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1.
Pediatr Pulmonol ; 2024 May 21.
Artigo em Inglês | MEDLINE | ID: mdl-38771207

RESUMO

INTRODUCTION: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non-eligible patients for CFTR modulators in the CF registry of Turkey (CFRT) to highlight their clinical needs. METHODS: This retrospective cohort study included CF patient data from the CFRT in 2021. The decision of eligibility for the CFTR modulator was determined according to the 'Vertex treatment-Finder' on the Vertex® website. Demographic and clinical characteristics of patients were compared between eligible (group 1) and ineligible (group 2) groups for CFTR modulators. RESULTS: Among the study population (N = 1527), 873 (57.2%) were in group 1 and 654 (42.8%) were in group 2. There was no statistical difference between groups regarding sex, meconium ileus history, diagnoses via newborn screening, FEV1 z-score, CF-associated complications, organ transplant history, and death. Patients in group 2 had a higher incidence of pancreatic insufficiency (87.7% vs. 83.2%, p = .010), lower median height z-scores (-0.87 vs. -0.55, p < .001), lower median body mass index z-scores (-0.65 vs. -0.50, p < .001), longer days receiving antibiotics due to pulmonary exacerbation (0 [interquartile range, IQR: 0-2] vs. 0 [IQR: 0-7], p = 0.001), and more non-invasive ventilation support (2.6% vs. 0.9%, p = 0.008) than patients in group 1. CONCLUSION: The ineligible group had worse clinical outcomes than the eligible group. This highlights their need for life-changing drugs to improve clinical outcomes.

3.
Mol Imaging Radionucl Ther ; 33(1): 40-42, 2024 Feb 22.
Artigo em Inglês | MEDLINE | ID: mdl-38390778

RESUMO

Sagliker syndrome (SS) is a rare, exaggerated form of chronic kidney disease (CKD)-mineral and bone disorder resulting from untreated secondary hyperparathyroidism due to CKD. Herein, we describe a 34-year-old male patient whose Tc-99m-methylene diphosphonate bone scintigraphy and Tc-99m-sestamibi parathyroid scintigraphy revealed hints of SS and exhibited its defining characteristics.

4.
Children (Basel) ; 11(1)2024 Jan 22.
Artigo em Inglês | MEDLINE | ID: mdl-38275444

RESUMO

OBJECTIVES: Septic arthritis (SA) is a serious bacterial infection that must be treated efficiently and timely. The large number of culture-negative cases makes local epidemiological data important. Accordingly, this study aimed to evaluate the etiology, clinical characteristics, and therapeutic approach of SA in children in Turkiye, emphasizing the role of real-time polymerase chain reaction (PCR) techniques in the diagnosis. METHODS: In this multi-center, prospective study, children hospitalized due to SA between February 2018 and July 2020 in 23 hospitals in 14 cities in Turkiye were included. Clinical, demographic, laboratory, and radiological findings were assessed, and real-time PCR was performed using synovial fluid samples. RESULTS: Seventy-five children aged between 3 and 204 months diagnosed with acute SA were enrolled. Joint pain was the main complaint at admission, and the most commonly involved joints were the knees in 58 patients (77.4%). The combination of synovial fluid culture and real-time PCR detected causative bacteria in 33 patients (44%). In 14 (18.7%) patients, the etiological agent was demonstrated using only PCR. The most commonly isolated etiologic agent was Staphylococcus aureus, which was detected in 22 (29.3%) patients, while Streptococcus pyogenes was found in 4 (5.3%) patients and Kingella kingae in 3 (4%) patients. Streptococcus pyogenes and Kingella kingae were detected using only PCR. Most patients (81.3%) received combination therapy with multiple agents, and the most commonly used combination was glycopeptides plus third-generation cephalosporin. CONCLUSIONS: Staphylococcus aureus is the main pathogen in pediatric SA, and with the use of advanced diagnostic approaches, such as real-time PCR, the chance of diagnosis increases, especially in cases due to Kingella kingae and Streptococcus pyogenes.

