Deniz Dogru

Effects of claritromycin on inflammatory parameters and clinical conditions in children with bronchiectasis.

Background:  The effects of the macrolides cannot be ascribed to their antibacterial action alone. Their immunoregulatory and anti‐inflammatory functions are significant too. They are frequently used in the treatment of diffuse panbronchiolitis and cystic fibrosis (CF).

Postinfectious Bronchiolitis obliterans in Children: Clinical and Radiological Profile and Prognostic Factors

Background: Postinfectious bronchiolitis obliterans (BO) designates a clinical syndrome of chronic airflow obstruction associated with inflammatory changes in the small airways. Objectives: The aim of this study was to determine the etiology, clinical and radiological features and define prognostic factors of postinfectious BO in children. Methods: We undertook a review of the medical records of 20 children with postinfectious BO, who were followed up between January 1994 and August 2001. Results: Postinfectious BO was diagnosed in 11 boys and 9 girls with a median age of 21.5 months (6–69 months) who were followed up for a median of 23 months. Cough and wheezing persisted since the initial lung infection in all patients. Postinfectious BO was diagnosed in an average of 6 months (1–42 months) after the acute illness. Adenoviruses were the most common etiologic agents identified serologically. The diagnoses of postinfectious BO was made by thoracic high-resolution computed tomography and clinical features. Corticosteroid therapy was used in 17 patients and supportive treatment was applied in all patients. Age at initial lung infection, gender, time of starting corticosteroid treatment, presence of bronchiectasis or atelectasis and etiologic agents identified on presentation did not predict severity in our study. Conclusions: Despite the prominent role of inflammation in the pathogenesis of postinfectious BO, the use of anti-inflammatory agents remains controversial. We could not identify any prognostic factors related to disease severity. In order to minimize associated complications, patients with postinfectious BO should be closely followed up and receive meticulous lung care.

Gorham-Stout Syndrome with chylothorax: successful remission by interferon alpha-2b.

Gorham–Stout syndrome is a rare disease and most often recognized in children and young adults. Chylothorax is a serious complication of Gorham–Stout syndrome. The treatment of either Gorham–Stout syndrome or chylothorax is still a dilemma. We described a 9‐year‐old girl with Gorham–Stout syndrome and chylothorax who was admitted to our hospital because of dyspnea, orthopnea, and bone pain, and treated with interferon alpha‐2b. Pediatr Pulmonol. 2009; 44:613–615.

Clinical features and treatment approaches in cystic fibrosis with pseudo-Bartter syndrome.

Abstract Introduction: Infants with cystic fibrosis (CF) are prone to develop episodes of hyponatraemic, hypochloraemic dehydration with metabolic alkalosis, which are biochemical hallmarks of the pseudo-Bartter syndrome (PB). Method: We reviewed the clinical and laboratory features and treatment aproaches of 29 children with CF and PB who were under follow-up in our institution from May 1992 to November 2003. Results: Of 241 patients with CF, PB was detected in 29 (12%) with a median age of 4 months at the time of the first attack. Most patients experienced vomiting, loss of appetite and dehydration during episodes of PB. All patients were managed with intravenous fluids and sodium chloride solutions. During follow-up, 12/29 cases required hospital admission for recurrent PB attacks. The oldest age at the time of the last attack was 48 months. Conclusions: CF should be considered in the differential diagnosis of metabolic alkalosis in young children. Vomiting and loss of appetite are important warning signs of possible PB in CF patients, particularly before 4 years of age. To prevent serious complications, it is crucial that parents and physicians recognise PB as early as possible.

Tracheomalacia and Bronchomalacia in 34 Children: Clinical and Radiologic Profiles and Associations with Other Diseases

We reviewed our experience on tracheomalacia (TM) and bronchomalacia (BM) in children who presented with chronic respiratory problems and evaluated their clinical and radiologic characteristics and their associations with other disorders. There were 26 males and 8 females with a median age of 9 months. The main symptoms were wheezing, persistent or recurrent pneumonia, and chronic cough. Atelectasis on chest radiograph was the most common sign. Of 23 children with TM, 1 had a double aortic arch, 1 had tracheoesophageal fistula, and 1 other had associated laryngomalacia. BM was found in 27 children and was predominantly seen on the right side. TBM was found in 16 cases (in an infant TBM was accompanied by pharyngeal dyskinesia and in another by laryngomalacia). Malacia disorders were associated with gastroesophageal reflux, cardiovascular anomalies, and tracheoeosophageal fistula. TM and BM should be considered in the differential diagnosis of children with chronic and recurrent respiratory symptoms. Early diagnosis of malacia disorders will prevent unnecessary use of antibiotics or antiasthmatic drugs, which are often abused to treat these children. In these patients, treatment for associated diseases should also be considered.

