Pediatric Allergy Immunology and Pulmonology最新文献

Background: This case report provides the general pediatrician with insight on a unique presentation of an already rare disease. Plastic bronchitis (PB) is an exceedingly rare disease that presents with the formation of casts in the endobronchial tree. This typically occurs in patients with congenital heart defects that have undergone repair, however, it is atypical to be seen in otherwise healthy patients. Influenza A, lymphatic abnormalities, and single ventricle physiology are the only proven causes of PB. Asthma, toxic inhalation, and acute chest syndrome, however, are a few of the many conditions that have been proposed to predispose patients toward developing PB. Case Presentation: Thus, it is important to discuss the case of a 9-year-old boy with a history of uncontrolled asthma who presented with cough, chills, weight loss, and fevers. This patient was initially treated with broad-spectrum antibiotics due to concerns for necrotizing pneumonia, but due to failure in improvement, a direct laryngoscopy and bronchoscopy was performed, revealing the diagnosis of PB. Although this patient had a history of uncontrolled asthma, this was the only predisposing respiratory condition that put him at risk of developing PB. This patient went on to be treated with corticosteroids, chest physiotherapy, inhaled fibrinolytics, and direct fibrinolytic therapy with marked improvement in symptoms and imaging. Conclusion: PB, though rare, is a condition that all pediatricians must keep in their minds when patients present with respiratory symptoms with an unclear etiology. The delay in diagnosis and treatment of patients with PB can be detrimental as expectoration of these casts can result in asphyxiation and death. This article goes on to remind all providers, at all levels, the importance of conducting a thorough history/physical examination, creating a broad differential, and treating each patient holistically.

Background: Although serum levels of squamous cell carcinoma antigen (SCCA) are elevated in atopic dermatitis (AD), their clinical utility has not been fully elucidated. Methods: Thirty-three cases of AD who admitted to our hospital were analyzed. Results: Baseline characteristics on admission were as follows: median age 19 months [interquartile range (IQR), 12-52 months], median objective severity scoring of atopic dermatitis (O-SCORAD) 19.2 (IQR, 4.2-36.0), and median serum SCCA levels 3.2 ng/mL (IQR, 2.1-6.8 ng/mL). O-SCORAD significantly correlated with serum SCCA levels (rs = 0.865, P < 0.001). In 9 cases whose information before and after treatment was available (median interval, 3 days; IQR 2-5 days), median serum SCCA levels significantly decreased from 8.0 to 2.0 ng/mL (P = 0.008) after the treatment. Conclusions: Serum levels of total SCCA rapidly declined in response to the treatment and could be used as a real-time biomarker in childhood AD.

Background: Guidelines such as Global Initiative for Asthma (GINA) recommend disease control as the mainstay of asthma management. Objective: To investigate which measure of asthma control best correlates with the GINA criteria for determining asthma control in children. Methods: Child asthma-patients at a tertiary hospital were enrolled in the study after evaluation of response to treatment. Asthma control test (ACT)/pediatric asthma control test (PACT), Pediatric Asthma Quality of Life Questionnaire (PAQLQ), fractional exhaled nitric oxide (FeNO), and lung function parameters were evaluated. Patients were examined by asthma specialists and control status was evaluated based on GINA. Results: The median age (interquartile range) of patients was 10.7 (8.4-12.9) years, 57.9% of patients were boys. Of 228 children, 84.2%, 9.6%, and 6.1% displayed "well-controlled", "partially controlled", and "uncontrolled" asthma, respectively, according to GINA. The patients with "partially controlled" and "uncontrolled" asthma were grouped as "not well-controlled." The cutoff levels were 22, 21, and 5.9 for PACT, ACT, and PAQLQ, respectively, for determining "well-controlled" asthma (P < 0.001). With these cutoff values, ACT exhibited higher comparability with GINA than PACT and PAQLQ (κ = 0.473, 0.221, and 0.150, respectively, P < 0.001). PAQLQ had higher agreement with GINA criteria in children ≥12 years old (κ = 0.326, P < 0.001 and κ = 0.151, P = 0.014, respectively). Correctly classified patients with PACT, ACT, and PALQLQ based on GINA with these cutoff levels were 93 (64.1%), 63 (75.9%), and 139 (62.9%), respectively. FeNO and lung function parameters were unsuccessful at revealing control status according to GINA. Conclusion: ACT is better than PACT for comparability with GINA. Better correlation of PAQLQ and ACT and better comparability of PAQLQ and GINA were evident in older children.

Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease of the central nervous system that is caused by John Cunningham virus (JCV). It occurs almost exclusively in immunosuppressed individuals, for example, patients with AIDS and hematological and lymphoreticular malignancies. In this article, we present a review of the literature and 2 case reports with PML. The first report examines a 15-year-old male (who presented with dedicator of cytokinesis 8 deficiency) who was diagnosed as having PML based on characteristic magnetic resonance imaging (MRI) lesions and a positive PCR for JCV in cerebrospinal fluid. He was transferred for bone marrow transplantation after stabilization with therapy of maraviroc and cidofovir. The second report examines a 6-year-old male who presented with encephalitis and was also diagnosed with AIDS. He was diagnosed with PML and started treatment with cidofovir. His clinical status and MRI findings deteriorated rapidly. In immunosuppressive patients who developed encephalopathy, JCV ought to be considered.

Background: 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. Methods: 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. Results: 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 (n = 7) or diaphragm pacing (n = 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 (n = 3) who reported symptoms such as headache or oxygen desaturations during sleep required an increase in ventilator settings. Conclusion: 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.

Introduction: 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. Case Presentation: 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. Conclusion: 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.

Background: 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. Conclusions: "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. Case: 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.

Introduction: 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. Case Presentation: 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. Conclusion: 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.

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.

Background: Myhre syndrome is a rare connective tissue disorder caused by heterozygous pathogenic variants in the SMAD4 gene. Although recognizing Myhre syndrome in early childhood is challenging, it is important to manage airway stenosis in patients with Myhre syndrome. Case Presentation: 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 SMAD4 was identified through exome sequencing. Tracheostomy was performed, and the patient has maintained stable respiration through a customized tracheostomy tube with a home ventilator. Conclusions: 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.