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1.
Clin Endocrinol (Oxf) ; 98(3): 332-341, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36536479

RESUMO

OBJECTIVE: Prader-Willi Syndrome (PWS) is the most common genetic cause of obesity. Prevention and management of obesity, which represents the main cause of morbidity and mortality in these patients, is essential. Ketogenic diet (KD) is used in the treatment of various disorders, however knowledge of its effect in PWS is lacking. The present study assesses the characteristics of patients with PWS who were on KD. DESIGN AND PATIENTS: This is a retrospective, cross-sectional descriptive study investigating the subjects with PWS, who had received KD for at least 6 months. RESULTS: Ten patients with PWS [median age 52.5 (47-77) months] complied with KD. The median treatment period was 16.5 [11-52] months. Of the daily calorie, 75%-85% were from fat, and 15%-25% from protein + carbohydrate. The baseline body weight standard deviation (SD) score before diet therapy was 2.10 [-1.11-4.11], whereas it was 0.05 [-0.92-1.2] at final evaluation (p = .007). The baseline median BMI SD score before diet therapy was 3.05 [-0.21-3.72], whereas it was 0.41 [-0.87-1.57] at final evaluation (p = .002). The height SD score remained unchanged. Mild hypercholesterolaemia was the most common biochemical abnormality during treatment with KD. CONCLUSION: Our results indicate that KD might have a favourable effect on weight management in PWS.


Assuntos
Dieta Cetogênica , Síndrome de Prader-Willi , Humanos , Criança , Pessoa de Meia-Idade , Síndrome de Prader-Willi/metabolismo , Estudos Retrospectivos , Estudos Transversais , Obesidade/metabolismo
2.
Eur J Paediatr Neurol ; 23(1): 143-151, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30497921

RESUMO

PURPOSE: Ketogenic diet (KD) is an effective non-pharmacological treatment for drug-resistant epilepsy. The aim of this study was to investigate the efficacy, tolerability and complications of olive oil-based KD in epileptic children. METHOD: In this single-center, prospective study, patients were followed up at 1, 3, 6 and 12 months after KD initiation. Initially, blood ketone levels were measured daily, and as needed thereafter to maintain the levels between 4 and 5 mmol/L. Patient demographics, seizure frequency, serum biochemistry, abdominal ultrasonography and adverse effects were recorded. Efficacy of KD was defined as ≥50% seizure reduction. RESULTS: A total of 389 patients with drug-resistant epilepsy receiving KD from 2012 to 2016 were included. One hundred patients (25.7%) stopped the diet for different reasons in the first year, and 369, 314, 225 and 160 patients have been receiving KD treatment for 1, 3, 6 and 12 months, respectively. At 1, 3, 6 and 12th months, 65.8% (243/369), 74.7% (235/314), 70.6% (159/225) and 83.1% (133/160) of the patients were responders, respectively. None of the children had an increased seizure-frequency. Hyperlipidemia (50.8%), selenium deficiency (26.9%), constipation (26.2%), sleep disturbances (20.0%), nephrolithiasis (3.0%), hyperuricemia (3.0) and hepatic side effects (2.6%) were the most common complications of KD. Previous adrenocorticotropic hormone (ACTH) use due to epileptic encephalopathy and presence of constipation at baseline or during KD treatment were found the predictors of treatment efficacy. CONCLUSION: KD is an effective and well-tolerated treatment option for patients with drug-resistant epilepsy. Previous history of ACTH use and constipation during KD treatment are important factors that affect the efficacy of KD treatment.


Assuntos
Dieta Cetogênica/efeitos adversos , Dieta Cetogênica/métodos , Epilepsia Resistente a Medicamentos/dietoterapia , Azeite de Oliva/uso terapêutico , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Estudos Prospectivos , Resultado do Tratamento , Turquia
3.
J Child Neurol ; 33(11): 718-723, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30062917

RESUMO

AIMS: To investigate the effect of ketogenic diet on motor function and daily living activities in children with epilepsy. METHODS: A total of 62 children (median age 5.0 years) were enrolled. Motor function was assessed using the Gross Motor Function Measure (GMFM), and daily living activities and cognitive functions were assessed using the Functional Independence Measure (WeeFIM) before treatment and 3, 6, and 12 months after ketogenic diet treatment. RESULTS: Significant improvement in total GMFM and WeeFIM scores ( P < .001) were found during the 12 months of ketogenic diet treatment. There was a positive correlation between total GMFM scores and WeeFIM scores at baseline (r= 0.792, P = .0001), and at 3 (r= 0.780, P = .0001), 6 (r= 0.744, P = .0001), and 12 months (r= 0.692, P = .0001) of treatment. Both the responder (50 patients, 80.7%) and nonresponder (12 patients, 19.3%) patient groups showed significantly higher GMFM and WeeFIM scores at 12 months of treatment compared to baseline values. A ≥50% reduction in seizure frequency was observed in 77.4%, 72.6%, and 80.7% of the patients after 3, 6, and 12 months of treatment, respectively. CONCLUSION: Ketogenic diet treatment improves motor functions and daily living activities in children with epilepsy during the 12 months of treatment.


