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Hyper-IgE syndrome (HIES) comprises a group of rare primary immunodeficiencies, which are characterized by extremely high serum IgE levels, eczema, recurrent skin and pulmonary infections.Signal transduction and activator of transcription 3( STAT3)-HIES is the most common type, which is caused by dominant-negative mutations in STAT3.STAT3-HIES confers broad innate and acquired immune defects, defects in skeletal, connective tissue, and vascular functions, causing a clinical phenotype including eczema, staphylococcal and fungal skin and pulmonary infections, scoliosis and minimal trauma fractures, vascular tortuosity and aneurysm.In this article, the advance in diverse clinical manifestations and management strategies of STAT3-HIES was summarized.
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Objective:To analyze the clinical features of bronchiectasis in children after severe adenovirus pneumonia and to provide clinical clues for the early diagnosis of bronchiectasis in children after severe adenovirus pneumonia.Methods:A retrospective study was made to analyze the clinical data of 26 children with bronchiectasis after severe adenovirus pneumonia treated in Guangzhou Women and Children′s Medical Center, Guangzhou Medical University from May 2016 to May 2021.Results:A total of 26 cases were reported, including 18 males and 8 females.The median onset age of severe adenovirus pneumonia was 23.0 (15.0, 48.0) months.A total of 23 cases suffered concurrent infections, and bacterial co-infection was the most common (16 cases). High resolution computed tomography (HRCT) showed multiple lobar solids in the lung with/without pleural effusion.During the acute phase, most of the cases were treated with intravenous immunoglobulin (21 cases), mechanical ventilation (20 cases), and systemic glucocorticoids (19 cases). The median age at diagnosis of bronchiectasis was 29.5 (21.0, 56.8) months, and the median time that the patients took to develop into acute adenovirus pneumonia was 6.0 (3.3, 13.0) months.Six cases suffered bronchiectasis alone, and 20 cases had bronchiectasis combined with post-infectious bronchiolitis obliterans (PIBO). Of these 20 cases, 3 cases developed bronchiectasis and PIBO simultaneously, and the remaining 17 cases developed bronchiectasis after PIBO.In the included 26 cases, diffuse bronchiectasis predominated (24 cases), most frequently involving the left lower lobes (21 cases) and right lower lobes (21 cases). Cylindrical bronchiectasis was the most common type (23 cases). All the patients had recurrent cough and wheezing during follow-up, and only 3 cases coughed up pus sputum without hemoptysis.All children had acute exacerbations, which were mostly caused by bacteria (21 cases). Nineteen cases combined with PIBO and 1 case with only bronchiectasis were rehospitalized.There was no cases of surgical resection or death.Conclusions:Bronchiectasis after severe adenovirus pneumonia mostly occurs in patients with or without PIBO.Multiple lobe involvement and co-infection may be a risk factor for PIBO patients to develop bronchiectasis.The clinical manifestations are mostly recurrent cough and wheezing, while sputum and hemoptysis are less common.Pediatricians should promptly perform chest HRCT for early diagnosis of the disease.
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Objective:To observe the therapeutic timing and dosage of intravenous immunoglobulin (IVIG) in children with severe adenovirus pneumonia.Methods:Clinical data of children with severe adenovirus pneumonia treated with IVIG at the Department of Respiratory, Guangzhou Women and Children′s Medical Center, Guangzhou Medical University from January 2019 to January 2020 were retrospectively analyzed.Participants were classified as early presenters (5-10 days of illness course) and later presenters (11-15 days of illness course) according to the timing of IVIG treatment.They were further subdivided into plan 1 group[1 g/(kg·d) IVIG for 2 days] and plan 2 group [0.4-0.5 g/(kg·d) IVIG for 3-5 days]. Continuous variables and categorical variables between groups were analyzed by the nonparametric Mann- Whitney U test and the Fisher′ s exact test, respectively. Results:A total of 202 patients with the median age of 12 (12, 36) months were recruited, involving 128 early presenters (63.37%) and 74 later presen-ters (36.63%). Later presenters had a longer duration of fever [18.00(14.00, 23.25) days vs.11.00(9.00, 14.00) days], more demands for mechanical ventilation (33.78% vs.20.31%), and higher incidence of bronchiectasis (9.46% vs.1.56%) than those of early presenters (all P<0.05). For early presenters, no significant differences were detected in the demand for advanced life support, outcomes and sequelae between plan 1 group and plan 2 group (all P>0.05). For later presenters, a shorter duration of fever [18.00(14.00, 21.00) days vs.21.00(15.50, 30.75) days] and lower usage of extracorporeal membrane oxygenation (ECMO, 2.13% vs.18.52%) were observed in the plan 1 group than that of plan 2 group (all P<0.05). The incidence of post-infectious bronchiolitis obliterans and bronchiectasis as pulmonary sequelae was comparable between plan 1 group and plan 2 group ( P>0.05). The incidence of adverse events was 5.77% during IVIG infusion, showing no significant difference between plan 1 group and plan 2 group ( P>0.05). Conclusions:Early treatment of IVIG are very important to improve the prognosis of children with severe adenovirus pneumonia.For later presenters, a high dosage of IVIG is effective in reducing the ECMO use and shortening the duration of fever, thus providing clinical benefits.
