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1.
Int Arch Allergy Immunol ; 184(10): 994-1002, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37494888

RESUMEN

INTRODUCTION: Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome are rare severe hypersensitivity reactions that lead to epithelial sloughing. Studies investigating the chronic multisystem effects of these syndromes and assessing patients in terms of quality of life (QOL), depression, and anxiety in the pediatric population are limited. In this study, we aimed to investigate the long-term effects of these diseases from a multisystem perspective. METHOD: Sixteen pediatric patients diagnosed with SJS, TEN, and SJS/TEN overlap syndrome were evaluated between September 2020 and March 2021. Physical and eye examinations were performed. To evaluate QOL and psychological status, Children's Dermatology Life Quality Index (CDLQI), Screen for Child Anxiety-Related Emotional Disorders (SCARED), and Children's Depression Inventory (CDI) were conducted. The patients' general characteristics, symptoms, and examination findings at their first admission were retrospectively obtained from the hospital's electronic records. RESULTS: Nineteen percent of the patients were female (n = 3). There were 7 patients (44%) with the diagnosis of SJS, 5 patients (31%) with TEN, and 4 patients (25%) with SJS/TEN overlap. The median follow-up time of the subjects was 6.5 years. The most common sequelae in the chronic period were skin changes (n = 13, 81%). Hyperpigmentation was the most common skin change (n = 9, 56%). In the last evaluation, 9 cases had eye involvement. In two cases, eye examination was normal in the acute phase, while ocular involvement was present in the chronic period. In 4 (50%) patients, there was height and/or weight percentile loss. Three patients' SCARED scores and 2 patients' CDI scores were high. According to the CDLQI survey, SJS, TEN, or SJS/TEN overlap syndrome had a small to moderate effect on the QOL in the 43% (n = 6) of the patients. The ANA values of 3 patients (60%) were positive at the follow-up and negative at the first admission. CONCLUSION: SJS, TEN, and SJS/TEN overlap syndrome may cause sequelae even after a long time of the onset of the disease. Patients' QOL and psychological status can be affected negatively. Ocular symptoms may develop in the follow-up, even without involvement in the acute period. Patients with SJS, TEN, and SJS/TEN overlap syndrome should be followed up in the chronic period and approached multidisciplinary.


Asunto(s)
Hipersensibilidad , Síndrome de Stevens-Johnson , Humanos , Niño , Femenino , Masculino , Síndrome de Stevens-Johnson/complicaciones , Síndrome de Stevens-Johnson/diagnóstico , Calidad de Vida , Estudios Retrospectivos , Piel , Hipersensibilidad/complicaciones
2.
Pediatr Pulmonol ; 58(9): 2505-2512, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37278544

RESUMEN

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.


Asunto(s)
Fibrosis Quística , Quinolonas , Humanos , Fibrosis Quística/complicaciones , Fibrosis Quística/tratamiento farmacológico , Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/uso terapéutico , Estudios Retrospectivos , Aminofenoles/uso terapéutico , Quinolonas/uso terapéutico , Mutación
3.
Turk J Pediatr ; 65(2): 257-268, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37114691

RESUMEN

BACKGROUND: We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Türkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV1) decline and LT candidates without rapid FEV1 decline in the last year to identify a preventable cause in patients with such rapid FEV1 decline. METHODS: All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV1 below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV1 decline of more than 20% in the previous year but had other indications for LT (Group 2). Demographic and clinical features were compared between the two groups. RESULTS: Of 1488 patients registered in CFRT, 58 had a need for LT. Twenty patients were included in Group 1 and others in Group 2. Our findings did not reveal any significant variations in treatment, chronic infection status, or complications between the two groups. The average weight z-score was significantly higher in Group 1. Positive correlations were detected between weight z-score and FEV1 in 2017 in Group 1 and between FEV1 values in 2017 and 2018 in Group 2. CONCLUSIONS: There appears to be a relationship between the nutritional status and weight z-scores of CF patients and pulmonary function, which may indirectly affect the need for lung transplantation referral.


