The significance of MUAC z-scores in diagnosing pediatric malnutrition: A scoping review with special emphasis on neurologically disabled children

This review by a panel of pediatric gastroenterology-hepatology-nutrition and pediatric neurology experts aimed to address the significance of mid-upper arm circumference (MUAC) assessment in diagnosis of pediatric malnutrition. Specifically, the potential utility of recently developed MUAC z-score tape in clinical practice for larger patient populations was addressed including the neurologically disabled children. In accordance with the evidence-based data, four statements were identified by the participating experts on the utility of MUAC z-score tape, including (1) MUAC z-scores correlate with body mass index (BMI) and weight for height/length (WFH/l) z-scores in diagnosing malnutrition; (2) MUAC z-score tape offers a higher sensitivity to diagnose the mild and moderate malnutrition and better ability to track the changes in nutritional status over time than the other single datapoint measurements; (3) Using single-step MUAC z-score tape in children with cerebral palsy (CP) seems to provide more reliable data on anthropometry; and (4) The clinical value of the tool in classifying secondary malnutrition in CP should be investigated in large-scale populations. In conclusion, enabling single-step estimation of nutritional status in a large-scale pediatric population regardless of age and within a wide range of weight, without formal training or the need for ancillary reference charts and calculators, MUAC z-tape offers a favorable tool for easier and earlier diagnosis of pediatric malnutrition. Nonetheless, further implementation of MUAC z-score screening in larger-scale and/or special populations is necessary to justify its utility in relation to other primary anthropometric indicators in diagnosis of malnutrition as well as in treatment monitoring in the community and hospital setting.Abbott Nutrition Turkey ; KAPPA Consultancy Training Research Ltd

Sturge-Weber Syndrome Type III

Sturge-Weber syndrome (SWS) is a neurogenetic disease with an incidence of 1 in 20.000-50.000 live births. The less common form, which can be difficult to diagnose and only involves leptomeningeal angioma, has been defined as Type III SWS. A 5.5-month-old male patient with normal neuromotor development presented with right sided partial seizures, which had been occurring frequently for the previous two days and could not be controlled. A cranial magnetic resonance imaging showed pathological contrasts in the cortical regions involving the left hemisphere and in the leptomeningeal structures. We aim to present the case of an infant with SWS, which unlike the classical form was unidentifiable in physical examination and diagnosed using imaging methods

Determining Risk Factors of Epilepsy in Children with Cerebral Palsy: A Retrospective Study

Aim: The aim of this study is to determine the risk factors of epilepsy development in children with cerebral palsy. Materials and Methods: Data of 234 cerebral palsy patients treated at Ege University Pediatrics Department, Child Neurology Division between January 2008 and December 2015 were evaluated retrospectively. All patients were classified into two groups; Group I: cerebral palsy without epilepsy (n=116) and Group II: cerebral palsy with epilepsy (n=118). The clinical and laboratory findings of the groups were compared to each other, a p value of less than 0.05 was considered as statistically significant. Results: There was no significant difference between the two groups in terms of gender, gestastional age, birth type, birth weight, risk factors for cerebral palsy development (pre-/peri-/postnatal), duration of neonatal intensive care stay and the need for mechanical ventilation (p>0.05). The risk factors of epilepsy were determined as the following; the presence of neonatal convulsions, focal clonic and generalized tonic neonatal seizures, an abnormal baseline rhythm on neonatal electroencephalography (EEG), discharge from neonatal intensive care unit with at least one antiepileptic drug, spastic bilateral (tetraplegic) cerebral palsy, epileptic activity on the sixth month EEG, abnormal cranial magnetic resonance imaging findings, mental retardation, microcephaly and visual problems. Conclusion: Epilepsy is a common problem in children with cerebral palsy. Therefore, cases of cerebral palsy, especially those with the determined risk factors should be closely monitored for epilepsy in order to ensure a timely diagnosis and proper treatment

European Association of Urology and European Society for Paediatric Urology Guidelines on Paediatric Urinary Stone Disease

