Postinfectious Bronchiolitis Obliterans

Childhood bronchiolitis obliterans (BO) is an uncommon complication that is characterized clinically by persistent and continuous obstructive respiratory symptoms, and has been described secondary to various etiologic factors, including drugs, exposure to toxic fumes, allergic reactions, collagen vascular disease or infections. BO occurs most commonly in children after an episode of acute bronchiolitis and is considered a long-term sequela of viral infection. Postinfectious Bronchiolitis Obliterans (PIBO) is characterized by persistent airway obstruction with functional and radiological evidence of small airway involvement that is in general unresponsive to bronchodilator treatment. Although the condition is relatively rare, and its exact incidence is unknown, it is important to keep it in mind. PIBO is complication of lower respiratory tract epithelial injury, and is often misdiagnosed, delaying recognition and potential treatment. A PIBO diagnosis is usually based on a few factors, including a good medical history, positive clinical findings, and lung function test and imaging results, although biopsy and histopathology remain as the optimum diagnostic approach. There have to date been few studies proposing treatments for the condition, and no accepted protocol exists in literature. There is usually a fixed airway obstruction in PIBO. Various treatment approaches have been extrapolated from studies of post bone marrow transplantations and lung transplant BO. The clinical course is variable, and good supportive therapy is essential, with anti-inflammatory therapy often being employed

Use of Flexible Bronchoscopy in Foreign Body Aspiration

Objective:Foreign body aspiration remains a serious health problem with a potential for severe consequences, and acute and chronic problems in children. It therefore demands immediate intervention. Rigid bronchoscopy has long been the method of choice for foreign body removal but is now being replaced by flexible bronchoscopy which offers reduced trauma and the ability to access distal bronchial regions. In the presented study we assessed the patients who underwent flexible bronchoscopy for foreign body removal in our clinic.Methods:We reviewed the records of 20 patients who underwent flexible bronchoscopy due to suspected foreign body aspiration and had a foreign body removed in our clinic. Patients were analyzed in terms of sociodemographic data, foreign bodies removed, method of foreign body removal, foreign body location and time to diagnosis.Results:Our study group included nine females and 11 males. The removed foreign body was organic in 19 of the 20 patients and inorganic in one patient. Hard organic food, such as hazelnuts, peanuts, seeds, almonds, and raw corn kernel were identified in 12 patients, a piece of fishbone in one patient and a piece of gelatin in another. The pieces of soft organic food identified were apple in one patient, egg in one patient, and boiled corn kernel in one patient and removed by suction. The foreign body was removed using forceps in nine patients, and a basket was used successfully in seven patients.Conclusion:Foreign bodies can be removed with minimal complication using flexible bronchoscopy, basket, and forceps in children

Development Of Novel Self-Assembled Polymeric Micelles From Partially Hydrolysed Poly(2-Ethyl-2-Oxazoline)-Co-Pei-B-Pcl Block Copolymer As Non-Viral Vectors For Plasmid Dna In Vitro Transfection

A new efficient, non-viral gene delivery cationic polymeric micellar system was developed by partial hydrolysis of poly(2-ethyl-2-oxazoline) (PEtOx) with two different hydrolysis percentages of PEtOx (30% and 60%) to reduce the disadvantages of the PEI. These self-assemble amphiphilic cationic micelles prepared from poly(2-ethyl-2-oxazoline)(30%)-co-poly(ethyleneimine)-block-poly(epsilon-caprolactone) (PEtOx(30%)-co-PEI-b-PCL) (PPP30) and poly(2-ethyl-2-oxazoline) (60%)-co-poly(ethyleneimine)-block-poly(epsilon-caprolactone) (PEtOx(60%)-co-PEI-b-PCL) (PPP60) block copolymers were successfully condensed with pEGFP-C3 plasmid DNA via electrostatic interactions to form micelle/DNA complexes with desirable particle sizes. All formulations showed low critical micelle concentration (CMC) values that means highly stable in serum containing medium. Polymeric micelles were also evaluated for their stability in the presence of serum and nuclease as well as cytotoxicity and transfection efficiency. All our results proved that our novel polymeric micellar system prepared by PPP60 block copolymer offer to be an efficient promising carrier for gene delivery applications. Moreover, these findings contribute to design and development of novel gene vectors with tunable and functionality features and also to reduce the cytotoxicity of PEI by partial hydrolysis of PEtOx an alternative synthesis method to produce linear PEI. [GRAPHICS] .WoSScopu

Comparison of refugee patients with cystic fibrosis and their counterpart children from Turkey during the war

