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Item Sustained hyperferritinemia in a child with macrophage activation syndrome secondary to systemic juvenile idiopathic arthritis - perforinopathy: case based review(TURKISH J PEDIATRICS, 2018-01-01) Cakan, Mustafa; Aktay-Ayaz, Nuray; Gemici, Hakan; Annayev, Agageldi; Citak, Agop; Akcay, Arzu; Ozturk, GulyuzSystemic juvenile idiopathic arthritis is a subtype of juvenile idiopathic arthritis and characterized by arthritis and many systemic features like fever, rash, hepatosplenomegaly, lymphadenopathy and serositis. Macrophage activation syndrome is the most dreadful complication of systemic juvenile idiopathic arthritis and can cause mortality and morbidity if not recognized and treated early and aggressively. Hemophagocytic lymphohistiocytosis (HLH) is characterized by diminished or absent activities of natural killer cells and cytotoxic T lymphocytes leading to cytokine storm and uncontrolled activation of T cells and macrophages. Primary (familial) HLH is a group of autosomal recessive disorders caused by mutations in the perforin and other related genes and distinctive for onset during early infancy and high rate of mortality. Secondary HLH may be caused by infectious, oncologic and rheumatologic disorders. The term Perforinopathy is used to describe cases with classical familial HLH and also for cases with familial HLH gene mutations but not following a classical familial HLH course. Herein we report a case of chronic perforinopathy in which clinical symptoms started with systemic juvenile idiopathic arthritis and severe macrophage activation syndrome that needed plasma exchange and extracorporeal membrane oxygenation during acute period and ongoing interleukin-1 blockage for sustained hyperferritinemia.Item The clinical characteristics and prognosis of pertussis among unvaccinated infants in the pediatric intensive care unit(TURKISH PEDIATRICS ASSOC, 2020-01-01) Sik, Guntulu; Demirbuga, Asuman; Annayev, Agageldi; Citak, AgopAim: To evaluate the clinical characteristics, risk factors, and prognosis of pertussis in the pediatric intensive care unit. Material and Methods: Patients who were hospitalized in pediatric intensive care unit between January 2017 and January 2019 and diagnosed as having pertussis were retrospectively evaluated. Samples were taken from tracheal aspirate material in intubated patients and nasopharyngeal swabs in the other patients. Samples for Bordetella pertussis were studied using multiplex real-time polymerase chain reaction. Results: Eighteen patients were admitted to our pediatric intensive care unit with a diagnosis of pertussis. Ten patients were female (55.5\%), and all patients were unvaccinated. The median age was 40 (range, 38-47.5) days and the median intensive care unit stay was 9 (range, 5-14) days. All patients had respiratory distress, 14 patients had cough (77.7\%), four patients had fever (22.2\%), and three patients had convulsions (16.6\%). Seven patients were intubated. Three patients died of multiple organ failure and cardiogenic shock despite extracorporeal treatment. Respiratory syncytial virus was found in two patients and rhinovirus was found in one patient. The median leukocyte count was significantly higher in non-survivors than in survivors. Blood exchange was performed in three patients due to hyperleukocytosis. Pulmonary hypertension was the most common cardiac pathology detected in echocardiographic examinations. Conclusion: We found that high leukocyte count, viral co-infection, and severe pulmonary hypertension were associated with mortality and morbidity in pertussis.Item Extracorporeal membrane oxygenation for the support of pediatric patients with acute fulminant myocarditis(TURKISH J PEDIATRICS, 2019-01-01) Sik, Guntulu; Annayev, Agageldi; Demirbuga, Asuman; Deliceo, Elif; Aydin, Selim; Erek, Ersin; Demir, Halil Ibrahim; Citak, AgopAcute fulminant myocarditis, is a severe, rapidly progressive disease. The clinical outcomes of children with severe acute myocarditis who are resist to medical treatment is not