5.
Dev Cell ; 58(24): 2914-2929.e7, 2023 Dec 18.
Artigo em Inglês | MEDLINE | ID: mdl-38113852

RESUMO

Low-grade chronic inflammation is a hallmark of ageing, associated with impaired tissue function and disease development. However, how cell-intrinsic and -extrinsic factors collectively establish this phenotype, termed inflammaging, remains poorly understood. We addressed this question in the mouse intestinal epithelium, using mouse organoid cultures to dissect stem cell-intrinsic and -extrinsic sources of inflammaging. At the single-cell level, we found that inflammaging is established differently along the crypt-villus axis, with aged intestinal stem cells (ISCs) strongly upregulating major histocompatibility complex class II (MHC-II) genes. Importantly, the inflammaging phenotype was stably propagated by aged ISCs in organoid cultures and associated with increased chromatin accessibility at inflammation-associated loci in vivo and ex vivo, indicating cell-intrinsic inflammatory memory. Mechanistically, we show that the expression of inflammatory genes is dependent on STAT1 signaling. Together, our data identify that intestinal inflammaging in mice is promoted by a cell-intrinsic mechanism, stably propagated by ISCs, and associated with a disbalance in immune homeostasis.


Assuntos
Mucosa Intestinal , Intestinos , Camundongos , Animais , Células-Tronco , Fenótipo , Inflamação
6.
Turk J Pediatr ; 65(3): 469-478, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37395966

RESUMO

BACKGROUND: The prevalence of community-acquired methicillin-resistant S. aureus (CA-MRSA) has been increasing worldwide. We aimed to investigate the prevalence of MRSA in community-acquired S. aureus infections, the risk factors for CA-MRSA infection and the clinical features of CA-MRSA. METHODS: A multi-center study with prospective and retrospective sections was conducted. Patients ≥ 3 months old and ≤18 years of age who were diagnosed with community-acquired S. aureus infections were included in this study and the patients` information were reviewed from the medical and microbiological database of the hospital. A standard question form about living conditions and exposure risk factors was administered to the parents of patients. The CA-MRSA infections were compared with the methicillin-susceptible S. aureus (CAMSSA) infections in terms of the queried risk factors and clinical variables. RESULTS: We identified 334 pediatric patients with S. aureus infection, 58 (17.4%) had an infection with CAMRSA. The refugee rate was higher in the CA-MRSA group. There was no significant difference regarding the exposure risk. The treatment modalities and outcomes were similar. CONCLUSIONS: The study was not able to show reliable clinical variables or epidemiological risk factors except for being a refugee for CA-MRSA infections. Empirical antibiotic treatment should therefore be determined according to the local CA-MRSA prevalence in patients presenting with a possible staphylococcus infection.


Assuntos
Infecções Comunitárias Adquiridas , Staphylococcus aureus Resistente à Meticilina , Infecções Estafilocócicas , Humanos , Criança , Lactente , Staphylococcus aureus , Estudos Retrospectivos , Estudos Prospectivos , Meticilina/uso terapêutico , Antibacterianos/uso terapêutico , Infecções Comunitárias Adquiridas/microbiologia , Infecções Estafilocócicas/tratamento farmacológico
7.
Int J Ment Health Addict ; : 1-27, 2023 May 10.
Artigo em Inglês | MEDLINE | ID: mdl-37363769

RESUMO

Exercise addiction (EA) refers to excessive exercise, lack of control, and health risks. The Exercise Addiction Inventory (EAI) is one of the most widely used tools in its assessment. However, the cross-cultural psychometric properties of the EAI could be improved because it misses three pathological patterns, including guilt, exercise despite injury, and experienced harm. Therefore, the present study tested the psychometric properties of the expanded EAI (EAI-3) in a large international sample. The EAI-3 was administered to 1931 physically active adult exercisers speaking five languages (Chinese, German, Italian, Japanese, and Turkish) and other measures for obsessive-compulsive behavior, eating disorders, and personality traits. The assessment structure and reliability of the EAI-3 were tested with factorial analyses and through measurement invariance across languages and sex. Finally, a cutoff point for dysfunction-proneness was calculated. The EAI-3 comprised two factors, reflecting the positive and pathological sides of exercise. The structure had excellent reliability and goodness-of-fit indices and configural and metric invariances of the scale were supported. However, three items caused violations in scalar invariance. The results of partial measurement invariance testing suggested an adequate fit for the data. Following sensitivity and specificity analysis, the EAI-3's cutoff score was 34 out of a maximum score of 48. This preliminary study suggests that the EAI-3 is a promising tool for screening EA in an international sample, with a robust and reliable structure comparable across languages and sex. In addition, the proposed cutoff could pave the way toward a consensus on a threshold to screen for EA.

