Why is CF called "cystic" and "fibrosis"?

Old Days. Back in the "old days, " patients with CF would frequently die, and the autopsy findings of the lungs showed scarring ("fibrosis") and bubbles ("cysts, " "cystic"). Thus, it was called cystic fibrosis. We know much more now about this disease, but the old name stuck.
History of the Dz. Back when infants were dying from an unknown disease, autopsies showed pathologic changes in the pancreases (pancrelipase) of these children. The description of the pathological tissue was that of showing "cystic" changes with subsequent "fibrosis" of the tissue, and thus the name stuck. The term "cystic fibrosis" describes the changes that occur to various organs as a result of the disease.

Related Questions

Please help! What is the cystic fibrosis vest called and what are the med names that are used with said vest?

TheVest. Com. One website to check is thevest. Com this is a type of physiotherapy used in CF to help mobilize airway secretions. I hope this helps.

What kind of condition is cystic fibrosis (c.F.)?

Progressive disease. Cystic fibrosis (CF) is a progressive, lifelong condition in which the glands that produce mucus, sweat, and intestinal secretions do not function properly. Thick mucus accumulates in the lungs, leading to breathing difficulty and infection. CF also impairs digestion, and affected males are usually infertile.
Genetic disease. It affects about 70, 000 people worldwide, primarily caucasian (~90%). The defective gene codes for a chloride channel that is present on the inner surface of the lungs, GI tract, pancreas, liver, and other organs. Lack of function of the channel results in thick, viscid mucus secretions in those organs. Obstruction of the airways results in infection and lung damage, and can be life shortening.

My son has hirschsprung disease. My wife tested neg. For cystic fibrosis. Are the two related? Should I be tested for CF? I'm 32 w/no issues.

Yes. There is a relationship between hirschsprung and CF. Both may present with passage of meconium problems at birth. Was your son tested for CF?
No, not related. Hirschsprung's disease is a congenital condition that leads to absence or lower numbers of nerve cells in the large intestine that are responsible for allowing the colon muscles to function, leading to constipation or inability to pass stool. Cystic fibrosis is an inherited recessive disorder of salt transport leading to thickened secretions. If your wife is negative, no need for you to be tested.
Susceptibility to. Hirschsprung Disease can be caused by a specific gene mutation on the long arm of Chromosome 10, but around 8 other gene mutations on different Chromosomes have also been found & the pattern of inheritance is complex. CF is caused by a gene mutation on chromosome 7. If mom is not a carrier, no need to test dad. Meconium ileus in CF differs from Hirschsprung disease.

Newborn boy has a severe mutation delf508 and a 5-t gt12. Was told that he might or might not develop mild cystic fibrosis. How does mild CF manifest?

Varies. Mild CF can vary from recurrent sinus infections, recurrent pneumonia, pancreatitis, fertility problems. One may have no evidence of CF until adulthood. Sometimes the first sign is recurrent pancreatitis in the second or third decade of life. Usually growth is not impaired.
Sweat test. It can be difficult to predict the course of CF. If the sweat chloride result is borderline or normal, then typically there are very few or no symptoms.

My wife & son passed california newborn screening for CF, does that mean odds are low for typical cystic fibrosis or mild form?

Newborn screening. For Cystic Fibrosis is done by Immunoreactive Trypsinogen, IRT. If the IRT level is ^, DNA tests for CF gene mutations are done on blood on the Guthrie card. CF Foundation-accredited Care Centers in CA look for an "intermediate" sweat chloride result in young infants who do not gain wt. & have bad gas, abdominal pain & loose or hard stools &/or wheezing/coughing for > 2 wks. To diagnose CRMS.

My son & wife tested neg for Cystic Fibrosis through screening test, not sweat test. However my son has hirschsprung disease. Should I test for CF - or it's high unlikely I have it? I'm 32. Feel fine

CF. If you are 32 and have no symptoms, you do not have CF. Do not worry. Hope this helps. Dr R.
I wouldn't. There is no relationship between Hirschprung disease and CF. If you wand to know if you are an asymptomatic carrier of CF, the tests are available. However, you don't get to age 30 without symptoms when you have the disease and your fertility would also be diminished. If your wife is not a carrier, you can not produce babies with CF.
Depends. You could still be a carrier. It would only matter if you and wife were carriers. There is no coorelation with hirschsprung's as different chromosomes are involved.
Rare association. Very rare association between Hirschsprung's disease and cystic fibrosis.