Ünsal Yılmaz

Multipl skleroz (MS), santral sinir sisteminin multifokal ve multifazik seyreden, başka bir hastalıkla açıklanamayan demiyelinizan bir hastalığıdır. Tanıda, 2017 McDonald kriterleri ile alanda ve zamanda yayılımın gösterilmesi gerekmektedir. Son yıllarda MS patogenezindeki gelişmeler doğrultusunda 2024 ECTRIMS önerileri tanı sürecine yeni biyobelirteçler (örneğin, kappa serbest hafif zincir) ve görüntüleme bulgularını (santral ven işareti, paramagnetik rim lezyonları) dahil etmeyi önermektedir. MS tanısı konulan çocuk hastalarda tedavi, akut atak yönetimi ve hastalık modifiye edici tedavileri kapsar. Erken tanı ve tedavi, uzun vadede özürlülüğü önlemek açısından kritik öneme sahiptir. Multidisipliner bir yaklaşım, bilişsel ve psikososyal desteğin sağlanması ve düzenli takip ile hastalık yönetimi optimize edilmelidir. Multiple Sclerosis (MS) is a demyelinating disease of the central nervous system that follows a multifocal and multiphasic course and cannot be explained by another condition. Diagnosis requires demonstrating dissemination in space and time according to the 2017 McDonald criteria. In recent years, advancements in MS pathogenesis have led to the 2024 ECTRIMS recommendations, which propose incorporating new biomarkers (e.g., kappa free light chains) and imaging findings (central vein sign, paramagnetic rim lesions) into the diagnostic process.In pediatric patients diagnosed with MS, treatment encompasses acute attack management and disease-modifying therapies. Early diagnosis and treatment are crucial in preventing long-term disability. A multidisciplinary approach, including cognitive and psychosocial support and regular follow-up, is essential for optimizing disease management

Multipl skleroz (MS), santral sinir sisteminin kronik, ilerleyici ve relapslarla seyreden demiyelinizan bir hastalığıdır. Vakaların %3-7’si pediatrik yaş grubunda başlar. Çocukluk çağı MS'i genellikle relapsing-remitting fenotipinde görülür ve erişkinlere kıyasla daha sık ve şiddetli ataklarla seyreder.Tedavi stratejileri akut atak tedavisi ve uzun süreli hastalık modifiye edici tedaviler olarak ikiye ayrılır. Akut ataklarda yüksek doz intravenöz metilprednizolon, intravenöz immünglobulin ve plazma değişimi tercih edilir. Uzun süreli tedavide interferon beta, glatiramer asetat ve teriflunomid gibi birinci basamak tedaviler, daha dirençli vakalarda fingolimod, dimetil fumarat ve monoklonal antikorlar (natalizumab, okrelizumab) kullanılır.Son yıllarda yüksek etkinlikli tedavilere erken başlanmasının hastalık progresyonunu azalttığı gösterilmiştir. Ancak çocuklarda tedaviye ilişkin kanıta dayalı veriler sınırlıdır. MS'li çocuk hastaların multidisipliner bir yaklaşımla izlenmesi, bilişsel ve psikososyal desteğin sağlanması önemlidir. Aşılama ve enfeksiyon taramaları tedavi sürecinin ayrılmaz bir parçasıdır. Multiple sclerosis (MS) is a chronic, progressive, and relapsing-remitting demyelinating disease of the central nervous system. Approximately 3-7% of cases have a pediatric onset. Pediatric MS predominantly follows a relapsing-remitting course, with more frequent and severe attacks compared to adult-onset MS.Treatment strategies are classified into acute attack management and long-term disease-modifying therapies. Acute attacks are primarily managed with high-dose intravenous methylprednisolone, intravenous immunoglobulin, and plasma exchange. Long-term treatment includes first-line therapies such as interferon-beta, glatiramer acetate, and teriflunomide, whereas more refractory cases require second-line agents like fingolimod, dimethyl fumarate, and monoclonal antibodies (natalizumab, ocrelizumab).Recent studies suggest that early initiation of high-efficacy treatments may mitigate disease progression. However, evidence-based data on pediatric MS treatment remain limited. A multidisciplinary approach is essential for managing pediatric patients, ensuring cognitive and psychosocial support. Vaccination and infection screening are integral components of the therapeutic process.