8.
BMC Pediatr ; 23(1): 121, 2023 03 18.
Artigo em Inglês | MEDLINE | ID: mdl-36932373

RESUMO

BACKGROUND: Antibiotic-associated diarrhea is one of the most frequent side effects of antimicrobial therapy. We assessed the epidemiological data of antibiotic-associated diarrhea in pediatric patients in our region. METHODS: The prospective multi-center study included pediatric patients who were initiated an oral antibiotic course in outpatient clinics and followed in a well-established surveillance system. This follow-up system constituded inclusion of patient by the primary physician, supply of family follow-up charts to the family, passing the demographics and clinical information of patient to the Primary Investigator Centre, and a close telephone follow-up of patients for a period of eight weeks by the Primary Investigator Centre. RESULTS: A result of 758 cases were recruited in the analysis which had a frequency of 10.4% antibiotic-associated diarrhea. Among the cases treated with amoxicillin-clavulanate 10.4%, and cephalosporins 14.4% presented with antibiotic-associated diarrhea. In the analysis of antibiotic-associated diarrhea occurrence according to different geographical regions of Turkey, antibiotic-associated diarrhea episodes differed significantly (p = 0.014), particularly higher in The Eastern Anatolia and Southeastern Anatolia. Though most commonly encountered with cephalosporin use, antibiotic-associated diarrhea is not a frequent side effect. CONCLUSION: This study on pediatric antibiotic-associated diarrhea displayed epidemiological data and the differences geographically in our region.


Assuntos
Antibacterianos , Pacientes Ambulatoriais , Criança , Humanos , Estudos Prospectivos , Antibacterianos/efeitos adversos , Combinação Amoxicilina e Clavulanato de Potássio/efeitos adversos , Cefalosporinas/efeitos adversos , Diarreia/induzido quimicamente , Diarreia/epidemiologia , Diarreia/tratamento farmacológico
9.
Childs Nerv Syst ; 39(5): 1335-1339, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36534133

RESUMO

Subdural empyema refers to the collection of purulent material in the subdural space and the most source of it is bacterial meningitis in infants while sinusitis and otitis media in older children. It has been very recently reported that coronaviruses (CoV) exhibit neurotropic properties and may also cause neurological diseases. CoV-related complications as hypercoagulability with thrombosis and associated inflammation, catastrophic cerebral venous sinus thrombose sand bacterial-fungal superinfections have been well documented in adult patients. Hereby, we describe 15-year-old and 12-year-old female children with subdural empyema after SARS-CoV2. The patients presented limitation of eye in the outward gaze, impaired speech, drowsiness, fever, vomiting and they also were tested positive for COVID-19. MRI indicated subdural empyema and surgical interventions were needed to relieve intracranial pressure and drain pus after receiving broad spectrum antibiotics treatments. The microbiological analysis of abscess material revealed Streptococcus constellatus which is extremely rare in an immunocompetent child and the patients received appropriate IV antibiotic therapy. Eventually, patients became neurologically intact. Pediatric patients with CoV infections should be closely monitored for neurological symptoms. Further research and more data on the correlation between CoV infections would provide better recognition and treatment options in an efficient manner in children.


Assuntos
COVID-19 , Empiema Subdural , Lactente , Adulto , Feminino , Humanos , Criança , Empiema Subdural/etiologia , Empiema Subdural/cirurgia , SARS-CoV-2 , RNA Viral , COVID-19/complicações , Abscesso/tratamento farmacológico , Antibacterianos/uso terapêutico
10.
An Bras Dermatol ; 98(2): 168-175, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36473757

RESUMO

OBJECTIVES: multi-system inflammatory syndrome in children (MIS-C) is an immune-mediated process that develops after infections like SARS-CoV-2. The authors aimed to reveal the mucocutaneous findings of patients diagnosed with MIS-C at presentation and evaluate the frequency of these mucocutaneous findings and their possible relationship with the severity of the disease. METHODS: A prospective study was conducted of 43 children admitted to a tertiary hospitals between January 2021 and January 2022 who met Centers for Disease Control and Prevention criteria for MIS-C. RESULTS: 43 children (25 [58.1%] male); median age, 7.5 years [range 0.5‒15 years]) met the criteria for MIS-C. The most common symptom was cutaneous rash 81.4%, followed by gastrointestinal symptoms 67.4%, oral mucosal changes 65.1%, and conjunctival hyperemia 58.1%. The most common mucosal finding was fissured lips at 27.9%, diffuse hyperemia of the oral mucosa at 18.6%, and strawberry tongue at 13.9%. Urticaria (48.8%) was the most common type of cutaneous rash in the present study's patients. The most common rash initiation sites were the trunk (32.6%) and the palmoplantar region (20.9%). The presence or absence of mucocutaneous findings was not significantly associated with disease severity. STUDY LIMITATIONS: The number of patients in the this study was small. CONCLUSIONS: The present study's prospective analysis detected mucocutaneous symptoms in almost 9 out of 10 patients in children diagnosed with MIS-C. Due to the prospective character of the present research, the authors think that the characteristic features of cutaneous and mucosal lesions the authors obtained will contribute to the literature on the diagnosis and prognosis of MIS-C.


