The evaluation of vitamin K status in children with febrile seizure

Background: Febrile seizure is the most common neurological disorder in childhood. The exact pathophysiology of febrile seizures is unknown. Recent studies showed the role of vitamin K in nonhematological and inflammatory disorders. This study aimed to investigate the serum vitamin K levels in children with febrile seizures. Aims: To evaluate vitamin K levels in children with febrile seizures. Study Design: Prospective case-control study. Methods: This multicenter study examined representative populations in 8 different cities in Turkey between April 1, 2018 and April 1, 2019. Blood samples were taken from all children at presentation. Vitamin K1, vitamin K2, tumor necrosis factor-alpha, interleukin 1 beta, and interleukin 6 levels were determined by enzyme-linked immunosorbent assay. Results: A total of 155 children were included in the study—84 children with febrile seizures and 71 children in febrile control group. Serum vitamin K1 and vitamin K2 levels were also higher in children with febrile seizures than in the controls. The results of statistical analysis showed that vitamin K1 and vitamin K2 levels were correlated with tumor necrosis factor-alpha, interleukin 1 beta, and interleukin 6 levels. The median vitamin K1 and vitamin K2 levels of children experiencing their first febrile seizure were higher than those in children with recurrent febrile seizures. Type of febrile seizure has no effect on serum vitamin K1 and vitamin K2 levels. Conclusion: In children with febrile seizures, vitamin K levels are higher than those in the control group. These new findings may contribute to elucidating the etiopathogenesis of febrile seizures

Serebral palsili hastalarda epilepsi beraberliğini ve epilepsi prognozunu etkileyen faktörler

TEZ7827Tez (Uzmanlık) -- Çukurova Üniversitesi, Adana, 2010.Kaynakça (s. 53-57) var.viii, 60 s. : res. ; 29 cm.Aim: This study was conducted between November 2006 and October 2009 to determine the factors predicting the presence and prognosis of epilepsy in cerebral palsy patients. Material and Method: We enrolled 42 cerebral palsy patients (19 female, 23 male ) in the first group and 56 cerebral palsy patients with epilepsy (23 female, 33 male) in the second group. The mean age of the first group was 7.7±3.3 (minimum 3, maximum 15), and 8.9±3.7 (minimum 3, maximum 16) for the second group. The subjects in the second group were considered to have good prognosis if they were free of seizures for the last year otherwise they were considered to have poor prognosis. Results: In the second group, neonatal epilepsy, family history of epilepsy and moderate to severe mental retardation was significantly higher than the first group (p0.05). The onset of seizures was found between 5 months and 13.5 years in the second group (average 3.4±3.6 years). In univariate analysis, neonatal seizure, epileptic activity in electroencephalography, polytherapy was found to be predictors of poor prognosis. Additionally, need for long term medication to control seizures affects prognosis badly. Prematurity, low birth weight, type of cerebral palsy, intelligence quotient, Palisano scoring, magnetic resonance imaging findings, type of seizure, gender and parental consanguinity were not related with prognosis. In logistic regression analysis, neonatal seizure and interictal epileptic activity in electroencephalography were found to be independent predictors of worse outcome. In addition logistic regression analysis revealed that the increasing age decreases the success of epilepsy treatment. In conclusion: Neonatal seizure, family history of epilepsy and mental retardation was found to be important and independent predictors of epilepsy formation in cerebral palsy. Neonatal seizure, interictal epileptic electroensefalogram, polytheraphy and long time to control seizures were independent predictors of poor prognosis for cerebral palsy patients with epilepsy.Amaç: Bu çalışma, serebral palsili hastalarda epilepsi beraberliğini ve epilepsi prognozunu etkileyen faktörleri belirlemek amacıyla Kasım 2006- Ekim 2009 tarihleri arasında yapıldı. Gereç ve Yöntem: Çalışmaya 42 serebral palsi (19 kız, 23 erkek), 56 serebral palsi+ epilepsi (23 kız, 33 erkek) tanısı alan 2 grup hasta alındı. Grup I olgularının yaş ortalaması 7,7±3,3 (minimum 3, maksimum 15), Grup II olgularının ise 8,9±3,7 (minimum 3, maksimum 16) idi. Grup II olgularından bir veya daha fazla antiepileptik ilaç ile bir yıl ve daha uzun süre nöbet kontrolü olanlar iyi prognozlu, nöbet kontrolü olmayanlar ise kötü prognozlu olarak değerlendirildi. Bulgular: Grup II olgularında yenidoğan nöbeti varlığı, ailede epilepsi öyküsü varlığı ve orta-ağır mental retarde olma olasılığı anlamlı olarak daha yüksek oranda bulundu (p0,05). Grup II olgularının nöbet başlama yaşı 5 ay ile 13,5 yıl arasında (ortalama: 3,4±3,6 yıl) bulundu. Tek değişkenli analizde, yenidoğan dönemde nöbet varlığı, elektroensefalografide epileptik aktivite varlığı ve politerapi almanın epilepsi prognozunu anlamlı derecede olumsuz etkilediği bulundu (p0,05). Lojistik regresyon analizi sonucunda ise yenidoğan nöbet öyküsünün varlığı ve interiktal elektroensefalografide epileptik aktivite olması bağımsız prognostik faktör olarak bulundu. Ayrıca yine lojistik regresyon analizinde, tedavi sırasında hasta yaşı büyüdükçe nöbetlerin kontrole girmesinin daha zorlaştığı saptandı (p<0,05). Sonuç: Serebral palsili hastalarda yenidoğan nöbeti, ailede epilepsi öyküsü olması ve mental retardasyon varlığının epilepsi gelişimi için önemli risk faktörü olduğunu; serebral palsili epileptik hastalarda yenidoğan nöbet öyküsü varlığının, epileptik interiktal elektroensefalografi bulgusunun, hastanın politerapi almasının ve nöbetin kontrole girmesi için geçen sürenin daha uzun olmasının nöbet prognozunu olumsuz yönde etkileyen bağımsız değişkenler olduğunu söyleyebiliriz