Effects of scoliosis on respiratory muscle strength in patients with neuromuscular disorders

BACKGROUND CONTEXT Neuromuscular disorders (NMD) are characterized by loss of lung volume and respiratory muscle weakness, but the effects of scoliosis on lung function are unclear. PURPOSE To compare pulmonary function and respiratory muscle strength in patients with NMD with and without scoliosis as well as in healthy controls. STUDY DESIGN/SETTING Prospective comparison of pulmonary function testing and respiratory muscle strength were made at the pediatric pulmonology and cardiopulmonary rehabilitation units of a university hospital. PATIENT SAMPLE Twenty-two patients with NMD and scoliosis, 17 patients with NMD without scoliosis, and 24 age- and sex-matched healthy controls. Outcome measures were compared in patients with NMD with and without scoliosis and healthy subjects using Student t test, Mann-Whitney U test, chi-square test, one-way analysis of variance (ANOVA), Kruskal-Wallis one-way ANOVA, Pearson correlation coefficients, and Spearman rank correlation, as appropriate. OUTCOME MEASURES 1) Pulmonary function: forced vital capacity (FVC), forced expiratory volume in 1 second (FEV(1)), peak expiratory flow rate (PEF), forced expiratory flow between 25% and 75% of FVC (FEF(25-75%)), and maximum expiratory flows at 75%, 50%, and 25% of FVC (MEF(75), MEF(50), and MEF(25), respectively); 2) oxygen saturation: pulse oxymeter reading; and 3) respiratory muscle strength: maximal inspiratory mouth pressure (MIP) and maximal expiratory mouth pressure (MEP). METHODS Pulmonary function, oxygen saturation, MIP, and MEP were measured and compared in patients with NMD, patients with and without scoliosis, and in healthy subjects. RESULTS The patients with NMD, both with and without scoliosis, had significantly lower PEF, MIP, MEP, % predicted MIP (%MIP), and % predicted MEP (%MEP) than those of healthy subjects (p<.05). The patients with NMD and scoliosis had significantly lower values than those with NMD without scoliosis and controls (p<.05) for FVC, FEV(1), and FEF(25-75%). CONCLUSION Both inspiratory and expiratory muscle strength were diminished in patients with NMD compared with healthy controls. Significant differences were also noted in pulmonary function in patients with NMD with or without scoliosis. This suggests that NMD may impact respiratory function independently of the effects of scoliosis. Clinicians treating patients with NMD should be aware of the possibility of compromised respiratory function in these patients to address possible complications.

Spontaneous Neonatal Chylothorax Treated with Octreotide in Turkey: A Case Report

Chylothorax, which is usually idiopathic, is the most common form of pleural effusion encountered in neonates. Herein we report a 2-week-old infant who had spontaneous chylothorax and was treated with octreotide. Neonatal chylothorax responded well to octreotide treatment, which appeared to shorten the duration of hospitalization. We conclude that octreotide should be considered in the treatment of neonatal chylothorax.

BALF nitrite as an indicator of inflammation in children with cystic fibrosis.

Background: A unique substance reflecting the degree of inflammation localized to the lower respiratory tract in patients with cystic fibrosis (CF) has been the concern of several investigators and nitric oxide has gained interest for this purpose in the last decades. Objectives: The aim of this study was to evaluate the cytokine and nitrite levels by showing the relationship between them in serum and bronchoalveolar lavage fluid (BALF) of patients with CF and patients in which flexible fiberoptic bronchoscopy (FFB) was applied because of indications other than infection. Methods: 20 children with CF with mean age 8.2 years and 10 children as control group with mean age 4.2 years were included in the study. Cultures for aerobes, anaerobes, fungi and mycobacteria, cell differentials, cytokine and nitrite measurements were made from BALF specimens. White blood cell (WBC) count, erythrocyte sedimentation rate (ESR), quantitative C-reactive protein (QCRP), cytokine and nitrite levels were measured from serum samples. Results: BALF neutrophil, TNF-α, IL-8 and nitrite levels were significantly higher in patients with CF than control patients. There was no correlation between serum and BALF cytokine and nitrite levels. However, there was a significantly positive correlation between BALF IL-8 and nitrite levels in patients with CF (r = 0.5) and also in control patients (r = 0.6). Conclusions: The results suggest that BALF nitrite levels reflect the degree of inflammation localized to lower respiratory tract and may be a useful indicator of airway inflammation for patients with CF.

Successful unilateral partial lung lavage in a child with pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material accumulates within the alveoli. A 4-year-old child with autoimmune PAP, who was successfully treated with a series of unilateral partial bronchoalveolar lavages by selectively ventilating the other lung with a cuffed endotracheal tube, is presented.

Use of recombinant human DNase in a premature infant with recurrent atelectasis

Presence of mucus-plugging and abundance of thickened pulmonary secretions in the neonatal period are risk factors for pulmonary infections and prolonged artificial ventilation. The viscoelastic properties of airway secretions was known to be largely due to the presence of highly polymerized DNA. 1 Recombinant human DNase (rhDNase) degrades highly viscosic DNA and was shown to be efficient in patients with cystic fibrosis (CF). 2,3 The use of rhDNase for the treatment of atelectasis in patients in the neonatal intensive care unit is a new concept, intended to liquefy the mucus when conventional therapies fail. 4 We present our experience with rhDNase use in a premature infant with recurrent atelectasis.