Talk:Cystic fibrosis

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[edit] Starting from first principles

I'm probably going out on a limb here, but is it not medically accurate to say introductorily that CF is caused by incompetence of exocrine glands? This explains the digestion and breathing problems.Jefferson61345 (talk) 07:59, 13 March 2008 (UTC)

[edit] Chloride in or out?

How come the CFTR article says that the protein transports chloride out of the cell (which I believe is correct) whereas this one says it moves it from outside to inside the cell? 129.67.50.206 16:33, 8 March 2007 (UTC)

Choride most definately gets trapped inside the cell. Hence the clinical symptoms of sticky mucus, as the reduced secretion of salt from the epithelial cells causes reduced water excretion. The salty sweat is likely caused by a CFTR independent process of chloride secretion in sweat glands, coupled with a reduced sodium chloride reabsorpton. This the commonly accepted theory, the writer who did this paragraph was a moron. I bet a lot of school kids doing research for biology have been buggered by this wiki heh. 129.31.65.236 21:04, 2 June 2007 (UTC)

I agree! Why hasn't this been fixed? —Preceding unsigned comment added by 131.172.4.44 (talk) 23:19, 17 April 2008 (UTC)


I think the problem is that the paragraph in question mentions the CFTR channels of the lungs and of the sweat ducts in the same sentence. Then, the following sentence refers specifically to the CFTR of the sweat duct, which brings Cl- from the lumen of the sweat duct into the duct cells. This is "reversed" relative to (I think) all other CFTRs in that the others behave mainly in a secretory manner; they allow Cl- to pass from inside the cell into the lumen or the ECF. I think this article is in need of an expert to clarify the matter. Quartertone (talk) 16:37, 23 April 2008 (UTC)

The CFTR gene can do both. In the airways it transports chloride out of the cell, however in the sweat ducts it transports chloride into the cell bringing sodium into the cell also. In CF the chloride remains trapped outside of the cell in the sweat which prevents Na+ from entering the cell = salty sweat. In the airways the decreased chloride output results in a sharp increase in Na+ being transported into the cells. This change in polarity causes water to move into the epithelial cells resulting in sticky mucous. (Robbins and cotran , Pathalogic basis of disease 7th ed. (Kumar, Abbas, Fausto) Alex

[edit] The austrian painter would be proud of it!

Article says: "to provide fertility for people with cystic fibrosis"

That cannot be true, else this looks like a major crime against humanity. Who has the right to create foetuses and then children, who are bound to die early due to inherited fatal CF disease, just to satisfy the vanity of some terminally ill people? This is worse than Mengele's death camp acts, here doctors are actually torturing people, as the kids who are borne to CF patients will predictable suffer a lot and continously, before they meet their terrible early death.

This is probably in the same amoral league what press reports say many US doctors are now helping dwarf or deaf or blind couples in USA with selective artifical insemination, to guarantee their children will also be dwarf-sized, deaf or sightless, just to satisfy the vanity of their parents. That should be a felony! 81.0.68.145 23:09, 6 April 2007 (UTC)

" kids who are borne to CF patients will predictable suffer a lot and continously, before they meet their terrible early death." You assume that all children by a person with CF will have CF themselves, that is not true. Couples in which both have CF is a rarity and discouraged due to cross contamination concerns; even then it's not your concern or problem but the concern of the couple. --ImmortalGoddezz 01:54, 7 April 2007 (UTC)
Furthermore, there is nothing in the article that says that doctors are selecting for the child to be born with CF. What you (the anonymous user) are talking about is the conscious choice of a minority of dwarf/deaf/blind couples, working in collaboration with their doctors, to select for, or guarantee that their child will be like them. --Kyoko 02:38, 7 April 2007 (UTC)

[edit] Changes

I removed this line added to the beginning of the article: 'The patient lacks a gene that codes for the trans membrane carrier of the chloride ion.patients often die due to to numerous infections of the respiratory tract.' This is certainly good info, but shouldn't start the article. --Marco Passarani 17:05, 3 May 2007 (UTC)

[edit] Grégory Lemarchal

I've added a bit about the death of the French singer Grégory Lemarchal. I hope this is deemed appropriate because his fame has undoubtedly increased the awareness of the disease in France and a special TV programme shown last night has so far raised more than 7.5 million euros. Surely this is notable as I have never heard of any other such programme dedicated to the fight against CF. User:AcerBen, 11:17, 5 May 2007 (GMT)

