An analysis of cystic fibrosis

The aim of this study is to present the first total number of tested children in the Federation of Bosnia and Herzegovina and the number of children with positive sweat test. During the study we determined the number of ill children, the median age of children with cystic fibrosis, date of initial diagnosis, an average amount of chloride in the sweat. In the period from March to Decemberwe have tested children. Female children were more affected then male children, in the ratio of 1:

An analysis of cystic fibrosis

The aim of this study is to present the first total number of tested children in the Federation of Bosnia and Herzegovina and the number of children with positive sweat test.

People with cystic fibrosis are at greater risk of getting lung infections because thick, sticky mucus builds up in their lungs, allowing germs to thrive and multiply. Lung infections, caused mostly by bacteria, are a serious and chronic problem for many people living with the disease. Cystic fibrosis (CF) occurs at increased frequency in Caucasians and individuals of Ashkenazi Jewish descent, but can occur in any ethnic group. It is a disorder of mucus production, primarily affecting the pulmonary, gastrointestinal and reproductive systems. Cystic fibrosis transmembrane conductance regulator (CFTR) is a membrane protein and chloride channel in vertebrates that is encoded by the CFTR gene.. The CFTR gene codes for an ABC transporter-class ion channel protein that conducts chloride and thiocyanate ions across epithelial cell urbanagricultureinitiative.comons of the CFTR gene affecting chloride ion channel function lead to dysregulation .

During the study we determined the number of ill children, the median age of children with cystic fibrosis, date of initial diagnosis, an average amount of chloride in the sweat.

In the period from March to Decemberwe have tested children. Female children were more affected then male children, in the ratio of 1: An average age of female children was 4.

The median concentration of chloride in the sweat measured by sweat test was for male children In the post-war period we started to use a sweat test. Male children tend to live longer than female children with CF.

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INTRODUCTION Cystic fibrosis CF or mucoviscidosis is a multisystem inflammatory disorder where the primary cause of morbidity and mortality is pulmonary destruction, characterized by chronic inflammation, bacterial colonization and frequent exacerbations 12. CF is an autosomal-recesive disease caused by mutations in the CF transmembrane conductance regulator CFTR protein at the chromosome 7q An analysis of cystic fibrosis mutation is a deletion of the three nucleotides that comprise the codon for phenylalanine at position Mutations of the CFTR gene affecting chloride ion channel function lead to dysregulation of epithelial fluid transport in the lung resulting in cystic fibrosis 3 - 7.

In the European Union, 1 in newborns is found to be affected by CF 567. CF occurs in one of every 3, live white births, and in 1 of every 15, live African American births, 1 of every 31, live Asian Americans births 28. This leads to obstruction of the airway lumen and a suitable basis for the development of pathogenic microorganisms 129.

In the thick layer of dense and tenacious secretions airway gradually create anaerobic conditions, which encourages bacteria to adjustment, especially pseudomonas, with the formation of alginate and mucous form of growth Cystic fibrosis is a chronic progressive disease that affects multiple organ systems and whose manifestations related primarily to the development of chronic lung disease and malnutrition.

Common CF symptoms are: Diagnosis is made by finding elevated levels of chloride in the sweat test. Sweat test is used to measure chloride and sodium levels in sweat. They can be performed at any time from 1 week of age, but adequate sweat volumes may not be obtained before 6 weeks of age.

Sweat electrolyte values in adults are often higher than children and need to be interpreted by an expert. The sweat glands are made to produce sweat with a mild chemical called pilocarpine and a little electricity pilocarpine iontophoresis 11 Sweat-testing involves application of a medication that stimulates sweating pilocarpine.

To deliver the medication through the skin, iontophoresis is used to, whereby one electrode is placed onto the applied medication and an electric current is passed to a separate electrode on the skin.

The resultant sweat is then collected on filter paper or in a capillary tube and analyzed for abnormal amounts of sodium and chloride. People with CF have increased amounts of sodium and chloride in their sweat The reference ranges of sweat chloride that are currently accepted for the diagnosis of CF in infants up to and including 6 months of age are: The reference ranges of sweat chloride that are currently accepted for the diagnosis of CF in people older than 6 months of age are: The test is carried out by collecting sweat from pilocarpine iontophoresis in two or more occasions and chemical determination of the concentration of chloride.

The conditions other than cystic fibrosis, which are associated with elevated concentrations of sweat electrolytes include malnutrition, adrenal insufficiency, glycogen storage diseases, anorexia, celiac disease, hypothyroidism, hypoparathyroidism, nephrogenic diabetes inspidus, ectodermal dysplasia, atopic eczema, and congenital metabolic disease.

Most of errors are caused by using unreliable methodology, irregular way of collecting sweat, technical errors and misinterpretation of results 12 We included all resident patients of the Federation of Bosnia and Herzegovina, consisting of Canton Sarajevo and 9 other cantons.

An analysis of cystic fibrosis

We selected all patients with positive sweat test, and also recorded age, gender, date of initial diagnosis, amount of chloride in the sweat test, the number of patients in Federation of Bosnia and Herzegovina.Call to request forms, or photocopy the Cystic Fibrosis Screening Questionnaire from the Genetics Appendix online.

References American College of Obstetricians and Gynecologists Committee on Genetics. ABOUT CYSTIC FIBROSIS. Learn about cystic fibrosis, a genetic disorder that affects the lungs, pancreas, and other organs, and how to treat and live with this chronic disease. Cystic fibrosis (CF) is a genetic disorder that usually affects the lungs and may also affect the pancreas, kidneys, liver, and intestine.

Long-term repercussions including difficulty in breathing and coughing as a result of recurrent lung infections can be seen in urbanagricultureinitiative.com: Coherent Market Insights. INTRODUCTION. Pancreatic insufficiency is the most common gastrointestinal complication of cystic fibrosis (CF), affecting approximately 85 percent of patients at some time in their lives [].The major consequences of pancreatic insufficiency are due to fat malabsorption, which is caused by decreased production of pancreatic enzymes.

Neonatal Sweat Test Newborn Screening For Cystic Fibrosis

Cystic fibrosis (CF) occurs at increased frequency in Caucasians and individuals of Ashkenazi Jewish descent, but can occur in any ethnic group. It is a disorder of mucus production, primarily affecting the pulmonary, gastrointestinal and reproductive systems. It is preferable not to perform additional testing on original tubes prior to PCR testing.

A completed screening questionnaire must accompany specimens. Call to request forms, or photocopy the Cystic Fibrosis Screening Questionnaire from the Genetics Appendix online.

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