Clinical Course of Children with Chronic Suppurative Lung Disease or Bronchiectasis Infected with .

Children with chronic wet cough and without cystic fibrosis (non-CF) may suffer from chronic suppurative lung disease (CSLD) or bronchiectasis. (Pa) can be one of the offending microbes in these children. The present study aimed to describe the clinical course of children with the above two conditions who were infected with Pa.

Physicians' ability to recognize adventitious lung sounds.

Background: Lung auscultation is an important tool for diagnosing respiratory diseases. However, the ability of observers to recognize respiratory sounds varies considerably and depends on the sound. The present study aimed to assess the auscultatory skills of healthcare professionals and medical students.

Asthma-Like Features and Anti-Asthmatic Drug Prescription in Children with Non-CF Bronchiectasis.

Bronchiectasis and asthma may share some characteristics and some patients may have both conditions. The present study aimed to examine the rationale of prophylactic inhaled corticosteroids (ICS) prescription in children with bronchiectasis. Data of children with radiologically established bronchiectasis were retrospectively reviewed.

In Vitro and In Vivo Preclinical Effects of Type I IFNs on Gliomas.

The interferons (IFNs) are a family of cytokines with diverse cellular actions such as control of cell proliferation and regulation of immune responses; therefore, they have been extensively studied as antitumor agents for a variety of malignancies, including gliomas. Type I IFNs exert their antitumor effects either directly, by targeting the tumor cells or the tumor stem cells, or indirectly, by regulating the anticancer activities of the immune system. More specifically, IFN-beta and IFN-alpha exhibit antiproliferative effects by p53 induction, CD8 T-lymphocyte and macrophage activation, chemokine secretion, and miR-21 downregulation.

Prognostic markers of pediatric meningococcal sepsis.

Having available tools to determine the prognosis of pediatric meningococcal sepsis at admission to the Intensive Care Unit or during the course of the disease constitutes a clinical necessity. Recently, new readily measurable circulating biomarkers have been described as an additional tool for severity classification and prediction of mortality in meningococcal disease. These biomarkers have been associated with increased risk of mortality scores and a number of organ failures in heterogeneous samples of critically ill children.

Androgen insensitivity syndrome: clinical features and molecular defects.

The end-organ resistance to androgens has been designated as androgen insensitivity syndrome (AIS), an X-linked disorder caused by mutations in the androgen receptor (AR) gene. It is generally accepted that defects in the AR gene prevent the normal development of both internal and external genital structures in 46,XY individuals, causing a variety of phenotypes ranging from male infertility to completely normal female external genitalia. Precise diagnosis requires clinical, hormonal and molecular investigation and is of great importance for appropriate gender assignment and management in general.

Sex-reversed phenotype in association with two novel mutations c.2494delA and c.T3004C in the ligand-binding domain of the androgen receptor gene.

Objective: To establish the diagnosis of complete androgen insensitivity syndrome (CAIS) in two patients with characteristic clinical and hormonal findings, relative family history in one of them, and unusual Müllerian remnants in the other.

Design: Case report.

Setting: Research laboratory in the Department of Medical Genetics at a university children's hospital.

The effects of erythropoetic activity and iron burden on hepcidin expression in patients with thalassemia major.

Hepcidin production is homeostatically regulated by iron stores, anemia and hypoxia. We evaluated the effect of iron overload and of ineffective erythropoeisis on hepcidin expression in patients with thalassemia major. Liver hepcidin mRNA levels correlated with hemoglobin concentration and inversely correlated with serum transferrin receptor, erythropoietin and non-transferrin-bound iron.

Binuclear copper(II) complexes of tolfenamic: synthesis, crystal structure, spectroscopy and superoxide dismutase activity.

The synthesis and characterization of copper(II) complexes with a potent non-steroidal anti-inflammatory drug, tolfenamic acid, Htolf, with formula [Cu(tolf)(2)L](2) (where L is H(2)O or DMF, N,N-dimethylformamide) were investigated. The crystal and molecular structure of [Cu(tolf)(2)(DMF)](2) was reported. Crystallographic data are as follows: monoclinic system, space group P2(1)/n with cell constants a=9.