Cystic Fibrosis : Scientifically Described As A Disease In 1936
Cystic fibrosis (for "disease of viscous mucus in French) or cystic fibrosis (for"
cystic fibrosis "implied" pancreas "in English) is a genetic disease affecting the glandular epithelia many organs.
This genetic disease is linked to mutations in the CFTR gene on chromosome 7, resulting in alteration of the CFTR (cystic fibrosis transmembrane acronym for conductance regulator). This protein is an ion channel permeable to chlorine, thiocyanate whose function is to regulate the transport of chloride across cell membranes. Its dysfunction causes increased viscosity of mucus and its accumulation in the respiratory and digestive tracts. The disease affects many organs, but the breathing is predominant and account for most of the morbidity.
The most common clinical form associated respiratory disorders, digestive disorders and growth disorders thrive. In chronic, progressive disease often expressed early in infancy although there are crude forms of delayed diagnosis.
The diagnosis is based on the sweat test confirmed by identification of genetic mutations. Neonatal screening, widespread in France since 2002 allows diagnosis and early treatment so that genetic counseling enables couples heterozygous known not to have another child sick.
There is no cure, but advances in management have improved the quality and expectancy of life of patients and in France, life expectancy at birth increased 7 years in 1965 to 47 years in 2005 .Known since the Middle Ages, the disease is described scientifically by the Swiss pediatrician Guido Fanconi in 1936 . She identified two years later by Dorothy Andersen Hansine as a disease entity reaching the pancreas where its historic name of cystic fibrosis. It retains this name in English: cystic fibrosis.
Cystic fibrosis, scientifically described as a disease in 1936, was in fact already long known. In the Middle Ages were known doom newborn whose mother had noticed the "salty kiss, that is to say, the salty taste left by a kiss on the forehead of the child .Welsh cites an old adage of the folklore of Northern Europe : "Woe to the child in whom a kiss on the forehead has a salty taste. He is bewitched and soon must die.
In 1606, the Spanish doctor Alonso y de los Ruyz FONTEC of past reports that the fingers on the forehead of the bewitched children have a salty taste . Rochholz in his almanac of 1857 wrote "The child will soon die whose forehead kiss is salty.. According to Busch One of the first medical descriptions of injuries encountered in pancreatic cystic fibrosis could be an autopsy report done by Professor Pieter Pauw, Leiden in the Netherlands in 1595, in which he describes a woman miserable 11 years supposed to spell and having an enlarged pancreas, hard and white.
In the nineteenth century Viennese physician Karel Rokitansky reported a fetus with a fatal case of meconium peritonitis , who was later identified as a complication of meconium ileus, neonatal intestinal obstruction present in some children with cystic fibrosis described in 1905 by Landsteiner .
In the early twentieth century the first observations involving lung disease, diarrhea and pancreatic anomaly with several cases in the same family. In 1912, Garrod describes families whose children have diarrhea fat and die of lung infection. These descriptions usually focus on digestive problems, steatorrhea and pancreatic disorders, and their authors are a form of disease cliaque although lung problems are also noted.
In 1936, in a thesis written in German and chaired by the Swiss pediatrician Guido Fanconi, the disease is described for the first time in children with disease cliaque supposed, under the name "cystic fibrosis and bronchiectasis . Cystic fibrosis was considered a distinct disease entity in 1938 by the American pediatrician Hansine Dorothy Andersen, a physician at Babies' Hospital in New York, who published an article titled "Cystic fibrosis of the pancreas and its relationship with disease cliaque .
While studying autopsies on infants she described the clinical and histological features of the disease, including neonatal intestinal obstruction, respiratory and digestive complications and histological lesions of pancreas specific. It linked up the disease to a deficiency in vitamin A and persisted to support this theory for many years although it was never confirmed.
The term cystic fibrosis, created from the words "mucus" and "viscous" was first used in 1943 by Dr. Sydney Farber , chief physician at Children's Hospital in Boston, to correct the name used Dorothy Andersen, centered on the pancreas. Farber was convinced that the disease was due to the widespread diffusion of viscous mucus. The term CF is widely used worldwide, particularly in France, and is sometimes preferred to the English term cystic fibrosis .
by: Laura Steinfield
The Quran Declares a great Scientific Fact, is NASA being able to discover it? The Unique Scientific Challenge of the Quran Easy To Get Too Wet Unscientific "humidifier Pneumonia" Scientific Careers: Crazy Inventors Or Helpful Professionals Scientific Awards: The Albert Einstein World Award Of Science Paper Industry More Innovative New Deal Comprehensively Implementing The Scientific Concept Of Scientific Careers: If You Want To Become Successful Scientific Grants: What Is It And How To Apply? Academic (scientific) Grants: Various Elements Of Application Scientific Careers: Do Not Listen To Others, Choose Your Path Is there Scientific Proof that God Exists? Part 3 Scientific knowledge of PING G15 Iron Levels Of Water Purity For Scientific Applications