Asunto(s)
Fibrosis Quística , Trasplante de Pulmón , Humanos , Fibrosis Quística/epidemiología , Fibrosis Quística/cirugía , Fibrosis Quística/complicaciones , Datos de Salud Recolectados Rutinariamente , Pulmón , Volumen Espiratorio Forzado , Derivación y Consulta
4.
J Asthma ; 60(8): 1566-1572, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-36525429

RESUMEN

Objective: Increased health literacy (HL) improves the management of chronic diseases. Data on the HL levels of adolescents with asthma are limited. In this study, we aimed to investigate the HL levels of adolescents with asthma and the effect of HL levels on asthma control.Methods: Our research included 81 adolescents with asthma and 47 age and sex-matched controls. The validated version of the European Health Literacy Survey Questionnaire (HLS-EU-Q16) was utilized to estimate the participants' health literacy levels. In addition, the Asthma Control Test (ACT) was used to determine the degree of asthma control.Results: No significant difference between the asthmatic adolescents (n = 45, 55.6%) and the control group (n = 28, 59.6%) has been established in terms of the number of participants who were considered to have adequate HL (p = 0.658). The difference between the patient and control groups in health care, disease prevention, health promotion, and overall HL scores was determined non-significant. According to the ACT scores, the overall median HL score was significantly higher in patients with controlled asthma {34.4 (14.6:50)} than in those with uncontrolled asthma {32.3 (16.7:48.9)} (p = 0.037). It was determined that there was a difference in the distribution of controlled asthma, uncontrolled asthma, and controls in HL subgroups (poor, problematic-limited, sufficient, and perfect HL) (p = 0.002).Conclusion: The level of HL is associated with asthma control. A significant proportion of asthmatic adolescents who participated in our research displayed low HL scores. Further studies should be conducted to increase the HL levels of adolescents to achieve better asthma control.


Asunto(s)
Asma , Alfabetización en Salud , Humanos , Adolescente , Asma/epidemiología , Encuestas Epidemiológicas , Encuestas y Cuestionarios , Promoción de la Salud
5.
Pediatr Pulmonol ; 57(5): 1245-1252, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-35102722

RESUMEN

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.


Asunto(s)
Síndrome de Bartter , Fibrosis Quística , Síndrome de Bartter/complicaciones , Fibrosis Quística/complicaciones , Fibrosis Quística/epidemiología , Fibrosis Quística/terapia , Regulador de Conductancia de Transmembrana de Fibrosis Quística , Femenino , Humanos , Recién Nacido , Masculino , Atención al Paciente , Sistema de Registros , Turquía/epidemiología
6.
Pediatr Int ; 64(1): e14888, 2022 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-34131975

RESUMEN

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.


Asunto(s)
Fibrosis Quística , Fibrosis Quística/diagnóstico , Fibrosis Quística/epidemiología , Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Humanos , Recién Nacido , Tamizaje Neonatal/métodos , Staphylococcus aureus , Tripsinógeno
7.
J Korean Med Sci ; 36(49): e339, 2021 Dec 20.
Artículo en Inglés | MEDLINE | ID: mdl-34931499

RESUMEN

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic is affecting people at any age and there is limited information about the effect of the COVID-19 pandemic on quality of life (QoL) in adolescents with asthma. In the present study, it was aimed to assess the attitudes of adolescents with asthma toward the COVID-19 pandemic and determine the effects of the pandemic on their QoL. METHODS: In total, 125 adolescents with asthma and 98 healthy adolescents participated in the present study. The questionnaire form consisted of three parts. In the first part, all the participants were asked whether they complied with the protective measures against COVID-19. The second part included questions for measuring the participants' level of concern about COVID-19, while the third part consisted of EUROHIS-QOL 8. RESULTS: The patient and control groups were similar in terms of the female/male ratio (55/70 and 48/50, respectively) and mean participant age (14.6 ± 2 and 15.1 ± 1.65 years, respectively) (P = 0.459 and P = 0.062, respectively). The prevalence of COVID-19 in the patients (n = 2, 1.6%) was lower than that in the controls (n = 6, 6.1%); however, the difference was not statistically significant (P = 0.142). The total EUROHIS-QOL score was significantly lower in the patients (31.2 ± 6.7) than in the controls (33.7 ± 4.4) (P < 0.001). The total QoL scores of asthmatic adolescents without other allergic disease (31.4 ± 6.7) was also lower than those of the controls (33.7 ± 4.4) (P = 0.009). Treatment disruption was significantly more common in patients who received subcutaneous immunotherapy (n = 20, 48.8%) than in those who did not (n = 8, 9.5%) (P < 0.001). Moreover, the patients had lower EUROHIS-QOL scores in the overall QoL, general health, finance, and home domains. CONCLUSION: Our results indicate that the mean QoL score of asthmatic adolescents during COVID-19 pandemic is lower than in the healthy population. Disruption in their treatment was most common in patients with asthma who were receiving subcutaneous immunotherapy.


