Case Report: First Occurrence of Plasmablastic Lymphoma in Activated Phosphoinositide 3-Kinase δ Syndrome

Activated phosphoinositide 3-kinase δ syndrome (APDS) is an autosomal dominant primary immunodeficiency caused by acquired gene function mutation (GOF). APDS has a variety of clinical phenotypes, particularly recurrent respiratory infections and lymphoproliferation. Here we report a pediatric patient with APDS who presented with recurrent respiratory infections, lymphoproliferation, hepatosplenomegaly, bronchoscopy suggesting numerous nodular protrusions in the airways and a decrease in both T and B lymphocytes, and progression to plasmablastic lymphoma (PBL) after 1 year. Whole exome sequencing revealed a heterozygous mutation in the PIK3CD gene (c.3061 G>A p.E1021K). This is the first reported case of APDS combined with PBL and pediatricians should follow up patients with APDS regularly to be alert for secondary tumours.

Tracheobronchoplasty and Diaphragmatic Plication under VV ECMO for Combined ECAC and Diaphragmatic Paralysis

The coexistence of expiratory central airway collapse and diaphragmatic paralysis presents a diagnostic and treatment challenge. Both entities are underrecognized causes of dyspnea, cough, sputum production, and orthopnea. Optimal treatment must be individualized and is best achieved by a multidisciplinary team. We present a case of a patient with profound functional impairment from dyspnea and hypoxemia due to expiratory central airway collapse, complicated by bronchiectasis from recurrent respiratory infections, and diaphragmatic paralysis.

THYMUS SONOMETRY AND RECURRENT RESPIRATORY INFECTIONS IN PRESCHOOL CHIKDREN

Introduction. The results of scientific research that have been received so far in terms of the relationship between health status of children of various age and thymus size are controversial. On the one hand, researchers mainly from the former Soviet Union note the presence of the clear association between thymomegaly and an increased tendency to recurrent respiratory infections among preschool children. On the other hand, according to current data of world medical science, a relatively larger thymus, on the contrary, is considered to be the sign of the optimal state of child's immune system. The study aimed at clarifying of the interdependence between the thymus physical parameters and the frequency of acute respiratory infections in children aged 1-6 years. Materials and Methods. Thirty-seven children (16 boys and 21 girls) aged from one to six years, undergoing inpatient treatment on acute respiratory infection, were involved in the clinical study. Two markers of acute respiratory infections recurrence (infections index, resistance index) were taken into account and several anthropometric parameters were calculated for each child. The participants also underwent ultrasound scan. The statistical processing of the obtained primary digital material was performed by IBM SPSS Statistics 26 licensed program. Results. The thymus size was within the reference values only in 14 (37.84%) examined children. In the majority of the patients (23 (62.16%)), it was below the mentioned values. The significant exceeding the thymic index values in the children of the younger age group as compared with the older children was found. There was no difference between the boys and girls in terms of thymus volume, mass and thymic index. Conclusions. There is а moderately pronounced inverse relationship between the age of the examined children and the thymic index, according to which this index values are decreased with increasing age. The integral indicators of acute respiratory infections recurrence among the preschool children are not correlated with the studied absolute and relative sonometric thymus parameters. The thymic index values are in the closest and inverse correlation with the body surface area among all the anthropometric parameters considered in the children.

Immunoprophylaxis of influenza, acute and recurrent respiratory infections during the COVID-19 pandemic

The article notes features of the last epidemic season (2020-2021) in the form of extremely low activity of influenza viruses with SARS-CoV-2 remaining dominant in the etiological structure of acute respiratory viral infections. Presented own data indicating the heterogeneity of SARS-CoV-2 (Alpha, Delta, B.1.1.317, B.1.1.397, B.1.1.523) isolated from hospitalized patients was noted. An increase in the etiological role of bocavirus, alpha-coronavirus and metapneumovirus with a decrease in the frequency of parainfluenza viruses, adenoviruses, rhinoviruses and respiratory syncytial virus was established. An unusual shift of the period of maximum activity of respiratory syncytial virus to the 20th week was noted. Attention is paid to the need during the ongoing pandemic COVID-19 to fully immunize children as part of the National Immunization Calendar, also emphasized the role of mandatory annual vaccination against influenza. It is noted that the maximum protective effect of immunoprophylaxis of influenza acute and recurrent respiratory infections can be achieved with a combination of vaccination with bacterial lysates. The article presents a review of the literature and our data demonstrating the safety, good tolerability, and high clinical and immunological efficiency of polyvalent mechanical bacterial lysate for the prevention of acute and recurrent respiratory infections in children. It is shown that the use of polyvalent mechanical bacterial lysate during the prevaccination period makes it possible to substantially reduce the frequency of intercurrent infections, which reduces the number of temporary medical withdrawals and increases the coverage of vaccinations against influenza in organized groups to 85.1%. The role of trained immunity as one of the possible mechanisms providing nonspecific immunoprophylaxis during influenza vaccination and the use of bacterial lysates is discussed.

