RESUMEN
BACKGROUND: Although it is a valuable option for children with drug-resistant epilepsy, ketogenic diet (KD) therapy is associated with several side effects. The frequency of kidney stones and risk factors for their development in epileptic children receiving KD is unclear. The aim of this study was to determine the frequency and risk factors for the development of renal stones in children receiving KD therapy. METHODS: A total of 95 patients receiving KD were identified. Of these, seven patients were excluded from the study due to the duration of KD being less than 12 months. The remaining 88 children were enrolled in the study. RESULTS: Renal stones were detected in 15 patients (17%), of which 12 (73.3%) received potassium citrate treatment. Two (13.3%) patients needed lithotripsy despite receiving potassium citrate treatment, and one of these, who received potassium citrate treatment for 5 months, developed acute vesicourethral reflux and underwent surgery. No patient discontinued KD due to renal stone development. The serum uric acid concentrations and urine calcium/creatinine ratio did not change significantly over the 24-month follow-up period. Age, gender, etiology, age at seizure onset, duration of KD, mobility status, use of topiramate or zonisamide, and the number of antiepileptic drugs used were not significantly different between patients with and without kidney stones. CONCLUSION: Renal stone appears to be a common adverse effect of KD therapy. Although adequate hydration and potassium citrate treatment are effective in most patients, lithotripsy and surgery may be required in a minority of patients.
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Dieta Cetogénica , Cálculos Renales , Niño , Humanos , Dieta Cetogénica/efectos adversos , Citrato de Potasio/efectos adversos , Ácido Úrico/uso terapéutico , Cálculos Renales/etiología , Factores de Riesgo , Resultado del TratamientoRESUMEN
BACKGROUND: Although Guillain-Barré syndrome (GBS) is now the most common cause of acute flaccid paralysis in children, information on the long-term follow-up of GBS is still limited. Identification of prognostic factors can play an important role in treatment strategies and the follow-up of patients. This study aimed to evaluate the effectiveness of monitoring the GBS disability score (DS) in predicting morbidity and mortality. METHODS: The patients were separated into two groups those with DS≥ or <3 on admission. These groups were compared in respect of demographic data, clinical and laboratory findings, and the DS recorded on admission and at first, third, sixth, 12th, and 24th months. RESULTS: The study included 44 patients (54.5% male, 45.5% female) with a median age of 5 years. The most common involvements during the disease were weakness, ataxia, neuropathic pain, cranial neuropathy, respiratory distress, autonomic dysfunction, and psychiatric symptoms, respectively. In patients with a DS of ≥3, the time from onset of symptoms to hospital admission was shorter, and the length of hospital stay was longer. Children with back pain and autonomic dysfunction had a DS of ≥3. A high 3-month DS was found to be a significant predictor for the development of sequelae. CONCLUSIONS: Although progressive muscle weakness and inability to walk are the most common symptoms of GBS, it should be kept in mind that atypical manifestations such as hemiplegia and ophthalmoplegia may also occur. For an objective assessment of clinical improvement during follow-up, the DS for motor functions can be used.
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Síndrome de Guillain-Barré , Humanos , Masculino , Niño , Femenino , Preescolar , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/terapia , Estudios Retrospectivos , Progresión de la Enfermedad , Hospitalización , Tiempo de InternaciónRESUMEN
OBJECTIVES: Status epilepticus (SE) is associated with significant morbidity and mortality in children. SE in the pediatric intensive care unit (PICU) are not well characterized. The aim of this study is to retrospectively investigate the clinical features and treatment of seizures in children admitted to the PICU of our hospital. METHODS: We retrospectively examined the clinical characteristics of patients aged between 1 month and 18 years who were admitted to our hospital with SE or who were diagnosed with SE after hospitalization and were followed up with continuous electroencephalographic monitoring between January 2015 and December 2019. RESULTS: A total of 88 patients with SE, 50 (56.8%) boys and 38 (43.2%) girls, were included. The median age was 24 months (interquartile range, 12-80 months). When we evaluate the continuous electroencephalographic monitoring data, 27 (30.7%) were lateralized, 20 (22.7%) were multifocal, 30 (34.1%) were generalized, and 11 (12.5%) were bilateral independent epileptic activity. Seventy nine patients (89.8%) were evaluated as convulsive status epilepticus (CSE) and 9 (10.2%) as nonconvulsive status epilepticus (NCSE). Pediatric Risk of Mortality (PRISM III) score and mortality of patients with NCSE were higher ( P = 0.004 and P = 0.046, respectively). Thirteen eight patients (43.1%) were diagnosed as SE, 38 patients (43.1%) as refractory SE, and 12 patients (13.6%) as super-refractory SE. The overall mortality rate was 10.2%. CONCLUSIONS: Status epilepticus is a neurological emergency that causes mortality and morbidity. Electroencephalographic monitoring is important for the recognition of seizures and rapid intervention. No superiority of second-line treatments or combined treatments was demonstrated in patients with SE.