Asunto(s)
Asma/epidemiología , Asma/psicología , COVID-19/epidemiología , Adolescente , Corticoesteroides/uso terapéutico , Asma/complicaciones , Actitud Frente a la Salud , COVID-19/complicaciones , COVID-19/psicología , Estudios de Casos y Controles , Niño , Femenino , Humanos , Masculino , Pandemias , Prevalencia , Calidad de Vida , Cuarentena , Encuestas y Cuestionarios , Adulto Joven
8.
Allergy Asthma Proc ; 42(5): e135-e144, 2021 Sep 01.
Artículo en Inglés | MEDLINE | ID: mdl-34474716

RESUMEN

Background: Several factors that increase the risk of severe food-induced anaphylaxis have been identified. Objective: We aimed to determine the demographic, etiologic, and clinical features of food-induced anaphylaxis in early childhood and also any other factors associated with severe anaphylaxis. Methods: We carried out a medical chart review of anaphylaxis cases from 16 pediatric allergy and immunology centers in Turkey. Results: The data of 227 patients with 266 food-induced anaphylaxis episodes were included in the study. The median (interquartile range) age of the first anaphylaxis episode was 9 months (6-18 months); 160 of these patients were boys (70.5%). The anaphylaxis episodes were mild in 75 cases (28.2%), moderate in 154 cases (57.9%), and severe in 37 cases (13.9%). The most frequent food allergens involved were cow's milk (47.4%), nuts (16.7%), and hen's egg (15.8%). Epinephrine was administered in only 98 (36.8%) of these anaphylaxis episodes. A logistic regression analysis revealed two statistically significant factors that were independently associated with severe anaphylaxis: the presence of angioedema and hoarseness during the anaphylactic episode. Urticaria was observed less frequently in patients who developed hypotension. In addition, confusion and syncope were associated with 25.9- and 44.6-fold increases, respectively, in the risk of concomitant hypotension. Conclusion: Cow's milk, nuts, and hen's egg caused the majority of mild and moderate-to-severe anaphylaxis episodes. The presence of angioedema and hoarseness in any patient who presents with a history of food-induced anaphylaxis should alert clinicians that the reaction may be severe. In addition, the presence of confusion, syncope, or stridor probably indicates concomitant hypotension.


Asunto(s)
Anafilaxia , Angioedema , Hipersensibilidad a los Alimentos , Hipotensión , Hipersensibilidad a la Leche , Alérgenos , Anafilaxia/diagnóstico , Anafilaxia/epidemiología , Anafilaxia/etiología , Animales , Bovinos , Hipersensibilidad al Huevo , Femenino , Hipersensibilidad a los Alimentos/complicaciones , Hipersensibilidad a los Alimentos/diagnóstico , Hipersensibilidad a los Alimentos/epidemiología , Ronquera , Humanos , Lactante , Masculino , Hipersensibilidad a la Leche/complicaciones , Hipersensibilidad a la Leche/diagnóstico , Hipersensibilidad a la Leche/epidemiología , Hipersensibilidad a la Nuez , Síncope , Turquía
9.
Int Arch Allergy Immunol ; 182(7): 607-614, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33601370