Since the outbreak of the Syrian civil war in 2011, the population of Arab refugees in Turkey has rapidly increased. While cystic fibrosis (CF) is believed to be rare among Arabs, recent studies suggest it is underdiagnosed. This study aims to present the demographic, clinical, and genetic characteristics of CF patients among Arab refugees in Turkey. Additionally, a comparison is made between the findings in the National CF Registry 2021 in Turkey (NCFRT) and the refugee CF patient group. The study included refugee patients between the ages of 0 and 18 years who were diagnosed with CF and received ongoing care at pediatric pulmonology centers from March 2011 to March 2021. The study examined demographic information, age at diagnosis, age of diagnosis of patients through CF newborn screening (NBS), presenting symptoms, CF transmembrane conductance regulator (CFTR) mutation test results, sputum culture results, weight, height, and body mass index (BMI) z score. Their results were compared with the NCFRT results. The study included 14 pediatric pulmonology centers and 87 patients, consisting of 46 (52.9%) boys and 41 (47.1%) girls. All of the patients were Arab refugees, with 80 (92%) being Syrian. All the patients were diagnosed in Turkey. The median age at diagnosis of patients was 22.33 (interquartile range, 1–258) months. The median age of diagnosis of patients through NBS was 4.2 (interquartile range, 1–12) months. The median age of older patients, who were unable to be included in the NBS program, was 32.3 (interquartile range, 3–258) months. Parental consanguinity was observed in 52 (59.7%) patients. The mutation that was most frequently found was F508del, which accounted for 22.2% of the cases. It was present in 20 patients, constituting 32 out of the total 144 alleles. There was a large number of genetic variations. CFTR genotyping could not be conducted for 12 patients. These patients had high sweat tests, and their genetic mutations could not be determined due to a lack of data. Compared to NCFRT, refugee patients were diagnosed later, and long-term follow-up of refugee CF patients had significantly worse nutritional status and pseudomonas colonization. Conclusion: Although refugee CF patients have equal access to NBS programs and CF medications as well as Turkish patients, the median age at diagnosis of patients, the median age of diagnosis of patients through NBS, their nutritional status, and Pseudomonas colonization were significantly worse than Turkish patients, which may be related to the difficulties of living in another country and poor living conditions. The high genetic heterogeneity and rare mutations detected in the refugee patient group compared to Turkish patients. Well-programmed NBS programs, thorough genetic studies, and the enhancement of living conditions for refugee patients in the countries they relocate to can have several advantages such as early detection and improved prognosis. What is Known: • Children who have chronic diseases are the group that is most affected by wars. • The outcome gets better with early diagnosis and treatment in patients with Cystic Fibrosis (CF). What is New: • Through the implementation of a newborn screening program, which has never been done in Syria previously, refugee patients, the majority of whom are Syrians were diagnosed with cystic fibrosis within a duration of 4 months. • Despite equal access to the newborn screening program and CF medications for both Turkish patients and refugee patients, the challenges of living in a foreign country have an impact on refugees

Childhood interstitial lung disease in Turkey: first data from the national registry

Abstract: The childhood interstitial lung diseases (chILD) Turkey registry (chILD-TR) was established in November 2021 to increase awareness of disease, and in collaboration with the centers to improve the diagnostic and treatment standards. Here, the first results of the chILD registry system were presented. In this prospective cohort study, data were collected using a data‐entry software system. The demographic characteristics, clinical, laboratory, radiologic findings, diagnoses, and treatment characteristics of the patients were evaluated. Clinical characteristics were compared between two main chILD groups ((A) diffuse parenchymal lung diseases (DPLD) disorders manifesting primarily in infancy [group1] and (B) DPLD disorders occurring at all ages [group 2]). There were 416 patients registered from 19 centers. Forty-six patients were excluded due to missing information. The median age of diagnosis of the patients was 6.05 (1.3–11.6) years. Across the study population (n = 370), 81 (21.8%) were in group 1, and 289 (78.1%) were in group 2. The median weight z-score was significantly lower in group 1 (− 2.0 [− 3.36 to − 0.81]) than in group 2 (− 0.80 [− 1.7 to 0.20]) (p < 0.001). When we compared the groups according to chest CT findings, ground-glass opacities were significantly more common in group 1, and nodular opacities, bronchiectasis, mosaic perfusion, and mediastinal lymphadenopathy were significantly more common in group 2. Out of the overall study population, 67.8% were undergoing some form of treatment. The use of oral steroids was significantly higher in group 2 than in group 1 (40.6% vs. 23.3%, respectively; p = 0.040). Conclusion: This study showed that national registry allowed to obtain information about the frequency, types, and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. What is Known: • Childhood interstitial lung diseases comprise many diverse entities which are challenging to diagnose and manage. What is New: • This study showed that national registry allowed to obtain information about the frequency, types and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. Also, our findings reveal that nutrition should be considered in all patients with chILD, especially in A-DPLD disorders manifesting primarily in infancy

Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?

Background: Cystic fibrosis (CF) is an autosomal recessive disorder caused by CF transmembrane conductance regulator (CFTR) genetic variants. CFTR modulators improve pulmonary function and reduce respiratory infections in CF. This study investigated the clinical and laboratory follow-up parameters over 1 year in patients with CF who could not receive this treatment. Methods: This retrospective cohort study included 2018 and 2019 CF patient data from the CF registry of Turkey. Demographic and clinical characteristics of 294 patients were assessed, who had modulator treatment indications in 2018 but could not reach the treatment. Results: In 2019, patients younger than 18 years had significantly lower BMI z-scores than in 2018. During the 1-year follow-up, forced expiratory volumes (FEV1) and FEV1 z-scores a trend toward a decrease. In 2019, chronic Staphylococcus aureus colonization, inhaled antipseudomonal antibiotic use for more than 3 months, oral nutritional supplement requirements, and oxygen support need increased. Conclusions: Patients who had indications for modulator treatments but were unable to obtain them worsened even after a year of follow-up. This study emphasized the importance of using modulator treatments for patients with CF in our country, as well as in many countries worldwide