Assuntos
Atividades Cotidianas , Dieta Cetogênica/métodos , Epilepsia Resistente a Medicamentos , Transtornos dos Movimentos/dietoterapia , Transtornos dos Movimentos/etiologia , Criança , Pré-Escolar , Epilepsia Resistente a Medicamentos/complicações , Epilepsia Resistente a Medicamentos/dietoterapia , Epilepsia Resistente a Medicamentos/psicologia , Feminino , Humanos , Masculino , Estudos Prospectivos , Estatísticas não Paramétricas , Inquéritos e Questionários , Fatores de Tempo , Resultado do Tratamento
4.
Epilepsia Open ; 3(2): 175-192, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29881797

RESUMO

Ketogenic dietary therapies (KDTs) are established, effective nonpharmacologic treatments for intractable childhood epilepsy. For many years KDTs were implemented differently throughout the world due to lack of consistent protocols. In 2009, an expert consensus guideline for the management of children on KDT was published, focusing on topics of patient selection, pre-KDT counseling and evaluation, diet choice and attributes, implementation, supplementation, follow-up, side events, and KDT discontinuation. It has been helpful in outlining a state-of-the-art protocol, standardizing KDT for multicenter clinical trials, and identifying areas of controversy and uncertainty for future research. Now one decade later, the organizers and authors of this guideline present a revised version with additional authors, in order to include recent research, especially regarding other dietary treatments, clarifying indications for use, side effects during initiation and ongoing use, value of supplements, and methods of KDT discontinuation. In addition, authors completed a survey of their institution's practices, which was compared to responses from the original consensus survey, to show trends in management over the last 10 years.

5.
Neurol Sci ; 39(1): 85-89, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29038947

RESUMO

Benefits of the ketogenic diet (KD) in epileptic patients are well known while less is known about the nutritional risks of the diet and its potential impacts on biochemical nutritional status. In this study, we aimed to evaluate the hematological parameters of patients who have drug-resistant epilepsy and are treated with KD. Fifty-three patients with drug-resistant epilepsy (mean age 7.4 ± 4.4 years [2-18], 23 [43.4%] female) were included in the study. Demographic and laboratory data of the patients were retrospectively analyzed at baseline and Month 6 and Month 12 of the treatment. Repeated measures ANOVA (post hoc Bonferroni correction) and Friedman test were used to assess the changes in data during the treatment. Mean hemoglobin levels increased by 0.594 g/dL after 6 months (p = 0.001) and by 0.602 g/dL after 12 months of the treatment (p = 0.002). Mean hematocrit level was found to be significantly increased at Month 6 and 12 of the treatment compared to baseline [F(2,94) = 8.9, p < 0.0001]. An increase in MCV levels was determined with the KD treatment [F(2,94) = 19.7, p < 0.0001]. Mean level of vitamin B12 was found to be significantly increased in Month 12 of treatments compared to Month 6 [F(1.686,72.479) = 3.472, p = 0.035]. There was no significant effect of KD on other hematological parameters (red blood cell, white blood cell and platelet counts, serum iron, total iron-binding capacity, transferrin saturation, and ferritin and folic acid levels). We can conclude that KD increases levels of hemoglobin, hematocrit, MCV, and serum vitamin B12 in patients with intractable epilepsy. Prospective, multi-center, longitudinal studies are needed to confirm our results.


Assuntos
Dieta Cetogênica , Epilepsia Resistente a Medicamentos/sangue , Epilepsia Resistente a Medicamentos/dietoterapia , Adolescente , Análise Química do Sangue , Criança , Pré-Escolar , Feminino , Testes Hematológicos , Humanos , Masculino , Estudos Retrospectivos
6.
Turk J Med Sci ; 47(3): 942-946, 2017 Jun 12.
Artigo em Inglês | MEDLINE | ID: mdl-28618748

RESUMO

BACKGROUND/AIM: Diabetes mellitus (DM) is one of the most common metabolic diseases seen in the world today. Diabetic neuropathy (DN) is a chronic complication of the disease that is rarely reported in children, since it has a relatively longer latency period. Our main objective in this study is to determine the incidence rate of DN in pediatric DM patients and assess the risk factors associated with DN. MATERIALS AND METHODS: Data from 111 patients from January 2011 to May 2014 were reviewed in a retrospective manner. Nerve conduction studies were performed as the gold standard in diagnosis. RESULTS: The incidence rate of symptomatic DN was 13.5% according to our study results. The EMG-diagnosed DN incidence rate was calculated as 22.5%. Following linear regression analysis, positive correlation was found between diabetes duration, diabetic ketoacidosis, and DN presence. CONCLUSION: Our study results demonstrate the fact that poor metabolic control, especially during early stages of the disease, is a major risk factor for neuropathy development. Planning prospective studies with long-term evaluations on nerve conduction in children with DM will be beneficial for this subject.