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Penicillium marneffei (PM) is the only thermally dimorphic species as a Penicillium that causes the transmitted penicilliosis marneffei (PSM). PM infection is more common in immunodeficient children.Due to the lack of specific clinical manifestations, PSM is easily misdiagnosed as pulmonary plasmacycosis, tuberculosis or pulmonary aspergillosis.Because of the rapid progress and multiple complications of transmitted PSM, its mortality remains high without timely and effective treatment.Therefore, early diagnosis and treatment are of great significance, and the diagnosis of PSM mainly relies on the immune function examination, measurement of serum markers, pathogen detection and imaging examinations.There is no standard antifungal regimen, and Amphoterus B and Itraconazole are used as the first-line treatment.This study aims to review the progress of diagnosis and treatment of PM infection in children.
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Human adenoviruses (HAdVs) are the common infectious pathogens in children, which mainly infect the respiratory tract, digestive tract and eyes.Severe HAdVs may even be life-threatening.HAdVs enter the host and cause infection by binding to the host epithelial cells.At the same time, they are recognized by various immune cells and then activate the immune defense response.This study aims to review the interaction between HAdVs and the host, and the immune defense mechanism, thus improving the understanding of the immune response to HAdVs.
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Primary immunodeficiencies (PIDs) are caused by one or more defects of the immune system.Children with PIDs are more likely to experience recurrent and/or severe infections and tend to develop a wide range of complications.Respiratory diseases are the main and initial manifestation in most cases.Pulmonary complications have significant morbidity and mortality in children with PIDs.The spectrum of pathogens usually varies among different types of PID.Early diagnosis and appropriate therapy can prevent or at least slow down the development and course of respiratory complications of PIDs.In this study, the main respiratory manifestations of common PIDs in children are reviewed.
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Objective:To analyze the clinical and imaging characteristics of pulmonary artery sling (PAS) in children and to explore its diagnosis and treatment strategies and risk factors.Method:s A retrospective study was performed in 98 pediatric patients with PAS who were admitted to Guangzhou Women and Children′s Medical Center, Guangzhou Medical University, from January 2011 to October 2018.All their clinical features, imaging examination, treatment and prognosis were reviewed and analyzed.Result:s (1) Among the 98 patients with PAS, 56 cases were male and 42 cases were female.The age range of onset was 0-19 months with the median age of 2 months, and the age range of diagnosis was 0-84 months with the median age of 4 months.(2) The main clinical manifestations were cough (61/98 cases, 62.24%), recurrent wheezing(47/98 cases, 47.96%), shortness of breath (43/98 cases, 43.88%), cyanosis (19/98 cases, 19.39%), and laryngeal stridor (8/98 cases, 8.16%) and so on.(3) Ninety-six patients underwent cardiac ultrasonography, and the diagnostic rate was 87.50%(84/96 cases), and 62 cases (62/96 cases, 64.58%) of them were found to be associated with other congenital cardiovascular anomalies, among which atrial septal defect was the most common (32/96 cases, 33.33%). (4) Chest CT and three-dimensional reconstruction were performed in 92 PAS children, and the diagnostic rate was 100.00%.Airway stenosis was mainly in the middle and lower sections of the main bronchus (76/92 cases, 82.61%), and bronchobridge was found in 13 cases (13/92 cases, 39.13%). Bronchoscopy was performed in 77 children with PAS, showing complete tracheal cartilage ring in 43 cases (43/77 cases, 55.84%), and tracheobronchomalacia in 13 cases (13/77 cases, 16.88%). (5) Among 67 cases who had received left pulmonary artery (LPA) reimplantation, 5 cases died after operation because of airway stenosis and weaning failure (LPA reconstruction was performed in 1 case, and both LPA reconstruction and tracheal intervention were performed in the other 4 cases), 24 cases lost to follow-up, 38 cases survived, and the respiratory symptoms were improved in 2 to 96 months of follow-up.There were 31 cases of non-surgical treatment, of which 18 cases died of respiratory failure, 7 cases lost to follow-up, and the rest 6 cases showed respiratory symptoms of different degrees in the follow-up period.(6) Multivariate regression analysis showed that conservative treatment was an independent risk factor affecting the prognosis of PAS ( OR=7.45, 95% CI: 1.23-48.68). Conclusions:The combination of cardiac ultrasound, chest CT and bronchoscopy is important to the diagnosis of PAS.LPA reconstruction is the main method to treat PAS, which can improve respiratory symptoms, but its fusion with tracheal intervention poses a high risk of death.Conservative treatment is an independent risk factor that affects the prognosis of PAS children.