A Case of Severe COVID-19 in a Patient with Good's Syndrome

Good’s syndrome is a rare adult-onset combined immunodeficiency. The association of hypogammaglobulinaemia with a history of recurrent infectious or autoimmune manifestations in a middle-aged patient with evidence of a mediastinal mass should lead to the clinical suspicion of Good’s syndrome. The mortality rate associated with infectious complications is high. Thus, although it is rare, the disease should be diagnosed early so that proper treatment can be started. Thymectomy and immunoglobulin replacement are the main therapeutic strategies. We describe the case of a patient with a history of thymoma and recurrent respiratory infections, with a late diagnosis of Good’s syndrome in the context of severe organizing pneumonia secondary to COVID-19.

Bronchial hygiene technique in patients with cystic fibrosis

Cystic Fibrosis is characterized by excess pulmonary secretions that cause recurrent respiratory infections, with consequent deterioration of gas exchange. Bronchial hygiene techniques aim to mobilize secretions from the peripheral airways so that they can be eliminated by coughing or tracheal aspiration. To identify the effects of different bronchial hygiene techniques on improving lung function in patients with Cystic Fibrosis. Through a systematic review of the literature, randomized controlled trials (RCTs) published between 2007 and 2021 were selected, according to the highest score in the PEDro score. The search involved the PEDro and PubMed databases, using the following keywords: bronchial hygiene. Six ECR`s were included. One study performedthe techniques during anesthesia and observed increased resistance and reduced compliance. Regarding FEV1, 3 RCTs with hospitalized patients showed improvement in lung function, regardless of the technique used. In outpatients, there was no improvement. Regarding secretion weight, the cough machine produced more secretion than autogenous drainage, as well as a drop in saturation after the 2-min walk test, and increased FEV1. Bronchial hygiene techniques in patients with cystic fibrosis have been shown to be effective in removing mucus, with consequent improvement in lung function and aerobic fitness.

Diagnostic Barriers in Children with Immunodeficiencies in Central Asia: A Case-Based Discussion

Common variable immunodeficiency (CVID) is a primary immune deficit (PID) mainly characterized by hypogammaglobulinemia. In addition to increased susceptibility to infections and several immune-mediated manifestations, patients with CVID frequently develop bronchiectasis because of recurrent respiratory infections. This occurrence could be more likely if the diagnosis of CVID is delayed, as it often happens in less resourced clinical settings. A 15-year-old female patient was admitted to a tertiary hospital in Kazakhstan for consultation regarding a previous and established diagnosis of bronchiectasis. The clinical history was characterized by recurrent respiratory infections for several years, in addition to the development of a mixed restrictive-obstructive respiratory syndrome. Therefore, she underwent chest computerized tomography, which confirmed the presence of multiple and bilateral bronchiectasis. The clinical discussion on this patient highlighted that serum immunoglobulins were never measured previously and, thus, their assessment was strongly recommended. Based on that, a diagnosis of CVID was finally achieved, and the patient started the appropriate immunoglobulin replacement therapy. To our knowledge, this report is the first English-language publication on CVID and bronchiectasis from Central Asia. Bronchiectasis is currently an important medical problem in developing countries and populations with low socioeconomic status, where the diagnosis of the underlying cystic fibrosis and non-cystic fibrosis comorbidities can be delayed and more difficult than in countries with more accessible health care systems and facilities. This case report emphasized this important clinical issue in Central Asia and should raise the medical attention and awareness of this health problem, in order to improve the diagnostic timing and rate.