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Electroencefalografía , Estado Epiléptico , Masculino , Niño , Femenino , Humanos , Preescolar , Lactante , Estudios Retrospectivos , Electroencefalografía/efectos adversos , Estado Epiléptico/diagnóstico , Estado Epiléptico/tratamiento farmacológico , Convulsiones , Unidades de Cuidado Intensivo PediátricoRESUMEN
AIM: Due to reactions, such as being ridiculed, blamed, or rejected, children with epilepsy and their families may consider epilepsy as something to be ashamed of, and therefore both the child and parents hide the disease from other people. No valid and reliable measurement in Turkish language that evaluates the level of this behavior, which will greatly affect the management of epilepsy, in both children and parents has been found in the literature. This study was carried out to test the validity and reliability of the Epilepsy Disclosure Scale (EDS) - Youth and Parent Versions in Turkey. MATERIALS AND METHOD: A descriptive, comparative, correlational, and methodological design was used in the study. The study was carried out with 200 youth with epilepsy between the ages of 8 and 18, who were registered in the pediatric neurology outpatient clinic of a university hospital located in the western region of Turkey, and their parents. The study data were collected using a Descriptive Information Form and the Turkish version of the EDS-Y and the EDS-P. The data were evaluated using content validity index, explanatory and confirmatory factor analyses, Cronbach's alpha, split-half, and item-total score correlation. FINDINGS: The total explained variance of the Turkish version of the EDS-Y consisting of one sub-dimension and six items was determined as 53.55%, and the total explained variance of the Turkish version of the EDS-P consisting of one sub-dimension and six items was determined as 59.39%. Cronbach's alpha values were 0.864 for the overall Turkish EDS-Y and 0.881 for the EDS-P. According to the confirmatory factor analysis, the model fit indices of both scales were found to be above 0.90 and the factor loads of all items were greater than 0.40. CONCLUSION: The Turkish versions of the EDS-Y and EDS-P scales have acceptable internal consistency reliability and content and construct validity and can be used by health professionals to evaluate the concealment of epilepsy from the perspectives of both young people and parents.
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Revelación , Epilepsia , Adolescente , Niño , Humanos , Lenguaje , Padres , Psicometría , Reproducibilidad de los Resultados , Encuestas y Cuestionarios , TurquíaRESUMEN
PURPOSE/AIM: Ehlers-Danlos syndrome (EDS) is a hereditary connective tissue disease. Epilepsy is not a common neurological finding in EDS. Here we report a pediatric patient with EDS comorbid with STXBP1 related epileptic encephalopathy as 'electrical status epilepticus during slow-wave sleep (ESES)' and whose refractory epileptic seizures were controlled with ketogenic diet. CASE REPORT: A 6-year-old girl who had EDS presented with refractory seizures and worsening cognitive functions. Her sleep electroencephalography (EEG) revealed electrical status epilepticus during slow-wave sleep (ESES). The epileptic encephalopathy panel revealed a de novo c.560C > T (p.pro187Leu) heterozygous mutation in the STXPB1 gene. Ketogenic diet treatment was started for her refractory seizures and seizures stopped in the third month of the 3:1 classical ketogenic diet. CONCLUSION: Our case is remarkable due to the coexistence of EDS and epileptic encephalopathy as well as ESES findings in STXBP1-associated epileptic encephalopathy and is therefore presented. Ketogenic diet would be beneficial on the management of refractory seizures in STXBP1-related epileptic encephalopathy and ESES.