Early rehabilitation of a child with intensive care unit acquired weakness secondary to membranoproliferative glomerulonephritis: A case report

Coşkun-Benlidayı İ, Başaran S, Gül-Mert G, Güzel R. Early rehabilitation of a child with intensive care unit acquired weakness secondary to membranoproliferative glomerulonephritis: A case report. Turk J Pediatr 2015; 57: 422-425.Intensive care unit acquired weakness presents with flaccid paralysis of the extremities and difficulty of weaning from the ventilator and it has occasionally been reported in children. We report the early rehabilitation of a 12-year-old girl with membranoproliferative glomerulonephritis who developed intensive care unit acquired weakness. She underwent an intensive rehabilitation procedure which included assistive range of motion, bed mobility, airway clearance and breathing techniques, neuromuscular electrical stimulation (NMES) to the quadriceps muscles and resistive therapeutic band exercises. Following the rehabilitation program, muscle strength (Medical Research Council sum score), ambulation (Functional Ambulation Category) and activity (Wee FIM) scores increased significantly.Coşkun-Benlidayı İ, Başaran S, Gül-Mert G, Güzel R. Early rehabilitation of a child with intensive care unit acquired weakness secondary to membranoproliferative glomerulonephritis: A case report. Turk J Pediatr 2015; 57: 422-425.Intensive care unit acquired weakness presents with flaccid paralysis of the extremities and difficulty of weaning from the ventilator and it has occasionally been reported in children. We report the early rehabilitation of a 12-year-old girl with membranoproliferative glomerulonephritis who developed intensive care unit acquired weakness. She underwent an intensive rehabilitation procedure which included assistive range of motion, bed mobility, airway clearance and breathing techniques, neuromuscular electrical stimulation (NMES) to the quadriceps muscles and resistive therapeutic band exercises. Following the rehabilitation program, muscle strength (Medical Research Council sum score), ambulation (Functional Ambulation Category) and activity (Wee FIM) scores increased significantly

Pyridoxine-dependent epilepsy in two Turkish patients in Turkey and review of the literature