Context: Paediatric stone disease is an important clinically entity and management is often challenging. Although it is known that the condition is endemic in some geographic regions of the world, the global incidence is also increasing. Patient age and sex; the number, size, location, and composition of the stone; and the anatomy of the urinary tract are factors that need to be taken into consideration when choosing a treatment modality. Objective: To provide a general insight into the evaluation and management of urolithiasis in the paediatric population in the era of minimally invasive surgery. Evidence acquisition: A nonsystematic review of the literature on management of paediatric urolithiasis was conducted with the aim of presenting the most suitable treatment modality for different scenarios. Evidence synthesis: Because of high recurrence rates, open surgical intervention is not the first option for paediatric stone disease, except for very young patients with very large stones in association with congenital abnormalities. Minimally invasive surgeries have become the first option with the availability of appropriately sized instruments and accumulating experience. Extracorporeal shockwave lithotripsy (SWL) is noninvasive and can be carried out as an outpatient procedure under sedation, and is the initial choice for management of smaller stones. However, for larger stones, SWL has lower stone-free rates and higher retreatment rates, so minimally invasive endourology procedures such as percutaneous nephrolithotomy and retrograde intrarenal surgery are preferred treatment options. Conclusions: Contemporary surgical treatment for paediatric urolithiasis typically uses minimally invasive modalities. Open surgery is very rarely indicated. Patient summary: Cases of urinary stones in children are increasing. Minimally invasive surgery can achieve high stone-free rates with low complication rates. After stone removal, metabolic evaluation is strongly recommended so that medical treatment for any underlying metabolic abnormality can be given. Regular follow-up with imaging such as ultrasound is required because of the high recurrence rates

Two Cases of Bladder Adenocarcinoma After Augmentation Cystoplasty

To draw attention to the disregarded malignancy risk after ileocystoplasty, we present two cases of adenocarcinoma. The first case was metastatic at initial diagnosis. Despite chemotherapy, the condition progressed and the patient died at the 9th month. The second patient has received cystectomy followed by chemotherapy and radiotherapy. Although the second patient was an immunosuppressed renal transplant, she was disease-free at the 27th month. As the malignancy risk after bladder augmentation is a proven fact, until the discovery of a proper diagnostic method, we recommend doing routine annual cystoscopic biopsy starting after the 10th year of ileocystoplasty

Efficacy of Antibiotic Coated Clean Intermittent Catheterization in Children with Neurogenic Bladder

Aim: The primary goal of urologic management in children with neurogenic bladder is to reduce the risk of urinary tract infection (UTI) and associated renal injury. We aimed to evaluate the use of antibacterial-coated clean intermittent catheterization (CIC) catheters for neurogenic bladder patients in comparison with standard catheters.  Material and Methods: We performed a retrospective study of 144 neurogenic bladder patients aged 6-16 years old, who received CIC at two major centers between January 2007 and June 2016. Group 1 consisted of children used antibacterial coated (chitosan) catheter (n=55), group 2 of children used standard CIC without antibacterial (n=42) and group 3 of children used standard CIC returned into antibiotic coated CIC (n=29). Febrile urinary tract infection and asymptomatic bacteriuria were evaluated among patients with antibacterial coated or standard catheters. We also focused on a subgroup of patients with high risk of urinary tract infection (grade 3> vesicoureteral reflux, previously scar formation in renal scintigraphy).  Results: Totally 126 patients (89 female, 37 male) were involved in this study. The mean age of the study group was 9.6±2.6 years (range 6 to 16) and the mean follow-up 58±14 months (min: 22, max: 69). There was no significant difference between three groups for asymptomatic bacteriuria and febrile UTI frequencies. However, febrile UTI frequencies and de nova scar formation in renal scintigraphy were higher in previously defined subgroup of patients with high risk of urinary tract infection in group 2 than group1 and 3.  Discussion: Both antibiotic coated and standard CIC can be used in children with neurogenic bladder with similar complication rates. Patients with high risk of urinary tract infection (higher than grade 3 vesicoureteral reflux, dilated ureter, previously de nova scar formation in renal scintigraphy) will benefit from antibacterialcoated catheters rather than standard ones

Antiepileptik ilaçların kemik mineralizasyonuna olumsuz etkilerinin patofizyolojisi ve erken tanısı