RESUMEN

BACKGROUND: Urticaria can be the only sign of a food allergy or can be seen together with other signs and symptoms of a food allergy. OBJECTIVE: To determine the demographic, etiologic, and clinical features of food-induced acute urticaria in childhood. METHODS: Patients suspected of food-induced acute urticaria were included in this prospective cross-sectional multicenter study. RESULTS: Two hundred twenty-nine urticaria cases were included in this study. Seventeen patients who did not meet the inclusion criteria of the study were excluded. Of the 212 included cases, 179 (84.4%) were diagnosed with definitive food-induced acute urticaria. The most common foods causing acute urticaria were cow's milk, hen's eggs, and nuts in 56.4, 35.2, and 19% of cases, respectively. The positive predictive value of a history of milk-induced acute urticaria together with a milk-specific IgE >5 kU/L for cow's milk-induced acute urticaria was 92% (95% CI: 81-96%). A history of cow's milk-induced and/or hen's egg-induced acute urticaria was consistent with a definitive diagnosis of food-induced urticaria (Chen's kappa: 0.664 and 0.627 for milk and eggs, respectively). Urticaria activity scores were higher in patients with food-induced acute urticaria (p = 0.002). CONCLUSION: Cow's milk, hen's eggs, and nuts were the most common allergens in the etiology of childhood food-induced acute urticaria. Although the urticaria activity score provides guidance for diagnosis, an oral food challenge is often essential for the definitive diagnosis of a patient with a history of food-induced acute urticaria.


Asunto(s)
Alérgenos/inmunología , Hipersensibilidad a los Alimentos/complicaciones , Hipersensibilidad a los Alimentos/inmunología , Alimentos/efectos adversos , Urticaria/diagnóstico , Urticaria/etiología , Preescolar , Estudios Transversales , Diagnóstico Diferencial , Femenino , Hipersensibilidad a los Alimentos/diagnóstico , Humanos , Masculino , Pronóstico , Evaluación de Síntomas
10.
Turk Pediatri Ars ; 55(2): 174-183, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32684763

RESUMEN

AIM: To determine the frequency of sinopulmonary infections, detect changes in the respiratory system, and measure functional capacity of the lungs in our patients with humoral immunodeficiency. MATERIAL AND METHODS: Fifty-six patients with humoral immunodeficiency were enrolled in this study. The clinical, laboratory, and radiologic data, and pulmonary function tests of the subjects were evaluated from their file records, retrospectively. RESULTS: The distribution of our patients was as follows: 25 patients had common variable immune deficiency, three patients had X-linked agammaglobulinemia, five patients had hyper immunoglobulin M syndrome, 19 patients had deficiency of immunoglobulin G subset, and four patients had selective immunoglobulin A deficiency. The most common symptom of the patients was chronic cough (n=47, 83.9%). The most common pathologies on high-resolution computed tomography of the chest were atelectasis and bronchiectasis (27.7%). The most common pathology in pulmonary function tests was the presence of moderate obstructive patterns along with restrictive patterns (n=6,12.5%). The FEV 1, FVC, and FEF 25-75 values were significantly lower in patients with common variable immunodeficiency compared with the patients who had IgG subset deficiencies (p=0.001, p=0.01, p=0.01). Among the patients who were treated with intravenous immunoglobulin, the age at the diagnosis of immunodeficiency was higher in patients with bronchiectasis (14.2±8.4 years) compared with those without bronchiectasis (10.1±11.4 years) (p=0.04). CONCLUSION: Clinical findings are not sufficient to monitor the structural and functional changes in the respiratory system, and patients should be evaluated using high-resolution computed tomography of the chest and pulmonary function tests.

11.
Pediatr Pulmonol ; 55(9): 2302-2306, 2020 09.
Artículo en Inglés | MEDLINE | ID: mdl-32453906

RESUMEN

BACKGROUND: A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs. METHODS: Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy. RESULTS: Of registered 1488 CF patients, genetic analysis was done for 1351. The numbers and percentages of patients and names of the drugs, that the patients are eligible for, are as follows: 122 (9.03%) for ivacaftor, 156 (11.54%) for lumacaftor-ivacaftor, 163 (11.23%) for tezacaftor-ivacaftor, and 57 (4.21%) for elexacaftor-tezacaftor-ivacaftor. Among 1351 genotyped patients total of 313 (23.16%) patients are eligible for currently licensed modulator therapies (55 patients were shared by ivacaftor and tezacaftor-ivacaftor, 108 patients were shared by lumacaftor-ivacaftor and tezacaftor-ivacaftor, and 22 patients were shared by tezacaftor-ivacaftor and elexacaftor-tezacaftor-ivacaftor groups). CONCLUSIONS: The present study shows that approximately one-fourth of the registered CF patients in Turkey are eligible for modulator drugs. As, frequent mutations that CF patients have in Turkey are different from North American and European CF patients, developing modulator drugs effective for those mutations is necessary. Furthermore, as modulator drugs are very expensive currently, financial support of the government in developing countries like Turkey is noteworthy.