Assuntos
COVID-19 , Doenças do Tecido Conjuntivo , Hiperemia , Criança , Humanos , Masculino , Lactente , Pré-Escolar , Adolescente , Feminino , COVID-19/complicações , COVID-19/epidemiologia , SARS-CoV-2 , Estudos Prospectivos , Pandemias , Síndrome de Resposta Inflamatória Sistêmica/epidemiologia
11.
Brief Bioinform ; 25(1)2023 11 22.
Artigo em Inglês | MEDLINE | ID: mdl-38180828

RESUMO

Complex biological processes in cells are embedded in the interactome, representing the complete set of protein-protein interactions. Mapping and analyzing the protein structures are essential to fully comprehending these processes' molecular details. Therefore, knowing the structural coverage of the interactome is important to show the current limitations. Structural modeling of protein-protein interactions requires accurate protein structures. In this study, we mapped all experimental structures to the reference human proteome. Later, we found the enrichment in structural coverage when complementary methods such as homology modeling and deep learning (AlphaFold) were included. We then collected the interactions from the literature and databases to form the reference human interactome, resulting in 117 897 non-redundant interactions. When we analyzed the structural coverage of the interactome, we found that the number of experimentally determined protein complex structures is scarce, corresponding to 3.95% of all binary interactions. We also analyzed known and modeled structures to potentially construct the structural interactome with a docking method. Our analysis showed that 12.97% of the interactions from HuRI and 73.62% and 32.94% from the filtered versions of STRING and HIPPIE could potentially be modeled with high structural coverage or accuracy, respectively. Overall, this paper provides an overview of the current state of structural coverage of the human proteome and interactome.


Assuntos
Proteoma , Humanos , Bases de Dados Factuais
12.
Heliyon ; 8(9): e10446, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36105477

RESUMO

Aim: This study aimed to determine the effects of various sociodemographic variables and experiences, unhelpful beliefs about stuttering, and perceived social support on psychological well-being in stuttering adults. Methods: Forty-five stuttering adults were included in our study, and sociodemographic data were collected using a stuttering experiences information form, the Unhelpful Thoughts and Beliefs About Stuttering (UTBAS)-6 scale, the perceived social support scale, and the Warwick-Edinburgh Mental Well-being Scale. Results: It was determined that the total score of the UTBAS scale, the high scores on the fear of negative evaluation, avoidance, self-doubt and insecurity, and the hopelessness subscales, and the low scores of the perceived social support scale were correlated with lower psychological well-being results in stuttering individuals. Conclusions: To support the mental well-being of individuals with stuttering, we believe it would be beneficial to provide mental assessment and supportive approaches, raise awareness to eliminate prejudice and stigmatizing attitudes toward individuals with stuttering in the family and wider society, and develop social support systems, alongside speech therapy.

13.
Mol Imaging Radionucl Ther ; 31(2): 151-153, 2022 Jun 27.
Artigo em Inglês | MEDLINE | ID: mdl-35771040

RESUMO

Pulmonary alveolar microlithiasis is a rare disease characterized by accumulating intraalveolar small calcium phosphate stones. The disease is slow and does not show any signs in the early stages, but the shortness of breath, cough, and right heart failure may develop as it progresses. Methylene diphosphonate used in bone scintigraphy shows high uptake of calcium deposits in the alveoli and causes diffuse increased radiopharmaceutical uptake in the lungs.