Gül-Mert G, İncecik F, Hergüner Mö, Ceylaner S, Altunbaşak Ş. Pyridoxine-dependent epilepsy in two Turkish patients in Turkey and review of the literature. Turk J Pediatr 2015; 57: 394-397.Pyridoxine-dependent epilepsy (PDE) is a rare autosomal recessive enzyme defect in the vitamin B6 metabolism characterized by intractable seizures which are usually resistant to all antiepileptic drugs but respond to pharmacological doses of pyridoxine. We present the clinical and molecular genetic findings of two patients with c.1597_1597delG mutations in ALDH7A1 gene. There are different clinical phenotypes in PDE: patients with complete seizure control with pyridoxine and normal development (group 1), patients with complete seizure control with pyridoxine and development delay (group 2), and patients with persistent seizures despite pyridoxine treatment and with development delay (group 3). Our two patients have persistant seizure despite pyridoxine treatment and with development delay. Pyridoxine-dependent epilepsy can be identified in any neonate with signs of encephalopathy and refractory seizures, with no evidence of hypoxic-ischemic damage or other underlying metabolic disturbance. Neurodevelopmental outcomes of patients with PDE is multifactorial; early diagnosis and treatment of these patients is vital.Gül-Mert G, İncecik F, Hergüner Mö, Ceylaner S, Altunbaşak Ş. Pyridoxine-dependent epilepsy in two Turkish patients in Turkey and review of the literature. Turk J Pediatr 2015; 57: 394-397.Pyridoxine-dependent epilepsy (PDE) is a rare autosomal recessive enzyme defect in the vitamin B6 metabolism characterized by intractable seizures which are usually resistant to all antiepileptic drugs but respond to pharmacological doses of pyridoxine. We present the clinical and molecular genetic findings of two patients with c.1597_1597delG mutations in ALDH7A1 gene. There are different clinical phenotypes in PDE: patients with complete seizure control with pyridoxine and normal development (group 1), patients with complete seizure control with pyridoxine and development delay (group 2), and patients with persistent seizures despite pyridoxine treatment and with development delay (group 3). Our two patients have persistant seizure despite pyridoxine treatment and with development delay. Pyridoxine-dependent epilepsy can be identified in any neonate with signs of encephalopathy and refractory seizures, with no evidence of hypoxic-ischemic damage or other underlying metabolic disturbance. Neurodevelopmental outcomes of patients with PDE is multifactorial; early diagnosis and treatment of these patients is vital

A Case with Recurrent Facial Palsy: Melkersson-Rosenthal Syndrome

Melkersson-Rosenthal sendromu (MRS), tekrarlayan fasiyal paralizi, orofasiyal ödem, dilde fissürle karakterize nadir bir nöromukokütanöz sendromdur.Oligosemptomatik ve monosemptomatik olgular, klasik triaddan sık görülür.Bulguların ikisinin görülmesi veya bulguların biri ve biyopside granülomatöz keilitin varlığı Melkersson-Rosenthal sendromu tanısı için yeterlidir. Bu yazıda Melkersson-Rosenthal sendromu tanısı alan 12 yaşında bir erkek sunulmuştur.Melkersson-Rosenthal syndrome (MRS) is a rare neuromucocutaneous syndrome characterized by reccurent facial paralysis, orofacial edema and fissured tongue. Oligosymptomatic and monosymptomatic forms are more common than the triad. The presence of two manifestations or one manifestation with granulomatous cheilitis in biopsy is sufficient to make diagnosis of Melkersson-Rosenthal syndrome. We present a 12 years-old male who is diagnosed MelkerssonRosenthal syndrome

Atypic subacute sclerosing panensefalitis in a six-year-old male

Subacute sclerosing panencephalitis is a rare, slow, and insidious neurodegenerative disease caused by measles. This disease mostly has a classic course. However, sometimes it can be presented with atypical manifestations. In this paper, we aim to present a six years old male patient that was hospitalized due to seizures and ataxia. Cerebral magnetic resonance imaging was normal on the first day of admission, but within a few days, the patient started to be apathetic. On the seventh day, magnetic resonance imaging showed hyperintense lesions in the thalamic, brainstem, and periventricular areas. Periodic epileptiform discharges were detected in the repeated electroencephalogram. Investigations from the cerebrospinal fluid showed markedly elevated measles virus IgG at 230U/ml consistent with the diagnosis of SSPE which should always be ruled out when a patient comes in with uncontrollable seizures, ataxia and apathy

Serebral palsili hastaların klinik bulguları ve kemik mineral metabolizmalarının değerlendirilmesi

