hikmet tekin nacaroglu

Primary complement deficiencies are rare diseases. To retrospectively evaluate the clinical and laboratory findings and complications of patients to increase awareness of pediatricians about complement deficiencies, which are rarely encountered. In this study, the clinical and immunological characteristics of 21 patients who consulted the Immunology Department of our hospital between 2003 and 2017 and were diagnosed with classical or alternative pathway complement deficiency were obtained from the file records. Ten patients with C1 inhibitor deficiency, four patients with factor I deficiency, three patients with properdin deficiency, two patients with C8 deficiency, one patient with C1q deficiency, and one patient with C4B deficiency were assessed. The mean age of the patients at diagnosis was 11.4±4.7 years, the age of onset of symptoms was 7.9±3.9 years, and the follow-up period was 6.7±3.9 years. Fourteen cases had a similar medical history in the family. All patients with C1q, factor I, properdin, C8, and C4B deficiencies presented with an infection, and vasculitic rash was present in two patients with factor I deficiency. In addition, immune complex glomerulonephritis was present in one patient with factor I deficiency. Meningococcal, Haemophilus influenzae type B, and pneumococcal vaccines were administered and prophylactic antibiotic treatment was initiated in all patients except patients with C1 inhibitor deficiency. Early diagnosis of complement deficiencies can facilitate prevention of life-threatening complications such as severe bacterial infections by considering prophylactic antibiotics and vaccines. In patients with C1 inhibitor deficiency, achieving an acurate early diagnosis will assist in the management and timely treatment of life-threatening attacks such as upper airway obstruction and improve outcomes.

We present a 13-year-old male childallergic to three different plants (Salvia officinalis, Mentha piperita and Origanum onites L.) of Lamiaceae family. The patient developed angioedema 20-30 minutes after eating chicken meat with cheddar cheese. There was no history of allergy. Oral food challenge (OFC) with both cheddar cheese and chicken meat was negative. Skin tests for inhalant allergens were negative. 3 weeks later, the patient was admitted with angioedema after drinking sage tea. OFC with sage was applied and angioedema was observed. It was recognized that the first trigger, chicken meat with cheddar cheese, included oregano (Origanum onites L.). OFC for oregano was positive. Prick to prick test for Lamiaceae herbs (oregano, sage, mint) was performed. A positive reaction was observed only to mint. OFC was repeated with fresh mint and angioedema developed after 16 hours. Diagnose of Lamiaceae allergy is complicated and cross-sensitivity is common. Skin prick test (prick to prick)revealed a positive response only to mint but not to oregano and sage. Commercial radioallergosorbent (RAST) tests are available only for a few members of the family. Finally, thediagnose is based mainly on OFC. Spices from Lamiaceae group should be considered as potential triggers of allergic reactions.

Osteoprotegerin (OPG) is a member of the tumor necrosis factor superfamily. Reduced OPG levels are related to obesity, insulin resistance, and non-alcoholic fatty liver disease (NAFLD).
The aim of this study was to evaluate the relationship between OPG levels, obesity, insulin resistance, and NAFLD in pediatric patients.
This was a prospective, cross-sectional, controlled study that was conducted in the department of pediatrics at Bagcilar training and research hospital in Istanbul, Turkey, between April and August 2015. The study was performed on 107 children with obesity and 37 controls aged 5 - 17 years. In the obese subset, 62 patients had NAFLD. Homeostatic model assessment-insulin resistance (HOMA-IR) was used to calculate insulin resistance. Insulin resistance was defined as a HOMA-IR value greater than 2.5. Plasma OPG levels were measured using enzyme-linked immunosorbent assays. NAFLD was diagnosed by hepatic ultrasound.
The mean age was 11.25 ± 3.38 years in the patient group and 10.41 ± 3.15 years in the control group. The OPG level in the obese group with the mean of 55.20 ± 24.55 pg/mL (median = 48.81 pg/mL) was significantly lower than that in the control group with the mean of 70.78 ± 33.41 pg/mL (median = 64.57 pg/mL) (P = 0.0001). The optimal cut-off point (sensitivity, specificity) of the OPG level for the diagnosis of obesity was ≤ 46, 19 pg/mL. According to logistic regression analysis, fasting insulin (P = 0.036) and OPG (P = 0.01) levels were most affected by obesity. In the obese patients, who had HOMA-IR We confirmed that serum OPG concentrations reduce in obese children. However, no correlation was identified between OPG and insulin resistance. OPG levels are not meaningful in the diagnosis of NAFLD in children with obesity.