[edit] Selenium defficiency while the mother is pregnant is the cause of cystic fibrosis (CF)

Sadly, most doctors have never even heard of selenium as an essential nutrient. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=3820518&dopt=Abstract

READ this to understand:

Frank clinical selenium deficiency has been described in cystic fibrosis (CF), and a relative deficiency has been proposed as contributing to the pathogenesis of the disease. Because of these possibilities, we investigated the relationship between overall nutritional status in CF with measures of selenium nutriture. Fifteen stable outpatients with CF (group I) were compared to 13 age-matched controls (group II) and 27 healthy adults (group III). Whole blood, plasma, and red blood cell selenium levels were reduced by 31%, 29%, and 33%, respectively, in CF patients vs controls (all p less than 0.001). In addition, both groups I and II showed significantly lower blood selenium levels than healthy adults (p less than 0.005). Nutritional assessment revealed CF patients to be undernourished, with significant decreases in serum albumin (p less than 0.025), weight-for-height deficit (p less than 0.01), and weight-for-age (p less than 0.025) vs controls. However, only the triceps skinfold (TSF) measurement correlated significantly with selenium status (r = 0.56: p less than 0.05 for whole blood selenium vs TSF). We conclude, based on the magnitude of decrement in blood selenium, that it is unlikely that selenium plays a significant primary pathogenic role in cystic fibrosis. However, these patients are at high risk for developing clinical selenium deficiencies. The measurement of blood selenium levels using appropriate age-matched normal standards should be mandatory in all CF patients with malnutrition, or in those requiring parenteral nutritional support.

Is it just me, or does the title of this section oppose the conclusion drawn by the study's authors? Quoting from above:
"We conclude, based on the magnitude of decrement in blood selenium, that it is unlikely that selenium plays a significant primary pathogenic role in cystic fibrosis."
The study only suggests that CF patients may be at risk for developing clinical Selenium deficiencies, not that Selenium deficiency causes CF. AncientBrit 02:08, 7 November 2007 (UTC)

[edit] 1 in 1,000?

The cff.org site says there are about 30,000 cases in the U.S. Given that there are about 300,000,000 people in the U.S., that would be 1 in 10,000 people, not 1 in 1,000 as Wikipedia states.

  • Noted. I couldn't verify the values given for the other regions either, so I updated it with info I found on CFRI's site PxT 19:18, 21 May 2007 (UTC)
The prevalence of a disease is influenced by how lethal it is. If all CF patients lived twice as long as they do now, the prevalence would be double what it is now. I think the 1:1,000 is the incidence of CF, but only in certain populations. Una Smith 04:03, 14 June 2007 (UTC)

[edit] Prenatal diagnosis

The article on prenatal diagnosis currently says In some genetic conditions, for instance cystic fibrosis, an abnormality can only be detected if DNA is obtained from the baby. Would someone here please rewrite that?! Una Smith 04:03, 14 June 2007 (UTC)

[edit] Adding a link to a CF-related web site

Hi, I would like to add a link to my CF web site. I think it would be a good and relevant link to add, because my CF site has been up for 10 years, and has had nearly a million visitors. It also ranks very well in Google. Today it ranks as number two, after the Canadian Cystic Fibrosis Foundation. It is always in the top ten. I had tried adding it myself before, but somebody removed it, and I don't know why. That is why I am asking for permission to add it this time. I am new to participating in the inner workings of Wikipedia (but I read the articles a lot), so bear with me for my newness to the situation. Also, is there a person who is in charge here, who makes the major decisions about the CF wiki page, or is it just a whole bunch of people who make the decisions?

Anyways, I hope that those concerned can take a look at my page, and make a decision whether to include it or not in the links area. Here is the URL: http://www3.nbnet.nb.ca/normap/CF.htm It has an excellent reputation, and is updated regularly. I have received many compliments on it, and it's usefulness.

I am a person with CF, who also happens to have been a Registered Nurse, so I do have a pretty good medical background, especially when it comes to CF. I will await any responses, and if nobody responds, I will recreate the link again, hoping it will be up to stay...

Thanks!