Asunto(s)
Aminofenoles/uso terapéutico , Aminopiridinas/uso terapéutico , Benzodioxoles/uso terapéutico , Agonistas de los Canales de Cloruro/uso terapéutico , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Fibrosis Quística/tratamiento farmacológico , Indoles/uso terapéutico , Pirazoles/uso terapéutico , Piridinas/uso terapéutico , Quinolinas/uso terapéutico , Quinolonas/uso terapéutico , Adolescente , Adulto , Niño , Preescolar , Estudios Transversales , Fibrosis Quística/genética , Combinación de Medicamentos , Femenino , Humanos , Lactante , Masculino , Mutación , Sistema de Registros , Turquía , Adulto Joven
12.
Pediatr Pulmonol ; 55(8): 2011-2016, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32364312

RESUMEN

BACKGROUND: Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population. METHODS: The data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017. Clinical features, diagnostic test results, colonization status, complications, and genetic test results were compared in patients with and without PBS. RESULTS: Totally 1170 patients were recorded into the registry in 2017 and 120 (10%) of them had PBS. The mean age of diagnosis and current age of patients were significantly younger and newborn screening positivity was lower in patients with PBS (P < .001). There were no differences between the groups in terms of colonization status, mean z-scores of weight, height, BMI, and mean FEV1 percentage. Types of genetic mutations did not differ between the two groups. Accompanying complications were more frequent in patients without PBS. CONCLUSION: PBS was detected as the most common complication in the registry. It could be due to warm weather conditions of our country. It is usually seen in younger ages regardless of mutation phenotype and it could be a clue for early diagnosis of CF.


Asunto(s)
Síndrome de Bartter/etiología , Fibrosis Quística/complicaciones , Adolescente , Adulto , Síndrome de Bartter/diagnóstico , Peso Corporal , Niño , Preescolar , Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Mutación , Tamizaje Neonatal , Fenotipo , Sistema de Registros , Turquía , Adulto Joven
13.
Pediatr Pulmonol ; 55(2): 541-548, 2020 02.
Artículo en Inglés | MEDLINE | ID: mdl-31710166

RESUMEN

BACKGROUND: Cystic fibrosis (CF) care has been implemented in Turkey for a long time; however, there had been no patient registry. For this purpose, the Turkish National CF Registry was established. We present the first results of registry using data collected in 2017. METHODS: The data were collected using a data-entry software system, which was accessed from the internet. Demographic and annually recorded data consisted of 15 and 79 variables, respectively. RESULTS: There were 1170 patients registered from 23 centers; the estimated coverage rate was 30%. The median age at diagnosis was 1.7 years (median current age: 7.3 years); 51 (4.6%) patients were aged over 18 years. Among 293 patients who were under 3 years of age, 240 patients (81.9%) were diagnosed through newborn screening. Meconium ileus was detected in 65 (5.5%) patients. Genotyping was performed in 978 (87.4%) patients and 246 (25.2%) patients' mutations were unidentified. The most common mutation was deltaF508 with an allelic frequency of 28%, followed by N1303K (4.9%). The median FEV1% predicted was 86. Chronic colonization with Pseudomonas aeruginosa was seen in 245 patients. The most common complication was pseudo-Bartter syndrome in 120 patients. The median age of death was 13.5 years in a total of 15 patients. CONCLUSIONS: Low coverage rate, lack of genotyping, unidentified mutations, and missing data of lung functions are some of our greatest challenges. Including data of all centers and reducing missing data will provide more accurate data and help to improve the CF care in Turkey in the future.