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Dieta Cetogénica , Síndrome de Ehlers-Danlos , Epilepsia Generalizada , Epilepsia , Estado Epiléptico , Niño , Síndrome de Ehlers-Danlos/complicaciones , Electroencefalografía , Epilepsia/complicaciones , Femenino , Humanos , Proteínas Munc18/genética , Convulsiones/complicaciones , Sueño , Estado Epiléptico/complicacionesRESUMEN
BACKGROUND: Epilepsy is a neurological disease that requires long-term treatment and monitoring and causes significant restrictions in physical, emotional, intellectual, and social life that negatively affect the quality of life of the individual. This study aimed to test the validity and reliability of the Quality of Life in Childhood Epilepsy Questionnaire in Turkey. METHODS: The study was conducted on 421 parents using a descriptive correlational method. The data of the study were collected using a Descriptive Information Form and the Quality of Life in Childhood Epilepsy Questionnaire. Data analysis and evaluation were performed using factor analysis, Cronbach's alpha, and item-total score correlation. FINDINGS: The scale consists of 16 items and four sub-dimensions. The four sub-dimensions recorded a variance of 87.83%. Cronbach's alpha coefficient of the Turkish version of the scale was 0.96. The two-month test-retest reliability evaluated with intra-class correlation was 0.85. Confirmatory factor analysis indicated, the model fit index results were recorded as follows: 0.93 as the Goodness-of-Fit Index; comparative fit index, 0.98 and non-normed fit index (NNFI), 0.97. CONCLUSIONS: The study determined that the Turkish version of the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-16) is a valid and reliable measurement tool when used to measure quality of life for Turkish children with epilepsy. PRACTICE IMPLICATIONS: It is recommended that the health-related quality of life should be evaluated to assess the treatment of children with epilepsy and to intervene early in potential risk factors associated with the disease management process. All healthcare professionals can use this scale in interventional studies aiming at evaluating or improving the quality of life of children with epilepsy.
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Epilepsia , Calidad de Vida , Niño , Epilepsia/diagnóstico , Humanos , Psicometría , Reproducibilidad de los Resultados , Encuestas y Cuestionarios , TurquíaRESUMEN
BACKGROUND: Sleep disorders are common in drug-resistant children with epilepsy and their mothers. Ketogenic diet therapy (KDT) may have positive effects on sleep quality. The aim of this study was to evaluate the sleep quality of children with epilepsy and their mothers after starting KDT. METHODS: Using a prospective cross-sectional model, pre- and post-KDT questionnaires were given to the study subjects. A children's sleep habits questionnaire was administered to children with epilepsy, and the Pittsburgh sleep questionnaire was administered to their mothers. Sociodemographic and some clinical categorical variables of the patient group were evaluated using descriptive statistics. Evaluation of the data was conducted using the Wilcoxon and paired t-tests as parametric and non-parametric tests. RESULTS: Of 24 patients scheduled to begin KDT between January 2019 and January 2020, 14 were included in the study. Regarding sleep quality, improvement was reported in 7 (50%) of 14 patients, deterioration in 5 (35.7%) patients, and no change was seen in 2 (14.3%) patients. Sleep quality was reported to improve in all working mothers. Seven (50%) patients reported no seizures and 6 (42.9%) patients reported more than 50% seizure reduction. Although there were improvements in sleep scores in both groups, these improvements were not statistically significant. A significant decrease in sleep anxiety was reported in children after the third month of the KDT (pâ¯=â¯0.09). CONCLUSIONS: The results of this study determined that three months of KDT offered significant improvement on the sleep anxiety of children with epilepsy. It was thought that paying attention to patient selection may lead to better sleep quality by increasing compliance to KDT. However, a larger scale study and longer term follow-up should be done.