Multipl Sklerozun Tanımı ve Tarihçesi Coşkun YARAR Multipl Skleroz Epidemiyolojisi Meryem Hilal ALTAŞ Nesrin CEYLAN Multipl Sklerozda Etiyoloji ve Genetik Özlem YILMAZ Pınar GENÇPINAR Multipl Sklerozun Patogenezi Hüseyin Bahadır ŞENOL Uluç YİŞ Pediatrik Multipl Sklerozun Klinik Özellikleri Mehmet BAŞTEMUR İbrahim ÖNCEL Pediatrik Multipl Skleroz Tanı Kriterleri Özge DEDEOĞLU Ayşegül Neşe ÇITAK KURT Klinik İzole Sendrom Elif DİDİNMEZ TAŞKIRDI Nihal OLGAÇ DÜNDAR Radyolojik İzole Sendrom Burçin GÖNÜLLÜ POLAT Meltem ÇOBANOĞULLARI DİREK Pediatrik Multipl Sklerozda Tanı Yöntemleri Nilay AKTAR ULUKAPI İsmail SOLMAZ Pediatrik Multipl Sklerozda Ayırıcı Tanı Mehmet BAŞTEMUR İbrahim ÖNCEL Multipl Skleroz Olgularında Radyolojik İllustrasyonlar İle Tanısal İpuçları Harun YILDIZ Multipl Skleroz Atak Tedavisi Fatma Pınar TABANLI Serkan KIRIK Ömer BEKTAŞ Multipl Sklerozda Hastalık Modifiye Edici Tedaviler Genel Tedavi Prensipleri Sevde SEÇER Bahadır KONUŞKAN Oral Tedaviler Büşra BIYIKLIOĞLU Bahadır KONUŞKAN Enjeksiyon Tedavileri Aslı ERCİLİ ERDAL Bahadır KONUŞKAN İnfüzyon Tedavileri Büşra BIYIKLIOĞLU Bahadır KONUŞKAN Multipl Skleroz ve Kök Hücre Tedavisi Mehmet CANPOLAT Pediatrik Multipl Sklerozda Tedavi Yönetimi Nur ÇALIŞKAN Ünsal YILMAZ Pediatrik Multipl Skleroz Tanı ve Tedavi Algoritması Nur ÇALIŞKAN Ünsal YILMAZ Multiple Skleroz Tedavisinde Potansiyel Komplikasyonlar ve Yönetimi Esma ŞENGENÇ Sema SALTIK Multiple Skleroz ve Aşılar Pınar Saka ÜMİT İsmail SOLMAZ Multiple Skleroz Morbilite Nedenleri ve Yönetimi Ezgi ÇAĞLAR Meltem ÇOBANOĞULLARI DİREK Multipl Sklerozda Prognoz ve Mortalite Meryem HİLAL ALTAŞ Nesrin CEYLAN

Objectives: West syndrome (WS) is an early childhood epileptic encephalopathy characterized by spasms, typically occurring within the first year of life. The International League Against Epilepsy reclassified WS as “infantile epileptic spasm syndrome” to enhance early diagnosis and treatment. It is marked by a triad of epileptic spasms, psychomotor retardation or regression, and hypsarrhythmia on EEG. The prognosis and response to classical anti-epileptic treatments are often poor, and factors influencing prognosis remain unclear. Methods: This study retrospectively analyzed 75 patients with WS over five years, assessing etiology, MRI findings, and neurodevelopmental outcomes according to ILAE guidelines. Results: The cohort comprised 35 females (46.7%) and 40 males (53.3%). The most common etiology was structural, observed in 41 patients (54.7%), followed by unknown causes in 19 patients (25.3%). Genetic, metabolic, and infectious causes were less common. Brain MRI findings were normal in 23 patients (30.7%). Treatment primarily involved Vigabatrin, which was used in 54.7% of cases, followed by Adrenocorticotropic hormone (ACTH) in 25.3%. Seizure control improved over time, with 24% of patients fully controlled at one year and 42.8% at five years. However, 28% showed no change in seizure frequency. The presence of structural abnormalities correlated with a poorer prognosis, while early and complete seizure control was associated with better outcomes. Mortality was 5.3%, with four patients passing away during the follow-up period. Conclusion: The study highlights that while the etiology remains a significant factor in the prognosis of WS, early intervention and effective seizure management are crucial for improving long-term outcomes.

Aim: The use of broad-spectrum antibiotics, changes in patient profile, prolonged life expectancy, increased use of medical devices and technologies such as catheters, intravenous fluids, gastrostomy and tracheostomy have led to changes in the number and types of microorganisms isolated from infections and cultures. There are no data on infections seen in pediatric palliative care. The aim of this study was to determine the frequency of infections and the pathogens produced in culture, to identify the sites of growth and to review what should be done. Materials and Methods: This was planned as a cross-sectional, retrospective study between 11/18/2018 and 09/01/2023. Sociodemographic data, reason for hospitalization, number of hospitalizations, primary diagnoses, comorbid conditions, medical devices and technology used, time and place of culture, number of cultures, microorganisms grown and factors associated with culture growth were examined. Statistical analysis was performed with the SPSS 18.0 program. p