- Recurrent respiratory tract infections or acute otitis media were not a risk factor for vocabulary development in children at 13 and 24 months of age. [Journal Article]
- APActa Paediatr 2018 Aug 20
- CONCLUSIONS: The child's gender and parental socioeconomic status played a more critical role in vocabulary development in the first two years than a high burden of RTIs or AOM. This article is protected by copyright. All rights reserved.
- Timing of infections in patients with primary immunodeficiencies treated with intravenous immunoglobulin (IVIg). [Journal Article]
- AAAllergy Asthma Clin Immunol 2018; 14:35
- CONCLUSIONS: We believe that this pilot study is the first reported prospective study to examine the timing of infections after IVIg infusion in individuals with CVID and XLA. Further multi-centered research with a larger sample size is required into the comparison of infection rates in primary immunodeficiency patients treated with IVIg versus subcutaneous immunoglobulin therapy, where serum IgG levels remain at steady state.
- Respiratory syncytial virüs infections in neonates and infants. [Review]
- TPTurk Pediatri Ars 2018; 53(2):63-70
- Respiratory syncytial virus is one of the major causes of respiratory tract infections during infancy with high rates of hospitalization and mortality during the first years of life. It is the most c...
Respiratory syncytial virus is one of the major causes of respiratory tract infections during infancy with high rates of hospitalization and mortality during the first years of life. It is the most common cause of acute bronchiolitis and viral pneumonia in children below two years of age and second the most common cause of postneonatal infant mortality all around the world following malaria. In addition, the virus has been causally linked to recurrent wheezing and associated with pediatric asthma. The respiratory syncytial virus infections tend to be severe in high risk patients such as patients below six months of age, with prematurity, congenital heart diseases, neuromuscular diseases and immune deficiencies. No specific treatment is available for respiratory syncytial virus infections to date. Severe cases require supportive therapy, mainly oxygen supplementation and hydration, and less frequently, ventilatory support. Because there is no vaccine to prevent respiratory syncytial virus infections or clinically effective treatment to administer to children with respiratory syncytial virus infection, immunoprophylaxis with palivizumab is currently the only method for reducing morbidity associated with severe respiratory syncytial virus in high-risk infants.
- Management of bronchiectasis in adults. [Journal Article]
- MJMed J Aust 2018 Aug 20; 209(4):177-183
- Once neglected in research and underappreciated in practice, there is renewed interest in bronchiectasis unrelated to cystic fibrosis. Bronchiectasis is a chronic lung disease characterised by chroni...
Once neglected in research and underappreciated in practice, there is renewed interest in bronchiectasis unrelated to cystic fibrosis. Bronchiectasis is a chronic lung disease characterised by chronic cough, sputum production and recurrent pulmonary exacerbations. It is diagnosed radiologically on high resolution computed tomography chest scan by bronchial dilatation (wider than the accompanying artery). The causes of bronchiectasis are diverse and include previous respiratory tract infections, chronic obstructive pulmonary disease, asthma, immunodeficiency and connective tissue diseases. A large proportion of cases are idiopathic, reflecting our incomplete understanding of disease pathogenesis. Progress in the evidence base is reflected in the 2017 European management guidelines and the 2015 update to the Australian guidelines. Effective airway clearance remains the cornerstone of bronchiectasis management. This should be personalised and reviewed regularly by a respiratory physiotherapist. There is now robust evidence for the long term use of oral macrolide antibiotics in selected patients to reduce exacerbation frequency. The routine use of long term inhaled corticosteroids and/or long-acting bronchodilators should be avoided, unless concomitant chronic obstructive pulmonary disease or asthma exists. The evidence for nebulised agents including hypertonic saline, mannitol and antibiotics is evolving; however, access is challenging outside tertiary clinics, and nebulising equipment is required. Smokers should be supported to quit. All patients should receive influenza and pneumococcal vaccination. Patients with impaired exercise capacity should attend pulmonary rehabilitation. There is an important minority of patients for whom aetiology-specific treatment exists. The prevalence of bronchiectasis is increasing worldwide; however, the burden of disease within Australia is not well defined. To this end, the Australian Bronchiectasis Registry began recruitment in 2016 and is interoperable with the European and United States bronchiectasis registries to enable collaborative research. The recent addition of a bronchiectasis diagnosis-related group to the Australian Refined Diagnostic Related Group classification system will allow definition of the disease burden within the Australian hospital system.
- Juvenile-onset recurrent respiratory papillomatosis with pulmonary involvement and carcinomatous transformation. [Journal Article]
- ACAutops Case Rep 2018 Jul-Sep; 8(3):e2018035
- Papilloma associated with recurrent respiratory papillomatosis (RRP), caused by human papilloma virus (HPV) infection types 6 and 11, is the most common benign neoplasm of the larynx. The clinical fe...
Papilloma associated with recurrent respiratory papillomatosis (RRP), caused by human papilloma virus (HPV) infection types 6 and 11, is the most common benign neoplasm of the larynx. The clinical features of RRP vary widely from mild to aggressive forms. RRP in children is known as juvenile-onset recurrent respiratory papillomatosis (JORRP). Its outcome may be poor or even fatal due to the high rate of recurrence and eventual spread to the entire respiratory tract. Pulmonary invasion is reported to occur in 3.3% of patients with RRP, and malignant transformation in 0.5% of patients. We report the case of a 39-year-old female patient with a diagnosis of JORRP from the age of 3 years, with extensive bilateral pulmonary involvement and malignant transformation. Analysis of the papilloma and carcinomatous tissues revealed the presence of HPV type 11, which is associated with rapid and aggressive progression. We discussed the case on the basis of a literature review on pulmonary invasion, malignant transformation, and HPV 11 aggressiveness.