Asunto(s)
Fibrosis Quística/epidemiología , Adolescente , Adulto , Niño , Preescolar , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Mutación , Tamizaje Neonatal , Pseudomonas aeruginosa , Sistema de Registros , Turquía/epidemiología
14.
Turk Pediatri Ars ; 51(3): 152-158, 2016 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-27738400

RESUMEN

AIM: Stevens Johnson syndrome and toxic epidermal necrolysis are severe acute mucocutaneous diseases. In this study, we evaluated the clinical aspects of Steven Johnson syndrome, toxic epidermal necrolysis and Stevens-Johnson syndrome/toxic epidermal necrolysis overlap patients who admitted to our clinics in the last five years. MATERIAL AND METHODS: Eleven patients diagnosed as Stevens-Johnson syndrome, toxic epidermal necrolysis and Stevens-Johnson syndrome/toxic epidermal necrolysis overlap in Department of Pediatric Allergy in Uludag University School of Medicine were included in this study. Clinical findings, laboratory tests and response to treatments were evaluated via electronic files. RESULTS: Two of the patients had Stevens-Johnson syndrome, four had Stevens-Johnson syndrome/toxic epidermal necrolysis overlap, and five had toxic epidermal necrolysis. The median period for drug usage was 10 days (2-44 days). Herpes simpleks virus IgM antibody was detected two patients. The median healing time was 38 days 26-94 days). Maculopapular eruptions and oral mucositis were seen in all patients. Vesicul or bullae, epidermal detachment and ocular involvement in 10 of patients. Wound care, H1 antihistamine and methyl prednisolon were used in all patients, intravenous immunoglobulin were used in 7 patients and cyclosporine in 1 patient. Sequel lesions developed in 2 of the patients and there was no death. CONCLUSION: Anticonvulsants, antibiotics and non steroid anti-inflammatory drugs play a major role in the etiology of Stevens-Johnson syndrome and toxic epidermal necrolysis. Anticonvulsants are associated with severe disease. The patients with proper wound care and treatment with immunosuppressive drugs can be recovered without or with minimal sequelae.

15.
Paediatr Int Child Health ; 36(1): 64-71, 2016 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-25203843

RESUMEN

BACKGROUND: For the adequate control of asthma in school-age children, it is recommended that teachers, school health personnel and administrators should have sufficient knowledge of how to manage asthma during school hours. AIM: To investigate asthma health care in elementary schools, and teachers' knowledge of childhood asthma and its management. METHODS: The extent of knowledge of childhood asthma in 2779 teachers in 141 elementary schools (children aged 6-14, grades 1-8) in Bursa, the fourth largest city in Turkey, was evaluated. Section I comprised questions about asthma health-care in schools, Section II teachers' knowledge of the main characteristics of asthma and Section III (Likert Scale) teachers' detailed knowledge of the signs, triggering factors, treatment and general knowledge of asthma. RESULTS: The findings of Section I demonstrated that the organisation of health-care for asthma in schools was insufficient. Of the teachers questioned, 14·7% were not even aware and only 1% and 9·6% of the teachers had been made aware by school health personnel and school records, respectively, of asthmatic children. Only 27·3% of the teachers stated that they were responsible for the health of an asthmatic child. The majority of teachers (70%) said that asthmatic children could use the medication (e.g. inhalers) themselves. In Section II, there were between 44·1% and 75·5% correct answers, while in Section III this figure ranged from 3·3% to 78·4%. The correct answer rate was 60·4% for Sections II and III combined. The results of Sections II and III showed that the teachers' knowledge of asthma was poor in many respects. Teachers who stated that they had asthma or had first-degree relatives with asthma, or those with 10 or more years' experience provided significantly more correct answers in Sections II and III combined than did those without these characteristics (P<0·001). CONCLUSIONS: There is a need to improve and standardise health care for asthma (asthma management policies) in schools. The implementation of asthma education programmes for teachers and other staff responsible for pupils' health should result in better control of this common disease.


