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1.
Bioorg Chem ; 143: 107068, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38181659

RESUMO

α-Amylase is a secretory enzyme commonly found in nature. The α-Amylase enzyme catalyzes the hydrolysis of α-D-(1,4)-glucosidic bonds in starch, glycogen, and polysaccharides. The chemical characterization of the composite carrier and the immobilized enzyme was performed, and the accuracy of the immobilization was confirmed by FTIR, SEM, and EDS analyses. The X-ray diffraction (XRD) analysis indicates that the magnetic nanoparticle retained its magnetic properties following the modification process. Based on the Thermogravimetric Analysis (TGA) outcomes, it was evident that the structural integrity of the FPT nanocomposite remained unchanged at 200°C. The optimal pH was determined to be 5.5, and no alteration was observed following the immobilization process. Purified α-amylases usually lose their activity rapidly above 50°C. In this study, Bacillus licheniformis α-Amylase enzyme was covalently immobilized on the newly synthesized magnetic composite carrier having more azole functional group. For novelty-designed immobilized enzymes, while there is no change in the pH and optimum operating temperature of the enzyme with immobilization, two essential advantages are provided to reduce enzyme costs: the storage stability and reusability are increased. Furthermore, our immobilization technique enhanced enzyme stability when comparing our immobilized enzyme with the reference enzyme in industrial applications. The activity of the immobilized enzyme was higher in presence of 1-3% H2O2.


Assuntos
Bacillus licheniformis , Compostos de Epóxi , Nanopartículas de Magnetita , Metacrilatos , Triazóis , Enzimas Imobilizadas/química , Bacillus licheniformis/metabolismo , Peróxido de Hidrogênio , Nanopartículas de Magnetita/química , Concentração de Íons de Hidrogênio , Estabilidade Enzimática , alfa-Amilases/metabolismo , Temperatura
2.
Eur J Pediatr ; 183(4): 1831-1838, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38265526

RESUMO

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.


Assuntos
Fibrose Cística , População do Oriente Médio , Refugiados , Recém-Nascido , Masculino , Criança , Feminino , Humanos , Lactente , Pré-Escolar , Adolescente , Fibrose Cística/diagnóstico , Fibrose Cística/epidemiologia , Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Turquia/epidemiologia , Triagem Neonatal/métodos
3.
Eur J Pediatr ; 183(1): 295-304, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37875631

RESUMO

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.


Assuntos
Doenças Pulmonares Intersticiais , Linfadenopatia , Criança , Humanos , Pulmão , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/terapia , Estudos Prospectivos , Sistema de Registros , Turquia/epidemiologia , Lactente , Pré-Escolar
4.
BMC Health Serv Res ; 24(1): 60, 2024 Jan 11.
Artigo em Inglês | MEDLINE | ID: mdl-38212713

RESUMO

BACKGROUND: Gradually, society has shifted more services online, with COVID-19 highlighting digital inequalities in access to services such as healthcare. Older adults can experience such digital inequalities, yet this group is also more likely to need medical appointments, compared to younger people. With the growing digitalisation of healthcare, it is increasingly important to understand how older people can best use communicative e-health services to interact with healthcare services. This is especially if older adults are to access, and actively interact with health professionals/clinicians due to their general health decline. This review aims to synthesise older adults' experiences and perceptions of communicative e-health services and, in turn, identify barriers and facilitators to using communicative e-health services. METHODS: A meta-ethnography was conducted to qualitatively synthesise literature on older adults' experiences of using communicative e-health services. A systematic search, with terms relating to 'older adults', 'e-health', 'technology', and 'communication', was conducted on six international databases between January 2014 and May 2022. The search yielded a total of 10 empirical studies for synthesis. RESULTS: The synthesis resulted in 10 themes that may impact older adults' perceptions and/or experiences of using communicative e-health services. These were: 1) health barriers, 2) support networks, 3) application interface/design, 4) digital literacy, 5) lack of awareness, 6) online security, 7) access to digital devices and the internet, 8) relationship with healthcare provider(s), 9) in-person preference and 10) convenience. These themes interlink with each other. CONCLUSION: The findings suggest older adults' experiences and perceptions of communicative e-health services are generally negative, with many reporting various barriers to engaging with online services. However, many of these negative experiences are related to limited support networks and low digital literacy, along with complicated application interfaces. This supports previous literature identifying barriers and facilitators in which older adults experience general technology adoption and suggests a greater emphasis is needed on providing support networks to increase the adoption and usage of communicative e-health services.


