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Öğe Evaluation of Clinical and Immunological Characteristics of Children with Common Variable Immunodeficiency(Hindawi Ltd, 2018) Alkan, Gulsum; Keles, Sevgi; Reisli, IsmailBackground. Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder (PID) that typically presents with hypogammaglobulinemia and impaired antibody production. Objectives. This study aimed to promote the awareness of CVID, whose clinical spectrum is quite broad. Methods. The demographic, clinical, and laboratory characteristics of 12 children (seven males and five females) with CVID were analyzed retrospectively. The patients were diagnosed using the diagnostic criteria of the European Society for Primary Immunodeficiencies. Results. The median disease onset age was 7.2 +/- 4.1 years, and the mean diagnosis age was 11.6 +/- 3.7 years. The diagnosis delay was 4.3 +/- 2.6 years, and the parental consanguinity rate was 75%. Most patients presented with recurrent infections, including upper respiratory tract infections (n = 8), lower respiratory tract infections (n = 9), and gastroenteritis (n = 5). In addition, growth retardation (n = 9) and bronchiectasis (n = 5) were common comorbidities. Two patients presented with autoimmune thrombocytopenia and anemia, and one patient exhibited lung empyema. All the patients had immunoglobulin G deficiencies. Conclusion. CVID is a heterogeneous disease, so the diagnosis is frequently delayed. In the CVID patients with pulmonary complications, relationships were seen with the diagnosis delay, symptom onset age, and lung infection prevalence. Overall, the early diagnosis and treatment of PIDs can preclude life-threatening complications.Öğe Posterior reversible encephalopathy syndrome in children: a case series(Aves, 2016) Emeksiz, Serhat; Kutlu, Nurettin Onur; Caksen, Huseyin; Alkan, Gulsum; Yikmaz, Hulya Seker; Tokgoz, HuseyinPosterior reversible encephalopathy syndrome is characterized by hypertension, seizure, headache, clouding of consciousness, and visual disturbance, and is diagnosed in the presence of typical lesions on magnetic resonance imaging. We retrospectively evaluated five patients who were diagnosed as having posterior reversible encephalopathy syndrome and followed up in Meram Medical Faculty, Pediatric Intensive Care and Hematology wards, between January 2010 and January 2014. We reviewed the demographic and clinical data, and neuroimaging findings. The primary diseases of the subjects included acute lymphocytic leukemia (n=2), Henoch-Scholein purpura (n=1), systemic lupus erythematous (n=1), and acute poststreptococcal glomerulonephritis (n=1). The mean age was 10 +/- 4.58 years (range, 5-14 years). Acute elevation of blood pressure was found in all patients (n=5). Initial neurologic manifestations included seizure, clouding of consciousness, headache, and visual disturbance. After the diagnosis was made through clinical evaluations and magnetic resonance imaging, complete clinical recovery was obtained in all patients with the appropriate therapeutic approach. In conclusion, posterior reversible encephalopathy syndrome should be considered in the differential diagnosis of patients who present with encephalopathy and underlying diseases such as nephritis, vasculitis, malignancy accompanied by hypertension, and a history of use of medication.Öğe Recurrent meningitis by Streptococcus pneumoniae in a girl with cochlear implant and head trauma despite 13-valent conjugated pneumococcal vaccine(Soc Argentina Pediatria, 2019) Emiroglu, Melike; Alkan, Gulsum; Feyzioglu, Bahadir; Aycan, Ahmet E.; Ceyhan, MehmetRecurrent bacterial meningitis is a very rare phenomenon in children. Skull base fractures and cochlear implant are the important predisposing factors and, Streptococcus pneumoniae is the most frequently isolated agent. Implementation of 13-valent conjugated pneumococcal vaccine (PCV13) has reduced the occurence of invasive pneumococcal diseases. Vaccination breakthrough is typically related to underlying predisposing conditions. Herein, we reported recurrent pneumococcal meningitis in a patient with a cochlear implant who experienced a head trauma after being fully vaccinated with PCV13. The patient experienced three meningitis episodes within one year. S. pneumoniae was determined on CSF culture in the first and third episodes and detected by PCR at the second episode. Neurosurgical intervention was performed after the third meningitis episode, and the patient had no recurrence problems for the following two years. To our knowledge, breakthrough S. pneumoniae serotype 1 meningitis after full PCV13 immunization has not been reported elsewhere in the literature.