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Publication Open Access A clinical scoring system to predict the development of bronchopulmonary dysplasia(Thieme Medical Publishers, 2015) Hayran, Mutlu; Derin, Hatice; Ovalı, Fahri; N/A; Gürsoy, Tuğba; Faculty Member; School of Medicine; 214691Objective: This study aims to develop a scoring system for the prediction of bronchopulmonary dysplasia (BPD). Methods: Medical records of 652 infants whose gestational age and birth weight were below 32 weeks and 1,500g, respectively, and who survived beyond 28th postnatal day were reviewed retrospectively. Logistic regression methods were used to determine the clinical and demographic risk factors within the first 72 hours of life associated with BPD, as well as the weights of these factors on developing BPD. Predictive accuracy of the scoring system was tested prospectively at the same unit. ResultsBirth weight, gestational age, gender, presence of respiratory distress syndrome, patent ductus arteriosus, intraventricular hemorrhage, hypotension were the most important risk factors for BPD. Therefore, a scoring system (BPD-TM score) ranging from 0 to 13 and grouped in four tiers (0-3: low, 4-6: low intermediate, 7-9: high intermediate, and 10-13: high risk) was developed based on these factors. Below the score of 4, 4.1% of infants (18/436), above the score of 9, 100% (29/29) of the infants developed BPD. The score was validated successfully in 172 infants. Conclusion: With this easy to use scoring system, one can predict the neonate at risk for BPD at 72 hours of life and direct preventive measures toward these infants.Publication Open Access A novel and simple machine learning algorithm for preoperative diagnosis of acute appendicitis in children(Springer, 2020) Türkmen, İnan Utku; Namlı, Gözde; Özturk, Çiğdem; Esen, Ayşe B.; Eray, Y. Nur; Akova, Fatih; Aydın, Emrah; Eroğlu, Egemen; Faculty Member; faculty Member; School of Medicine; 32059; N/AIntroduction: there is a tendency toward nonoperative management of appendicitis resulting in an increasing need for preoperative diagnosis and classification. For medical purposes, simple conceptual decision-making models that can learn are widely used. Decision trees are reliable and effective techniques which provide high classification accuracy. We tested if we could detect appendicitis and differentiate uncomplicated from complicated cases using machine learning algorithms. Materials and methods: we analyzed all cases admitted between 2010 and 2016 that fell into the following categories: healthy controls (Group 1); sham controls (Group 2); sham disease (Group 3), and acute abdomen (Group 4). The latter group was further divided into four groups: false laparotomy; uncomplicated appendicitis; complicated appendicitis without abscess, and complicated appendicitis with abscess. Patients with comorbidities and whose complete blood count and/or pathology results were lacking were excluded. Data were collected for demographics, preoperative blood analysis, and postoperative diagnosis. Various machine learning algorithms were applied to detect appendicitis patients. Results: there were 7244 patients with a mean age of 6.84 +/- 5.31 years, of whom 82.3% (5960/7244) were male. Most algorithms tested, especially linear methods, provided similar performance measures. We preferred the decision tree model due to its easier interpretability. With this algorithm, we detected appendicitis patients with 93.97% area under the curve (AUC), 94.69% accuracy, 93.55% sensitivity, and 96.55% specificity, and uncomplicated appendicitis with 79.47% AUC, 70.83% accuracy, 66.81% sensitivity, and 81.88% specificity. Conclusions: machine learning is a novel approach to prevent unnecessary operations and decrease the burden of appendicitis both for patients and health systems.Publication Open Access A rare case of juvenile amyotrophic lateral sclerosis(Turkish National Pediatric Society, 2021) Bodur, Muhittin; Toker, Rabia Tütüncü; Okan, Mehmet Sait; Başak, Ayşe Nazlı; Faculty Member; Koç University Research Center for Translational Medicine (KUTTAM) / Koç Üniversitesi Translasyonel Tıp Araştırma Merkezi (KUTTAM); School of Medicine; 1512Background: amyotrophic lateral sclerosis (ALS) is a chronic motor neuron disease characterised by progressive weakness in striated muscles resulting from the destruction of neuronal cells. The term juvenile ALS (JALS) is used for patients whose symptoms start before 25 years of age. JALS may be sporadic or familial. Case: here, we present a sporadic case of JALS because of its rarity in children. The heterozygous p.Pro525Leu (c.1574C>T) variation was identified in the fused in sarcoma (FUS) gene. Conclusion: the p.Pro525Leu mutation in the FUS gene has been detected in patients with ALS, characterised by early onset and a severely progressive course.Publication Metadata only A rare complication after an interventional procedure using the common