Asunto(s)
Asma/terapia , Atención a la Salud/organización & administración , Conocimientos, Actitudes y Práctica en Salud , Servicios de Salud Escolar/estadística & datos numéricos , Maestros/normas , Adolescente , Adulto , Anciano , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Instituciones Académicas , Encuestas y Cuestionarios , Turquía
16.
J Asthma ; 49(8): 868-74, 2012 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-22953785

RESUMEN

BACKGROUND: The Childhood Asthma Control Test (C-ACT) has been proposed to be a simple, patient-based test that is able to reflect the multidimensional nature of asthma control. In this analysis, the aim was to evaluate the perceptions of physicians and caregivers concerning C-ACT and its predictive value for future asthma-related events. METHOD: In a multicenter prospective design, 368 children aged 4-11 years with asthma who were either well- or not well-controlled were included in the study. The study participants were evaluated during three visits made at 2-month intervals and the Turkish version of C-ACT was completed each month. Parents completed questionnaires concerning their perception of asthma (before and after the study) and the C-ACT (after the study). Physicians completed a survey about their perception of a control-based approach and the C-ACT. RESULTS: The C-ACT scores increased from visit 1 to visit 3, with improvement seen in all domains of the test. At the end of the study period, the parents more strongly agreed that asthma could be controlled completely and that asthma attacks and nocturnal awakenings due to asthma were preventable (p < .05). Most of the parents reported that the C-ACT helped them to determine asthma treatment goals for their children and also that the C-ACT improved communication with their physicians. The physicians indicated that a control-centered approach was more convenient (95%) and simpler (94.5%) than a severity-centered approach and provided better disease control (93.4%). A higher C-ACT score was associated with a decreased risk of asthma attack and emergency department admittance in the 2 months following the administration of C-ACT. Conclusion. Our findings indicated that the C-ACT improved both parental outlook on asthma control and the communication between the physician and parents. There was a good correlation between the C-ACT score and the level of asthma control achieved, as described by the physician. Additionally the C-ACT score was predictive of future asthma-related events. These findings suggest that the C-ACT may have an important role in asthma management in the future.


Asunto(s)
Asma/prevención & control , Asma/psicología , Padres/psicología , Médicos/psicología , Asma/diagnóstico , Actitud del Personal de Salud , Niño , Preescolar , Femenino , Humanos , Modelos Logísticos , Masculino , Análisis Multivariante , Valor Predictivo de las Pruebas , Estudios Prospectivos , Pruebas de Función Respiratoria , Encuestas y Cuestionarios , Turquía
17.
Environ Monit Assess ; 164(1-4): 391-402, 2010 May.
Artículo en Inglés | MEDLINE | ID: mdl-19387854

RESUMEN

Pollen concentrations in the atmosphere of Istanbul, a city located between two continents, has been monitored for 1 year as part of a larger research program. The sampling sites were located in two different continents: the Asian part (AS) and the European part (EP). The sampling was performed in AS and EP of the city by using Hirst type volumetric method, and pollen grains of 58 and 62 taxa were identified in the two parts, respectively. The pollen spectrum reflected the floristic diversity of the region. The main pollen producers at the sites were characterized by some allergenic pollen and were identified as Cupressaceae/Taxaceae, Urticaceae, Pistacia sp., Quercus sp., Platanus sp., Fraxinus sp., and Xanthium sp. These pollen types contributed to the total pollen sum with a percentage of more than 80% at both monitoring sites. The highest amount of pollen grains was recorded in April. The greatest number of species was recorded in May, when 42 types (AS) and 44 types (EP) were present.


Asunto(s)
Contaminantes Atmosféricos/análisis , Polen , Clima , Monitoreo del Ambiente , Plantas , Estaciones del Año , Especificidad de la Especie , Turquía
18.
Environ Monit Assess ; 158(1-4): 365-80, 2009 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-18951137

RESUMEN

A continuous aerobiological survey of the atmosphere of Bursa was carried out from 1st January 2003 to 31st December 2004 by means of the volumetric method using a Lanzoni trap. During 2 years, a total of 57,124 pollen grains/m(3), which belonged to 66 taxa and 869 unidentified pollen grains, were recorded. In the region investigated, Pinus sp., Olea sp., Platanus sp., Cupressaceae/Taxaceae, Quercus sp., Poaceae, Moraceae, Urticaceae, and Castanea sp. were responsible for the greatest amounts of pollen. During the study period, the pollen concentration reached the highest level in May. A correlation analyses was made between the daily fluctuations of the main pollen types and meteorological parameters.


