Masks of hypersensitivity pneumonitis in children.

Hypersensitivity pneumonitis (HP), also known as extrinsic allergic alveolitis, is the most common interstitial lung disease in children, but remains rarely recognized in the pediatric population. Early recognition of triggering factors and a high index of suspicion of HP could lead to timely diagnosis and individualized treatment. This study aimed to present four clinical cases of HP reported between 2012 and 2022 in Lithuania to improve the suspicion of the disease in children.

Case report: Unilateral pulmonary artery agenesis and Kommerell's diverticulum in 1-year old girl.

Background: Unilateral pulmonary artery agenesis (UPAA) and Kommerell's diverticulum (KD) are two rare embryologically unrelated congenital vascular malformations rarely diagnosed in children. This is the first report of our knowledge of the unique combination for a child as patients are at a high risk of pulmonary hypertension and rupture of the diverticulum. Our aim is to present the case of a pediatric patient with UPAA and KD with the short literature review and to highlight the importance of early diagnostics of rare congenital vascular malformations. Case report: A 1-year-old girl presented to the emergency department with prolonged cough and variable wheezing. A hypoplastic left lung was suspected in the radiographic image of the chest. A transthoracic echocardiogram revealed absence of the left pulmonary artery and right arch of aorta and anomaly of subclavian arteries was suspected. The diagnosis was confirmed by computed tomography scans of the chest that demonstrated elongation of the aorta and an aberrant right subclavian artery with KD, as well as absence of the left pulmonary artery. The patient is being followed up for the development of pulmonary hypertension and compression of vascular structures to the airways as well as any indications for surgical intervention because of the KD. Conclusions: UPAA and KD are two very rare congenital vascular anomalies usually diagnosed in adults. A high risk of pulmonary hypertension and rupture of diverticulum is noted for adult patients. This case provides us with an exclusive possibility to follow up a patient with an extremely rare combination of the two vascular anomalies with insufficiently known future complications and outcomes.

Bacille Calmette-Guérin Vaccine-induced Osteomyelitis in Immunocompetent Children: A 10-year Case Series in Lithuania.

The Bacille Calmette-Guérin (BCG)-induced osteomyelitis is an extremely rare systemic adverse reaction after BCG vaccination in immunocompetent children and the correct diagnosis is frequently missed. We present 4 clinical cases of BCG-induced osteomyelitis reported over a 10-year period in a high-TB incidence country Lithuania. A brief review of clinical, management and treatment features of the disease is given.

Short course of systemic corticosteroids in wheezy children: still an open question.

INTRODUCTION: We performed a real-life clinical study to identify the main indications for the prescription of short-course treatment with systemic glucocorticosteroids (GCS) for steroid naive children with acute virus-induced wheezing as well as to analyze the influence of such treatment on patients' serum cortisol level, other blood tests results and the length of stay in the hospital. MATERIAL AND METHODS: The data of 44 patients who had acute wheezing, had no bacterial infection and were otherwise healthy were analyzed: 26 children received treatment with GCS and 18 children did not. Full blood count, biochemistry tests (Na, K, glucose) and blood cortisol levels of all patients were analyzed during treatment. RESULTS: The main indications for the short-term administration of systemic GCS were increased work of breathing, recurrent wheezing, clinical signs of atopy and a family history of asthma. Systemic GCS increased a sodium concentration (p = 0.014), decreased a cortisol level (p = 0.038), leukocyte (p = 0.043), neutrophil (p = 0.045), and eosinophil (p < 0.001) count in blood serum. The major reduction in the eosinophil count was observed in allergic children (p = 0.023). Older age was a risk factor for cortisol suppression (p = 0.018). The average length of stay in the hospital was longer in the intervention group (p = 0.039). CONCLUSION: Even short-course treatment with systemic GCS decreases the serum cortisol level and has a significant effect on other blood tests results. Systemic GCS used for acute virus-induced wheezing treatment did not prove to reduce the average length of stay in the hospital. Objective criteria for initiation of such treatment are still lacking, which might consequently lead to the overuse of corticosteroids.

Lower Airway Virology in Health and Disease-From Invaders to Symbionts.