Assuntos
Diabetes Mellitus Tipo 1/epidemiologia , Neuropatias Diabéticas/epidemiologia , Adolescente , Criança , Pré-Escolar , Diabetes Mellitus Tipo 1/complicações , Neuropatias Diabéticas/diagnóstico , Neuropatias Diabéticas/etiologia , Neuropatias Diabéticas/fisiopatologia , Eletromiografia , Feminino , Hemoglobinas Glicadas/análise , Humanos , Masculino , Estudos Retrospectivos , Fatores de Risco
7.
J Pediatr Endocrinol Metab ; 30(4): 411-416, 2017 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-28076316

RESUMO

BACKGROUND: Ketogenic diet (KD), which is high in fat and low in carbohydrates, mimics the metabolic state of starvation and is used therapeutically for pharmacoresistant epilepsy. It is known that generation of triiodothyronine (T3) from thyroxine (T4) decreases during fasting periods. The aim of this study was to evaluate the thyroid function of children receiving KD for at least 1 year due to drug-resistant epilepsy. METHODS: A total of 120 patients [63 males, 52.5%; mean age 7.3±4.3 years, median interquartile range (IQR): 7.0 (4-10 years)] treated with KD for at least 1 year were enrolled. Seizure control, side effects, and compliance with the diet were recorded, and free T3, free T4, and thyroid-stimulating hormone (TSH) levels were measured at baseline and at post-treatment months 1, 3, 6, and 12. The Mann-Whitney U-test, repeated measures analysis of variance (ANOVA) with post-hoc Bonferroni correction, and logistic regression analysis were used for data analysis. RESULTS: Hypothyroidism was diagnosed and L-thyroxine medication was initiated for eight, seven and five patients (20 patients in total, 16.7%) at 1, 3, and 6 months of KD therapy, respectively. Logistic regression analysis showed that baseline TSH elevation [odds ratio (OR): 26.91, 95% confidence interval (CI) 6.48-111.76, p<0.001] and female gender (OR: 3.69, 95% CI 1.05-12.97, p=0.042) were independent risk factors for development of hypothyroidism during KD treatment in epileptic children. CONCLUSIONS: KD causes thyroid malfunction and L-thyroxine treatment may be required. This is the first report documenting the effect of KD treatment on thyroid function. Thyroid function should be monitored regularly in epileptic patients treated with KD.


Assuntos
Biomarcadores/sangue , Dieta Cetogênica/efeitos adversos , Epilepsia Resistente a Medicamentos/dietoterapia , Hipotireoidismo/sangue , Tireotropina/sangue , Tiroxina/sangue , Tri-Iodotironina/sangue , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Hipotireoidismo/diagnóstico , Hipotireoidismo/etiologia , Lactente , Masculino , Prognóstico
8.
Biol Trace Elem Res ; 178(1): 1-6, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27873289

RESUMO

The aim of the present study was to evaluate serum selenium levels in children receiving olive oil-based ketogenic diet (KD) for intractable seizures for at least 1 year. Out of 320 patients who were initiated on KD, patients who continued receiving KD for at least 12 months were enrolled. Sixteen patients who had selenium deficiency at the time of starting KD were excluded. Finally, a total of 110 patients (mean age 7.3 ± 4.2 years) were included. Serum selenium levels were measured at baseline and at 3, 6, and 12 months after treatment initiation by using atomic absorption spectroscopy. Selenium deficiency was defined as a serum selenium level <48 µg/L at each visit. Repeated measure ANOVA with post hoc Bonferroni correction was used for data analysis. Mean duration of KD was 15.3 ± 4.3 months. Mean serum selenium levels were significantly lower at 6 and 12 months of KD treatment (66.2 ± 23.3 and 57.2 ± 16.2 µg/L, respectively) compared to pre-treatment levels (79.3 ± 25.7 µg/L) (p = 0.001). On the other hand, selenium levels did not show any significant difference at 3 months of KD treatment (70.0 ± 21.2 µg/L) compared to baseline levels (p = 0.076). A total of 54 patients (49.1%) were diagnosed with selenium deficiency, and oral selenium medication was initiated for these patients. No relevant clinical findings were detected, and echocardiographic findings were normal in all patients. The decline of the serum selenium concentrations after 6 and 12 months of ketogenic diet suggests that patients on this highly prescriptive dietary treatment need close monitoring of this trace element.