carotid: carotid pseudoaneurysm in an infant(Cambridge University Press (CUP), 2023) N/A; Biçer, Mehmet; Kızılkaya, Mete Han; Ödemiş, Ender; Gündoğmuş, Cemal Aydin; Faculty Member; Doctor; Faculty Member; Doctor; School of Medicine; N/A; School of Medicine; N/A; Koç University Hospital; 310599; N/A; 194545; N/AThe carotid artery is a valuable vascular access that can be used in patients who have undergone repetitive interventional and surgical procedures and premature babies. In the past, cut-down was used but nowadays, mostly the procedure is performed under ultrasonographic guidance. Complications such as bleeding, haematoma, and pseudoaneurysm may occur when the carotid artery is used as a vascular access for the procedures such as aortic balloon valvuloplasty, coarctation balloon angioplasty, or after interventional or surgical treatments to the carotid artery. Although pseudoaneurysm is very rare, prompt diagnosis and accurate treatment planning are life-saving. In this article, the diagnosis and treatment of pseudoaneurysm in the left common carotid after transcatheter coarctation balloon angioplasty in a 6-month-old infant will be presented.Publication Open Access A rare presentation of adrenal adenoma in infancy: isolated Cushing's syndrome(Turkish National Pediatric Society, 2020) Yeğen, Gülçin; Şal, Oğuzhan; Oğuzkurt, Nigar Pelin; Undergraduate Student; School of MedicineAdrenocortical tumors are rare in children. Most of these tumors present with endocrinological manifestations, majority of which with virilizing features alone or in combination with over production of other adrenal hormones. However, it is uncommon of an adrenocortical tumor to present solely with Cushing's syndrome. In this paper we discuss the clinical presentation and management of a 5-month-old infant who had presented with Cushing's syndrome due to a functioning adrenocortical adenoma without androgen and mineralocorticoid excess, and made a brief review on the clinical and histopathological characteristics of adrenocortical tumors.Publication Open Access A single-center report of COVID-19 disease course and management in liver transplanted pediatric patients(Wiley, 2021) Yüksel, Muhammed; Aktürk, Hacer; Mızıkoğlu, Özlem; Toroslu, Ertuğ; Arıkan, Çiğdem; Researcher; Faculty Member; Researcher; Faculty Member; Koç University Research Center for Translational Medicine (KUTTAM) / Koç Üniversitesi Translasyonel Tıp Araştırma Merkezi (KUTTAM); School of Medicine; Koç University Hospital; N/A; N/A; N/A; N/A; 240198Background: in 2019, SARS-CoV-2 causing COVID-19 emerged. Severe COVID-19 symptoms may evolve by virtue of hyperactivation of the immune system. Equally, immunocompromised patients may be at increased risk to develop COVID-19. However, treatment guidelines for children following liver transplantation are elusive. Methods: as a liver transplantation center, we diagnosed and followed up 10 children (male/female: 8/2) with a median age of 8.5 years (IQR: 5.2-11.0), with COVID-19 post-liver transplant between March 2019 and December 2020. COVID-19 diagnosis was based on PCR test and or florid X-ray findings compatible with COVID-19 in the absence of other cause. We retrospectively collected clinical and laboratory data from electronic patient records following written consent from patients/parents. Results: nine patients were diagnosed as definitive (PCR positive) with one patient being diagnosed as probable COVID-19. Seven patients recovered without any support whereas three were admitted for non-invasive oxygenation. Lymphopenia and/or high levels of serum IL-6 were detected in four patients. Six patients mounted anti-SARS-CoV-2 antibodies at median 30 days (IQR: 26.5-119.0) following COVID-19 diagnosis. Antibiotic therapy, favipiravir, anakinra, and IVIG were used as treatment in 4,1,1 and 2 patients, respectively. Furthermore, we kept the tacrolimus with or without everolimus but stopped MMF in 2 patients. Importantly, liver allograft function was retained in all patients. Conclusions: we found that being immunocompromised did not affect disease severity nor survival. Stopping MMF yet continuing with tacrolimus was an apt treatment modality in these patients.Publication Open Access A systematic review of recent and ongoing clinical trials in patients with the neurofibromatoses(Elsevier, 2022) Bedolla, Edwin Nieblas; Armstrong, Amy E.; Hirbe, Angela C.; Acar, Simge; Undergraduate Student; School of MedicineIntroduction: the neurofibromatoses comprise three different genetic conditions causing considerable morbidity and mortality: neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis (SWN). This review summarizes recent and ongoing clinical trials involving patients with neurofibromatoses to better understand the current state of clinical trial research centered around these conditions and inform areas of need. Methods: a search was conducted using the Cochrane Central Register of Controlled Trials and