Semiha Bahceci Erdem

Side Effects of Leukotriene Receptor Antagonists in Asthmatic Children

Background: 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. Objectives: The objective of our study was to evaluat the observed side effects of LTRAs used in patients with astma. Patients and Methods: 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. Results: 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. Conclusions: 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.

Tolerance development in food protein-induced allergic proctocolitis: single centre experience

BACKGROUND Food protein-induced allergic proctocolitis (FPIAP) is characterised by inflammation of the distal colon in response to one or more food proteins. It is a benign condition of bloody stools in a well-appearing infant, with usual onset between one and four weeks of age. OBJECTIVE Our objective was to examine the clinical properties of patients with FPIAP, tolerance development time as well as the risk factors that affect tolerance development. METHODS The clinical symptoms, offending factors, laboratory findings, methods used in the diagnosis and tolerance development for 77 patients followed in the Paediatric Allergy and Gastroenterology Clinics with the diagnosis of FPIAP during January 2010-January 2015 were examined in our retrospective cross-sectional study. RESULTS The starting age of the symptoms was 3.3±4.7 months (0-36). Milk was found as the offending substance for 78% of the patients, milk and egg for 13% and egg for 5%. Mean tolerance development time of the patients was 14.7±11.9 months (3-66 months). Tolerance developed before the age of one year in 40% of the patients. Tolerance developed between the age of 1-2 years in 27%, between the age of 2-3 years in 9% and after the age of 3 years in 5% of the patients. CONCLUSIONS Smaller onset age and onset of symptoms during breastfeeding were found associated with early tolerance development. In the majority of the patients, FPIAP resolves before the age of one year, however in some of the patients this duration may be much longer.

Asplenia in children with congenital heart disease as a cause of poor outcome.

The absence of a spleen is a well-known risk factor for severe bacterial infections, especially due to encapsulated bacteria. Congenital asplenia can be part of multiple congenital abnormalities as in heterotaxy including Ivemark syndrome with congenital anomalies of the heart or great vessels, or it can be isolated, which is extremely rare. In these cases, asplenia is an important factor effecting mortality. In this report, the clinical courses of five children with asplenia and concomitant minor or complex cardiac anomalies are presented. The ages of the children ranged between 1.5 and 17 months at the time of diagnosis. All of the cases had had a history of hospitalisation for infectious diseases before the diagnosis. The patient who was diagnosed at 17 months old had a history pneumonia, urinary tract infection, and bacterial meningitis beginning at five months old. Three children had complex cardiac anomalies, one child had ventricular septal defect, and one child had atrial septal defect. Howell-Jolly bodies were determined in peripheral blood smear in all of the patients. The diagnoses of asplenia were confirmed with spleen scintigraphy. One of the patients with complex cardiac anomalies died a short time after diagnosis, because of cardiac failure. The rest of the four patients were vaccinated for encapsulated bacteria and were taken under antibiotic prophylaxis. These children did not need hospitalisation for infectious diseases during the follow-up period (5-40 months). In asplenic children, early diagnosis, antibiotic prophylaxis, and immunisation for encapsulated bacteria can decrease the risk of morbidity and mortality.

DRESS syndrome associated with type 2 diabetes in a child

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is an uncommon, life-threatening drug reaction. The basic findings are skin rash, multiorgan involvement, and eosinophilia. Most of the aromatic anticonvulsants, such as phenytoin, phenobarbital and carbamazepine can induce DRESS. Herein we report a 14-year-old patient with DRESS syndrome related to carbamazepine use. The patient presented with signs of involvement of the skin, lungs, liver, and microscopic hematuria. Carbamazepine treatment was discontinued; antihistamines and steroids were started. Hyperglycemia, commencing on the first dose of the steroid given, persisted even after the discontinuation of steroids and improvement of other signs. There were no signs of pancreatitis or type 1 diabetes clinically in laboratory tests. Her blood glucose levels were regulated at first with insulin and later with metformin. Within 1 year of follow-up, still regulated with oral antidiabetics, she has been diagnosed with type 2 diabetes. Formerly, long-term sequelae related to “drug rash with eosinophilia and systemic symptoms syndrome” such as hepatic and renal failure, type 1 diabetes mellitus, Graves disease, autoimmune hemolytic anemia, and lupus have also been reported. However, up to date, no cases with type 2 diabetes have been reported as long-term sequelae. To our knowledge, this is the first case in the literature presenting with type 2 diabetes as long-term sequelae.