Assuntos
Dieta Cetogênica , Epilepsia Resistente a Medicamentos/sangue , Epilepsia Resistente a Medicamentos/dietoterapia , Selênio/sangue , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Selênio/administração & dosagem , Selênio/deficiência
9.
Seizure ; 43: 32-38, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27866088

RESUMO

PURPOSE: Long-term ketogenic diet (KD) treatment has been shown to induce liver steatosis and gallstone formation in some in vivo and clinical studies. The aim of this retrospective study was to evaluate the hepatic side effects of KD in epileptic children. METHOD: A total of 141 patients (mean age: 7.1±4.1years [2-18 years], 45.4% girls), receiving KD at least one year for intractable epilepsy due to different diagnoses (congenital brain defects, GLUT-1 deficiency, West syndrome, tuberous sclerosis, hypoxic brain injury, etc.) were included in the study. Serum triglyceride, cholesterol, aminotransferase, bilirubin, protein and albumin levels and abdominal ultrasonography were recorded before and at 1, 3, 6, and 12 months following after diet initiation. RESULTS: The mean duration of KD was 15.9±4.3months. At one month of therapy, three patients had elevated alanine and aspartate aminotransferase levels. These patients were receiving ketogenic diet for Doose syndrome, idiopathic epilepsy and GLUT-1 deficiency. Hepatosteatosis was detected in three patients at 6 months of treatment. Two of these patients were treated with KD for the primary diagnosis of tuberous sclerosis and one for Landau Kleffner syndrome. Cholelithiasis was detected in two patients at 12 months of treatment. They were receiving treatment for West syndrome and hypoxic brain injury sequelae. CONCLUSION: Long-term ketogenic diet treatment stimulates liver parenchymal injury, hepatic steatosis and gallstone formation. Patients should be monitored by screening liver enzymes and abdominal ultrasonography in order to detect these side effects.


Assuntos
Dieta Cetogênica/métodos , Hepatopatias/dietoterapia , Hepatopatias/etiologia , Abdome/diagnóstico por imagem , Adolescente , Antropometria , Bilirrubina/sangue , Criança , Pré-Escolar , Colesterol/sangue , Epilepsia Resistente a Medicamentos/sangue , Epilepsia Resistente a Medicamentos/complicações , Epilepsia Resistente a Medicamentos/dietoterapia , Epilepsia Resistente a Medicamentos/etiologia , Feminino , Humanos , Hepatopatias/diagnóstico por imagem , Estudos Longitudinais , Masculino , Estudos Retrospectivos , Fatores de Tempo , Transaminases/sangue , Triglicerídeos/sangue , Ultrassonografia
10.
Neuropediatrics ; 47(3): 157-61, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27043293

RESUMO

Background The association between ketogenic diet (KD) and prolonged QT interval, life-threatening ventricular arrhythmias, and sudden death is controversial. Aim We aimed to prospectively evaluate the effect of KD on electrocardiography (ECG) measures in children with refractory epilepsy. Method A total of 70 children with drug-resistant epilepsy who received a KD for at least 12 months were included in the study. The standard 12-lead electrocardiography was performed in all patients before the beginning and in the 12th month of KD. Heart rate, P-wave duration and dispersion, corrected QT interval and QT dispersion, and Tp-e interval were measured. Results All ECG-derived parameters, but P-wave dispersion increased after 12 months of KD compared with the baseline values. However, these changes were not statistically significant. Conclusion A 12-month long 3:1 KD treatment exerts no deleterious effect on cardiac repolarization measures.


Assuntos
Arritmias Cardíacas/induzido quimicamente , Dieta Cetogênica/efeitos adversos , Epilepsia Resistente a Medicamentos/dietoterapia , Adolescente , Erros Inatos do Metabolismo dos Carboidratos/complicações , Criança , Pré-Escolar , Epilepsia Resistente a Medicamentos/etiologia , Eletrocardiografia , Epilepsias Mioclônicas/dietoterapia , Feminino , Seguimentos , Frequência Cardíaca , Humanos , Hipóxia-Isquemia Encefálica/complicações , Lactente , Síndrome de Landau-Kleffner/complicações , Síndrome de Lennox-Gastaut/dietoterapia , Masculino , Malformações do Desenvolvimento Cortical/complicações , Proteínas de Transporte de Monossacarídeos/deficiência , Estudos Prospectivos , Espasmos Infantis/dietoterapia , Estado Epiléptico/dietoterapia , Esclerose Tuberosa/complicações , Adulto Jovem
11.
Brain Dev ; 38(9): 842-7, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27066714