Assuntos
Atenção à Saúde , Serviços de Saúde , Humanos , Idoso , Pessoal de Saúde , Aceitação pelo Paciente de Cuidados de Saúde , Antropologia Cultural
5.
Thorax ; 78(6): 587-595, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36808083

RESUMO

BACKGROUND: The majority of patients with childhood interstitial lung disease (chILD) caused by pathogenic variants in ATP binding cassette subfamily A member 3 (ABCA3) develop severe respiratory insufficiency within their first year of life and succumb to disease if not lung transplanted. This register-based cohort study reviews patients with ABCA3 lung disease who survived beyond the age of 1 year. METHOD: Over a 21-year period, patients diagnosed as chILD due to ABCA3 deficiency were identified from the Kids Lung Register database. 44 patients survived beyond the first year of life and their long-term clinical course, oxygen supplementation and pulmonary function were reviewed. Chest CT and histopathology were scored blindly. RESULTS: At the end of the observation period, median age was 6.3 years (IQR: 2.8-11.7) and 36/44 (82%) were still alive without transplantation. Patients who had never received supplemental oxygen therapy survived longer than those persistently required oxygen supplementation (9.7 (95% CI 6.7 to 27.7) vs 3.0 years (95% CI 1.5 to 5.0), p=0.0126). Interstitial lung disease was clearly progressive over time based on lung function (forced vital capacity % predicted absolute loss -1.1% /year) and on chest CT (increasing cystic lesions in those with repetitive imaging). Lung histology pattern were variable (chronic pneumonitis of infancy, non-specific interstitial pneumonia, and desquamative interstitial pneumonia). In 37/44 subjects, the ABCA3 sequence variants were missense variants, small insertions or deletions with in-silico tools predicting some residual ABCA3 transporter function. CONCLUSION: The natural history of ABCA3-related interstitial lung disease progresses during childhood and adolescence. Disease-modifying treatments are desirable to delay such disease course.


Assuntos
Transportadores de Cassetes de Ligação de ATP , Doenças Pulmonares Intersticiais , Criança , Adolescente , Lactente , Humanos , Estudos de Coortes , Transportadores de Cassetes de Ligação de ATP/genética , Transportadores de Cassetes de Ligação de ATP/metabolismo , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/genética , Doenças Pulmonares Intersticiais/terapia , Pulmão/metabolismo , Tomografia Computadorizada por Raios X , Mutação
6.
Eur J Pediatr ; 182(3): 1067-1076, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36565324

RESUMO

We aimed to evaluate cutoff values of immunoreactive trypsinogen (IRT)/IRT and determine relationship between IRT values and clinical characteristics of children with cystic fibrosis (CF). This study is cross-sectional study. Data of children with positive newborn screening (NBS) between 2015 and 2021 were evaluated in three pediatric pulmonology centers. Age at admission, sex, gestational age, presence of history of meconium ileus, parental consanguinity, sibling with CF, and doll-like face appearance, first and second IRT values, sweat chloride test, fecal elastase, fecal fat, biochemistry results, and age at CF diagnosis were recorded. Sensitivity and specificity of IRT cutoff values were evaluated. Of 815 children with positive NBS, 58 (7.1%) children were diagnosed with CF. Median values of first and second IRT were 157.2 (103.7-247.6) and 113.0 (84.0-201.5) µg/L. IRT values used in current protocol, sensitivity was determined as 96.6%, specificity as 17.2% for first IRT, and 96.6% sensitivity, 20.5% specificity for second IRT. Positive predictive value (PPV) was determined as 7.1%. When cutoff value for first IRT was estimated as 116.7 µg/L, sensitivity was 69.0% and specificity was 69.6%, and when cutoff value was set to 88.7 µg/L for second IRT, sensitivity was 69.0% and specificity was 69.0%. Area under curve was 0.757 for first and 0.763 for second IRT (p < 0.001, p < 0.001, respectively). PPV was calculated as 4.3%.    Conclusion: Although sensitivity of CF NBS is high in our country, its PPV is significantly lower than expected from CF NBS programs. False-positive NBS results could have been overcome by revising NBS strategy. What is Known: • Although immunoreactive trypsinogen elevation is a sensitive test used in cystic fibrosis newborn screening, its specificity is low. • In countries although different algorithms are used, all strategies begin with the measurement of immunoreactive trypsinogen in dried blood spots. What is New: • In our study, it was shown that use of the IRT/IRT protocol for cystic fibrosis newborn screening is not sufficient for the cut-off values determined by the high number of patients. • Newborn screening strategy should be reviewed to reduce false positive newborn screening results.


