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    22q11.2 deletion syndrome: 20 years of experience from two pediatric immunology units and review of clues for diagnosis and disease management
    (Codon Publications, 2021) Ozen, Selime; Akcal, Omer; Taskirdi, Ilke; Haci, Idil Akay; Karaca, Neslihan Edeer; Gulez, Nesrin; Kutukculer, Necil
    Introduction and objectives: The purpose of this study was to evaluate patients diagnosed with 22q11.2 deletion syndrome and determine the clues directing to diagnosis and evaluation of immunological findings for excellent management of the disease. Material and methods: Thirty-three pediatric patients with 22q11.2 deletion syndrome diagnosed between 1998 and 2019 at Pediatric Immunology Division of Ege University Faculty of Medicine and SBU Izmir Dr Behcet Uz Children's Education and Research Hospital were evaluated. Results: This study includes the largest case series reported from Turkey. Congenital cardiac anomalies were the most common pathology associated with the syndrome (90.9%). Hypocalcemic symptoms were observed in 13 patients (40%). Twenty-two of the 33 (66.6%) patients were diagnosed before two years of age. Autoimmune diseases, dysmorphic facial findings, recurrent infections, growth retardation, and speech impairment were other clues for diagnosis in older patients. Clinical spectrum and immunological abnormalities of this syndrome are quite variable. All T-cell subset counts were less than 5th percentile below median by age in one patient (3%) and 10 patients had normal all T-cell subset counts (30.3%). Overall, 69.6% of the patients had normal IgG, IgA, and IgM levels and two patients had panhypogammaglobulinemia. Recurrent infections were revealed in 75.7% of the patients during follow-up. Conclusions: Presence of cardiac anomaly is more helpful in the diagnosis, especially under two years of age. Patients with immunologically high or standard risk did not show any difference in terms of numbers and severity of infections and autoimmunity. (C) 2021 Codon Publications. Published by Codon Publications.
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    Hyponatremia in Children with Acute Lymphoblastic Leukemia
    (Galenos Yayincilik, 2020) Karapinar, Deniz Yilmaz; Sahin, Akkiz; Ozen, Selime; Ozkaya, Pinar Yazici; Sivis, Zuhal Onder; Akinci, Ayse Burcu; Karapinar, Bulent
    Aim: Hyponatremia is a common electrolyte abnormality in hospitalized patients. Administration of isotonic maintenance fluids is recommended to prevent hyponatremia. the present study was conducted to evaluate the frequency and severity of hyponatremia in children with acute lymphoblastic leukemia (ALL). Materials and Methods: the frequency, severity and possible causes of hyponatremia in children with ALL throughout their entire intensive treatment were retrospectively evaluated. All children in this study received isotonic fluids as maintenance IV treatment during the hospitalization period. Results: in a five-year period, 618 hyponatremia episodes seen in 92 children with ALL (median age 59 months), treated with ALLIC 2002 protocol were entered into the study. the median number of hyponatremia episodes per patient was 6. All patients had at least one hyponatremia episode of which 83.2% were classified as mild, 13.2% as moderate, 2.9% as severe and 0.6% as very severe. the median duration of hyponatremia episodes was 5 (range between 1-43) days. the total duration of all hyponatremia episodes of each patient varied from 6 to 138 days with a median of 30 days. in 241 episodes of 68 children, there was inadequate salt intake secondary to oral feeding intolerance, nausea, vomiting and oral aphthous stomatitis. in four patients, seizure was seen during the hyponatremia period and thought to be secondary to hyponatremic encephalopathy. No patient developed central pontine myelinolysis. Conclusion: Hyponatremia is very frequent in ALL patients. Despite the use of isotonic IV fluids, it seems it cannot be completely prevented.
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    INVISIBLE INGESTED FOREIGN BODY: ALUMINUM CAN TOP
    (Elsevier Science Inc, 2014) Saz, Eylem Ulas; Ozen, Selime; Ecevit, Cigdem Omur; Ozgenc, Funda