14.
Hum Vaccin Immunother ; 18(5): 2044707, 2022 11 30.
Artigo em Inglês | MEDLINE | ID: mdl-35714279

RESUMO

INTRODUCTION: Health care workers (HCWs) are disproportionately exposed to infectious diseases and play a role in nosocomial transmission, making them a key demographic for vaccination. HCW vaccination rates are not optimal in many countries; hence, compulsory vaccination policies have been implemented in some countries. Although these policies are effective and necessary under certain conditions, resolving HCWs' hesitancies and misconceptions about vaccines is crucial. HCWs have the advantage of direct contact with patients; hence, they can respond to safety concerns, explain the benefits of vaccination, and counter antivaccine campaigns that escalate during pandemics, as has been observed with COVID-19. METHOD: A short survey was carried out in May-June 2020 on the vaccination status of HCWs working with pediatric patients with COVID-19. The survey inquired about their vaccination status (mumps/measles/rubella [MMR], varicella, influenza, and diphtheria/tetanus [dT]) and willingness to receive hypothetical future COVID-19 vaccines. The respondents were grouped according to gender, age, occupation, and region. RESULTS: In total, 4927 HCWs responded to the survey. Most were young, healthy adults. The overall vaccination rates were 57.8% for dT in the past 10 years, 44.5% for MMR, 33.2% for varicella, and 13.5% for influenza. Vaccination rates were the highest among physicians. The majority of HCWs (81%) stated that they would be willing to receive COVID-19 vaccines. CONCLUSION: Although vaccination rates for well-established vaccines were low, a majority of HCWs were willing to receive COVID-19 vaccines when available. Education and administrative trust should be enhanced to increase vaccination rates among HCWs.


Assuntos
COVID-19 , Varicela , Vacinas contra Influenza , Influenza Humana , Sarampo , Adulto , COVID-19/prevenção & controle , Vacinas contra COVID-19 , Criança , Pessoal de Saúde , Humanos , Influenza Humana/prevenção & controle , Sarampo/prevenção & controle , SARS-CoV-2 , Vacinação
15.
Clin Neurol Neurosurg ; 218: 107270, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35623138

RESUMO

PURPOSE: Levetiracetam (LEV) is an anti-seizure drug (ASD). A consensus has not been reached regarding its effects on bone health. This cross sectional study was planned to assess short, medium and long-term effects on bone density and blood parameters that are associated with bone metabolism. METHODS: The sample consisted of 47 patients with epilepsy, who had been on LEV monotherapy for more than six months. All participants were over 18 years of age and had no other risk factors for osteoporosis. None of them used any other anti-seizure drug before. Bone mineral density (BMD) was evaluated with dual energy X-ray absorptiometry (DEXA) and biochemical markers associated with bone health were measured. Patients were divided into three groups depending on how long they had been on LEV for (Group A: <1 year; Group B: 1-5 years, Group C: >5 years) and compared with each other in terms of BMD scores and blood parameters. RESULTS: The mean age of patients was 32. 6 ± 13.3 and 20 patients were female (42.5%). The mean onset age of epilepsy was 28.1 ± 13.4 years. Average LEV period of consumption was 2.7 ± 2.7 years and mean daily dose was 1041.7 ± 393.9 milligrams. Lumbar BMD scores of the group with LEV usage < 1 year were significantly lower than those of the group with LEV usage of 1-5 years (p < 0.05). Lumbar vertebra scores were found to be lower in group of LEV usage duration of < 1 year when compared with LEV usage duration > 5 years but the difference was not statistically significant. CONCLUSION: We argue that LEV might have a negative effect on bone densitometry at the lumbar level in short-time usage for less than one year. Furthermore, no deleterious impact on bone metabolism was observed in long-term treatment. LEV seems as a rational drug for treatment of epilepsy patients, particularly for those with osteoporosis, since the comparative results of one year and longer than 5-years usage data did not show any statistically significant difference.


Assuntos
Epilepsia , Osteoporose , Piracetam , Adolescente , Adulto , Anticonvulsivantes/efeitos adversos , Densidade Óssea , Estudos Transversais , Epilepsia/tratamento farmacológico , Feminino , Humanos , Levetiracetam/uso terapêutico , Masculino , Osteoporose/induzido quimicamente , Osteoporose/tratamento farmacológico , Piracetam/efeitos adversos , Resultado do Tratamento , Adulto Jovem
16.
J Paediatr Child Health ; 58(6): 1069-1078, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35199895