RESUMO

OBJECTIVE: The ketogenic diet (KD) has been referred to as an "effective therapy with side effects" for children with intractable epilepsy. Among the most recognized adverse effects, there are cardiac conduction abnormalities, vascular and myocardial dysfunction. However, very limited and controversial data are available regarding the effects of the KD on cardiac functions. We sought to analyze the mid-term effect of ketogenic diet on cardiac functions in patients with intractable epilepsy who received a ketogenic diet for at least 12months using conventional and relatively new imaging techniques. METHODS: This prospective study included 61 patients with intractable epilepsy who received ketogenic diet for at least 12months. Clinical examinations, serum carnitine and selenium levels as well as electrocardiographic and echocardiographic examinations were scheduled prior to the procedure and at 1, 3, 6 and 12months. We utilized two-dimensional, M-mode, colored Doppler, spectral Doppler and pulsed wave tissue Doppler imaging techniques to investigate ventricular systolic and diastolic functions of this subgroup of patients. RESULTS: In our study, there was no significant difference after 1year of KD therapy compared to baseline values-except a significantly decreased A wave velocity-in terms of pulse wave Doppler echocardiographic measurements of the diastolic function. The tissue Doppler measurements obtained from the lateral wall of tricuspide and mitral annuli were not different at baseline and at month 12 of the treatment, as well. CONCLUSION: The ketogenic diet appears to have no disturbing effect on ventricular functions in epileptic children in the midterm.


Assuntos
Dieta Cetogênica , Epilepsia Resistente a Medicamentos/dietoterapia , Epilepsia Resistente a Medicamentos/fisiopatologia , Coração/fisiopatologia , Pré-Escolar , Dieta Cetogênica/efeitos adversos , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Ecocardiografia , Eletrocardiografia , Feminino , Seguimentos , Coração/diagnóstico por imagem , Humanos , Cetonas/sangue , Masculino , Estudos Prospectivos
12.
J Child Neurol ; 31(9): 1120-6, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-27080042

RESUMO

Because of the lack of studies comparing the efficacy and safety of levetiracetam and valproate before the induction of general anesthesia in the treatment of convulsive refractory status epilepticus in children, we aimed to compare the effectiveness of these antiepileptic drugs in patients with convulsive status epilepticus admitted to the Pediatric Intensive Care Unit between 2011 and 2014. Forty-six (59%) of the 78 patients received levetiracetam, and 32 (41%) received valproate for the treatment of refractory status epilepticus. The response rate was not significantly different between the 2 groups. Although no adverse event was noted in patients who received levetiracetam, 4 (12.5%) patients in the valproate group experienced liver dysfunction (P = .025). According to our results, levetiracetam and valproate may be used in the treatment of refractory status epilepticus before the induction of general anesthesia. Levetiracetam appears as effective as valproate, and also safer.


Assuntos
Anticonvulsivantes/administração & dosagem , Piracetam/análogos & derivados , Estado Epiléptico/tratamento farmacológico , Ácido Valproico/administração & dosagem , Administração Intravenosa , Adolescente , Anticonvulsivantes/efeitos adversos , Criança , Pré-Escolar , Protocolos Clínicos , Cuidados Críticos , Feminino , Humanos , Lactente , Unidades de Terapia Intensiva Pediátrica , Levetiracetam , Masculino , Segurança do Paciente , Piracetam/administração & dosagem , Piracetam/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento , Ácido Valproico/efeitos adversos
13.
Pediatr Neurol ; 56: 30-34, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26774551

RESUMO

OBJECTIVE: We aimed to determine the midterm effect of a ketogenic diet on serum lipid levels, carotid intima-media thickness, and the elastic properties of the carotid artery and the aorta in patients with intractable epilepsy. METHODS: A total of 52 children aged between 12 months and 18 years with intractable epilepsy who started the ketogenic diet from September 2014 to September 2015 were included into this prospective study. Carotid intima-media thickness and the elastic properties of the carotid artery and the aorta were assessed by echocardiography in all cases before beginning of the ketogenic diet and after at least 12 months on the ketogenic diet. RESULTS: Twenty-one patients at the third month and 25 patients at the first year of the ketogenic diet were seizure free. A reduction of greater than 90% in the seizure frequency was achieved in three patients at the sixth month and in five patients at the first year of the treatment. The serum levels of total cholesterol, low-density lipoprotein, and triglyceride were increased significantly at a median of 12.6 months (range: 12 to 13.5 months) of the ketogenic diet treatment, whereas serum levels of high-density lipoprotein did not change. Carotid intima-media thickness, aortic and carotid strain, the stiffness index, distensibility, and elastic modulus did not change after 12 months of the ketogenic diet therapy. CONCLUSION: Olive oil-based ketogenic diet appears to have no disturbing effect on the carotid intima-media thickness and the elastic properties of the aorta and the carotid artery in epileptic children, although it may be associated with increased concentrations of serum lipids.