Diagnostic values for egg white specific IgE levels with the skin prick test in Turkish children with egg white allergy

BACKGROUND The diagnostic values for the skin prick test (SPT) diameters and egg white-specific IgE (EW-sIgE) levels that will allow us to predict the result of the oral food challenge test (OFC) in the diagnosis of egg white allergy vary by the community where the study is carried out. OBJECTIVE This study aimed to determine the diagnostic values of SPT and EW-sIgE levels in the diagnosis of egg white allergy. METHODS 59 patients followed with the diagnosis of egg allergy September 2013 to September 2015 were included in our retrospective cross-sectional study. The patients were investigated in terms of egg and anaphylaxis history or the requirement of the OFC positivity. The demographic, clinical and laboratory findings of the cases were recorded, and they were compared with the patients with the suspected egg allergy but negative OFC (n=47). RESULTS In the study, for all age groups, the value of 5mm in SPT was found to be significant at 96.4% positive predictive value (PPV) and 97.8% specificity and the value of 5.27kU/L for EW-sIgE was found to be significant at 76% PPV and 86.6% specificity for egg white. The diagnostic power of the SPT for egg white (AUC: 72.2%) was determined to be significantly higher compared to the diagnostic power of the EW-sIgE (AUC: 52.3%) (p<0.05). CONCLUSION Along with the determination of the diagnostic values of communities, the rapid and accurate diagnosis of the children with a food allergy will be ensured, and the patient follow-up will be made easier.

Association of Celiac Disease With Idiopathic Pulmonary Hemosiderosis; Lane Hamilton Syndrome

Introduction: 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. Case Presentation: 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. Conclusions: 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.

Can mean platelet volume and neutrophil-to-lymphocyte ratio be biomarkers of acute exacerbation of bronchiectasis in children?

Introduction Bronchiectasis (BE) is a parenchymal lung disease evolving as a result of recurrent lung infections and chronic inflammation. Although it has been shown in adult studies that mean platelet volume (MPV) and neutrophil-to-lymphocyte ratio (NLR) can be used as biomarkers of airway inflammation, knowledge is limited in the paediatric age group. The aim of our study is to investigate the potential of MPV and NLR as biomarkers that may indicate acute exacerbations of non-cystic fibrosis BE in children. Material and methods Children with non-cystic fibrosis BE (n = 50), who were followed in the division of Paediatric Pulmonology of our hospital between June 2010 and July 2015, were involved in the present retrospective cross-sectional study. Haemogram values during acute exacerbations and non-exacerbation periods, and a control group were compared. Results In children with bronchiectasis, the average leukocyte count (p < 0.001), platelet count (p = 0.018), absolute neutrophil count (p < 0.001), and NLR (p < 0.001) were higher, as expected, when compared with the control group. NLR values, in the period of acute exacerbation were significantly higher than the values of both the non-exacerbation periods (p = 0.02) and the control group (p < 0.001). In contrast, MPV values in the period of acute exacerbation did not exhibit a significant difference from those of non-exacerbation periods (p = 0.530) and the control group (p = 0.103). Conclusions It was concluded that leukocyte count, platelet count, absolute neutrophil count, and NLR can be used to show chronic inflammation in BE, but only NLR and absolute neutrophil count can be used as biomarkers to show acute exacerbations.

