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.     

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

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 approaches 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.     

Immunohistochemical analysis of c-myc, c-jun and estrogen receptor in normal, hyperplastic and neoplastic endometrium

To evaluate the role of c-jun and c-myc proto-oncogenes in normal, hyperplastic and neoplastic endometrium in relation to estrogen receptor (ER) status and to investigate whether these genes can be related to other histopathological features of endometrial carcinoma, 32 endometrial carcinomas, 38 endometrial hyperplasias and 22 cyclic endometria (10 proliferative and 12 secretory) were evaluated histologically. Endometrial hyperplasia cases were classified as simple and complex hyperplasia without atypia, and atypical hyperplasia. Endometrial carcinoma cases were subtyped according to the International Society of Gynecological Pathologists. Modified FIGO system was used for both grading and staging. Immunohistochemical examination was performed using antibodies to ER-alpha, c-myc and c-jun with streptavidin-biotin-peroxidase technique. The mean percentage of ER-alpha positive cells changed cyclically during the menstrual cycle, and it was the highest (96%) and the lowest (31.6%) in proliferative and carcinomatous endometrium, respectively. There was a statistically significant difference between proliferative and secretory phases and proliferative and carcinomatous endometrium in relation to ER-alpha staining (p<0.05). There was also a statistically significant difference with respect to ERalpha reactivity between secretory phase and each hyperplastic group, as well as between the carcinoma group and each hyperplastic group (p<0.05). Although not significant, the mean percentage of c-myc expressing cells in the carcinoma group was higher (15.3%) than that of proliferative phase and hyperplastic groups. The mean percentage of c-jun positive cells in proliferative endometrium was slightly higher than in secretory endometrium, and it was the highest in atypical hyperplastic endometrium (28.3%), but there was no statistically significant difference between the groups. In carcinoma cases, a positive correlation was observed between c-jun positivity and tumor grade (p=0.027, r=0.3908), but such a correlation with c-myc was not found. A positive correlation was detected between ER-alpha and c-myc expression (p=0.038, r=0.3686). A progressive loss of ER seems to be correlated with increasing malignant transformation. C-myc expression might play a role in the development of endometrial carcinoma via ER. The association between c-jun and ER appears to be lost in endometrial carcinoma. The relationship between c-myc, c-jun and ER appears to be altered in endometrial carcinoma compared to that of menstrual endometrium.     

Gastric organo-axial malrotation coexisting respiratory symptoms

Gastric malrotation is defined as a torsion of stomach around its short or long axis. It is a rare disease in childhood. Gastric malrotation may present either as a surgical emergency or as chronic abdominal symptoms. There is limited data about the respiratory symptoms associated with gastric malrotation. The aim of this study was to review the records of 14 children who presented with respiratory symptoms and diagnosed as gastric organo-axial malrotation. Between August 2005 and August 2007, 14 children diagnosed as having gastric organo-axial malrotation participated in this study. There were 11 boys and three girls with a mean age of 7.1 months. All patients were symptomatic. Presenting symptoms included wheezing in four patients, recurrent pneumonia in four, chronic cough in two, chronic cough and apnea in two, recurrent pneumonia and chronic cough in one, and chronic cough and failure to thrive in one. All of our patients had organo-axial gastric malrotation. Gastroesophageal reflux was found in nine patients (64.2%). Six patients were treated with surgery and antireflux medication and the remaining eight patients with antireflux medication. In conclusion, it is probable that gastric malrotation associated with massive or occult gastroesophageal reflux could be responsible for the respiratory symptoms such as chronic cough, recurrent pneumonia, wheezing, and apnea.     

A critical role for phospholipase C in protective immunity conferred by listeriolysin O-deficient Listeria monocytogenes

Attenuated recombinant Listeria monocytogenes (Lm) strains are a promising class of vaccine vectors that trigger protective antigen-specific CD8 T cells. Listeriolysin O (LLO) is an important Lm virulence determinant allowing the bacterium to escape from the endocytic vacuole into the cell cytoplasm in phagocytic cells. However in non-phagocytic cells, Lm phospholipase C can also mediate cytoplasmic entry. The ability of LLO-deficient Lm to confer long-term protection to infection is uncertain. Herein, we demonstrate that LLO-deficient Lm mutants can prime protective immunity to subsequent Lm infection and that Lm phospholipase C is required for protective immunity conferred by LLO-deficient Lm.     

Hypercalciuria and nephrocalcinosis in cystic fibrosis patients

The objective of this study was to determine the frequency of nephrocalcinosis and hypercalciuria in cystic fibrosis (CF) patients, and to search possible causes of this phenomenon. Forty-three CF children (24 boys, 19 girls; mean age 64.9 months, range 5 months-18 years) were included in this study. Plasma sodium, potassium, chloride, BUN, creatinine, calcium, phosphorus, magnesium, alkaline phosphatase; spot urine sodium, potassium, chloride, creatinine, calcium, magnesium; and serum 25-hydroxyvitamin-D levels were measured in all patients. Urine samples were examined for microscopic hematuria. Fractional sodium, potassium, chloride excretion and estimated glomerular filtration rate (GFR) were calculated. All patients underwent renal ultrasonography. Hypercalciuria, nephrocalcinosis and microscopic hematuria were detected in 15 patients (34.2%), 10 patients (23.2%) and two patients (5%), respectively. There was no significant but borderline correlation between 25-hydroxyvitamin-D levels and hypercalciuria (r: 0.308, p:0.05). There were no correlations between Shwachman clinical scoring system results and hypercalciuria (r: 0.221, p: 0.148) and age and hypercalciuria (r: -0.229, p: 0.135). Patients with chronic Pseudomonas colonization showed no hypercalciuria or nephrocalcinosis. There was no difference for plasma biochemical results, renal function tests, hypercalciuria and nephrocalcinosis between CF patients who had or had not experienced pseudo Bartter's syndrome (PBS) before. There was no relation between detected CF mutations of the patients and hypercalciuria and nephrocalcinosis. These results suggested that it is a primary abnormality of calcium metabolism in the kidney.     

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

Pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material acumulates 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.     

Ultrasonography in carpal tunnel syndrome: comparison with electrophysiological stage and motor unit number estimate

The purpose of this study was to document the ultrasonographic measurement differences in median nerve size between patients with carpal tunnel syndrome (CTS) and controls, and to correlate these findings with electrophysiological stage and motor unit number estimation (MUNE), thereby allowing us to test the validity of ultrasound as a diagnostic modality for assessing the severity of CTS. High-resolution sonography and electrophysiological studies were performed on 41 wrists of 27 patients and compared with findings on 40 wrists of 20 healthy individuals. On ultrasonographic views, cross-sectional area and flattening ratio in proximal, middle, and distal tunnel segments of the median nerve were measured both by calculating ellipsoid area by large and small cross-sectional diameters and by automated ellipsoid area calculation. We compared electrophysiological stage and MUNE with proximal, middle, and distal cross-sectional area and other ultrasonographic findings. All correlations between electrophysiological stage and cross-sectional areas in these different segments of the median nerve were significant with both measurement methods. Negative correlations were seen between MUNE and cross-sectional area in the proximal and middle segments, whereas no significant correlation was detected in the distal segment. Our results indicate that there are close correlations between the ultrasonographic findings and electrophysiological stage. Ultrasound also reflects the reduction in the number of axons estimated by the MUNE method. Therefore, we suggest that the ultrasonographic findings reflect the severity of disease in patients with CTS.