cystic fibrosis

Cystic fibrosis

Amyotrophia (5)
Chromosomal aberration (1)
Cystic fibrosis (17)
Genetic disease (4)
Mongolism (5)
Muscular dystrophy (8)
Thrombasthenia (1)

Synopsis: 5. medicine & health: 1. diseases: Diseases: Genetic or hereditary diseases: Cystic fibrosis:

Nature 01858.txt

Growth factor makes a comeback in cystic fibrosis: Nature Newsthe stunted development common to cystic fibrosis begins at birth

and could be a direct consequence of a growth-hormone deficiency caused by the disease. In both people and pigs, newborns with cystic fibrosis tend to have abnormally low levels of a hormone called insulin-like growth factor 1 (IGF1),

according to a study published this week in the Proceedings of the National Academy of Sciences1.

whether a patient with cystic fibrosis will have growth problems later in life, says David Stoltz, a physician in the Department of Internal medicine at the University of Iowa, Iowa City,

Cystic fibrosis is a deadly genetic disease: many patients don't live past the age of 30. Scientists identified the culprit gene some two decades ago (see'Human genetics:

In the 1990s, researchers found that individuals with cystic fibrosis have low levels of IGF1 in their blood2,

and growth problems are caused not directly by the cystic fibrosis gene, but rather are by-products of malnutrition and lung inflammation.

Stoltz and his collaborators took advantage of a pig model of cystic fibrosis that they debuted in 20084.

The researchers then screened for IGF1 in samples of dried blood from 23 human newborns with cystic fibrosis.

The cystic fibrosis gene CFTR codes for a protein called the cystic fibrosis transmembrane regulator, which helps to move chloride ions across cell membranes.

that problems with insulin secretion associated with cystic fibrosis are stalled responsible for the IGF1 production in newborns insulin regulates the production of IGF1 in the liver in utero and throughout life.

In any case, the findings point to IGF1 as a potential therapy for cystic fibrosis particularly because regulators in the United states and Europe have approved already synthetic IGF1 for the treatment of severely short stature.

which IGF1 is being administered to adults with cystic fibrosis. But before rushing to treat infants with the hormone,

Nature 03468.txt

and pharmaceutical laboratories, has bred so far about 275 Â pigs some with cystic fibrosis, others with heart disease, arrhythmia or cancer,

In 2009, the company submitted its first application to the FDA for approval of its cystic fibrosis pig model."

ScienceDaily_2014 17910.txt

but can cause opportunistic or hospital-acquired infections in people with weakened immune systems or with cystic fibrosis.

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