Assuntos
Artérias Carótidas/patologia , Espessura Intima-Media Carotídea , Dieta Cetogênica/efeitos adversos , Epilepsia Resistente a Medicamentos/dietoterapia , Adolescente , Anticonvulsivantes/uso terapêutico , Pressão Sanguínea/fisiologia , Criança , Pré-Escolar , Epilepsia Resistente a Medicamentos/sangue , Feminino , Seguimentos , Humanos , Lactente , Lipídeos/sangue , Masculino , Estudos Retrospectivos , Estatísticas não Paramétricas , Adulto Jovem
14.
Neurol Sci ; 37(3): 465-70, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26700799

RESUMO

Ketogenic diet (KD) is one of the most effective therapies for intractable epilepsy. Olive oil is rich in monounsaturated fatty acids and antioxidant molecules and has some beneficial effects on lipid profile, inflammation and oxidant status. The aim of this study was to evaluate the serum lipid levels of children who were receiving olive oil-based KD for intractable seizures at least 1 year. 121 patients (mean age 7.45 ± 4.21 years, 57 girls) were enrolled. At baseline and post-treatment 1, 3, 6, and 12 months body mass index-SDS, total cholesterol, high-density lipoprotein (HDL) cholesterol, low-density lipoprotein (LDL) cholesterol and triglyceride levels were measured. Repeated measure ANOVA with post hoc Bonferroni correction was used for data analysis. The mean duration of KD was 15.4 ± 4.1 months. Mean total cholesterol, LDL-cholesterol and triglyceride levels were significantly higher at 1st, 3rd, 6th and 12th months of the KD treatment, compared to pre-treatment levels (p = 0.001), but showed no difference among during-treatment measurements. Mean body mass index-SDS and HDL-cholesterol levels were not different among the baseline and follow-up time points (p = 0.113 and p = 0.067, respectively). No child in this study discontinued the KD because of dyslipidemia. Even if rich in olive oil, high-fat KD causes significant increase in LDL-cholesterol and triglyceride levels. More studies are needed to determine the effect of KD on serum lipids in children using different fat sources in the diet.


Assuntos
Colesterol/sangue , Dieta Cetogênica/métodos , Azeite de Oliva/administração & dosagem , Convulsões/sangue , Convulsões/dietoterapia , Triglicerídeos/sangue , Adolescente , Índice de Massa Corporal , Criança , Pré-Escolar , Dieta Hiperlipídica/efeitos adversos , Dieta Hiperlipídica/métodos , Dieta Cetogênica/efeitos adversos , Feminino , Seguimentos , Humanos , Hiperlipidemias/sangue , Hiperlipidemias/dietoterapia , Masculino , Azeite de Oliva/efeitos adversos , Fatores de Tempo , Resultado do Tratamento
15.
Pediatr Neurol ; 53(3): 233-237.e1, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26302701

RESUMO

OBJECTIVE: Our primary aim was to determine the short-term effects of a ketogenic diet on cardiac ventricular function in patients with refractory epilepsy. METHODS: Thirty-eight drug-resistant epileptic patients who were treated with a ketogenic diet were enrolled in this prospective study. Echocardiography was performed on all patients before beginning the ketogenic diet and after the sixth month of therapy. Two-dimensional, M-mode, color flow, spectral Doppler, and pulsed-wave tissue Doppler imaging measurements were performed on all patients. RESULTS: The median age of the 32 patients was 45.5 months, and 22 (57.8%) of them were male. Body weight, height, and body mass index increased significantly at the sixth month of therapy when compared with baseline values (P < 0.05). Baseline variables assessed by conventional M-mode echocardiography showed no significant difference at month 6 (P > 0.05). Doppler flow indices of mitral annulus and tricuspid annulus velocity of patients at baseline and month 6 showed no significant differences (P > 0.05). Tricuspid annular E/A ratio was lower at month 6 (P < 0.05). Although mitral annulus tissue Doppler imaging studies showed no significant difference (P > 0.05), there was a decrease in Ea velocity and Ea/Aa ratio gathered from tricuspid annulus at month 6 compared with baseline (P < 0.05). CONCLUSION: A 6-month duration ketogenic diet does not impair left ventricular functions in children with refractory epilepsy; however, it may be associated with a right ventricular diastolic dysfunction.