Assuntos
Fibrose Cística , Recém-Nascido , Criança , Humanos , Fibrose Cística/diagnóstico , Triagem Neonatal/métodos , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Tripsinogênio , Estudos Transversais , Sensibilidade e Especificidade
7.
Perfusion ; 38(1): 186-192, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-34590527

RESUMO

INTRODUCTION: Chronic obstructive pulmonary disease (COPD) is considered a major risk factor for postoperative complications after transcatheter aortic valve implantation (TAVI). To date, there is no clear consensus on the best anesthesia management for these patients. We aimed to investigate the effects of types of anesthesia on clinical outcomes in patients with severe COPD undergoing TAVI. METHODS: This is a single-center, retrospective study comparing conscious sedation (CS) versus general anesthesia (GA) in 72 patients with severe COPD who underwent TAVI. The primary endpoints were 30-day all-cause mortality and postoperative pulmonary complications. RESULTS: The main outcome of interest of this study was that the frequency of pulmonary complications was statistically higher in the GA group (21.4% vs 3.3%, p = 0.038). These differences are most likely attributed to the GA because of prolonged mechanical ventilation, and longer ICU stay (2 (1.2-3) vs 2.5 (2-4) days, p = 0.029) associated with an increased risk of nosocomial infections. There were no significant differences in procedure complications and 30-day mortality between the two groups (GA; 19% vs CS; 13.3%, p = 0.521). One-year survival rates, compared by Kaplan-Meier analysis, were similar between groups (log-rank p = 0.733). CONCLUSION: In aortic stenosis patients with severe COPD undergoing TAVI, the use of GA compared with CS was associated with higher incidences of respiratory-related complications, and longer ICU length of stay. CS is a safe and viable option for these patients and should be considered the favored approach.


Assuntos
Estenose da Valva Aórtica , Doença Pulmonar Obstrutiva Crônica , Substituição da Valva Aórtica Transcateter , Humanos , Substituição da Valva Aórtica Transcateter/efeitos adversos , Substituição da Valva Aórtica Transcateter/métodos , Sedação Consciente/efeitos adversos , Sedação Consciente/métodos , Estudos Retrospectivos , Resultado do Tratamento , Anestesia Geral/efeitos adversos , Anestesia Geral/métodos , Estenose da Valva Aórtica/complicações , Estenose da Valva Aórtica/cirurgia , Fatores de Risco , Doença Pulmonar Obstrutiva Crônica/complicações , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Valva Aórtica/cirurgia
8.
Sensors (Basel) ; 23(10)2023 May 20.
Artigo em Inglês | MEDLINE | ID: mdl-37430842

RESUMO

This paper provides a novel methodology for human-driven decision support for capacity allocation in labour-intensive manufacturing systems. In such systems (where output depends solely on human labour) it is essential that any changes aimed at improving productivity are informed by the workers' actual working practices, rather than attempting to implement strategies based on an idealised representation of a theoretical production process. This paper reports how worker position data (obtained by localisation sensors) can be used as input to process mining algorithms to generate a data-driven process model to understand how manufacturing tasks are actually performed and how this model can then be used to build a discrete event simulation to investigate the performance of capacity allocation adjustments made to the original working practice observed in the data. The proposed methodology is demonstrated using a real-world dataset generated by a manual assembly line involving six workers performing six manufacturing tasks. It is found that, with small capacity adjustments, one can reduce the completion time by 7% (i.e., without requiring any additional workers), and with an additional worker a 16% reduction in completion time can be achieved by increasing the capacity of the bottleneck tasks which take relatively longer time than others.