Bu çalışmada, Çukurova Üniversitesi Çocuk Nöroloji Polikliniği’nde izlenen serebral palsili hastaların klinik ve demografik özelliklerinin belirlenmesi, bu hastalarda kemik mineral metabolizması bozukluklarının saptanması ve bu sorunlara farkındalığın yaratılması amaçlanmıştır. Çukurova Üniversitesi Tıp Fakültesi, Çocuk Nöroloji Polikliniği’nde Ocak 2014 - Ocak 2015 tarihleri arasında, serebral palsi tanılarıyla izlenen, takipleri düzenli yapılan 2-18 yaş arası toplam 102 hasta çalışmaya dahil edildi. Çalışmaya dahil edilen olguların demografik bilgileri, etiyolojileri, klinik ve laboratuvar bulguları, eşlik eden problemleri kaydedildi. Hastaların ortalama yaş değeri 96.83 ± 53.54 ay (24-207 ay) olup, erkek/kız oranı 1.2 idi. Olguların %23.5’i zamanında doğumdu ve %50’nin doğum ağırlığı 2500 gr.’ın üzerindeydi. En sık (%38.2) kuadriplejik tipte olgu saptandı ve etiyolojide en sık natal faktörler (%56.9) rol alıyordu. Ayrıntılı etiyolojik değerlendirmede; asfiksi (%49) ve DDA (%28.4) en sık faktörler olarak belirlendi. En sık beyin MRG anormalliği PVL (%25) olarak saptandı. Mental retardasyon %88.2, konuşma problemleri %72.5, malnütrisyon %67.6, görme problemleri %28.4, epilepsi %46.4 oranla en sık eşlik eden problemlerdi. Düzenli fizik tedavi alan hastaların osteokalsin, IGF1, IGFBP3 ve PTH düzeylerinin diğer hastalara göre anlamlı olarak daha iyi olduğu görüldü (p&lt;0.05). D vitamin düzeyleri ile güneş ışığı maruziyeti, arasında anlamlı ilişki saptanmadı (p&gt; 0.05). Güneş ışığından doğru yararlanma ve düzenli dinamik fizyoterapinin kemik sağlığı üzerinde daha etkili olduğu görülmüştür. Zor doğum ve perinatal asfiksi öyküsü olan bebeklerin nöromotor gelişimlerinin düzenli olarak takip edilmesi tanı ve tedavideki gecikmeleri engelleyebilir.</p

Developing a Core Set of Outcome Measures for Behcet Disease: Report from OMERACT 2016

Objective. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group has been working toward developing a data-driven core set of outcome measures for use in clinical trials of Behcet's syndrome [Behcet disease (BD)]. This paper summarizes the group's work through OMERACT 2016, discussions during the meeting, and the future research agenda

Evaluation of immunization status in patients with cerebral palsy: a multicenter CP-VACC study

Children with chronic neurological diseases, including cerebral palsy (CP), are especially susceptible to vaccine-preventable infections and face an increased risk of severe respiratory infections and decompensation of their disease. This study aims to examine age-appropriate immunization status and related factors in the CP population of our country. This cross-sectional prospective multicentered survey study included 18 pediatric neurology clinics around Turkey, wherein outpatient children with CP were included in the study. Data on patient and CP characteristics, concomitant disorders, vaccination status included in the National Immunization Program (NIP), administration, and influenza vaccine recommendation were collected at a single visit. A total of 1194 patients were enrolled. Regarding immunization records, the most frequently administrated and schedule completed vaccines were BCG (90.8%), hepatitis B (88.9%), and oral poliovirus vaccine (88.5%). MMR was administered to 77.3%, and DTaP-IPV-HiB was administered to 60.5% of patients. For the pneumococcal vaccines, 54.1% of children received PCV in the scope of the NIP, and 15.2% of children were not fully vaccinated for their age. The influenza vaccine was administered only to 3.4% of the patients at any time and was never recommended to 1122 parents (93.9%). In the patients with severe (grades 4 and 5) motor dysfunction, the frequency of incomplete/none vaccination of hepatitis B, BCG, DTaP-IPV-HiB, OPV, and MMR was statistically more common than mild to moderate (grades 1-3) motor dysfunction (p = 0.003, p < 0.001, p < 0.001, p < 0.00, and p < 0.001, respectively). Physicians' influenza vaccine recommendation was higher in the severe motor dysfunction group, and the difference was statistically significant (p = 0.029)