Bu tezin, veri tabanı üzerinden yayınlanma izni bulunmamaktadır. Yayınlanma izni olmayan tezlerin basılı kopyalarına Üniversite kütüphaneniz aracılığıyla (TÜBESS üzerinden) erişebilirsiniz.63 ÖZET Uzun süreli antiepileptik ilaç kullanımının kemik mineralizasyonuna olumsuz etkileri sonucu sekonder rahitis gelişebilmektedir. Bu çalışmada sekonder rahitisin erken tanısında kemik dansitometresinin (DEXA yöntemi) duyarlılığını ve patofizyolojisinden sorumlu olduğu öne sürülen mekanizmaları araştırmak amaçlanmıştır. Valproat, karbamazepin, fenobarbital tedavisi alması planlanan 30 hastadan tedavi öncesi ve tedavinin 18.ayında serum Ca, P, alkalen fosfataz, parathormon, osteokalsin, kalsitonin, 1.25 (OH)2 D3 ve idrar Ca/Kreatinin, P/Kreatinin düzeyleri bakılmış ve dansitometrik ölçümleri (BMC/BMD) yapılmıştır. Serum Ca düşüklüğü hastaların %20'sinde, alkalen fosfataz yüksekliği %27'sinde bulunurken, DEXA yöntemi ile kemik mineralizasyonunda azalma hastaların %50'sinde saptanmıştır. BMC/BMD değerleri ile yaş, ağırlık ve boy arasında kuvvetli pozitif korelasyon saptanmıştır. BMD ölçümlerinin çocuğun yaşına oranlarak elde edilen tedavi öncesi ve tedavinin 18. ayındaki BMD/yaş oranları karşılaştırıldığında, artması veya en azından azalmaması gereken bu oranların hastaların yarısında azaldığı saptanmıştır. Biyokimyasal parametreler ile (serum Ca, P, alkalen fosfataz, parathormon, osteokalsin, kalsitonin ve idrar Ca/Kreatinin, P/Kreatinin) BMC/BMD değerleri anlamlı bir korelasyon bulunmamıştır. Çalışmaya alınan 16 hastada tedavi öncesi ve tedavinin 18. ayında ölçülen 1.25 (OH)2 D3 düzeyleri karşılaştırıldığında tedavi ile anlamlı bir düşme olduğu saptanmıştır. Bu bulgu antiepileptik ilaçların kemik mineralizasyonuna olumsuz yan etkilerinin gelişiminde 1.25 (OH)2 D3 eksikliğinin önemli bir rolü olduğunu düşündürmüştür. Hastalarımızda günde 400 ünite D vitamini profilaksisine rağmen, yüksek oranda BMD azalması saptanması bu problemin önemine dikkat çekmiştir. Antiepileptik ilaç alan çocuklarda biyokimyasal inceleme yöntemleri yanısıra kemik dansitometresi ile anterior posteriordan L2-4 seviyesinde kısasüreli yıllık BMC/BMD ölçümleri yapılmasının gelişebilecek sekonder rahitisin erken tanısı için uygun olacağı sonucuna varılmıştı

KORPUS KALLOSUM ANOMALİLERİ

SEREBRAL HEMİSFERLER ARASINDAKİ TEMEL BİRLEŞTİRİCİ YOL OLAN KORPUS KALLOSUM (KK) 10. VE 20. GESTASYONEL HAFTALAR BOYUNCA GELİŞİR

Çocuk acil serviste Konvülzif Status Epileptikus'un yönetimi: Yeni Ege Pediatrik Status Epileptikus Protokolü (YEPSEP)