9.
J Clin Nurs ; 32(13-14): 3412-3420, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35818330

RESUMO

AIMS AND OBJECTIVES: The aim of this study was to evaluate the clinical features of children with tracheostomy and the predictors of psychological status of their primary caregivers in order to determine the associations between the children's clinical course with their caregivers' psychological status. BACKGROUND: The caregivers of children with tracheostomy are responsible for providing basic tracheostomy care at home. All these responsibilities may be associated with significant changes in family members' lifestyles, daily routines and family dynamics. DESIGN: This study is a cross sectional study. METHODS: Data of the family's socioeconomic status and clinical status of children with tracheostomy were noted in four paediatric pulmonology centers. The Beck Depression Inventory, Maslach Burnout Inventory, Zarit Caregiver Burden Scale, and Rosenberg Self-esteem Scale were used for psychological measurements of the caregivers. The STROBE checklist was used for this study. RESULTS: Eighty-five children and their primary caregivers were enrolled in the study. The children's median age was 4.1 years. Thirty-eight of them were dependent on home ventilators. Twenty-one had bacterial colonisation. All children's primary caregivers were their mothers. Beck Depression Inventory scores of mothers of children with colonisation were higher. Number of hospitalizations in previous 6 months was related to mothers' emotional exhaustion and depersonalization scores. Duration of children's hospitalizations in previous 6 months was positively correlated to mothers' emotional exhaustion, depersonalization, and Beck Depression Inventory scores. CONCLUSIONS: Mothers of children with tracheostomy may experience psychological conditions, such as high levels of depression, burnout, burden and low levels of self-esteem. Frequent and long-term hospitalizations of children correlated with mother's depression and burnout. Therefore, primary caregivers should be evaluated and supported psychologically. RELEVANCE TO CLINICAL PRACTICE: Preventing mothers of children with tracheostomy from experiencing psychological conditions such as depression, burnout, burden and low self-esteem can also increase the quality of care for children.


Assuntos
Esgotamento Profissional , Mães , Feminino , Humanos , Criança , Pré-Escolar , Mães/psicologia , Depressão/psicologia , Traqueostomia , Estudos Transversais , Esgotamento Psicológico , Cuidadores/psicologia , Progressão da Doença
10.
Turk J Med Sci ; 53(3): 761-770, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37476899

RESUMO

BACKGROUND: Considering its early bactericidal activity, isoniazid (H) is an important first-line agent in tuberculosis (TB) treatment.The aim of this study was to evaluate the treatment regimens and results of H-resistant pulmonary TB patients. METHODS: We retrospectively evaluated treatment regimens and results of 188 H-resistant pulmonary TB patients who were treated in our center between January 2015 and December 2017. Treatment regimens applied were noted and treatment outcomes were recorded. The long-term results were evaluated. RESULTS: Totally 174 (92.6%) of 188 patients with H-resistant pulmonary TB achieved treatment success. Ninety-seven patients (51.6%)were cured and 77 patients (41.0%) completed treatment. Five patients (2.7%) had treatment failure. Four patients (2.1%) having treatment success relapsed during one-year follow-up. Eighteen patients (9.6%) had unfavorable outcomes, including treatment failure in five (2.7%), death in nine (4.8%), and relapse in four patients (2.1%). The treatment success rate was found to be statistically higher in group 1 (9-month regimen 2HREZ/7HRE) compared with those in group 2 (9HREZ) (97.4% vs. 85.9%; p = 0.010). Group 3 (HREZFQ) and group 1 had statistically significant favorable outcomes, compared to group 2 (group 2 vs. group 3, p = 0.048; group 1 vs. group 2, p = 0.022). Interestingly, no relapse and acquired rifampicin resistance in patients occurred who received an FQ-containing regimen. DISCUSSION: Our study results show higher treatment success and positive results with the treatment regimen containing FQ and that treatment with HREZ for nine months is associated with a lower treatment success rate.


