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1.
Clin Endocrinol (Oxf) ; 98(3): 332-341, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-36536479

RESUMEN

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.


Asunto(s)
Dieta Cetogénica , Síndrome de Prader-Willi , Humanos , Niño , Persona de Mediana Edad , Síndrome de Prader-Willi/metabolismo , Estudios Retrospectivos , Estudios Transversales , Obesidad/metabolismo
2.
Neurol Sci ; 39(1): 85-89, 2018 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-29038947

RESUMEN

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.


Asunto(s)
Dieta Cetogénica , Epilepsia Refractaria/sangre , Epilepsia Refractaria/dietoterapia , Adolescente , Análisis Químico de la Sangre , Niño , Preescolar , Femenino , Pruebas Hematológicas , Humanos , Masculino , Estudios Retrospectivos
3.
Neuropediatrics ; 47(3): 157-61, 2016 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-27043293

RESUMEN

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.


Asunto(s)
Arritmias Cardíacas/inducido químicamente , Dieta Cetogénica/efectos adversos , Epilepsia Refractaria/dietoterapia , Adolescente , Errores Innatos del Metabolismo de los Carbohidratos/complicaciones , Niño , Preescolar , Epilepsia Refractaria/etiología , Electrocardiografía , Epilepsias Mioclónicas/dietoterapia , Femenino , Estudios de Seguimiento , Frecuencia Cardíaca , Humanos , Hipoxia-Isquemia Encefálica/complicaciones , Lactante , Síndrome de Landau-Kleffner/complicaciones , Síndrome de Lennox-Gastaut/dietoterapia , Masculino , Malformaciones del Desarrollo Cortical/complicaciones , Proteínas de Transporte de Monosacáridos/deficiencia , Estudios Prospectivos , Espasmos Infantiles/dietoterapia , Estado Epiléptico/dietoterapia , Esclerosis Tuberosa/complicaciones , Adulto Joven
4.
Neurol Sci ; 37(3): 465-70, 2016 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-26700799

RESUMEN

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.


Asunto(s)
Colesterol/sangre , Dieta Cetogénica/métodos , Aceite de Oliva/administración & dosificación , Convulsiones/sangre , Convulsiones/dietoterapia , Triglicéridos/sangre , Adolescente , Índice de Masa Corporal , Niño , Preescolar , Dieta Alta en Grasa/efectos adversos , Dieta Alta en Grasa/métodos , Dieta Cetogénica/efectos adversos , Femenino , Estudios de Seguimiento , Humanos , Hiperlipidemias/sangre , Hiperlipidemias/dietoterapia , Masculino , Aceite de Oliva/efectos adversos , Factores de Tiempo , Resultado del Tratamiento
5.
Eur J Paediatr Neurol ; 23(1): 143-151, 2019 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-30497921

RESUMEN

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.


Asunto(s)
Dieta Cetogénica/efectos adversos , Dieta Cetogénica/métodos , Epilepsia Refractaria/dietoterapia , Aceite de Oliva/uso terapéutico , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Estudios Prospectivos , Resultado del Tratamiento , Turquía
6.
J Child Neurol ; 33(11): 718-723, 2018 10.
Artículo en Inglés | MEDLINE | ID: mdl-30062917

RESUMEN

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.


Asunto(s)
Actividades Cotidianas , Dieta Cetogénica/métodos , Epilepsia Refractaria , Trastornos del Movimiento/dietoterapia , Trastornos del Movimiento/etiología , Niño , Preescolar , Epilepsia Refractaria/complicaciones , Epilepsia Refractaria/dietoterapia , Epilepsia Refractaria/psicología , Femenino , Humanos , Masculino , Estudios Prospectivos , Estadísticas no Paramétricas , Encuestas y Cuestionarios , Factores de Tiempo , Resultado del Tratamiento
7.
Epilepsia Open ; 3(2): 175-192, 2018 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-29881797

RESUMEN

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.

8.
Biol Trace Elem Res ; 178(1): 1-6, 2017 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-27873289

RESUMEN

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.


Asunto(s)
Dieta Cetogénica , Epilepsia Refractaria/sangre , Epilepsia Refractaria/dietoterapia , Selenio/sangre , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Selenio/administración & dosificación , Selenio/deficiencia
9.
Turk J Med Sci ; 47(3): 942-946, 2017 Jun 12.
Artículo en Inglés | MEDLINE | ID: mdl-28618748

RESUMEN

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.