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Adenovirus pneumonia is a common infectious disease of the respiratory tract in children, and severe cases developed post-infectious bronchiolitis obliterans (PIBO), which is chronic airflow obstruction syndrome caused by small airway inflammatory injury.The major clinical manifestations of PIBO are persistent cough, wheezing and dyspnea after acute stage of adenovirus pneumonia, and nowadays there is no specific treatment.PIBO has a prolonged course and very poor prognosis without early intervention, and it influences patients on health and life seriously.The article reviewed the early recognition and the therapeutic progress of adenovirus pneumonia complicated with PIBO.
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Adenovirus pneumonia is a common infectious disease of the respiratory tract in children, and severe cases developed post﹣infectious bronchiolitis obliterans (PIBO),which is chronic airflow obstruc﹣tion syndrome caused by small airway inflammatory injury. The major clinical manifestations of PIBO are persistent cough,wheezing and dyspnea after acute stage of adenovirus pneumonia,and nowadays there is no specific treatment. PIBO has a prolonged course and very poor prognosis without early intervention,and it in﹣fluences patients on health and life seriously. The article reviewed the early recognition and the therapeutic progress of adenovirus pneumonia complicated with PIBO.
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Objective To analyze the clinical and anatomic characteristics of bridging bronchus (BB) malformation in children,and to explore its diagnostic strategy,treatment and risk factors.Methods A retrospective study was performed in 23 pediatric patients with BB who were admitted to Guangzhou Women and Children's Medical Center form May 2010 to October 2016.All their clinical features,imaging examination,treatment and prognosis were reviewed and analyzed.Results (1) Among the 23 patients with BB,15 were males and 8 were females.The age range of onset was 0 to 4 years old with a median age of 3.17 months.(2)The main clinical manifestations were cough (23/23 cases,100.0％),recurrent wheezing (20/23 cases,87.0％),cyanosis (8/23 cases,34.8％) and feeding diffficulty/slow body weight growth (6/23 cases,24.6％).(3) There were 17 cases of type Ⅰ and 4 cases of type Ⅱ according to Wells,and the other 2 cases were anterior BB.There were 2 cases with atypical anatomical morphology in type Ⅰ and type Ⅱ respectively.(4) Twenty-one cases were associated with bronchial stenosis in which the origin of left main bronchus complicated with BB stenosis was the most common(5 cases).Bronchoscopy revealed bronchomalacia in 5 of the 16 patients.Sixteen patients associated with cardiac or vascular anomalies,11 of whom were compound anomalies,and the most common type was sling left pulmonary artery (SLPA) (12 cases).(5) Nine of 12 patients with SLPA received surgical management,ages ranging from 1 month to 8 years old.The respiratory symptoms remitted gradually or disappeared from 4 to 17 months after surgical management.Two died and one has been lost to follow-up among the remaining 3 patients with SLPA.Conclusions BB is a rare tracheobronchial malformation that is often associated with stenosis or bronchomalacia,and cardiac or vascular anomalies.The severity of the disease mainly depends on whether there is airway stenosis or malacia and its extent.The definitive diagnosis of BB requires the combination of different imaging modalities.The combination with simple SLPA could get better curative effect.