Amaç: Ege Üniversitesi Çocuk Hastanesi Acil Servis Ünitesinde pediatrik konvülzif status epileptikus (SE) tanılı çocuk hastalarin yönetiminde Yeni Ege Pediatrik Status Epileptikus Protokolü (YEPSEP) etkinliğini değerlendirmek. Hastalar ve Yöntemler: Bu retrospektif tez çalışmasında Ege Üniversitesi Tıp Fakültesi'nde 01 Ocak 2016–31 Aralık 2019 tarihleri arasında nöbet nedeni çocuk acil servise başvuran tüm hastaların tıbbi kayıtları incelendi. SE tanısı konulan ve YEPSEP uygulanan 300 çocuk hasta ( 171 kız , 129 erkek ; yaş ortalaması 60 ay) çalışmaya dahil edildi. Nöbet tipi, süresi, etyolojisi, antikonvülzan ilaç uygulamaları, yan etkileri, komplikasyonlar, yatış süreleri ve hastaların prognozları değerlendirildi. Bulgular: Çalışma örnekleminde yaş grupları dağılımı aşağıdaki şekilde oluşmuştur: 1 ay-1; %10, 1-5 yaş: %45.3, 5-10 yaş: %24.3, >10 yaş: %20. Uzamış nöbet tanılı 7 hasta (%2.3) çıkartıldığında SE'lı olguların dağılımı %43.3'ü erken SE , %30.0'ı gerçekleşmiş SE, %13.0'ı refrakter SE, %11.3'ü superrefrakter SE olarak gerçekleşmiştir. Ortanca nöbet süresi 23 dk olarak bulundu (IQR 11 – 63 dk, minimum 5 – maksimum 165 dk). Etyolojiye göre hastaların %43.0'ı "idiopatik/kriptojenik", %4.0'ı "akut semptomatik", %19.3'ü "uzak semptomatik", %14.3'ü "progresif ensefalopati" ve %19.3'ünde de "febril SE" olarak tanımlandı. Çalışmaya alınan hastaların %72.7'sinde en az bir kronik hastalığı olduğu görüldü; epilepsi: %69.0, nörometabolik hastalık : %18.0, intrakraniyal kanama + VPŞ: %10.0 , serebral felç : %2.7. Kronik hastalığı olanlarda istatistiksel olarak anlamlı derecede daha yüksek refrakter SE geliştiği görüldü (p=0.033). YEPSEP protokolünde yer alan 1. Basamak ilaç uygulamaları ile hastaların %37'sinde SE sonlandırılabilmiştir. Tedaviye yanıt alınamayan 189 (%63.0) hastada ikinci basamak tedaviye geçilmiştir. İkinci basamakta en sık tercih edilen ilaç levetirasetam (%58.2) olmuştur. Difenilhidantion ve valproik asid infüzyonları sırası ile %22.8 ve %19.0 oranlarında uygulanmıştır. İkinci basamak ilaç tedavi uygulaması ile hastaların %85.3'ünde SE kontrolü sağlanmıştır. Üçüncü basamak tedavi %14.7 hastaya uygulanmış ve SE sonlandırılmıştır. YEPSEP tedavi basamakları değerlendirildiğinde hastaların %25.3'ünde komplikasyon gerçekleştiği ve bunların çoğunun solunum depresyonu olduğu görüldü . Hastaların %59.3'ü acil serviste izlem yapılıp taburcu edilirken %27.7'si servise, %13.0'ı da çocuk yoğun bakım ünitesine yatırılarak izlenmiştir. Çalışma örnekleminde mortalite oranı %0.7 olarak belirlendi. Acil serviste YEPSEP protokolu ile yönetilen hasta grubumuzda yeni epilepsi tanısı erken dönem izlemde %14.7 olarak gerçekleşmiştir. Sonuç: Pediatrik SE uygun tedavi protokolleri ile yönetildiğinde başarılı bir şekilde sonlandırılabilmekte ve mortalite oranları düşmektedir. YEPSEP bu retrospektif kesitsel çalışmada etkin bir kurumsal SE tedavi protokolü olarak değerlendirilmiştir. YEPSEP' in etkinliği ve güvenilirliği EEG monitorizasyonu eşliğinde prospektif ve randomize kontrollü yeni klinik çalışmalar ile desteklenmelidir.Aim: We aimed to evaluate the effectiveness of the New Ege Pediatric Status Epilepticus Protocol (NEPSEP) in the management of pediatric patients diagnosed with pediatric convulsive status epilepticus (SE) in the Emergency Department (ED) of Ege University Children's Hospital. Material ; Methods: We enrolled all patients admitted to Ege University Medical Faculty of Pediatric Emergency Department with seizures between 01 January 2016–31 December 2019. Three-hundred pediatric patients (171 female, 129 males; mean age 60 months) diagnosed with SE and undergoing NEPSEP were included in the study. Seizure type, duration, etiology, anticonvulsant drug administration, side effects, complications, length of stay and prognosis were evaluated. Results: The distribution of age groups in the study sample was as follows: 10% of patients were one month-1 year; 45.3% 1-5 years; 24.3% 5-10 years, and 20% were > 10 years old. Seven patients (2.3%) diagnosed with prolonged seizures were excluded,. The distribution of SE cases was 43.3% early SE, 30.0% established SE, 13.0% refractory SE, 11.3% superrefractory SE. The median seizure duration was 23 minutes (IQR 11 - 63 min, min 5 - max 165 min). According to the etiology, 43.0% of the patients were defined as "idiopathic / cryptogenic", 4.0% "acute symptomatic", 19.3% "remote symptomatic", 14.3% "progressive encephalopathy" and 19.3% were "febrile SE". It was observed that 72.7% of the patients had one chronic disease at least; 69.0% epilepsy, 18.0% neurometabolic disease, 10.0% intracranial hemorrhage + VPS, and 2.7% were cerebral stroke. Children with refractory SE has significantly higher rate of chronic disease (p = 0.033). In the NEPSEP protocol, SE could be terminated in 37% of the patients with the first-step drug. Second-line treatment was started in 189 (63.0%) patients who did not respond to treatment. The most preferred drug in the second step was levetiracetam (58.2%). Diphenylhidantion and valproic acid infusions were administered at rates of 22.8% and 19.0%, respectively. SE control was achieved in 85.3% of the patients with the application of second-line treatment. Third-line treatment was applied to 14.7% of patients, and SE was discontinued. We evaluated NEPSEP steps, and the complications were seen in 25.3%, and most of them had respiratory depression. While 59.3% of the patients were followed up in the ED and discharged, 27.7% were admitted to the ward, and 13.0% pediatric intensive care unit. Although the mortality rate was 0.7%, the new diagnosis of epilepsy rate was 14.7% in the early follow-up. Conclusion: If the pediatric SE is managed with appropriate treatment protocols, it can be successfully terminated, and mortality rates decrease. We defined that the NEPSEP was an effective institutional SE treatment protocol in this retrospective cross-sectional study. The effectiveness and reliability of NEPSEP should be supported by new prospective and randomized controlled clinical studies accompanied by EEG monitoring