Asunto(s)
Diabetes Mellitus Tipo 1/epidemiología , Neuropatías Diabéticas/epidemiología , Adolescente , Niño , Preescolar , Diabetes Mellitus Tipo 1/complicaciones , Neuropatías Diabéticas/diagnóstico , Neuropatías Diabéticas/etiología , Neuropatías Diabéticas/fisiopatología , Electromiografía , Femenino , Hemoglobina Glucada/análisis , Humanos , Masculino , Estudios Retrospectivos , Factores de Riesgo
10.
J Pediatr Endocrinol Metab ; 30(4): 411-416, 2017 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-28076316

RESUMEN

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.


Asunto(s)
Biomarcadores/sangre , Dieta Cetogénica/efectos adversos , Epilepsia Refractaria/dietoterapia , Hipotiroidismo/sangre , Tirotropina/sangre , Tiroxina/sangre , Triyodotironina/sangre , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Hipotiroidismo/diagnóstico , Hipotiroidismo/etiología , Lactante , Masculino , Pronóstico
11.
Brain Res Bull ; 69(6): 656-9, 2006 May 31.
Artículo en Inglés | MEDLINE | ID: mdl-16716834

RESUMEN

Since their introduction, atypical neuroleptic agents have been discovered to have some beneficial effects beyond their effectiveness as neuroleptic drugs. Among these initially unexpected effects are their potential effects as mood stabilizers in bipolar disorder and their efficacy in improving long-term outcome in schizophrenia. These effects recently raised the question whether these drugs may also have some neuroprotective effect in the brain. To examine this matter, in this study we evaluated the neuroprotective effect of risperidone after permanent focal cerebral ischemia. Anaesthetized male C57BL/6j mice were submitted to permanent thread occlusion of the middle cerebral artery (MCA). Risperidone (0.1, 1 or 10 mg/kg) or vehicle was applied intraperitoneally just after permanent ischemia. Twenty-four hours after permanent ischemia, brain injury was evaluated by triphenyltetrazolium chloride staining (TTC). Risperidone (0.1, 1 and 10 mg/kg) showed significant neuroprotection after permanent focal cerebral ischemia.


Asunto(s)
Lesiones Encefálicas/tratamiento farmacológico , Lesiones Encefálicas/etiología , Isquemia Encefálica/complicaciones , Antagonistas de Dopamina/uso terapéutico , Risperidona/uso terapéutico , Animales , Lesiones Encefálicas/patología , Relación Dosis-Respuesta a Droga , Masculino , Ratones , Sales de Tetrazolio , Factores de Tiempo
12.
J Child Neurol ; 31(9): 1120-6, 2016 08.
Artículo en Inglés | MEDLINE | ID: mdl-27080042

RESUMEN

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.


Asunto(s)
Anticonvulsivantes/administración & dosificación , Piracetam/análogos & derivados , Estado Epiléptico/tratamiento farmacológico , Ácido Valproico/administración & dosificación , Administración Intravenosa , Adolescente , Anticonvulsivantes/efectos adversos , Niño , Preescolar , Protocolos Clínicos , Cuidados Críticos , Femenino , Humanos , Lactante , Unidades de Cuidado Intensivo Pediátrico , Levetiracetam , Masculino , Seguridad del Paciente , Piracetam/administración & dosificación , Piracetam/efectos adversos , Estudios Retrospectivos , Resultado del Tratamiento , Ácido Valproico/efectos adversos
13.
Brain Dev ; 38(9): 842-7, 2016 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-27066714

RESUMEN

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.


Asunto(s)
Dieta Cetogénica , Epilepsia Refractaria/dietoterapia , Epilepsia Refractaria/fisiopatología , Corazón/fisiopatología , Preescolar , Dieta Cetogénica/efectos adversos , Epilepsia Refractaria/diagnóstico por imagen , Ecocardiografía , Electrocardiografía , Femenino , Estudios de Seguimiento , Corazón/diagnóstico por imagen , Humanos , Cetonas/sangre , Masculino , Estudios Prospectivos
14.
Seizure ; 43: 32-38, 2016 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-27866088

RESUMEN

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.


Asunto(s)
Dieta Cetogénica/métodos , Hepatopatías/dietoterapia , Hepatopatías/etiología , Abdomen/diagnóstico por imagen , Adolescente , Antropometría , Bilirrubina/sangre , Niño , Preescolar , Colesterol/sangre , Epilepsia Refractaria/sangre , Epilepsia Refractaria/complicaciones , Epilepsia Refractaria/dietoterapia , Epilepsia Refractaria/etiología , Femenino , Humanos , Hepatopatías/diagnóstico por imagen , Estudios Longitudinales , Masculino , Estudios Retrospectivos , Factores de Tiempo , Transaminasas/sangre , Triglicéridos/sangre , Ultrasonografía
15.
Pediatr Neurol ; 56: 30-34, 2016 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-26774551

RESUMEN

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.