Assuntos
Dieta Cetogênica , Epilepsia Resistente a Medicamentos/dietoterapia , Epilepsia Resistente a Medicamentos/fisiopatologia , Função Ventricular/fisiologia , Pré-Escolar , Eletrocardiografia , Feminino , Humanos , Masculino , Estudos Prospectivos
16.
J Child Neurol ; 30(12): 1646-50, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25855687

RESUMO

The aim of this prospective study is to investigate the effect of a 6-month-long ketogenic diet on carotid intima-media thickness, carotid artery, and aortic vascular functions. Thirty-eight drug-resistant epileptic patients who were being treated with ketogenic diet were enrolled. Fasting total cholesterol, high-density lipoprotein, low-density lipoprotein, triglycerides, total cholesterol, and glucose concentrations were measured and echocardiography was performed in all patients before the beginning of ketogenic diet and at the sixth month of treatment. The body weight, height, body mass index, serum levels of triglyceride, total cholesterol, and low-density lipoprotein increased significantly at month 6 when compared to baseline values (P < .05). Carotid intima-media thickness, elastic properties of the aorta, and carotid artery did not change at the sixth month of therapy compared to baseline values. A 6-month-long ketogenic diet has no effect on carotid intima-media thickness and elastic properties of the carotid artery and the aorta.


Assuntos
Aorta/fisiopatologia , Artérias Carótidas/fisiopatologia , Espessura Intima-Media Carotídea , Dieta Cetogênica , Epilepsia Resistente a Medicamentos/dietoterapia , Epilepsia Resistente a Medicamentos/fisiopatologia , Fatores Etários , Aorta/diagnóstico por imagem , Análise Química do Sangue , Pressão Sanguínea , Estatura , Índice de Massa Corporal , Peso Corporal , Artérias Carótidas/diagnóstico por imagem , Pré-Escolar , Dieta Cetogênica/efeitos adversos , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Ecocardiografia , Elasticidade , Feminino , Seguimentos , Humanos , Masculino , Estudos Prospectivos , Fatores de Tempo , Resultado do Tratamento
17.
Pediatr Neurol ; 51(5): 688-95, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25172096

RESUMO

BACKGROUND: Levetiracetam has been proven to be effective in both partial and generalized seizures in children. However, few studies have reported its efficacy in the treatment of acute repetitive seizures. We aimed to investigate the efficacy and safety of levetiracetam in children with acute repetitive seizures. METHODS: The medical records of children from the age of 1 month-18 years who received levetiracetam because of acute repetitive seizures in the pediatric intensive care unit between 2010 and 2013 were reviewed retrospectively. RESULTS: Of the 133 patients, levetiracetam terminated seizures in 104 (78.2%). Side effects such as agitation and aggression were observed in three patients (2.2%). The likelihood of treatment failure was increased by four times by younger age at seizure onset; by six times in the individuals with neurological abnormalities; and by 22 times in the patients with West syndrome. The patients who used levetiracetam as the first treatment option for acute repetitive seizures had a longer duration of epilepsy, a higher rate of neurological abnormality, and a higher proportion of medically resistant epilepsy compared with the individuals who used levetiracetam as an add-on treatment to the other intravenous antiepileptic drugs. However, no differences were detected between these two groups in terms of treatment response. CONCLUSIONS: Intravenous levetiracetam appears to be effective and safe in the treatment of acute repetitive seizures. Randomized clinical trials are needed to determine whether intravenous levetiracetam may replace other antiepileptic drugs as the first-line therapy in the management of acute repetitive seizures.


Assuntos
Anticonvulsivantes/administração & dosagem , Piracetam/análogos & derivados , Convulsões/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Eletroencefalografia , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Injeções Intravenosas , Levetiracetam , Imageamento por Ressonância Magnética , Masculino , Piracetam/administração & dosagem , Curva ROC , Estudos Retrospectivos , Convulsões/classificação , Estatísticas não Paramétricas
18.
Turk Patoloji Derg ; 30(2): 111-7, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24638197

RESUMO

OBJECTIVE: In this study, it was aimed to describe the clinical, histopathological and genetic features of 20 patients with gamma sarcoglycanopathy confirmed by muscle biopsies and genetic analysis. MATERIAL AND METHOD: We retrospectively reviewed 20 patients from whom muscle biopsy specimens were obtained between 2007 and 2012. All patients were clinically diagnosed as muscular dystrophy and biopsy materials were collected from five different centers of neurological disorders. All DNAs were extracted from muscle tissues or blood samples of patients and genetic tests (mutation analyses for gamma sarcoglycan gene and deletion-duplication analyses for all 4 sarcoglycan genes) were performed. RESULTS: The mean age of the patients was 7.6 years (2 -21 years). Only one case (5%) was older than 14 years. The mean CPK level was 10311 U/L (1311 - 35000 U∕L). There were 4 siblings in these series. Expression defects of gamma sarcoglycan staining were determined in (15 males, and 5 females) all patients with muscle biopsy specimens. But only in 9 of them, disease-causing defects could be determined with genetic analyses. CONCLUSION: The present study has demonstrated that both examination of muscle biopsy specimens and DNA analysis remain important methods in the differential diagnosis of muscular dystrophies. Because dystrophinopathies and sarcoglycanopathies have similar clinical manifestation.