- TNFRSF13B/TACI Alterations in Turkish Patients with Common Variable Immunodeficiency and IgA Deficiency. [Journal Article]
- AJAvicenna J Med Biotechnol 2018 Jul-Sep; 10(3):192-195
- CONCLUSIONS: Modifier genes as well as their combination with other genetic or environmental factors may play an important role in the development of the immunodeficiency phenotype.
- Exposure to environmental tobacco smoke by healthy children aged below five (Preliminary study). [Journal Article]
- TPTurk Pediatri Ars 2018; 53(1):37-44
- CONCLUSIONS: In these regularly-monitored preschool children, we found frequent exposure to cigarette smoke. This study contributes to national data and will aid in increasing the awareness for the deleterious effects of passive smoking on child health.
- Structural basis for prolidase deficiency disease mechanisms. [Journal Article]
- FJFEBS J 2018 Aug 01
- Prolidase is a metallopeptidase that cleaves iminodipeptides containing a proline (Pro) or hydroxyproline (Hyp) residue at their C-terminal end. The disease prolidase deficiency (PD) is a rare recess...
Prolidase is a metallopeptidase that cleaves iminodipeptides containing a proline (Pro) or hydroxyproline (Hyp) residue at their C-terminal end. The disease prolidase deficiency (PD) is a rare recessive human disorder characterized by reduced prolidase activity. PD manifests itself by a wide range of severe clinical symptoms, most commonly as skin ulceration, recurrent infections of the respiratory tract, and mental retardation. Several mutations in the PEPD gene have been identified that are responsible for the loss or the reduction of prolidase activity. In contrast, the structural basis of enzyme inactivation has so far remained elusive. In this study, we present high resolution crystal structures of a number of human prolidase (HsProl) variants, in which single amino acids are either substituted by others or deleted. The observed implications of the mutations on the three-dimensional structure of HsProl are reported and discussed and related to their enzymatic activity. The resulting structures may be divided into four groups depending on the presumed effect of the corresponding mutations on the reaction mechanism. The four possible inactivation mechanisms, which could be elucidated, are disruption of the catalytic Mn2 (OH- )-center, introduction of chain disorder along with the displacement of important active site residues, rigidification of the active site, and flexibilization of the active site.
- Impact of mold on mast cell-cytokine immune response. [Editorial]
- JBJ Biol Regul Homeost Agents 2018 Jul-Aug; 32(4):763-768
- Molds include all species of microscopic fungi, the spores of which are small molecules, ubiquitous, mostly found in soil with higher rainfall and high humidity, in the atmosphere of urban and rural ...
Molds include all species of microscopic fungi, the spores of which are small molecules, ubiquitous, mostly found in soil with higher rainfall and high humidity, in the atmosphere of urban and rural settings and in decaying vegetation. They originate from pathogenic fungi and have a crucial role in inflammatory response, causing a broad range of diseases. Immune suppressed subjects may develop mycoses caused by opportunistic common pathogenic fungi. Mast cells (MCs) are immune cells involved in the pathophysiology of infected skin, lung, and organs, where there is an increase of angiogenesis. Airways fungi infections can induce allergic lung disease mediated by MCs and other immune cells. In addition, fungal infection may cause and/or aggravate asthma inflammation. Spores are able to navigate in the airways of the lung and can be recognized trough toll-like receptor (TLR) signaling by the innate immune cells including MCs. Activated MCs release preformed mediators including histamine, proteases (tryptase, chimase), pro-inflammatory cytokines/chemokines and they also generate arachidonic acid products. MCs activated by fungi provoke an increases of PGD2 levels and lead to hypersensitivity diseases which present signs such as irritation of the respiratory tract and eyes, recurrent sinusitis, bronchitis, cough and neurological manifestations including fatigue, nausea, headaches and brain fog. Therefore, fungi activate the innate immune response through the TLRs, leading to the release of myeloid differentiation factor 88 (MyD88) which, with a series of cascade reactions, induces the stimulation of AP-1 and NF-kB with subsequent activation of inflammatory IL-1 family members. Here, we report that fungi can activate MCs to secrete pro-inflammatory cytokines which may be inhibited by IL-37, a new anti-inflammatory IL-1 family member.
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- The psoriasis risk allele HLA-Cw0602 shows evidence of association with chronic or recurrent streptococcal tonsillitis. [Journal Article]
- IIInfect Immun 2018 Jul 23
- Pharyngeal tonsillitis is one of the most common upper respiratory tract infections and Group A streptococcus is the most important bacterial pathogen causing it. While most patients experience tonsi...
Pharyngeal tonsillitis is one of the most common upper respiratory tract infections and Group A streptococcus is the most important bacterial pathogen causing it. While most patients experience tonsillitis only rarely, a subset of patients suffer from recurrent or chronic tonsillitis or pharyngitis. The predisposing factors for recurring or chronic form of this disease are not yet fully understood but genetic predisposition has been suggested. A genetic association study using Illumina's Immunochip SNP array was performed to search for new genetic biomarkers in pharyngeal tonsillitis. Over 100,000 SNPs relevant to immune-mediated diseases were analyzed in a cohort of 95 patients subjected to tonsillectomy due to recurrent/chronic tonsillitis and 504 controls. Genetic association between the cases and controls showed strongest association with two peaks in the HLA locus (OR 3.7-4.7, p=4.9-5.7x10-6). Further analysis with imputed classical HLA alleles suggested the known psoriasis risk allele HLA-C*06:02 as a risk factor for tonsillitis (p=4.8 x 10-4, OR 2.3). In addition, the imputed HLA haplotype HLA-C*06:02/HLA-B*5701, a reported risk haplotype in psoriasis, had the strongest risk for tonsillitis, too (p=8.0 x 10-3, OR 5.5). These findings further support the previously reported link between streptococcal throat infections and psoriasis.