well known. We studied the clinical courses of patients with acute fulminant myocarditis supported by extracorporeal membrane oxygenation (ECMO). We performed a retrospective chart review of six children with acute fulminant myocarditis who were treated with ECMO. Demographic information, clinical and vital signs, as well as laboratory results were investigated. The median age of 63 months (13-140 months), the mean ECMO duration was 164 hours (79-402 hours), and median intensive care unit stay was 24 days. The most common symptoms were chest pain (66\%) and fever (66\%). Severe arrhythmia were seen in two patients. One patient received extracorporeal cardiopulmonary resuscitation. In two patients, right femoral arteries and right femoral veins were used, in others, right common carotid artery and right internal jugular veins were used. Five patients (83.3 \%) survived to discharge. ECMO can be used effectively in pediatric patients with acute fulminant myocarditis to support the circulation while awaiting myocardial recovery. Timely use of ECMO can improve the survival rate and may be associated with better outcomes.Item Viral Infections Among Patients with Acute Lower Respiratory Tract Infections in the Pediatric Intensive Care Unit(AVES YAYINCILIK, IBRAHIM KARA, 2020-01-01) Sik, Guntulu; Demirbuga, Asuman; Annayev, Agageldi; Cabiri, Asli; Deliceo, Elif; Citak, AgopObjective: We aimed to determine the frequency of viral pathogen and clinical characteristics of patients hospitalized in the pediatric intensive care unit with the diagnosis of acute lower respiratory tract infection. Material and Methods: Eighty patients with laboratory-confirmed viral infections among children admitted to the pediatric intensive care unit (PICU) between November 2016 and September 2017 with a suspicion of viral infection were included. Diagnosis was made using a respiratory viral panel including adenovirus (AV), metapneumovirus (MV), parainfluenza virus (PIV) 1, PIV 2, PIV 3, PIV 4, influenza virus (IV) A and IVB, rhinovirus (RV), respiratory syncytial virus (RSV) A and RSV B, and multiplex polymerase chain reaction (PCR). Tracheal aspiration specimens were obtained from intubated patients and nasopharyngeal swab specimens were obtained from the remaining patients. Results: A total of 514 children were admitted to our PICU. Of 123 patients with lower respiratory tract infection, specimens from a nasopharyngeal swab or tracheal aspiration were obtained and tested using a viral infection panel and multiplex PCR. Mean age of the patients was 6.1 +/- 3.6 months, 60\% (n= 48) of the children were boys. Ninety-three positive results were obtained from 80 patients. The most common viral pathogens were RSV (A + B) (n= 36, 45\%), RV (n= 26, 32.5\%), PIV 1 (n= 7, 8.7\%), AV (n=6, 7.5\%), human MV (n= 5, 6.296), IVA (n= 4, 5\%), and IVB (n= 4, 5\%). The most common dual infection was RV and RSV B. Viral pathogen detection was the highest in december (n= 15) and february (n= 13). High-flow oxygen therapy was needed in 57.5\% of patients, and 12.5\% had non-invasive mechanical ventilation. Twenty-seven (33.7\%) patients had to be intubated due to insufficient ventilation with noninvasive methods. The most commonly detected viral pathogen among the intubated patients was RSV B. The average intensive care length of stay for all children was 10.1 +/- 3.6 days. Conclusion: Viral infections are common among critically ill children in PICUs. Hospital length of stay, morbidity, and mortality increase with underlying chronic diseases or dual infections. Early diagnosis of viral infections decreases unnecessary antibiotic use.Item Clinical and laboratory predictors of survival for pediatric patients on non-postcardiotomy extracorporeal membrane oxygenation (ECMO)(TURKISH J PEDIATRICS, 2020-01-01) Sik, Guntulu; Demirbuga, Asuman; Annayev, Agageldi; Temur, Bahar; Aydin, Selim; Demir, Halil Ibrahim; Erek, Ersin; Citak, AgopExtracorporeal membrane oxygenation (ECMO) is used in pediatric patients with severe cardiopulmonary failure who do not respond to conventional therapy