2021-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8329726/pdf/ped.2021.0059.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39244583","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Relationships Between Bronchoscopy, Microbiology, and Radiology in Noncystic Fibrosis Bronchiectasis.","authors":"Mustafa Atilla Nursoy,&nbsp;Ayse Ayzit Kilinc,&nbsp;Fatouma Khalif Abdillahi,&nbsp;Feyza Ustabas Kahraman,&nbsp;Lina Muhammed Al Shadfan,&nbsp;Bilge Sumbul,&nbsp;Sabriye Sennur Bilgin,&nbsp;Fatma Betul Cakir,&nbsp;Hayrettin Daskaya,&nbsp;Erkan Cakir","doi":"10.1089/ped.2020.1319","DOIUrl":"https://doi.org/10.1089/ped.2020.1319","url":null,"abstract":"<p><p><b><i>Background:</i></b> Published data on the correlations of bronchoscopy findings with microbiological, radiological, and pulmonary function test results in children with noncystic fibrosis (CF) bronchiectasis (BE) are unavailable. The aims of this study were to evaluate relationships between Bronchoscopic appearance and secretion scoring, microbiological growth, radiological severity level, and pulmonary function tests in patients with non-CF BE. <b><i>Methods:</i></b> Children with non-CF BE were identified and collected over a 6-year period. Their medical charts and radiologic and bronchoscopic notes were retrospectively reviewed. <b><i>Results:</i></b> The study population consisted of 54 female and 49 male patients with a mean age of 11.7 ± 3.4 years. In the classification according to the bronchoscopic secretion score, Grade I was found in 2, Grade II in 4, Grade III in 9, Grade IV in 17, Grade V in 25, and Grade VI in 46 patients. When evaluated according to the Bhalla scoring system, 45 patients had mild BE, 37 had moderate BE, and 21 had severe BE. Microbial growth was detected in bronchoalveolar lavage fluid from 50 of the patients. Forced expiratory volume in 1 s (FEV₁) and functional vital capacity decreased with increasing bronchoscopic secretion grade (<i>P</i> = 0.048 and <i>P</i> = 0.04), respectively. The degree of radiological severity increased in parallel with the bronchoscopic secretion score (<i>P</i> = 0.007). However, no relationship was detected between microbiological growth rate and radiological findings (<i>P</i> = 0.403). <b><i>Conclusions:</i></b> This study showed that bronchoscopic evaluation and especially scoring of secretions correlate with severe clinical condition, decrease in pulmonary function test, worsening in radiology scores, and increase in microbiological bacterial load in patients. Flexible endoscopic bronchoscopy should be kept in mind in the initial evaluation of non-CF BE patients.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":"34 2","pages":"46-52"},"PeriodicalIF":0.9,"publicationDate":"2021-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8266562/pdf/ped.2020.1319.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38900419","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}