Asunto(s)
Arterias Carótidas/patología , Grosor Intima-Media Carotídeo , Dieta Cetogénica/efectos adversos , Epilepsia Refractaria/dietoterapia , Adolescente , Anticonvulsivantes/uso terapéutico , Presión Sanguínea/fisiología , Niño , Preescolar , Epilepsia Refractaria/sangre , Femenino , Estudios de Seguimiento , Humanos , Lactante , Lípidos/sangre , Masculino , Estudios Retrospectivos , Estadísticas no Paramétricas , Adulto Joven
16.
Pediatr Neurol ; 53(3): 233-237.e1, 2015 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-26302701

RESUMEN

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.


Asunto(s)
Dieta Cetogénica , Epilepsia Refractaria/dietoterapia , Epilepsia Refractaria/fisiopatología , Función Ventricular/fisiología , Preescolar , Electrocardiografía , Femenino , Humanos , Masculino , Estudios Prospectivos
17.
J Child Neurol ; 30(12): 1646-50, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-25855687

RESUMEN

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.


Asunto(s)
Aorta/fisiopatología , Arterias Carótidas/fisiopatología , Grosor Intima-Media Carotídeo , Dieta Cetogénica , Epilepsia Refractaria/dietoterapia , Epilepsia Refractaria/fisiopatología , Factores de Edad , Aorta/diagnóstico por imagen , Análisis Químico de la Sangre , Presión Sanguínea , Estatura , Índice de Masa Corporal , Peso Corporal , Arterias Carótidas/diagnóstico por imagen , Preescolar , Dieta Cetogénica/efectos adversos , Epilepsia Refractaria/diagnóstico por imagen , Ecocardiografía , Elasticidad , Femenino , Estudios de Seguimiento , Humanos , Masculino , Estudios Prospectivos , Factores de Tiempo , Resultado del Tratamiento
18.
Pediatr Neurol ; 50(4): 343-6, 2014 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-24485016

RESUMEN

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.


Asunto(s)
Dieta Cetogénica , Epilepsia/dietoterapia , Epilepsia/fisiopatología , Corazón/fisiopatología , Adolescente , Niño , Preescolar , Electrocardiografía , Femenino , Frecuencia Cardíaca/fisiología , Humanos , Lactante , Masculino , Factores de Tiempo , Resultado del Tratamiento
19.
Seizure ; 23(4): 252-9, 2014 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-24370319

RESUMEN

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.


Asunto(s)
Anticonvulsivantes/efectos adversos , Epilepsia/tratamiento farmacológico , Adolescente , Anticonvulsivantes/clasificación , Síntomas Conductuales/inducido químicamente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Estadísticas no Paramétricas , Insuficiencia del Tratamiento , Resultado del Tratamiento
20.
J Child Neurol ; 29(11): 1473-8, 2014 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-24097851

RESUMEN

The aim of this study is to evaluate the types and clinical characteristics of peripheral facial palsy in children. The hospital charts of children diagnosed with peripheral facial palsy were reviewed retrospectively. A total of 81 children (42 female and 39 male) with a mean age of 9.2 ± 4.3 years were included in the study. Causes of facial palsy were 65 (80.2%) idiopathic (Bell palsy) facial palsy, 9 (11.1%) otitis media/mastoiditis, and tumor, trauma, congenital facial palsy, chickenpox, Melkersson-Rosenthal syndrome, enlarged lymph nodes, and familial Mediterranean fever (each 1; 1.2%). Five (6.1%) patients had recurrent attacks. In patients with Bell palsy, female/male and right/left ratios were 36/29 and 35/30, respectively. Of them, 31 (47.7%) had a history of preceding infection. The overall rate of complete recovery was 98.4%. A wide variety of disorders can present with peripheral facial palsy in children. Therefore, careful investigation and differential diagnosis is essential.


Asunto(s)
Parálisis Facial/epidemiología , Adolescente , Parálisis de Bell/complicaciones , Parálisis de Bell/diagnóstico , Parálisis de Bell/epidemiología , Parálisis de Bell/fisiopatología , Niño , Preescolar , Diagnóstico Diferencial , Parálisis Facial/diagnóstico , Parálisis Facial/etiología , Parálisis Facial/fisiopatología , Femenino , Humanos , Lactante , Masculino , Mastoiditis/complicaciones , Mastoiditis/diagnóstico , Mastoiditis/epidemiología , Mastoiditis/fisiopatología , Pronóstico , Estudios Retrospectivos
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