RESUMO

AIM: Multisystem inflammatory syndrome in children (MIS-C) may cause shock and even death in children. The aim of this study is to describe the clinical features, laboratory characteristics and outcome of children diagnosed with MIS-C in 25 different hospitals in Turkey. METHODS: The retrospective study was conducted between 8 April and 28 October 2020 in 25 different hospitals from 17 cities. Data were collected from patients' medical records using a standardised form. Clinical and laboratory characteristics and outcomes according to different age groups, gender and body mass index percentiles were compared using multivariate logistic regression analysis. RESULTS: The study comprised 101 patients, median age 7 years (interquartile range (IQR) 4.6-9.3); 51 (50.5%) were boys. Reverse-transcriptase polymerase chain reaction (PCR) assay was positive in 21/100 (21%) patients; 62/83 (74.6%) patients had positive serology for SARS-CoV-2. The predominant complaints were fever (100%), fatigue (n = 90, 89.1%), and gastrointestinal symptoms (n = 81, 80.2%). Serum C-reactive protein (in 101 patients, median 165 mg/L; range 112-228), erythrocyte sedimentation rate (73/84, median 53 mm/s; IQR 30-84) and procalcitonin levels (86/89, median 5 µg/L; IQR 0.58-20.2) were elevated. Thirty-eight patients (37.6%) required admission to intensive care. Kawasaki disease (KD) was diagnosed in 70 (69.3%) patients, 40 of whom had classical KD. Most patients were treated with intravenous immunoglobulin (n = 92, 91%) and glucocorticoids (n = 59, 58.4%). Seven patients (6.9%) died. CONCLUSION: The clinical spectrum of MIS-C is broad, but clinicians should consider MIS-C in the differential diagnosis when persistent fever, fatigue and gastrointestinal symptoms are prominent. Most patients diagnosed with MIS-C were previously healthy. Immunomodulatory treatment and supportive intensive care are important in the management of cases with MIS-C. Glucocorticoids and intravenous immunoglobulins are the most common immunomodulatory treatment options for MIS-C. Prompt diagnosis and prompt treatment are essential for optimal management.


Assuntos
COVID-19 , Síndrome de Linfonodos Mucocutâneos , COVID-19/complicações , Criança , Fadiga , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Estudos Retrospectivos , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica , Turquia/epidemiologia
17.
Pediatr Pulmonol ; 57(5): 1245-1252, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35102722

RESUMO

BACKGROUND: Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease. METHODS: Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data. RESULTS: There were 1170 people with CF in 2017 and 1637 in 2019 CF registry. Eight hundred and fourteen people were registered in both 2017 and 2019 of whom z-scores of heights and BMI were significantly higher in 2019 (p = 0.002, p =0.039, respectively). Inhaled hypertonic saline, bronchodilator, and azithromycin usages were significantly higher in 2019 (p =0.001, p = 0.001, p = 0.003, respectively). The percent predicted of forced expiratory volume in 1 sec and forced vital capacity were similar in 2017 and 2019 (88% and 89.5%, p = 0.248 and 84.5% and 87%, p =0.332, respectively). Liver diseases and osteoporosis were significantly higher, and pseudo-Bartter syndrome (PBS) was significantly lower in 2019 (p = 0.011, p = 0.001, p = 0.001, respectively). CONCLUSIONS: The z-scores of height and BMI were higher, the use of medications that protect and improve lung functions was higher and incidence of PBS was lower in 2019. It was predicted that registry system increased the care of people with CF regarding their follow-up. The widespread use of national CF registry system across the country may be beneficial for the follow-up of people with CF.


Assuntos
Síndrome de Bartter , Fibrose Cística , Síndrome de Bartter/complicações , Fibrose Cística/complicações , Fibrose Cística/epidemiologia , Fibrose Cística/terapia , Regulador de Condutância Transmembrana em Fibrose Cística , Feminino , Humanos , Recém-Nascido , Masculino , Assistência ao Paciente , Sistema de Registros , Turquia/epidemiologia
18.
Clin EEG Neurosci ; 53(1): 45-53, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34558322