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Dieta Cetogénica , Epilepsia , Preparaciones Farmacéuticas , Niño , Estudios Transversales , Epilepsia/complicaciones , Femenino , Humanos , Madres , Estudios Prospectivos , Calidad del SueñoRESUMEN
BACKGROUND: Ketogenic diet (KD) remains a valuable treatment option for children with drug-resistant epilepsy. However, it may cause many well-known adverse effects such as dyslipidemia or kidney stones. But, its effects on thyroid functions are largely unknown. PURPOSE: The aim of this study was to investigate the effects of the KD on thyroid functions in children with drug-resistant epilepsy. METHOD: A total of 66 children (35 females) aged 3-193 months (median, 52 months) with drug-resistant epilepsy who received a KD for at least 12 months were enrolled in the study. All children were started on KD with 3:1 ratio which was then adjusted as clinically necessary. Serum free-thyroxine (FT4) and thyroid stimulating hormone (TSH) concentrations were measured before starting treatment and at the first, sixth and twelfth months of treatment. Changes in FT4 and TSH concentrations over 12 months were analyzed. RESULTS: Median serum FT4 and TSH concentrations, and the frequencies of patients with low FT4 and high TSH concentrations did not change significantly in the study sample over the 12-month study period. Serum FT4 levels increased significantly and TSH concentrations decreased insignificantly in four patients receiving L-thyroxine replacement therapy. During the 12-month treatment period, BMI-SDS increased, and the number of antiepileptic drugs decreased significantly. CONCLUSION: It appears that KD therapy does not impair thyroid functions in children with drug-resistant epilepsy. KD can be used safely along with L-thyroxine replacement even in children with pre-existing subclinical hypothyroidism.
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Dieta Cetogénica , Epilepsia Refractaria , Preparaciones Farmacéuticas , Niño , Epilepsia Refractaria/tratamiento farmacológico , Femenino , Humanos , Glándula Tiroides , Tirotropina , TiroxinaRESUMEN
BACKGROUND: Vincristine (VCR), which is a key component of chemotherapy, is important for survival. VCR is associated with a well-known side effect, including neurotoxicity. AIMS: The aim of this study was to evaluate the features of vincristine-induced peripheral neuropathy (VIPN) and the effectiveness of pyridoxine plus pyridostigmine therapy in children with acute lymphoblastic leukemia. METHODS: The WHO and NCI CTCAE neurotoxicity scorings were used to evaluate VIPN at diagnosis, in the first month, and after the third month of the treatment. The clinical features of 23 patients having acute lymphoblastic leukemia with VIPN during the period of July 2013-February 2016 were prospectively evaluated. RESULTS: The mean age was 72.8 ± 51.6 months, and 26.1%, 56.5%, and 17.4% were in standard, moderate, and high-risk groups, respectively. Neuropathy frequently occurred at induction (82.6%) and reinduction (17.4%) of the protocol. Drop foot (82.6%), leg pain (82.6%), and difficulty in walking (82.6%) were observed. The mean total cumulative dose of neuropathy occurrence was 5.6 ± 2.03 mg/m2. Our study showed that both the WHO and NCI CTCAE scorings were significantly improved via pyridoxine plus pyridostigmine therapy. CONCLUSION: The WHO and NCI CTCAE scorings may be used for evaluating neuropathy at diagnosis and follow-up of neurotoxicity with treatment. Pyridoxine plus pyridostigmine therapy may be an effective option in the treatment of VIPN.
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Antineoplásicos Fitogénicos , Enfermedades del Sistema Nervioso Periférico , Leucemia-Linfoma Linfoblástico de Células Precursoras , Niño , Preescolar , Humanos , Lactante , Enfermedades del Sistema Nervioso Periférico/inducido químicamente , Enfermedades del Sistema Nervioso Periférico/tratamiento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Bromuro de Piridostigmina/uso terapéutico , Piridoxina/uso terapéutico , Vincristina/efectos adversosRESUMEN
Neurofibromatosis type 1 (NF1) is caused by mutations in the NF1 gene. This retrospective study aims to evaluate the clinical manifestations and brain magnetic resonance images (MRI) analysis in 60 genetically confirmed NF1 patients. The results of next-generation sequencing (NGS), Sanger sequencing, and MLPA of NF1 gene were evaluated. A total of 54 different variants were identified. Fourteen out of them were novel variants (25.9%). Patients who complied with NIH criteria had most frequently frameshift variants (11/32 patients), and those with only CALMs had missense variants (9/28 patients). Neurofibromatosis type 1 bright objects (NBOs) on T2-weighted MRI were detected in 42 patients (42/56; 75%). These brain lesions were detected mostly in basal ganglia and in cerebellar vermis. NBOs were detected more in the patients who complied with NIH criteria (80.6%) compared to those who were only CALMs (68%). While frameshift variants (33.3%) were the most common type variants in the patients who had NBOs, the most common variants were splicing (35.7%) and missense (35.7%) variants in the patients whose MRIs were normal. Frameshift variants (11/28 patients; 39.3%) were the most common in the patients with more than one brain locus involvement. Therefore, we consider that frameshift variants may be associated with increased incidence of NBOs and involvement of more than one brain locus. In addition, NBOs may occur less frequently in the patients with splicing variants. To our knowledge, this is the first study evaluated the relationship between NF1 gene variants and NBOs. Future studies may help us understand the etiology of NBOs.