Unusual Radiological Sign in Bronchial Atresia

Bronchial atresia is usually diagnosed by incidentally detecting opacitiy at hilar ragion and hyperinflation around this opacity on chest X-ray. It may rarely be detected as air sac like atresic bronchus. The breath sounds in the right hemithorax were heard less when compared to the left hemithorax in the auscultation of a 16-year-old male patient with allergic rhinitis. The patient had no pulmonary complaints, and this finding was not recorded in his previous follow-up. In order to determine the etiology of hyperinflation seen on chest X-ray, computed tomography was performed. Hyperinflation was identified in the lower lobe superior segment of the right lung, which could be secondary to bronchial atresia. It was confirmed that in the evaluation of computed tomography with three-dimensional reconstruction, lower lobe superior segment bronchus of the right lung was atresic and contrary to expected mucus opacity in the distal of atresia, dilated bronchus was filled with air. This case was especially presented to lay emphasis on careful auscultation and share its unusual radiological presentation which had been reported twice before.

Five years of experience in transient hypogammaglobulinemia of infancy

Objective: Transient hypogammaglobulinemia of infancy (THI) is a transient primary immune deficiency stemming from delay in immunoglobulin synthesis which spontaneously, and completely resolves with age. In this study, cases which were followed with the initial diagnosis of hypogammaglobulinemia and received the diagnosis of THI were retrospectively evaluated. Methods: A total of 193 patients, who received the diagnosis of THI in the Department of Immunology of Dr. Behçet Uz Children’s Hospital, and Pediatric Surgery between January 2011 and December 2015, were included in the study. Clinical, laboratory and demographic data, prophylactic treatments they received, their follow-up periods, and age of onset of disease resolution were retrospectively analyzed. Results: In the study, 70.5% of 193 cases were male. The age of onset of symptoms was 11.7±7.0 month, the age of hypogammaglobulinemia recovery was 30.6±10.5 month. The cases were most frequently admitted (40.9%) for the recurrent upper respiratory tract infection. Only one case had immunodeficiency in the family history. At the admission, the immunoglobulin levels were as follows; IgG: 420.2±110.6 mg/dL, IgA: 29.8±23.3 mg/dL, IgM: 71±3.1 mg/dL. In vaccine responses; anti tetanus 75.9% (60/79), anti HBs 91.6% (141/154) and anti hemophilus influenza type B was 86.4% (51/59) positive. Twenty five (13%) of the patients received only antibiotic prophylaxis, 19 of them (9.8%) received only IV gamma globulin replacement, and 6 of them ( 3.1%) received both. There was no statistically significant difference between age of recovery and infection frequency, clinical presentation, vaccination response, IV gamma globulin replacement and antibiotic prophylaxis (p>0.05). Conclusion: THI is usually recovers spontaneously around 3 years of age. There is no relationship between age of recovery and clinical presentation, vaccine responses, IV gamma globulin replacement and antibiotic prophylaxis. The cases must be monitored regularly for correct diagnosis.

Risk of systemic allergic reactions to allergen immunotherapy in a pediatric allergy clinic in Turkey

OBJECTIVES Even though allergen immunotherapy is an effective treatment method that has been used on rhinitis, asthma and venom anaphylaxis for over 100 years, systemic reactions (SRs) limit the use of this treatment method. We classified SRs associated with subcutaneous immunotherapy (SCIT) according to the World Allergy Organization Subcutaneous Immunotherapy Systemic Reaction Grading System. Risk factors for the SRs were assessed. METHODS In this study 67,758 injections to 1350 children with allergic rhinitis and/or asthma were analyzed throughout January 1999-December 2014. RESULTS A total of 51 systemic reactions were observed in 39 patients (0.075% per injection, %3 per patient). Mean age of SRs observed patients was 13±2.6 years (range 9.5-16 years) and 64.1% were male, 35.9% were female. 51.3% of SRs were grade 1, 38.5% grade 2, 7.7% grade 3 and 2.6% grade 4. SRs were early onset in 41% of the patients and delayed onset in 59%. 76.9% of SRs were seen during maintenance therapy and 56.4% during peak pollen season. In 28.2% of cases previous local reactions and in 30.8% previous grade 1 reactions were determined. There was no fatal outcome from any of the SRs. CONCLUSION SCIT related SRs are generally of mild severity. Although only 10% of the SRs were grade 3 or 4, there is a still a small risk of severe reactions. 76.9% of SRs were observed during maintenance therapy. Delayed-onset SRs rate in our study is 59%. So both clinicians and parents should be alert about the delayed reactions after SCIT.