RESUMO

Introduction and aim: A proportion of patients diagnosed with genetic generalized epilepsy (GGE) experience eye-closure sensitivity (ECS), the underlying pathogenesis of which is unknown. In this study, we compare magnetic resonance spectroscopy (MRS) findings of healthy volunteers with patients diagnosed with GGE, with and without ECS, to detect possible explanatory differences between groups. Materials and methods: A total of 33 patients diagnosed with GGE: 17 with ECS and 16 without, and 12 healthy volunteers are included. MRS measurements of N-acetyl-aspartate (NAA), choline (Cho), and creatine (Cr) were made of bilateral occipital lobes and thalamus, and values of patients with GGE were compared with those of normal controls, and within subgroups with different clinical variables, using appropriate statistical tests. Results: Left occipital NAA and NAA/Cr levels were found to be significantly higher in the ECS group than in the control group. In the ECS epilepsy group, a significant moderate positive correlation was noted between left thalamic Cr and duration of drug therapy (r = .539, P = .047) and left thalamic Cr and age at epilepsy onset (r = .564, P = .036). Additionally, left thalamic NAA and NAA/Cr levels were observed to be lower in GGE patients compared to healthy subjects, although not to a statistically significant degree. Conclusion:The differences in MRS-measurable metabolites in the left occipital lobe in those with ECS epilepsy suggest an association between the ECS mechanism and the left occipital lobe. Our results also support the multifocal thalamocortical pathway disorder in the pathophysiology of GGE based on the observation of cellular dysfunction in the thalamus.


Assuntos
Eletroencefalografia , Epilepsia Generalizada , Creatina , Humanos , Imageamento por Ressonância Magnética , Espectroscopia de Ressonância Magnética
19.
J Mycol Med ; 32(1): 101229, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-34911023

RESUMO

BACKGROUND: Invasive Trichosporon infections are rarely seen opportunistic fungal infections in children and mainly affect immunocompromised patients. This multicenter retrospective study has rewieved the characteristics, risk factors, treatment modalities and outcomes of bloodstream infections caused by Trichosporon species in children diagnosed over the past ten years in Turkey. METHODS: The study was performed with the participation of 12 of 55 hospitals invited from Turkey. In each center, the patients with bloodstream infections caused by Trichosporon spp. between January 2010 and December 2020 were retrospectively ascertained and the results were reported to the study coordinator by means of a simple case report. Data were collected on patient demographics, underlying condition(s), treatment of.infections caused by Trichosporon spp, and 7 and 30- day mortality rates. RESULTS: A total of 28 cases with fungemia caused by Trichosporon spp. were included in the study. The most common underlying disease was paediatric cancers (39.3%). T. asahii infections were detected in 78.5 % (n=22) of patients. A various spectrum of antifungal treatment regimens were used including intravenous amphotericin B monotherapy in 35.7%, intravenous amphotericin B and voriconazole combination in 32.1% and intravenous voriconazole monotherapy in 28.6% of the patients. The overall mortality rate was 28.5 %. The mortality rates were 12.5% in the voricanozole, 30% in the amphotericin B and 33.3% in combined voriconazole -amphotericin B arms CONCLUSIONS: Invasive Trichosporon infections with an important impact of patients quality of life are almost related to underlying diseases with an overall mortality rate of 28.5%. Voriconazole was found to be associated with lower mortality rates when compared with other treatment regimens.


Assuntos
Sepse , Trichosporon , Antifúngicos/uso terapêutico , Criança , Humanos , Qualidade de Vida , Estudos Retrospectivos , Turquia/epidemiologia
20.
J Adolesc ; 93: 134-145, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34749167

RESUMO

INTRODUCTION: Social networking sites such as Instagram have provided young people with unprecedented opportunities for social comparison, and such behaviour can have implications for identity development. Although initial evidence suggests that there may be developmental differences in terms of how such behaviour informs identity development during adolescence and emerging adulthood, all previous research has been conducted in highly individualistic cultural contexts (i.e., the UK and the US). METHOD: To shed further light on these possible developmental differences and to determine whether results replicate amongst young people from more collectivist cultural contexts, cross-sectional survey data were collected from 1,085 (M age = 18.87, SD = 2.57; Female = 77.8%) adolescents and emerging adults in Romania and Serbia between December 2019 and March 2020. The relationships between social comparisons of ability and opinion on Instagram and three key identity processes (i.e., commitment, in-depth exploration, and reconsideration of commitment) were then examined. RESULT: Hierarchical multiple regression analyses identified significant age differences in terms of how social comparisons of ability and opinion on Instagram associated with identity commitment and in-depth exploration. Furthermore, possible cultural differences were identified in terms of how social comparisons of opinion on Instagram associated with the identity processes. CONCLUSION: Overall, results suggest that whilst social comparisons on Instagram can elicit self-focus and prompt further exploration, developmental and cultural factors may influence how such behaviour informs identity development during adolescence and emerging adulthood.


Assuntos
Comparação Social , Adolescente , Adulto , Estudos Transversais , Feminino , Humanos
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