Norma Kennedy Plourde, 46, with CF (Oct. 7, 2007) Normaplourde 17:41, 7 October 2007 (UTC)

[edit] Diabetes clarification

The type of diabetes experienced by CF sufferers is a unique form, possessing elements of both Type 1 and Type 2. One term I have seen used is CFRD (cystic fibrosis related diabetes).

Would the article benefit from the inclusion of a separate section on CFRD?

My original information comes from the parents of a CF sufferer in his 20s who has just developed CFRD, but there are online resources available - CFWW has a useful overview page at www.cfww.org/pub/newsletter/nl01a/nl01af.htm. I don't know that I am qualified to write this up. AncientBrit 15:30, 17 October 2007 (UTC)

Diabetes is already mentioned in the article however it's not referred to as CFRD. I'll see about editing it for clarification. --ImmortalGoddezz 16:47, 17 October 2007 (UTC)
Great - thanks very much. I think CFRD could do with an entry of its own - it's a separate and distinct form of diabetes that may have a different cause (compared with T1 and T2). AncientBrit 17:11, 19 October 2007 (UTC)

[edit] Grammar issue

Re. the line: "In her paper, she described the characteristic cystic fibrosis of the pancreas correlated it with the lung and intestinal disease prominent in CF." This line doesn't read clearly. "...and correlated..." would be more clear, if that's the intent. Cheers, Kevin 70.52.216.160 08:43, 22 October 2007 (UTC)

[edit] worth adding this to Treatment?

Hello, I was editing the article on bronchiectasis, and I found this section of text:

Mannitol dry inhalation powder, under the name Bronchitol, has been approved by the FDA for use in cystic fibrosis patients with bronchiectasis. The original orphan drug indication approved in February 2005 allowed its use for the treatment of bronchiectasis. The original approval was based on the results of Phase II clinical studies showing the product to be safe, well-tolerated, and effective for stimulating mucus hydration/clearance, thereby improving quality of life in patients with chronic obstructive lung diseases like bronchiectasis. Long-term studies are underway as of 2007 to ensure the safety and effectiveness of the treatment.[1]

The original reference link is here. Is it worth adding this to the CF article, or is this too specific and better left out? Thanks. --Kyoko 15:14, 1 November 2007 (UTC)

too specific. There are a lot of drugs that have come out/are coming out (eg. Tobramycin, Pulmozyme, Zithromycin, Aztreonam inhaled, etc) that seem to really help CFers however they aren't all mentioned in the article and it'd be tedious to insert them all in there. Especially since some people are sensitive to some drugs and some aren't and we really can't list all the drugs anyway since it'd be an impossible task. --ImmortalGoddezz 15:21, 1 November 2007 (UTC)
Yeah, that was my concern as well, that it might have been too specific a population to list in a general article. Thanks for your input. --Kyoko 15:27, 1 November 2007 (UTC)

[edit] CF and lung transplants

Hello, I reverted this edit, where an anonymous user had struck through content about CF and lung transplants. The anon user is correct that CF is a genetic disorder; however, the lungs of a person with CF are frequently colonised by pathogenic bacteria that could potentially infect a transplanted lung if only one lung and not two were transplanted. I hope this clarifies things. --Kyoko 23:58, 11 November 2007 (UTC)

[edit] Lung transplants no help for cystic fibrosis: study

Lung transplants no help for cystic fibrosis: study Ling.Nut (talk) 02:59, 23 November 2007 (UTC)

That article specifically mentions for patients who receive transplants under 18.. which doesn't account for those who receive transplants after 18. Being quite honest I think it's a bunch of bull. The main reasons for getting a transplant is to both extend life and to improve the quality of life for as long as it is extended. Only one of which is measured and reported upon in the article; quality of life isn't, which would be hard to quantitatively measure anyway. Also transplant isn't a 'treatment', it is a last measure step taken to prevent death just a little bit longer. Anyway I don't think this should be included in the article unless there's a specific study published (eg. chest journal, lung, pub med) with specific statistics, so far when looking it up I can't find it. Anyway when looking it up I come across this so obviously it is disputed information and it looks like the study hasn't taken everything into account from the looks of it. --ImmortalGoddezz 04:06, 23 November 2007 (UTC)

This is the original study. JFW | T@lk 01:54, 29 November 2007 (UTC)