Assuntos
Tuberculose Resistente a Múltiplos Medicamentos , Tuberculose Pulmonar , Humanos , Isoniazida/uso terapêutico , Antituberculosos/uso terapêutico , Estudos Retrospectivos , Seguimentos , Tuberculose Pulmonar/tratamento farmacológico , Tuberculose Resistente a Múltiplos Medicamentos/tratamento farmacológico , Resultado do Tratamento , Recidiva
11.
J Pediatr Hematol Oncol ; 44(2): e474-e478, 2022 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-34001788

RESUMO

Thoracic air leak syndromes (TALS) are very rare among the noninfectious pulmonary complications (PCs). They can either be idiopathic or have several risk factors such as allogeneic hematopoietic stem cell transplantation (allo-HSCT), graft versus host disease and rarely pulmonary aspergillosis. We present a 14-year-old girl with hypoplastic myelodysplastic syndrome who developed graft versus host disease on day 60, TALS on day 150, bronchiolitis obliterans syndrome on day 300, pulmonary aspergillosis on day 400 and COVID-19 pneumonia on day 575 after allo-HSCT. This is the first report of a child who developed these subsequent PCs after allo-HSCT. Therefore, the manifestations of these unfamiliar PCs like TALS and COVID-19 pneumonia, and concomitant pulmonary aspergillosis with management options are discussed.


Assuntos
COVID-19/complicações , Doença Enxerto-Hospedeiro/patologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Síndromes Mielodisplásicas/terapia , Pneumonia Viral/patologia , Aspergilose Pulmonar/patologia , Enfisema Pulmonar/patologia , Adolescente , COVID-19/virologia , Feminino , Doença Enxerto-Hospedeiro/etiologia , Humanos , Síndromes Mielodisplásicas/patologia , Pneumonia Viral/etiologia , Prognóstico , Aspergilose Pulmonar/etiologia , Enfisema Pulmonar/etiologia , Fatores de Risco , SARS-CoV-2/isolamento & purificação
12.
Eur J Pediatr ; 181(8): 3093-3101, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-35705877

RESUMO

The exact immunological mechanisms of post infectious bronchiolitis obliterans (PIBO) in childhood are not fully known. It has been shown that the inflammasome and IL-18 pathway play important roles in the pathogenesis of lung fibrosis. We aimed to investigate the role of caspase-1, IL-18, and IL-18 components in PIBO. From January to May 2020, children with PIBO, children with history of influenza infection without PIBO, and healthy children were asked to participate in the study in three pediatric pulmonology centers. Serum caspase-1, IL-18, IL-18BP, IL-18R, and INF-γ levels were measured by ELISA and compared between the 3 groups. There were 21 children in the PIBO group, 16 children in the influenza group, and 39 children in the healthy control group. No differences in terms of age and gender between the 3 groups were found. IL-18 and IL-18BP levels were higher in the healthy control group (p = 0.018, p = 0.005, respectively). IL-18R was higher in the PIBO group (p = 0.001) and caspase-1 was higher in the PIBO and influenza group than the healthy control group (p = 0.002). IFN-γ levels did not differ between the 3 groups. IL-18BP/IL-18 was higher in the influenza group than the PIBO group and the healthy control group (p = 0.003). CONCLUSIONS: Caspase-1 level was increased in patients with PIBO which suggests that inflammasome activation may have a role in fibrosis; however, IL-18 level was found to be low. Mediators other than IL-18 may be involved in the inflammatory pathway in PIBO. Further immunological studies investigating inflammasome pathway are needed for PIBO with chronic inflammation. WHAT IS KNOWN: • Post infectious bronchiolitis obliterans (PIBO) is a rare, severe chronic lung disease during childhood which is associated with inflammation and fibrosis which lead to partial or complete luminal obstruction especially in small airways. • The exact immunological mechanisms of PIBO in childhood are not fully known. WHAT IS NEW: • Inflammasome activation persists even years after acute infection and may play a role in fibrosis in PIBO. • Mediators other than IL-18 may be involved in these inflammatory pathway.