Assuntos
Sarcoglicanopatias/genética , Sarcoglicanopatias/patologia , Sarcoglicanas/genética , Adolescente , Sequência de Aminoácidos , Sequência de Bases , Criança , Pré-Escolar , DNA/análise , Análise Mutacional de DNA , Feminino , Humanos , Masculino , Dados de Sequência Molecular , Músculo Esquelético/patologia , Reação em Cadeia da Polimerase , Estudos Retrospectivos , Adulto Jovem
19.
Pediatr Neurol ; 50(4): 343-6, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24485016

RESUMO

BACKGROUND: Limited data are available on the effects of a ketogenic diet on dispersion duration of P-wave and QT-interval measures in children. We searched for the changes in these measures with serial electrocardiograms in patients treated with a ketogenic diet. METHODS: Twenty-five drug-resistant patients with epilepsy treated with a ketogenic diet were enrolled in this study. Electrocardiography was performed in all patients before the beginning and at the sixth month after implementation of the ketogenic diet. Heart rate, maximum and minimum P-wave duration, P-wave dispersion, and maximum and minimum corrected QT interval and QT dispersion were manually measured from the 12-lead surface electrocardiogram. RESULTS: Minimum and maximum corrected QT and QT dispersion measurements showed nonsignificant increase at month 6 compared with baseline values. Other previously mentioned electrocardiogram parameters also showed no significant changes. CONCLUSIONS: A ketogenic diet of 6 months' duration has no significant effect on electrocardiogram parameters in children. Further studies with larger samples and longer duration of follow-up are needed to clarify the effects of ketogenic diet on P-wave dispersion and corrected QT and QT dispersion.


Assuntos
Dieta Cetogênica , Epilepsia/dietoterapia , Epilepsia/fisiopatologia , Coração/fisiopatologia , Adolescente , Criança , Pré-Escolar , Eletrocardiografia , Feminino , Frequência Cardíaca/fisiologia , Humanos , Lactente , Masculino , Fatores de Tempo , Resultado do Tratamento
20.
Seizure ; 23(4): 252-9, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24370319

RESUMO

PURPOSE: Limited data are available for the effectiveness of the antiepileptic drugs in children in daily clinical practice. The aim of this study was to investigate the efficacy and tolerability of the first prescribed old and new antiepileptic drugs in children with newly diagnosed idiopathic epilepsy during a 12-month period. METHOD: A total of 289 children (141 females and 148 males) who received phenobarbital (n=33), valproate (n=142), carbamazepine (n=42), oxcarbazepine (n=38), or levetiracetam (n=34) as the first-line treatment, were enrolled in the study. Seizure control and the occurrence of adverse events were assessed during a treatment period of 12 months. RESULTS: Overall, 245 (84.8%) patients remained seizure-free during the study period. The rate of seizure control did not differ significantly between the drug groups (p=0.099). Forty-four (15.2%) patients including 1 (3.0%) treated with phenobarbital, 22 (15.5%) with valproate, 7 (16.7%) with carbamazepine, 10 (26.3%) with oxcarbazepine, and 4 (11.8%) with levetiracetam had treatment failure. There was no significant difference between seizure-free and failure groups in terms of age, gender, seizure type, and drugs used. Overall, 80 (27.7%) patients had adverse events, of those the most common ones were behavioral problems, nausea and/or vomiting, weight gain, and learning difficulties. The reasons for treatment failures were lack of seizure control in 29 (10.0%) patients and intolerable adverse events in 15 (5.2%) patients. CONCLUSION: It appears that old (phenobarbital, valproate and carbamazepine) and new antiepileptic drugs (oxcarbazepine and levetiracetam) have similar efficacy and tolerability profiles. Institutional ethic number is 28.3.2013/14.


Assuntos
Anticonvulsivantes/efeitos adversos , Epilepsia/tratamento farmacológico , Adolescente , Anticonvulsivantes/classificação , Sintomas Comportamentais/induzido quimicamente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Estatísticas não Paramétricas , Falha de Tratamento , Resultado do Tratamento
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