Clinical Profile and Outcomes of Primary Immunodeficiency and Malignancy in Childhood at a Tertiary Oncology Center in Developing Country.

Primary immune deficiencies are a group of heterogenous genetic disorders characterized by frequent infections, autoimmunity and malignancy. In this study, we aimed to evaluate clinical characteristics, outcomes of children with malignancy developed on background of primary immunodeficiency and compare survival rates of patients between malignant lymphoma with primary immunodeficiency and without immunodeficiency from tertiary oncology center in a developing country. A total 23 patients with primary immunodeficiency and malignancy were evaluated retrospectively. A total of 26 malignancies (first or second) in 23 patients were determined. The median age at the time of the first malignancy was 8 years (ranges 2-18 years) with increased male ratio (M/F:14/9). Non-Hodgkin lymphoma (n = 17; 65%) was the most common malignancy, followed by Hodgkin lymphoma (n = 5), anaplastic ependymoma (n = 1), spinal glioblastoma multiforme (n = 1), retinoblastoma (n = 1) and intracranial hemangiopericytoma (n = 1). The median follow-up time of patients was 25 months (ranges between 1 and 189 months). The 5-year overall survival rate of patients with malignant lymphoma associated with primary immunodeficiency (41%) were lower than immunocompetent patients with malignant lymphoma (80%) (p = 0.000). The 5-year overall survival of patients was diagnosed between 2021 and 2013 years (62%) was higher than previous years (22%) (p = 0.03). In conclusion, non-Hodgkin lymphomas were the most common histopathologic type in patients with malignancy associated with primary immunodeficiency in the present study. The survival of patients with malignant lymphoma associated with primary immunodeficiency has improved in recent years, yet it is still lower than immunocompetent patients with lymphoma and new targeted drugs are required for better survival rates.

Hypernatremic Dehydration in Breastfed Term Infants: Retrospective Evaluation of 159 Cases.

OBJECTIVES: The aim of this study was to reveal the frequency, presenting complaints, risk factors, complications, and ways for prevention of hypernatremic dehydration (HD) among term breastfed infants. METHODS: The files of 159 breastfed term infants hospitalized because of HD between the years 2009 and 2014 were examined retrospectively in the Neonatal Intensive Care Unit of Sivas State Hospital, Turkey. The patients were classified according to serum sodium (Na) levels, group 1 (Na: 146-149 mEq/L, n = 68) and group 2 (Na ≥150 mEq/L, n = 91). RESULTS: The most common complaint was fever (67.9%), and the most common physical finding was oral mucosal dryness (76%). There were positive correlations between serum Na levels and weight loss, hospital stay, admission age, admission to neonatal unit after discharge, serum urea levels, and body temperature (p < 0.05). The normalization period of Na levels was significantly longer (21.7 ± 8.8 versus 29.3 ± 17.8 hours, p = 0.03), and Na reduction rate was faster in group 2 (0.41 ± 0.3 versus 0.50 ± 0.3 mEq/L/hour, p = 0.02). Bradycardia was seen more commonly in group 2 (1.5% versus 16.5%, p = 0.002). CONCLUSIONS: HD is a significant condition that should be treated appropriately to avoid serious complications.

The Impact of Transfusion and Chelation on Oxidative Stress in Immigrant Syrian Children with ß-Thalassemia.

Iron overload in ß-thalassemia major and intermedia patients leads to oxidative stress and causes to formation of lipid hydroperoxides. Thiobarbituric acid reactive substances (TBARS) are a well established method for screening and monitoring of lipid peroxidation. We aimed to investigate serum TBARS and its relationship with biochemical and hematologic parameters of Turkish and immigrant Syrian ß-thalassemia children reflecting the effects of this socioeconomic condition on follow up of these patients. Lipid peroxidation products (TBARS) of Turkish (TR) (n = 62, from the cities of Gaziantep and Sivas, Turkey) and Syrian (SYR) (n = 34, from Gaziantep, Turkey) ß-thalassemia patients aged 2-17 years and 58 healthy subjects aged 2-16 years were studied. Liver and renal function tests, serum ferritin levels, white blood cell, absolute neutrophil and platelet counts, hemoglobin (Hb) levels of the patients were analyzed. Serum TBARS concentrations were found to be elevated in ß-thalassemia patients compared to healthy subjects (mean: 12.47 ± 8.53 vs. 9.78 ± 7.09, p = 0.045). In SYR patients mean pretransfusional Hb level (7.26.2.04 vs. 8.49 ± 1.01, p = 0.002) was lower and ferritin levels (5983.56 ± 5065.56 vs. 3234.60 ± 2237.82, p = 0.001), liver enzymes (ALT: 77.82 ± 76.48 vs. 42.13 ± 51.50, p = 0.005) were higher when compared to TR group. Positive correlation between TBARS and ferritin levels (p = 0.029, r = 0.231) and liver enzymes (for ALT p < 0.001, r = 0.373) was observed. ß-thalassemia patients are under more oxidative stress than healthy subjects. Liver is one of the major organs which are mainly affected by oxidative stress. War and migration might have caused inappropriate transfusion conditions and insufficient chelation therapy in the SYR group.