Assuntos
Bronquiolite Obliterante , Caspase 1 , Interleucina-18 , Bronquiolite Obliterante/sangue , Bronquiolite Obliterante/etiologia , Bronquiolite Obliterante/genética , Bronquiolite Obliterante/imunologia , Estudos de Casos e Controles , Caspase 1/sangue , Caspase 1/genética , Caspase 1/imunologia , Criança , Fibrose/sangue , Fibrose/genética , Fibrose/imunologia , Humanos , Inflamassomos/imunologia , Inflamação/sangue , Inflamação/genética , Inflamação/imunologia , Influenza Humana/sangue , Influenza Humana/complicações , Influenza Humana/genética , Influenza Humana/imunologia , Peptídeos e Proteínas de Sinalização Intercelular/sangue , Peptídeos e Proteínas de Sinalização Intercelular/genética , Peptídeos e Proteínas de Sinalização Intercelular/imunologia , Interleucina-18/sangue , Interleucina-18/genética , Interleucina-18/imunologia
13.
Pediatr Int ; 64(1): e14888, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34131975

RESUMO

BACKGROUND: Cystic fibrosis (CF) is a lethal recessive genetic disease caused by loss of function associated with mutations in the CF trans-membrane conductance regulator. It is highly prevalent (approximately 1 in 3,500) in Caucasians. The aim of this study was to compare demographic and clinical features, diagnostic tests, treatments, and complications of patients with CF whose newborn screening (NBS) with twice-repeated immune reactive trypsinogen testing was positive, normal, and not performed. METHODS: In this study, 359 of all 1,488 CF patients recorded in the CF Registry of Turkey in 2018, who had been born through the process of NBS, were evaluated. Demographic and clinical features were compared in patients diagnosed with positive NBS (Group 1), normal (Group 2), or without NBS (Group 3). RESULTS: In Group 1, there were 299 patients, in Group 2, there were 40 patients, and in Group 3, there were 20 patients. Among all patients, the median age at diagnosis was 0.17 years. The median age at diagnosis was higher in Groups 2 and 3 than in Group 1 (P = 0.001). Fecal elastase results were higher in Group 2 (P = 0.033). The weight z-score was lower and chronic Staphylococcus aureus infection was more common in Group 3 (P = 0.017, P = 0.004, respectively). CONCLUSIONS: Frequency of growth retardation and chronic S. aureus infection can be reduced with an early diagnosis using NBS. In the presence of clinical suspicion in patients with normal NBS, further analyses such as genetic testing should be performed, especially to prevent missing patients with severe mutations.


Assuntos
Fibrose Cística , Fibrose Cística/diagnóstico , Fibrose Cística/epidemiologia , Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Humanos , Recém-Nascido , Triagem Neonatal/métodos , Staphylococcus aureus , Tripsinogênio
14.
J Paediatr Child Health ; 57(10): 1605-1611, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34004018

RESUMO

AIM: We aimed to investigate sleep disturbances in children with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) and typically developing (TD) children during the COVID-19 pandemic. METHODS: Primary care givers of children with CF and PCD aged 3-16 years were asked to enrol in the study. Primary care givers of TD children were included as control group. The Sleep Disturbance Scale for Children (SDSC) was used, and questions related to sleep habits during the pandemic were asked. Results of the three groups were compared. RESULTS: Primary care givers of 33 children with CF, 16 children with PCD and 66 TD children were included in the study. There were no differences in terms of age and gender between the three groups. Changes in sleep patterns during the pandemic were more common among TD children and their families, with 75% of the children and 80% of their families sleeping later than before. The sleep initiation and maintenance disorder scores were higher in TD children (P = 0.001), whereas the sleep breathing disorder scores were higher in children with PCD (P = 0.001), and the sleep hyperhidrosis scores were higher in children with CF and PCD (P = 0.011). No relationships were found between sleep parameters and clinical findings of children with lung disease. CONCLUSIONS: Children's sleep habits have changed during the pandemic. Children with chronic lung diseases and even TD children may experience sleep disturbances during this period.


