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Öğe A case with Wolf-Parkinson-White syndrome first presented with a devastating event: aborted sudden cardiac death(Turkish Soc Cardiology, 2009) Uelger, Zuelal; Karapinar, Buelent; Akyol, Bedir; Arslan, Mehmet Tayyip; Levent, Ertuerk; Oezyuerek, Arif RuhiÖğe Chronic inflammatory demyelinating polyradiculopathy: an atypical pediatric case complicated with phrenic nerve palsy(Turkish J Pediatrics, 2007) Polat, Muzaffer; Tosun, Ayse; Serdaroglu, Guel; Caglayan, Engin; Karapinar, Buelent; Goekben, Sarenur; Tekguel, HasanAn atypical form of chronic inflammatory demyelinating polyneuropathy (CIDP) complicated with phrenic nerve palsy is presented with clinical and electrophysiologic features. A seven-year-old girl had initial presentation mimicking Guillain-Barre syndrome based on electrophysiologic characteristics. Between 7-11 years of age, she had five recurrences of subacute onset of weakness which usually developed over at least 2-4 months and progressed to loss of ambulation and to respiratory insufficiency. Radiologic examinations revealed unilateral phrenic nerve palsy associated with CIDP. Our patient demonstrated the rare association of CIDP and phrenic nerve palsy, resulting in diaphragmatic paralysis and respiratory failure.Öğe Single-center experience: Use of recombinant factor VIIa for acute life-threatening bleeding in children without congenital hemorrhagic disorder(Taylor & Francis Inc, 2008) Yilmaz, Deniz; Karapinar, Buelent; Balkan, Can; Akisue, Mete; Kavakli, KaanCoagulopathy is an important cause of mortality in critically ill children. Traditional therapies to correct coagulopathy lead to great time delays and cause fluid overload in patients. The authors report the effectiveness and safety of the activated recombinant factor VII (rFVIIa) administration in a series of 13 nonhemophiliac children with acute, life-threatening bleeding. In this retrospective study, the records of the patients who were not diagnosed with congenital hemorrhagic disorder and were administered rFVIIa due to any other reason in Ege University Faculty of Medicine, Department of Pediatrics, between February 2002 and February 2007 were reviewed retrospectively. Thirteen nonhemophiliac patients with acute life-threatening bleeding and ages ranging from 2 days to 15 years received rFVIIa over a 5-year period. Three patients were diagnosed with hemaphagocytic lymphohistiocytosis, 4 with prematurity, sepsis, and disseminated intravascular coagulation (DIC), 5 with sepsis, multiple organ dysfunction syndrome, and DIC, and 1 with acute liver failure. Severe bleeding resulted from pulmonary (n=3), lower gastrointestinal system (n=2), esophagus varices (n=1), pulmonary and gastrointestinal system (n=4), pulmonary, gastrointestinal system, and intracranial hemorrhage (n=1), and gastrointestinal system and intracranial hemorrhage (n = 2). Median frequency of rFVIIa administration was 3 per patient (range 2{15) and median dose of rFVIIa was 90 g/kg, ranging from 60 to 135 g/kg each administration. All of the patients were given fresh frozen plasma and if necessary platelet transfusion (n = 10) or fibrinogen concentrate (n = 3) before administration of rFVIIa. In 6 patients, lack of success to control bleeding by conventional methods was the only cause to start rFVIIa. In 7 patients, the need for volume restriction was also a significant contributing factor in deciding to start rFVIIa. Median PT was 32.9 s (range: 19{65) before rFVIIa administration and it was decreased to 11.6 s (range: 10.7{12.8), 2{3 h after rFVIIa infusion. Bleeding was stopped completely in 10 patients at least for 24 h and decreased in 3 patients 30{45 min after rFVIIa administration. Two patients had thrombotic complications attributed to rFVIIa administration. No other complication was observed in the other patients. In this retrospective study, rFVIIa was found to be effective at controlling severe hemorrhagic symptoms of different etiologies in children without congenital hemorrhagic disorder. In addition to the rapid control of bleeding, administration of this agent improved fluid balance and led to a reduction in blood product requirements in critically ill children. However, survival was still poor (23%), and 2/13 (15.4%) patients developed venous and arterial thrombosis within 3 h of treatment. The authors emphasize that in acquired, acute life-threatening bleeding, simultaneous administration of rFVIIa with conventional treatment may contribute to patient survival. However, the risk of thromboembolism should be considered before this treatment is given.Öğe An unusual cause of multiple organ dysfunction syndrome in the pediatric intensive care unit: Hemophagocytic lymphohistiocytosis(Lippincott Williams & Wilkins, 2009) Karapinar, Buelent; Yilmaz, Deniz; Balkan, Can; Akin, Mehmet; Ay, Yilmaz; Kvakli, KaanObjective: To report our experience in children with primary or secondary hemophagocytic lymphohistiocytosis (HLH) presented with multiple organ dysfunction syndrome (MODS) in pediatric intensive care unit (PICU). Design: The records of patients with a diagnosis of HLH and MODS between January 2005 and January 2008 were reviewed. The patients' characteristics, treatment modalities, and outcomes were assessed. Setting: PICU of Ege University Hospital. Patients/Subjects: Twelve children who were hospitalized in the PICU met the diagnostic criteria for HLH, and presented with MODS were entered into the study. Results: The median age of the patients was 3 years (range, 2 months-15.5 years). Six patients had a history of parental consanguinity and two had an affected sibling. Five of the patients were classified as primary HLH. All of the patients had hepatosplenomegaly, elevated ferritin levels, hypofibrinogenemia, anemia, thrombocytopenia, and hemophagocytosis in bone marrow examination at presentation. The median Pediatric Logistic Organ Dysfunction score of the patients at onset was 51 (range, 12-62). Four patients had six, four had five, two had four, and the remaining two had three organ dysfunctions. Organ dysfunction, other than hematologic dysfunction which was present in all patients, was most commonly seen in hepatic (n = 11, 91.7%), respiratory (n = 11, 91.7%), and cardiovascular systems (n = 10, 83.3%). Although nine patients showed neurologic dysfunction including convulsion and coma, renal failure was detected in five patients. Eleven patients were supported with mechanical ventilation and four patients required hemodialysis. Eight patients were treated according to the HLH 2004 treatment protocol, consisting of cyclosporine A, etoposide, and dexamethasone. The remaining four patients received only intravenous immunoglobulin and supportive treatment. Seven of the patients died. Conclusion: HLH is a frequently lethal disease and with a clinical presentation similar to severe sepsis, MODS, disseminated intravascular coagulation, or septic shock, which are frequent diagnoses in the PICU. In the PICU, HLH should be considered in the case of prolonged fever, splenomegaly, cytopenia, and MODS. It is important for pediatricians and particularly pediatric intensivists to know the diagnostic criteria and possible clinical presentations of HLH so treatment is initiated promptly. (Pediatr Crit Care Med 2009; 10:285-290)
