Background Mutations in DOCK8 cause a combined immunodeficiency (CID) also classified as autosomal-recessive hyper-IgE syndrome (HIES). Recognizing patients with CID / HIES is of clinical importance due to a difference in prognosis and management. Objectives Define the clinical features that distinguish DOCK8 deficiency from other forms of HIES and CIDs; study the mutational spectrum of DOCK8 deficiency; and report on the frequency of specific clinical findings. Methods Eighty-two patients from 60 families with CID and the phenotype of autosomal-recessive HIES with (64 patients) and without (18 patients) DOCK8 mutations were studied. Support vector machines were used to compare clinical data from 35 patients with DOCK8 deficiency with 10 AR-HIES patients without a DOCK8 mutation and 64 patients with STAT3 mutations. Results DOCK8-deficient patients had a median IgE of 5,201 IU, high eosinophil levels of usually at least 800/µl (92% of patients), and low levels of IgM (62%). About 20% of patients were lymphopenic, mainly due to low CD4+ and CD8+ T cells. Fewer than half of the patients tested produced normal specific antibody responses to recall antigens. Bacterial (84%), viral (78%), and fungal (70%) infections were frequently observed. Skin abscesses (60%) and allergies (73%) were common clinical problems. In contrast to STAT3 deficiency, there were few pneumatoceles, bone fractures, and teething problems. Mortality was high (34%). A combination of five clinical features was helpful in distinguishing patients with DOCK8 mutations from those with STAT3 mutations. Conclusions DOCK8 deficiency is likely in patients with severe viral infections, allergies, and/or low IgM levels, who have a diagnosis of HIES plus hypereosinophilia and upper respiratory tract infections in the absence of parenchymal lung abnormalities, retained primary teeth, and minimal trauma fractures.

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Abdominal re-approximation anchor system and VAC dressing can be used separately or in conjunction with each other for closure of delayed open abdomen successfully.

Objective: We aimed to determine the effects of intravenous iron therapy on blood parameters in pediatric patients who do not tolerate oral iron therapy for any reason. Patients and Methods: The patient group consisted of candidates for elective operations requiring blood transfusions in order to raise hemoglobin (Hb) concentrations rapidly and for whom oral iron administration is useless and compliance with long-term treatment is definitely impossible due to sociocultural factors. Sixty-two children were included in the study. Venous blood samples were taken at diagnosis, and after 1 week and 1, 2 and 3 months. Hb, hematocrit, erythrocyte indices (mean erythrocyte volume, mean erythrocyte Hb and mean erythrocyte Hb concentration), serum iron (SI) levels, iron binding capacity, transferrin receptor (CD71) and serum ferritin levels were measured. Iron sucrose was used as an intravenous iron preparation. Results: All children showed improvements in iron deficiency anemia. A statistically significant elevation occurred between the time of diagnosis and week 1 (p<0.05) in nearly all parameters. SI was raised until at least 1 month of therapy. There was no significant difference between transferrin receptors measured before and after the intravenous iron therapy. Ferritin did not exceed the values achieved in the 1st month. Mild side effects were encountered in only 8 (12.9%) patients. Treatment was not discontinued because of side effects in any case. The patients in the control group were given an oral form containing ferroglycine sulfate. Conclusion: Intravenous iron therapy can replace oral therapy in patients whose blood parameters must be raised rapidly and in situations where oral iron administration would not be appropriate for any reason. However, reinforcement with oral iron therapy or additional intravenous doses would be appropriate.

This study aims to examine the effect of zinc supplementation on free-radical formation and antioxidant system in individuals who are actively engaged in wrestling as a sport. The study registered a total of 40 male subjects, of whom 20 were wrestlers and 20 were sedentary individuals. The subjects were equally allocated to four groups: group 1, zinc-supplemented sportsmen group; group 2, sportsmen group without supplementation; group 3, zinc-supplemented sedentary group; group 4, sedentary group without supplementation. Blood samples were collected from all subjects twice, once at the beginning of the study and once again at the end of 8-week procedures. The blood samples collected were analyzed to determine the levels of malondialdehyde (MDA), serum glutathione (GSH), serum glutathione peroxidase (GPx) activity, serum superoxide dismutase (SOD) activity (ELISA colorimetric method) and zinc (colorimetric method). No difference was found between MDA levels of the study groups in the beginning of the study. The highest MDA value at the end of the study was obtained in group 4 (p < 0.01). MDA levels in group 2 were established to be significantly higher than those in groups 1 and 3 (p < 0.01). GSH level, GPx, and SOD activities and zinc level measured in the beginning of the study were not different between groups. Measurements performed at the end of the study showed that groups 1 and 3 (zinc-supplemented groups) had the highest GSH level, GPx, and SOD activities and zinc level (p < 0.01). These parameters were not different in the groups without supplementation (groups 2 and 4). Results obtained at the end of the study indicate that zinc supplementation prevents production of free radicals by activating the antioxidant system. In conclusion, physiologic doses of zinc supplementation to athletes may beneficially contribute to their health and performance.