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Neurofibromatosis 1 , Encéfalo/diagnóstico por imagen , Genes de Neurofibromatosis 1 , Humanos , Imagen por Resonancia Magnética , Neurofibromatosis 1/diagnóstico por imagen , Neurofibromatosis 1/genética , Neurofibromina 1 , Estudios RetrospectivosRESUMEN
INTRODUCTION AND PURPOSE: Neuronal ceroid lipofuscinoses (NCLs) is a group of congenital metabolic diseases where the neurodegenerative process with the accumulation of ceroid and lipofuscin autofluorescent storage materials is at the forefront. According to the age of presentation, NCLs are classified as congenital, infantile (INCL), late infantile (LINCL), juvenile (JNCL), and adult (ANCL) NCLs. In our study, it was aimed to discuss the clinical and molecular characteristics of our patients diagnosed with NCL. MATERIAL AND METHOD: This is a descriptive cross-sectional study which was conducted in 14 patients from 10 unrelated families who were diagnosed with different types of NCL based on clinical presentation, neuroimaging, biochemical measurements, and molecular analyses, at the department of pediatric metabolism between June 2015 and June 2020. RESULTS: A total of 14 patients were diagnosed with different types of NCL. Of those, 4 patients were diagnosed with NCL7 (4/14; 30%), 3/14 (23%) with NCL1, 3/14 (23%) with NCL2, 2/14 (14.2%) with NCL13, and 1/14 (7.1%) with NCL10. Eleven pathogenic variants were detected, 5 of which are novel (c.721G>T [p.Gly241Ter] and c.301G>C [p.Ala146Pro] in MFDS8 gene; c.316C>T [p.Gln106Ter] in PPT1 gene; c.341C>T [p.Ala114Val] in TPP1 gene; c.686A>T [p.Glu229Val] in CTSD gene) CONCLUSION: This study is one of the pioneer comprehensive researches from Turkey that provides information about disease-causing variants and clinical presentation of different and rare types of NCLs. The identification of novel variants and phenotypic expansion is important for genetic counselling in Turkey and expected to improve understanding of NCLs.
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Lipofuscinosis Ceroideas Neuronales , Adulto , Niño , Estudios Transversales , Humanos , Lipofuscinosis Ceroideas Neuronales/diagnóstico por imagen , Lipofuscinosis Ceroideas Neuronales/genética , Tripeptidil Peptidasa 1 , TurquíaRESUMEN
This study used a descriptive, correlational, and cross-sectional research design to evaluate the effect of seizure self-efficacy of children with epilepsy on their perceived stigma of seizure. The study was conducted with 303 children with epilepsy. The data of the study were collected using "the Seizure Self-Efficacy Scale for Children" and "the Scale for Perceived Stigma in Children with Epilepsy". The mean age of the children included in the study was 12.65⯱â¯2.37. The correlation between seizure self-efficacy of the children and their perceived seizure stigma was examined; a strong, significant, and negative correlation was found. It is recommended that the awareness of all health professionals should be increased in approaching children with epilepsy and that self-efficacy and stigma should be addressed.
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Epilepsia/psicología , Convulsiones/psicología , Autoeficacia , Estigma Social , Adolescente , Adulto , Niño , Estudios Transversales , Epilepsia/diagnóstico , Femenino , Humanos , Masculino , Convulsiones/diagnósticoRESUMEN
Background and aim: The number of reports on the role of tubulin gene mutations (TUBA1A, TUBB2B, and TUBB3) in etiology of malformations of cortical development has peaked in recent years. We aimed to determine tubulin gene defects on a patient population with simple and complex malformations of cortical development, and investigate the relationship between tubulin gene mutations and disease phenotype. Materials and methods: We evaluated 47 patients with simple or complex malformations of cortical development, as determined by radiological examination, for demographic features, clinical findings and mutations on TUBA1A, TUBB2B, and TUBB3 genes. Results: According to the magnetic resonance imaging findings, 19 patients (40.5%) had simple malformations of cortical development and 28 (59.5%) patients had complex malformations of cortical development. Focal cortical dysplasia was the most common simple malformation, lissencephaly was the most common coexisting cortical malformation, and corpus callosum anomalies were the most common coexisting extracortical neurodevelopmental abnormalities. None of the patients had genetic alterations on TUBA1A, TUBB2B, and TUBB3 genes causing protein dysfunction. On the other hand, the frequencies of some polymorphisms were higher when compared to the literature. Conclusion: It is crucial to identify the etiology in patients with malformations of cortical development in order to provide appropriate genetic counseling and prenatal diagnosis. We consider that multicenter studies with higher patient numbers and also including other malformations of cortical development-related genes are required to determine underlying etiological factors of malformations of cortical development patients.