Asunto(s)
Contaminantes Atmosféricos/análisis , Atmósfera/análisis , Monitoreo del Ambiente/métodos , Polen , Alérgenos/análisis , Olea , Pinus , Poaceae , Turquía
19.
Pediatr Allergy Immunol ; 20(1): 72-80, 2009 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-18363634

RESUMEN

Successful management of childhood asthma requires a thorough idea of the economic impact of asthma and its determinants, as policy makers and physicians inevitably influence the outcome. The aim of this study was to define the cost of childhood asthma in Turkey and its determinants. In April 2006, a multi-center, national study was performed where data regarding cost and control levels were collected. Asthmatic children (6-18 yr) with at least a 1-yr follow-up seen during a 1-month period with scheduled or unscheduled visits were included. The survey included a questionnaire-guided interview and retrospective evaluation of files. Cost and its determinants during the last year were analyzed. A total of 618 children from 12 asthma centers were surveyed. The total annual cost of childhood asthma was US$1597.4 +/- 236.2 and there was a significant variation in costs between study centers (p < 0.05). Frequent physician visits [odds ratio (95% confidence intervals)] [2.3 (1.6-3.4)], hospitalization [1.9 (1.1-3.3)], asthma severity [1.6 (1.1-2.8)], and school absenteeism due to asthma [1.5 (1.1-2.1)] were major predictors of total annual costs (p < 0.05 for each). The comparable cost of asthma among Turkish children with that reported in developed countries suggests that interventions to decrease the economic burden of pediatric asthma should focus on the cost-effectiveness of anti-allergic household measures and on improving the control levels of asthma.


Asunto(s)
Asma/economía , Asma/terapia , Asma/epidemiología , Niño , Análisis Costo-Beneficio , Estudios Transversales , Recolección de Datos , Femenino , Estudios de Seguimiento , Humanos , Masculino , Estudios Retrospectivos , Factores de Riesgo , Encuestas y Cuestionarios , Turquía/epidemiología
20.
Am J Rhinol ; 20(1): 53-63, 2006.
Artículo en Inglés | MEDLINE | ID: mdl-16539296

RESUMEN

BACKGROUND: Wheezing is the most common symptom of childhood respiratory tract illnesses. It is important not only for its associated acute morbidity, but also for the fact that early childhood wheezing confers a high risk for asthma. Epidemiological studies from various countries show that 10-15% of children <1 year of age and 25% of children <5 years of age have wheezing-associated respiratory tract illness, and one-third of these children develop asthma later in life. METHODS: In this retrospective study, we evaluated the association between a history of wheezing and prenatal, postnatal, familial, and environmental risk factors in 858 7-year-old children, randomly selected from seven primary schools in Bursa, Turkey, by means of an easy-to-understand questionnaire form. Among these children, 12.4% had a history of early transient wheezing, 7.1% had persistent wheezing, and 7.7% had late onset wheezing; 72.8% had no wheezing symptoms and 33.3% of children who experienced wheezing during the first 3 years of life had physician-diagnosed asthma. RESULTS: Notable risk factors associated with wheezing were as follows: male gender, lower socioeconomic status, premature birth, maternal smoking during pregnancy, bottle-feeding before 2 months of age, dampness and mold at home, hospitalization due to any respiratory illness in infancy, history of croup between 6 months and 5 years of age, frequent upper respiratory infections during the first 3 years of life, allergic eczema in the child, and any allergic disease in the mother or siblings. CONCLUSION: This study shows that the high rates of reported wheezing in the 858 primary school children in Bursa are clearly attributable to important risk factors that have long been recognized and discussed by researchers worldwide, and this suggests that all efforts at primary prevention may be insufficient.


Asunto(s)
Asma/epidemiología , Ruidos Respiratorios , Enfermedades Respiratorias/epidemiología , Contaminación del Aire Interior , Alimentación con Biberón , Lactancia Materna , Niño , Femenino , Humanos , Modelos Logísticos , Masculino , Prevalencia , Estudios Retrospectivos , Factores de Riesgo , Distribución por Sexo , Factores Socioeconómicos , Encuestas y Cuestionarios , Turquía/epidemiología
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