G.D. Tugcu

Omalizumab Treatment for Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis

Background: Allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) is characterized by destructive changes in the airways. Long-term treatment with oral corticosteroids is often required for repeated exacerbations. Because elevated total IgE is a cardinal abnormality of ABPA, omalizumab has been used sporadically to decrease corticosteroid dose or totally replace corticosteroids. Objective: The aim of this report is to describe our experience with omalizumab treatment in patients with CF and ABPA. Methods: We conducted a review of 6 CF patients with ABPA receiving omalizumab. All patients were treated with oral prednisolone and itraconazole. Omalizumab was started if the patient was not responding to steroid treatment, which was determined according to serum IgE levels and/or clinical findings or depending on if there were side effects caused by steroid treatment. Results: The mean age of patients at the beginning of omalizumab treatment was 16.1 years. One patient had a new diagnosis of ABPA; however, the others had the first to third exacerbation when treated with omalizumab. The mean duration of ABPA by the time that treatment with omalizumab started was 13 ± 12.4 months (range = 2-29 months). With omalizumab treatment, IgE levels were decreased in all patients, and Aspergillus-specific IgE levels were decreased in 4 patients; however, FEV1(% predicted) improved only in 2 patients who had mild disease. Corticosteroids were reduced in the first, second, and third months of omalizumab treatment in 2, 1, and 3 patients, respectively. In 2 patients, steroid treatment was stopped. None of the patients suffered from side effects of omalizumab. The mean duration of omalizumab treatment was 12.5 months (range = 6-18 months). Conclusions: This study showed steroid-sparing effect, decreasing IgE levels, and improvement in respiratory symptoms in 6 CF patients with omalizumab treatment. Although this is a small sample of the population, omalizumab may be an alternative therapy for ABPA in CF patients who fail to respond to systemic corticosteroids or have serious adverse effects.

A review of the etiology and clinical presentation of non-cystic fibrosis bronchiectasis: A tertiary care experience

INTRODUCTION Non-cystic fibrosis(CF) bronchiectasis has been recognized in children for the past 200 years. Early childhood pneumonia and underlying conditions such as immunodeficiency, primary ciliary dyskinesia(PCD), and congenital lung pathology should be considered in the etiology. The aim of our study was to describe the clinical characteristics, laboratory, and radiological findings of a large population of patients with non-CF bronchiectasis at a tertiary center. METHODS We analyzed the clinical findings of 187 patients diagnosed with non-CF bronchiectasis over a period of 10 years (January 2005-December 2015) at the Hacettepe University Faculty of Medicine Department of Pediatric Pulmonology. RESULTS The median age at the time of diagnosis of non-CF bronchiectasis was 8 years (1-18 years). Consanguinity was positive in 59.4% (n = 111) of patients and 19.8% (n = 37) of patients had a positive family history for non-CF bronchiectasis. Common causes were PCD in 51.3% (n = 96), immunodeficiency in 15% (n = 28), history of tuberculosis in 5.9% (n = 11), post-infectious complication in 3.2% (n = 6) and other anomalies in 2.1% (n = 4) of patients. The frequency of pulmonary lobe involvement was as follows: 71.1% left-lower lobe, 59.4% right lower lobe, 54% right-middle lobe, 26.8% left lingula, 13.9% right upper lobe, and 9.6% left upper lobe. CONCLUSIONS Diagnosis of non-CF bronchiectasis is often delayed because of a failure to recognize the significance of symptoms. Through clinical investigation, including a HRCT scan of the chest, sweat test, studies of immune function, and ciliary function in a child with a prolonged suppurative cough, remains important. In Turkey, the most common causes of non-CF bronchiectasis are PCD and immunodeficiency, related to a high frequency of consanguinity.

Impact of Surgery on Growth, Pulmonary Functions, and Acute Pulmonary Exacerbations in Children with Non-Cystic Fibrosis Bronchiectasis

Background Treatment decisions for the management of bronchiectasis include medical treatment, such as antibiotics, chest physiotherapy, and surgical procedures. Here, we aimed to review the effect of lung resection on longitudinal growth, clinical course of patients depending on annual exacerbation rates, and pulmonary function tests (PFTs) and compare them with the results of only medically treated children with non‐cystic fibrosis (non‐CF) bronchiectasis. Methods The medical records of patients with non‐CF bronchiectasis were retrospectively analyzed. Patients who underwent lobectomy/segmentectomy/pneumonectomy were categorized as “surgery group” (n = 29). Age‐ and gender‐matched patients who were only medically treated were selected as “medical group” (n = 33). Annual data of patients were included till the end of postoperative second year in the surgery group and third year of medical treatment in the medical group. Results Mean baseline height z ‐score was lower in the surgery group, and mean baseline PFT values were all lower in the surgery group than those in the medical group (p < 0.05). In the surgery group, mean values of height z ‐score were ‐1.68 ± 0.92 at the time of surgery and improved to ‐1.42 ± 1.22 and ‐1.34 ± 1.05 in the first and second postoperative years, respectively, and annual intravenous antibiotic requirements decreased significantly (p < 0.05); however, mean body mass index (BMI) z ‐score values and PFT parameters did not change significantly. In the medical group, height z ‐score mean values and PFT parameters showed nonsignificant improvement but annual exacerbation frequency, annual intravenous, and oral antibiotic requirements decreased significantly. Conclusion Surgical management of non‐CF bronchiectasis has no significant effect on BMI z ‐scores, annual exacerbation frequencies, oral antibiotic requirements and lung function tests; but can lead to significant improvement on height z ‐scores and decrease need of annual intravenous antibiotic requirements for acute severe exacerbations despite small number of patients in this study.