Assuntos
COVID-19 , Transtornos da Motilidade Ciliar , Fibrose Cística , Criança , Fibrose Cística/complicações , Fibrose Cística/epidemiologia , Humanos , Pandemias , SARS-CoV-2 , Sono
15.
J Trop Pediatr ; 67(2)2021 05 17.
Artigo em Inglês | MEDLINE | ID: mdl-34059924

RESUMO

Mycobacterium abscessus appears to be increasing cause of pulmonary infection in children with underlying risk factors including cystic fibrosis, chronic lung disease and immunodeficiency syndromes. We present a case of pulmonary M. abscessus infection in a pediatric patient with primary ciliary dyskinesia and he was successfully treated with parenteral amikacin, linezolid and oral clarithromycin combined with inhaled amikacin. Clinical improvement was observed after adding inhaled amikacin to the treatment.


Assuntos
Infecções por Mycobacterium não Tuberculosas , Mycobacterium abscessus , Amicacina , Antibacterianos/uso terapêutico , Criança , Claritromicina/uso terapêutico , Humanos , Masculino , Testes de Sensibilidade Microbiana , Infecções por Mycobacterium não Tuberculosas/diagnóstico , Infecções por Mycobacterium não Tuberculosas/tratamento farmacológico
16.
Nurs Health Sci ; 23(2): 362-371, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33433046

RESUMO

This study aims to investigate the relationship between the professional values and professional quality of life of nurses working in mental health units. This descriptive, cross-sectional, and correlational study was conducted with a sample of 120 nurses working in mental health units of one regional, one public, and one university hospital in western Turkey. Data were collected using an introductory information form, the Nurses Professional Values Scale-Revised, and the Professional Quality of Life-IV Scale. A statistically positive relationship was found between nurses' professional values, compassion satisfaction, and burnout. Nurses' professional values, education level, whether considering changing units, and allocation of time to social life were significant predictors of professional quality of life, explaining 44% of the total variance for Compassion Satisfaction and 24% for Burnout. Whether considering changing units was the most important predictor of compassion satisfaction, and truth value was the most important predictor of burnout. Nurses providing care based on professional values were found to have higher compassion satisfaction and burnout levels, and nurses with higher compassion satisfaction were found to experience less compassion fatigue.


Assuntos
Esgotamento Profissional/psicologia , Fadiga de Compaixão/psicologia , Transtornos Mentais/enfermagem , Enfermeiras e Enfermeiros/psicologia , Enfermagem Psiquiátrica , Qualidade de Vida/psicologia , Adulto , Estudos Transversais , Empatia , Feminino , Humanos , Satisfação no Emprego , Masculino , Saúde Mental , Inquéritos e Questionários , Turquia
17.
J Paediatr Child Health ; 56(10): 1537-1543, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32610373

RESUMO

AIM: In this study, we aimed to determine the psychosocial status of primary care givers of children with cystic fibrosis (CF) and its relationship with the clinical scores of children with CF. METHODS: Thirty-six patients with CF and their primary care givers were assessed. A personal information form, as well as the Parent Attitude Research Instrument, Maslach Burnout Inventory, Beck Depression Scale and modified Shwachman-Kulczycki Score, were used for data collection. RESULTS: All the CF children's primary care givers were their mothers. The mothers' occupation and educational level affected their child-rearing attitudes (P < 0.05). Furthermore, the frequency of hospital visits and hospitalizations was associated with increased emotional exhaustion, depression and negative attitudes towards child-rearing (P < 0.05). Higher levels of emotional exhaustion in mothers were associated with increased depersonalization and depression, while personal accomplishment was associated with lower levels of depression (P < 0.05). Depression was present in 69.4% of mothers, and its severity was correlated with their children having a poor clinical status. Moreover, increased depression was associated with increased negative attitudes towards child-rearing (P < 0.05). CONCLUSION: The rate of depression is high in mothers of children with CF. These mothers displayed a negative attitude towards child-rearing, along with the disease, which was more severe in their children. These mothers should undergo routine psychosocial screening, and support should be given to those in need.