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Malformaciones del Desarrollo Cortical , Tubulina (Proteína)/genética , Adolescente , Niño , Preescolar , Consanguinidad , Femenino , Humanos , Lactante , Desequilibrio de Ligamiento , Imagen por Resonancia Magnética , Masculino , Malformaciones del Desarrollo Cortical/diagnóstico por imagen , Malformaciones del Desarrollo Cortical/genética , Polimorfismo de Nucleótido Simple/genéticaRESUMEN
BACKGROUND: The ketogenic diet (KD) has been frequently used for the patients with drug-resistant epilepsy in recent years. The management of these patients in emergency departments (EDs) has some difficulties due to the special needs of KD. We aimed to determine the characteristics and the management of the patients on the KD in the pediatric ED setting. METHODS: Patients who were on the KD and admitted to the ED were included in the study. Demographic, clinical, and laboratory data of all patients were retrospectively reviewed and recorded. RESULTS: There were 105 emergency admissions of 27 patients. The median age of all patients was 55.0 (IQR: 29.0-91.0) months. The most common symptom was vomiting (43.8%). Four patients had upper gastrointestinal bleeding, and one patient had hyperammonemic acute hepatic failure while receiving KD. Of the patients, 41.9% had seizure-related ED admission. Infections were present in 41.9% of the ED visits. The frequency of status epilepticus was significantly lower in the patients who were on the KD for more than 6â¯months (pâ¯<â¯0.01). In 42.9% of all ED admissions, dextrose containing maintenance fluids was administered mistakenly; although ketosis rate was lower, no seizure was observed in this group. CONCLUSION: The patients on the KD can be admitted to EDs with intercurrent illnesses or adverse effects of the KD. For accurate management, emergency physicians must be aware of the common reasons for ED admission of these patients and the effects of the KD.
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Dieta Cetogénica/efectos adversos , Epilepsia Refractaria/dietoterapia , Servicio de Urgencia en Hospital/estadística & datos numéricos , Utilización de Instalaciones y Servicios/estadística & datos numéricos , Niño , Preescolar , Epilepsia Refractaria/complicaciones , Femenino , Estudios de Seguimiento , Humanos , Masculino , Estudios Retrospectivos , Estado Epiléptico/epidemiología , Estado Epiléptico/etiología , Estado Epiléptico/prevención & control , Resultado del TratamientoRESUMEN
AIM: The practical value of using fibre-enriched enteral feeding regimens to rehabilitate malnourished children remains inconclusive. This study determined the usage patterns, gastrointestinal tolerance, anthropometrics and safety of high-fibre enteral feeding in malnourished children with growth failure. METHODS: This Turkish observational study between February 2013 and June 2015 comprised 345 paediatric patients from 17 centres with malnutrition-related growth failure, with a weight and height of <2 SD percentiles for their age. Changes in anthropometrics, gastrointestinal symptoms, defecation habits and safety data relating to adverse events were analysed during the six-month follow-up period. RESULTS: Most subjects (99.7%) were supplemented with enteral feeding. The absolute difference and 95% confidence interval values for the Z scores of height for age, weight for age, weight for height and body mass index for height increased significantly in four months to six months to 0.21 (0.09-0.32), 0.61 (0.51-0.70), 0.81 (0.56-1.06) and 0.70 (0.53-0.86), respectively (p < 0.001 for each). The percentage of patients with normal defecation frequency significantly increased from 70.3% to 92.8% at the four months to six months visit (p = 0.004). Adverse events occurred in 15 (4.3%) of patients. CONCLUSION: Using a six-month high-fibre enteral feeding was associated with favourable outcomes in anthropometrics, appetite, gastrointestinal tolerance and safety in malnourished children.