Studies of human airway virome are relatively recent and still very limited. Culture-independent microbial techniques showed growing evidence of numerous viral communities in the respiratory microbial ecosystem. The significance of different acute respiratory viruses is already known in the pathogenesis of chronic conditions, such as asthma, cystic fibrosis (CF), or chronic obstructive lung disease (COPD), and their exacerbations. Viral pathogens, such as influenza, metapneumovirus, parainfluenza, respiratory syncytial virus, or rhinovirus, have been associated with impaired immune response, acute exacerbations, and decrease in lung function in chronic lung diseases. However, more data have attributed a role to Herpes family viruses or the newly identified Anelloviridae family of viruses in chronic diseases, such as asthma, idiopathic pulmonary fibrosis (IPF), or CF. Impaired antiviral immunity, bacterial colonization, or used medication, such as glucocorticoids or antibiotics, contribute to the imbalance of airway microbiome and may shape the local viral ecosystem. A specific part of virome, bacteriophages, frames lung microbial communities through direct contact with its host, the specific bacteria known as Pseudomonas aeruginosa or their biofilm formation. Moreover, antibiotic resistance is induced through phages via horizontal transfer and leads to more severe exacerbations of chronic airway conditions. Morbidity and mortality of asthma, COPD, CF, and IPF remains high, despite an increased understanding and knowledge about the impact of respiratory virome in the pathogenesis of these conditions. Thus, more studies focus on new prophylactic methods or therapeutic agents directed toward viral⁻host interaction, microbial metabolic function, or lung microbial composition rearrangement.

Relevance of nasal potential difference in diagnosis of cystic fibrosis among children.

OBJECTIVE. The aim of this study was to estimate the significance of nasal potential difference (NPD) in the diagnosis of cystic fibrosis (CF) in children with clinical symptoms suggestive of the disease, positive sweat test results, and/or genetically confirmed diagnosis. MATERIAL AND METHODS. NPD measurements according to the modifications by Alton were performed in 50 children with clinical CF symptoms supported by positive sweat test results, 50 children with other obstructive lung diseases, and 50 healthy children. A subgroup of 17 children with the diagnosis confirmed by 2 identified mutations in the CF transmembrane regulatory gene was analyzed individually. RESULTS. The mean NPD value recorded in 50 children with clinical symptoms of CF supported by positive sweat test results and/or genetic analysis was -28.0 mV [SD, 10.2]. The mean NPD value in the subgroup of children with 2 identified mutations in the CF gene (n=17) was more negative than in the subgroup of children with unrecognized mutations (n=33) (-37.1 mV [SD, 7.0] vs. -23.4 mV [SD, 8.3], P<0.001). The mean NPD value in patients with other obstructive lung diseases and healthy children was significantly more positive than in the group of CF children with positive sweat test results and/or identified mutations (-18.1 mV [SD, 3.6] and -15.5 mV [SD, 4.3] vs. -28.0 mV [SD, 10.2], P<0.001). The NPD cut point value for the genetically confirmed diagnosis of CF was -35.0 mV (sensitivity, 93.9%; specificity, 88.2%), while in general, the NPD prognostic value was -24.0 mV (sensitivity, 58.0%; specificity, 98.0%). CONCLUSIONS. The NPD measurement is a valuable tool for the diagnosis of CF in children, but further studies are necessary to establish NPD values related to the CF genotype and to reduce the intrasubject variability of this test.

A mediastinal mass mimicking asthma symptoms.

Extramedullary myeloid sarcoma is a rare form of myelogenous leukemia. It can involve any anatomical body part. Mediastinal involvement is reported in only few cases. We report on a case of extramedullary myeloid sarcoma presenting as a mediastinal mass in a previously healthy nonleukemic male teenager with primary asthmatic complaints and the signs of superior vena cava syndrome.

[Consensus on asthma diagnosis and treatment in children and adults]. / Vaiku ir suaugusiuju astmos diagnostikos ir gydymo sutarimas.

Asthma is a chronic airway disease that is a growing problem of public health. The current consensus is based on the Global Initiative for Asthma (GINA) and other guidelines for asthma, and adapted for Lithuania. This Consensus provides physicians with recommendations for asthma management in children and adults.

[Current treatment options for acute bronchiolitis in children]. / Naujas poziuris i vaiku uminio bronchiolito gydyma.

Bronchiolitis is the most common lower respiratory tract infection in infants and is responsible for the majority of pediatric hospital admissions in winter. Respiratory syncytial virus has been identified as the main causative agent, causing 50-90% of the cases of bronchiolitis. Despite significant advances in pharmacotherapy, the management of infants with bronchiolitis has changed little over the years from supplemental oxygen and good fluid management. Approaches to therapy vary widely all over the world and are controversial. This paper reviews current treatment options for bronchiolitis, including the use of bronchodilators, epinephrine, steroids and ribavirin. Most recent advances, including immunotherapy and intensive care, are discussed.