clinicaltrials.gov databases. Inclusion and exclusion criteria were designed to identify clinical trials focused on patients with NF1, NF2, or SWN completed in or after 2010 and in process as of December 31, 2021. Information was collected using standardized guidelines. Results: a total of 134 clinical trials were included, with 75 (56%) completed and 59 (44%) in process. For completed trials, 74% (n = 56) involved patients with NF1, and of those based on specific tumors (n = 26, 46%), the majority focused on plexiform neurofibromas (PNs) (n = 12, 46%). For ongoing trials, 79% (n = 47) involve patients with NF1, and of those based on specific tumors (n = 29, 61%), the majority are focused on PNs (n = 13, 45%). Conclusion: both recent and ongoing clinical trials have primarily focused on patients with NF1 and the treatment of PNs. This research has led to the first FDA-approved drug for NF1-PN and has changed management of these tumors, allowing for systemic therapy rather than reliance on only a surgical modality. Trials evaluating comorbid psychiatric conditions and quality of life among patients with any of the neurofibromatoses appear less common. These areas may warrant focus in future studies to improve clinical management.Publication Open Access Allergen immunotherapy for IgE-mediated food allergy: protocol for a systematic review(BioMed Central, 2016) Dhami, S.; Nurmatov, U.; Pajno, G. B.; Fernandez-Rivas, M.; Muraro, A.; Roberts, G.; Akdiş, C.; Alvaro-Lozano, M.; Beyer, K.; Bindslev-Jensen, C.; Burks, W.; du Toit, G.; Ebisawa, M.; Eigenmann, P.; Knol, E.; Makela, M.; Nadeau, K. C.; O'Mahony, L.; Papadopoulos, N.; Poulsen, L.; Sampson, H.; Santos, A.; van Ree, R.; Timmermans, F.; Sheikh, A.; N/A; Saçkesen, Cansın; Faculty Member; School of Medicine; 182537Background: The European Academy of Allergy and Clinical Immunology (EAACI) is in the process of developing the EAACI Guidelines for Allergen Immunotherapy (AIT) for IgE-mediated food allergy. We seek to critically assess the effectiveness, cost-effectiveness and safety of AIT in IgE-mediated food allergy. Methods: We will undertake a systematic review, which will involve searching international biomedical databases for published, in progress and unpublished evidence. Studies will be independently screened against pre-defined eligibility criteria and critically appraised using established instruments. Data will be descriptively and, if possible and appropriate, quantitatively synthesised. Discussion: The findings from this review will be used to inform the development of recommendations for EAACI's Guidelines on AIT.Publication Open Access Ambulatory arterial stiffness index is increased in obese children(Turkish National Pediatric Society, 2020) Gayret, Özlem Bostan; Taşdemir, Mehmet; Erginöz, Ergin; Bilge, İlmay; Doctor; Undergraduate Student; Faculty Member; School of Medicine; N/A; N/A; 198907Background and objectives: one way to measure arterial stiffness is the ambulatory arterial stiffness index (AASI), which is the relationship between diastolic and systolic ambulatory blood pressure (BP) over 24-hours. Methods: we studied the difference in AASI between obese and lean children. AASI was calculated from 24-hour ambulatory blood pressure monitoring in 53 obese children (33 girls) and compared with age-matched 42 healthy subjects (20 girls). Hypertension was defined according to the criteria of the American Heart Association. To evaluate inflammation, the blood level of high-sensitive C-reactive protein was measured. Results: the mean age was 10.6 +/- 2.83 years in obese children and 11.3 +/- 3.17 years in healthy subjects. Hypertension was determined in three (5.6%) obese children. The median heart rate-SDS, pulse pressure and blood pressure values did not differ between the two groups. The mean AASI was significantly higher in obese children compared to healthy subjects (0.42 +/- 0.15 vs. 0.29 +/- 0.18, p <0.001). AASI significantly correlated with nighttime SBP-SDS, nighttime SBP-load, systolic and diastolic nocturnal dipping, with no independent predictor. Conclusion: this study confirms that AASI is increased in obese children. AASI calculation is a useful, cost-effective, and an easy method to evaluate arterial stiffness. Early detection of increased arterial stiffness can help clinicians come up with preventive measures in the management of patients.Publication Metadata only Ambulatory blood pressure correlates with MRI-based renal volume in children and adolescents with autosomal dominant polycystic kidney disease(Springer, 2022) Yilmaz, Kubra; Saygili, Seha; Akgun-dogan, Ozlem; Yildirim, Zeynep Nagehan Yuruk; Cicek, Rumeysa Yasemin; Oner, Huseyin Adil; Aksu, Bagdagul; Akyel, Nazli Gulsum; Oguzhan, Ozge; Dursun, Hasan; Yavuz, Sevgi; Cicek, Neslihan; Agbas, Ayse; Nayir, Ahmet Nevzat; Konukoglu, Dildar; Kurugoglu, Sebuh; Sever, Lale; Canpolat, Nur; Caliskan, Salim; N/A; Akıncı, Nurver; Doctor; N/A; Koç University Hospital; N/AN/A