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Trastornos de la Nutrición del Niño/terapia , Fibras de la Dieta , Nutrición Enteral , Trastornos de la Nutrición del Lactante/terapia , Antropometría , Niño , Preescolar , Defecación , Femenino , Enfermedades Gastrointestinales/epidemiología , Humanos , Lactante , Masculino , Turquía/epidemiologíaRESUMEN
BACKGROUND: Seizure-related self-efficacy is the belief individuals have that they can perform the necessary actions to cope effectively with their seizures. Determining, developing, strengthening, and maintaining the perception of self-efficacy in children with epilepsy facilitates the child's disease management and their ability to cope with it. This study aimed to assess the impact of epilepsy-related parental fears during the COVID-19 period on the seizure self-efficacy of their children. METHODS: A total of 321 children with epilepsy and their parents participated in this descriptive, correlational, and cross-sectional study. Data were collected through the Descriptive Information Form, the Seizure Self-Efficacy Scale for Children (SSES-C), and the Epilepsy-Related Fears in Parents Questionnaire (EFPQ). Descriptive statistics, including frequency, percentage, and mean scores, were used to analyze the characteristics of the children and their parents. The Shapiro-Wilk test was utilized to assess the normality of the scale data. Pearson correlation analysis examined the relationship between parents' epilepsy-related fears and their children's seizure self-efficacy, while multiple regression analysis determined the effect of parental fears on children's seizure self-efficacy. RESULTS: The mean age of children included in the study was 12.65±2.37 years. Analysis revealed a strong and significant negative correlation between parents' epilepsy-related fears during the COVID-19 period and the seizure self-efficacy of their children. In the model created with regression analysis, The mean scores of parents on the short-term fears of parents about epilepsy of the EFPQ explained 85 % of children's seizure self-efficacy. The mean scores of parents on the long-term fears of parents about epilepsy of the EFPQ explained 85 % of children's seizure self-efficacy. It was determined that all of these variables together explained 85 % of the seizure self-efficacy of children with epilepsy. CONCLUSION: The findings of the study underscore the importance of addressing parents' fears regarding epilepsy, emphasizing the need for healthcare professionals to be aware of and provide support for these concerns. Future studies should focus on interventions to enhance the seizure self-efficacy of children with epilepsy.
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COVID-19 , Epilepsia , Miedo , Padres , Autoeficacia , Humanos , COVID-19/psicología , Estudios Transversales , Masculino , Epilepsia/psicología , Femenino , Miedo/psicología , Niño , Padres/psicología , Encuestas y Cuestionarios , Adolescente , Convulsiones/psicología , Adulto , Adaptación Psicológica , SARS-CoV-2 , PreescolarRESUMEN
OBJECTIVE: Advanced glycation end products (AGEs) are irreversible macromolecules formed by nonenzymatic reactions due to chronic hyperglycemia. The aim of this study was to assess the relationship between AGEs and the microvascular complications of children and adolescents with type 1 diabetes mellitus (T1DM). MATERIALS AND METHODS: Twenty-six T1DM patients with microvascular complications and 58 complication-naive patients who were similar regarding age, sex, and pubertal status enrolled in the study. Anthropometric, biochemical, ophthalmologic, and neurologic variables were compared with serum AGEs levels by the fluorescence method. RESULTS: There was no significant difference observed between the patients with complications and those without complications in terms of serum levels of AGEs and other biochemical parameters. However, the duration of T1DM and urine microalbumin-creatinine ratio (uACR) were significantly higher in the complication-positive group (P < .001). Serum levels of AGEs were found to be similar when retinopathy, peripheral, and optic neuropathy were separately compared with the complication-naive group (P > .05). However, patients with nephropathy had significantly higher serum levels of AGEs than patients without complications (P = .023). In addition, there was a significant positive correlation between serum AGEs levels and uACR (P = .042) but not other parameters (P > .05). CONCLUSION: This study is the first to evaluate the association between serum AGEs levels and microvascular complications in children and adolescents with T1DM. Our study highlights that serum AGEs levels are significantly correlated with nephropathy but not with retinopathy and neuropathy. Further long-term studies with a larger sample size are required to establish a better relationship between diabetic complications and AGEs. Cite this article as: Kirkgöz T, Acar S, Küme T, et al. Evaluation of serum advanced glycation end product levels and microvascular complications in children and adolescents with type 1 diabetes mellitus. Turk Arch Pediatr. 2024;59(1):31-37.