Assuntos
Fibrose Cística , Mães , Criança , Educação Infantil , Depressão/etiologia , Emoções , Feminino , Humanos , Relações Mãe-Filho
18.
J Trop Pediatr ; 66(4): 412-418, 2020 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-31774539

RESUMO

OBJECTIVE: To objectively investigate the effect of passive smoking on pneumonia and disease severity in children aged less than 5 years by using cotinine as an indicator of passive smoking. METHODS: Between December 2015 and April 2016, children aged less than 5 years with pneumonia and age-matched healthy controls were included in this study, which was conducted at three tertiary pediatric pulmonology centers. A questionnaire was given to the parents regarding demographic data and smoking status at home. Urinary cotinine/creatinine ratio (CCR) was measured. The data from the pneumonia and control groups, as well as children with mild and severe pneumonia within the pneumonia group, were compared. RESULTS: A total of 227 subjects were included in the study; there were 74 children in the pneumonia group and 153 in the control group. The mean age of all the children was 33.4 ± 1.28 months. Of all subjects, 140 were male and 102 were exposed to passive smoking by their parents at home. There were statistically significant differences in age, number of people in the home, and mother's and father's age between the control and pneumonia groups (p < 0.05). No difference was found in the CCR in the control and pneumonia group (p > 0.05). Age and urinary CCR were significantly different between children with mild and severe pneumonia (p < 0.05). CONCLUSION: We showed that passive smoking exposure was associated with the development of severe pneumonia in children. Further studies are needed to examine the underlying cause in detail.


Assuntos
Cotinina/urina , Pneumonia/urina , Poluição por Fumaça de Tabaco/efeitos adversos , Adulto , Estudos de Casos e Controles , Criança , Pré-Escolar , Tosse/etiologia , Feminino , Febre/etiologia , Humanos , Lactente , Masculino , Pais , Pneumonia/epidemiologia , Pneumonia/etiologia , Índice de Gravidade de Doença , Inquéritos e Questionários , Poluição por Fumaça de Tabaco/análise
19.
J Trop Pediatr ; 66(1): 85-94, 2020 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-31204435

RESUMO

INTRODUCTION: No clear information exists about the factors affecting pleural thickening following parapneumonic effusion in children. We aimed to investigate factors that affect the resolving time of pleural thickening after parapneumonic effusion. METHODS: Between the years of 2007-18, 91 patients, which were followed due to diagnosis of pleural thickening after parapneumonic effusion, were assessed. Ages, complaints, physical examination findings, laboratory results, chest x-ray and ultrasonography findings, treatments, duration of treatment and recovery time of the patients were examined terms in of pleural thickening resolving time. RESULTS: The mean age of patients was 7.5 ± 5.0 years. Pleural thickening resolving time was 151 ± 6.8 days. The resolving time for pleural thickening was delayed with older ages, longer duration of complaints, fever before hospital admission and treatment, lower oxygen saturation at the time of admission, crackles in the physical examination, higher white blood cell count and pleural fluid density (p = 0.018, p = 0.001, p = 0.021, p = 0.020, p = 0.024, p = 0.025, p = 0.021, p = 0.019). In addition, the amount of effusion measured by thorax ultrasonography, fibrinolytic usage, and complications had a role in the delayed resolving time (p = 0.034, p = 0.001, p = 0.034). Pleural thickening resolved in 80% of the patients. CONCLUSION: In this report, 80% of pleural thickening, following parapneumonic effusion resolved within 5 months. Patients who do not have a complication during follow-up are not required to monitor with frequent chest x-ray. Patients with a higher amount of pleural effusion, complications and need for fibrinolytic treatment should be followed more carefully.


Assuntos
Empiema Pleural/complicações , Pleura/patologia , Derrame Pleural/complicações , Assistência ao Convalescente , Criança , Pré-Escolar , Feminino , Fibrinolíticos/uso terapêutico , Seguimentos , Humanos , Pneumopatias/complicações , Pneumopatias/tratamento farmacológico , Masculino , Pleura/diagnóstico por imagem , Doenças Pleurais/diagnóstico por imagem , Doenças Pleurais/tratamento farmacológico , Doenças Pleurais/patologia , Radiografia Torácica , Estudos Retrospectivos
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