Henoch-Schonlein Purpura (HSP) is the most widespread systemic vasculitis during childhood. Gastrointestinal tract retention and gastrointestinal bleeding are among its major complications. Neutrophil-Lymphocyte ratio (NLR) and platelet-lymphocyte ratio (PLR) are indicators related to inflammatory diseases. This study evaluated the relationship between NLR or PLR and gastrointestinal bleeding in HSP.
The study consisted of 119 patients and 40 healthy children in the same age group. White Blood Cell (WBC) count, hemoglobin level, platelet count, mean platelet volume (MPV), neutrophil count and lymphocyte count were recorded. The NLR and PLR were calculated based on the results of complete blood count tests performed during the first visit to the hospital.
The average neutrophil count and NLR of the patients with HSP were found to be significantly increased compared to the control group (P = 0.0001). No significant difference was observed between the PLR average of HSP and control groups (P = 0.053). Platelet count average (P = 0.0001) and PLR (P = 0.001) of the patients with gastrointestinal system (GIS) bleeding were found to be statistically significantly increased compared to those who did not have gastrointestinal bleeding. No significant difference was found in the NLR of the patients with and without gastrointestinal bleeding (P = 0.060).
While the NLR was significantly increased in patients with HSP in this study, the PLR was found to be more significant in patients with gastrointestinal bleeding. Similar to NLR, PLR may also be used as an inflammatory indicator among children with HSP, who have gastrointestinal bleeding.

Leukotriene receptor antagonists (LTRAs) are drugs which have been widely used more than ten years. As the use of LTRAs increases, our knowledge with respect to their side effects increases as well. The objective of our study was to evaluat the observed side effects of LTRAs used in patients with astma. Patients and 1024 patients treated only with LTRAs owing to asthma or early wheezing were included in the study for a five-year period. The observed side effects of LTRAs in these patients were retrospectively investigated. The side effects were divided into two parts as psychiatric and non-psychiatric. Among the 1024 cases included in the study, 67.5% of the patients out of 41 with side effects were male, 32.5% were female and the average age was 6.5 years. The rate of patients with asthma was 63.41% and 36.58% of the patients had early wheezing. It was determined that sex, age and diagnosis (early wheezing or asthma) of the patients were ineffective in the emergence of side effects. The average period for the emergence of side effects was the first month. It was observed that hyperactivity was the most frequently observed psychiatric side effect and that abdominal pain was the non-psychiatric side effect. The side effects of LTRAs were common in children. Therefore, patients must be informed at the beginning of the treatment and they must be evaluated at certain intervals.

Idiopathic Pulmonary Hemosiderosis (IPH) is a rare cause of alveolar hemorrhage, which is seen primarily in childhood. Celiac disease is defined as a chronic, immune-mediated enteropathy of the small intestine, caused by exposure to dietary gluten in genetically pre-disposed individuals. Association of IPH and celiac disease is known as Lane Hamilton syndrome. There are limited number of case reports of this syndrome in literature. Although there were no growth and developmental delay and gastrointestinal symptoms like chronic diarrhea, chronic constipation, vomiting, abdominal bloating and pain in the two patients with IPH, they were diagnosed with Lane Hamilton Syndrome. After initiation of gluten-free diet, their IPH symptoms disappeared and hemoglobin levels were observed to return to normal. Even if there were no gastrointestinal symptoms in a patient with IPH, celiac disease should be investigated. These patients may benefit from gluten free diet and IPH symptoms may disappear.