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BACKGROUND & OBJECTIVES: It has been hypothesized that abnormal levels of serum nerve growth factor (NGF) may represent a serological marker for autistic children who may develop cognitive impairment, regression and finally epilepsy. The objective of this preliminary study was to measure serum NGF concentrations of autistic children and compare these levels with those of healthy children. METHODS: Consecutive children who were referred to the Paediatric Neurology and Child Psychiatry Policlinics of Dr. Behçet Uz Child Disease and Pediatric Surgery Training and Research Hospital, Turkey between February and September 2008 were included in the study. Serum samples were analyzed for NGF levels using ChemiKine NGF Sandwich ELISA Kit. Comparisons between the study and the control groups were made using student's t test and Chi-square test. RESULTS: Forty-nine autistic children and an equal number of healthy children (control group) were included in the study. No significant difference was found between the study and the control groups in terms of children's age, while number of boys was significantly higher (P<0.05) in the study group. Average serum NGF concentrations were 46.94 ± 51.40 and 32.94 ± 12.48 pg/ml in the study and control group, respectively. Serum NGF concentrations were significantly higher (P<0.05) in the study group compared with the control group. INTERPRETATION & CONCLUSIONS: Our preliminary findings show that enhanced serum NGF concentration may be used as a potential diagnostic tool in autism, however, further studies including a large number of patients are required to confirm the findings.
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Trastorno Autístico/sangre , Factor de Crecimiento Nervioso/sangre , Trastorno Autístico/patología , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Población , TurquíaRESUMEN
PURPOSE: To compare electroencephalography (EEG) features of newly diagnosed drug-naive childhood absence epilepsy (CAE) and juvenile absence epilepsy (JAE) patients and analyze their response to anti-seizure medications (ASMs). METHOD: EEG characteristics between CAE and JAE patients and responders and non-responders to ASM at baseline and 12 months were compared, and the changes from baseline were analysed. RESULTS: A total of 62 patients (32 CAE and 30 JAE) were included. Discharges in baseline awake and sleep EEGs and interictal and polyspike discharges in baseline sleep EEGs were more frequent in JAE patients. Although the median discharge densities (discharge containing seconds per minute) were similar in baseline awake and sleep EEGs between the groups, the median was higher in the JAE group at 12 months and decreased significantly in both groups at 12 months compared to the baseline values. Responses to initial ASMs were 94% and 77% in the CAE and JAE groups, respectively. In initial sleep EEGs of non-responders with JAE, focal onset generalized spike and slow wave discharges (GSWDs) were more frequent, and the median ictal and interictal discharge densities were higher. CONCLUSION: JAE patients had more frequent disorganized discharges at baseline in both awake and sleep EEGs and interictal and polyspike discharges in sleep EEGs than those of CAE patients. Improvement in EEG was more pronounced in CAE patients than in JAE patients. Focal-onset GSWDs and higher ictal and interictal discharge densities on baseline EEG were associated with a poor response to initial ASMs in JAE patients.
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Epilepsia Tipo Ausencia , Humanos , Niño , Epilepsia Tipo Ausencia/diagnóstico , Epilepsia Tipo Ausencia/tratamiento farmacológico , Vigilia , Electroencefalografía , SueñoRESUMEN
Aycan Ünalp, Yigithan Güzin, Bülent Ünay, Ayse Tosun, Dilek Çavusoglu, Hande Gazeteci Tekin, Semra Hiz Kurul, Ebru Arhan, Selvinaz Edizer, Gülten Öztürk, Uluç Yis, Ünsal Yilmaz, Turkish Rare Epilepsies Study Group, Clinical and genetic evaluations of rare childhood epilepsies in Turkey's national cohort, Epileptic Disorders, 2023, (https://doi.org/10.1002/epd2.20150) The above article, published online on 16 August 2023 on Wiley Online Library (www.onlinelibrary.wiley.com), has been retracted by agreement between the authors, the Editor-in-Chief, Sándor Beniczky, and John Wiley & Sons Ltd. The authors asked for a retraction based on an experimental error which would alter the results of the study if corrected.