Ayşe Tana Aslan

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.

The role of chemokines in exercise-induced bronchoconstriction in asthma

BACKGROUNDnThe pathogenesis of exercise-induced bronchoconstriction in asthma is incompletely understood, and the role of chemokines has not been investigated.nnnOBJECTIVEnTo investigate the involvement of the CC chemokines eotaxin, regulated upon activation normal T-cell expressed and secreted (RANTES), thymus and activation-regulated chemokine (TARC), and the CXC chemokine interferon-gamma-inducible protein 10 (IP-10) in exercise-induced bronchoconstriction.nnnMETHODSnFour groups were enrolled: asthmatic children with positive (n = 15) and negative (n = 15) responses to exercise, children with cystic fibrosis (n = 14), and healthy children (n = 11). Levels of eotaxin, RANTES, TARC, and IP-10 were determined in plasma before, immediately after, and 6 and 24 hours after exercise challenge using enzyme-linked immunosorbent assay. Transcriptional activity was measured using reverse transcriptase-polymerase chain reaction.nnnRESULTSnExercise did not induce any significant changes in systemic chemokine levels. A significant difference was observed only in the preexercise IP-10 levels among groups (P = .045). There was a significant difference in peripheral blood eosinophil counts among groups (P = .003). In asthmatic children with a positive response to exercise, there was an inverse correlation between eosinophil counts and eotaxin levels (r = -0.616; P = .01) and between forced expiratory volume in 1 second and TARC levels (r = -0.865; P = .001). Reverse transcriptase-polymerase chain reaction studies did not show any difference in the transcription of the chemokines.nnnCONCLUSIONSnExercise does not cause any changes in the systemic expression of eosinophilic chemokines. Peripheral blood eosinophils may be a determinant of the exercise response, and eotaxin and TARC may be associated with eosinophil counts and forced expiratory volume in 1 second in children with a bronchoconstrictor response to exercise.

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.

TAP1 and TAP2 gene polymorphisms in childhood cystic echinococcosis.

The incidence of cystic echinococcosis (CE) due to Echinococcus granulosus is as high as 2000-2500 patients per year in Turkey. Whether genetic characteristics of the Turkish population cause a tendency to the disease is currently unknown. We aimed at studying the role of TAP gene polymorphisms in Turkish children with cystic echinococcosis. For an overview of allelic distribution of TAP1 and TAP2 genes, genotypes of 85 patients with CE and 100 controls were studied. To determine the genotype-phenotype correlation, 81 of the patients whose clinical data were available were analyzed. For TAP1-637, Asp/Gly heterozygosity was significantly more prevalent in CE patients than in controls (20 vs. 4%, odds ratio 6.0), while Gly/Gly homozygosity was less frequent (5 vs. 14%). For TAP2-379, Ile/Val heterozygosity was significantly more prevalent in CE patients than in controls (14 vs. 1%, odds ratio 16.27), while Ile/Ile homozygosity was less frequent (13 vs. 25%). TAP1-637 and TAP2-379 polymorphisms may have a role in causing genetic tendency for CE in children. The data may reflect the genetic properties of the Turkish population or may reveal the minor role of TAP gene polymorphisms in CE.

Prenatal period to adolescence : the variable presentations of congenital cystic adenomatoid malformation

Background: Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare but potentially life‐threatening pulmonary anomaly.