Talk:Cystic fibrosis/Archive 2

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Archive 1 Archive 2

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)

Because it's correct. According to the logic that Cl- becomes trapped in EPITHELIAL cells of patients with cystic fibrosis (i.e. with inhibited or non-present CFTR protein channel), the protein (CFTR) is NOT able to transport Cl- out of the pulmonary epithelial cell as it would normally do. I agree with the comment below though; the distinction between sweat duct and epithelial mechanism should be made clear. —Preceding unsigned comment added by 70.55.142.202 (talk) 21:15, 24 March 2011 (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

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)
You're right on. As a person with Cystic Fibrosis who would like to have a child one day, I would certainly have my partner screened beforehand. Hillthekhore (talk) 14:23, 18 July 2008 (UTC)
The time a child is guaranteed to be born with cystic fibrosis is if both parents suffer from the disease, a situation which I am sure rarely occurs. I doubt that many cystic fibrosis patients, knowing full well the difficulties of their disease, would choose purposely to create a child which would suffer from the same. A CF patient can quite safely have children with a person who is not a CF carrier. --Kate M., 6:32 PM, Aug. 2, 2008

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)

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)

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)

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)

3-15-09 I have just read the article given by the CF org. They state that the ratio is about 1 in 3900, by USER:Trisha Williams —Preceding unsigned comment added by 99.153.185.205 (talk) 03:15, 15 March 2009 (UTC)

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)

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)

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)

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)

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)

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)

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)

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)

Alex Deford

It seems like the life of Alex Deford, daughter of Sports Illustrated writer Frank Deford and the subject of his book Alex: The Life of a Child would be worth a mention here, maybe in the Public Awareness section. Anybody? —Preceding unsigned comment added by 71.93.244.9 (talk) 01:36, 3 August 2008 (UTC)

Sure, put it in. Nihil novi (talk) 04:55, 3 August 2008 (UTC)

Etymology

I changed the history of the disease to reflect the cited reference by Andersen. Cystic in this case originally referred to microscopic fluid filled areas of the pancreas. It's true that the adjective cystic can be used to describe aspects of the biliary tree, but only because the gall bladder resembles a large fluid filled sac itself. The medical term for fibrosis of the bilary tree would be cholecystic (or choledochocystic) fibrosis, and is not what Andersen was refering to.

Ashkenazi Jews?

Some needs to add a citation for that little bit about the Ashkenazi Jews having the disease often. In any case, wouldn't they fall under western European peoples? It seems odd to list a general blanket statement like "western European populations" then list a specific people group like the Ashkenazi Jews....

Pstanton 23:47, 25 November 2008 (UTC) —Preceding unsigned comment added by Pstanton (talkcontribs)

I noticed this was previously discussed in the archived discussions.--99.240.122.45 (talk) 02:03, 26 November 2008 (UTC)
Ashkenazi Jews are the world's classic case of ascertainment bias, because they are the world's most screened population, because carrier testing was first initiated for Tay-Sachs disease in 1971 among AJ. The population carrier rate in the AJ population is essentially the same as that of the general European population. Is there anything noteworthy? Why should this even be reported? Metzenberg (talk) 12:26, 22 April 2009 (UTC)

This article discusses incidence of CF in Ashkenazi Jews.

    • Kerem B, Chiba-Falek O, Kerem E. Cystic fibrosis in Jews: frequency and mutation distribution. Genet Test. 1997;1(1):35-9. Review. PMID 10464623

If it is appropriate that we should report CF incidences among all European ethnic groups (eg. Finns, Irish, Germans, Slovenians, etc. then it also makes sense to report this information about Ashkenazi Jews as well. Otherwise, what is relevant is that the incidence differs across continents (Europe, South Asia, Africa). That may have significance for whatever heterozygote advantage it is that CF carriers have. Metzenberg (talk) 05:11, 23 April 2009 (UTC)

Ireland

I have removed the claim that 1 in 19 in Ireland suffer from CF. This is incorrect. I checked the site of the CF foundation of Ireland and it notes that 1 in 19 people in Ireland carry the gene for CF. For a child to have it, both parents have to carry the gene. Even then, the chance of onset is 25%. So, the probability that any person in Ireland has CF is something like (1/19)*(1/19)*1/4, or 0.0007%. This means that about 0.7 people in 1000 would have CF, which is more in line with numbers in Western Europe. —Preceding unsigned comment added by 68.101.153.164 (talk) 17:22, 26 November 2008 (UTC)

National Post's Politicization of Race and CF

Hello,

Some suspect information has made it into this article that is not properly researched, mostly driven by the National Post (a right-leaning newspaper from Canada). Much of the controversy stems from the definition of "causasian" which is used in a variety of different manners in the scholarly literature. Please note the following references:

[1] Kabra SK, Kabra M, Lodha R, Shastri S. Cystic fibrosis in India. Pediatr Pulmonol. 2007 Dec;42(12):1087-94.

"Cystic fibrosis (CF) was considered to be non-existent in Indian subcontinent. Reports in last one decade have suggested that cystic fibrosis occurs in India but its precise magnitude is not known. Studies on migrant Indian population in United States and United Kingdom estimate frequency of CF as 1:10,000 to 1:40,000. The clinical features are similar to that reported in Caucasian population." In this context, CF occurs at approximately the same rate as in African populations, but note that "Caucasian" is used to contrast with "Indian" (which comprises most of South Asia).

[2] Kabra SK, Kabra M, Shastri S, Lodha R. Diagnosing and managing cystic fibrosis in the developing world. Paediatr Respir Rev. 2006;7 Suppl 1:S147-50. Epub 2006 Jun 6.

"Cystic fibrosis (CF), earlier believed to be non existent in non Caucasians, is now a pan ethnic disease, having being reported from various regions of the world over last one decade."

Again, a contrast between "Causasian" and "non Causasian". Obviously, "caucasian" has different uses in different countries. However, rates of CF are high in "Causasian" (white) and negligable in those from South Asia, North Africa, etc. More research is needed to elucidate the exact epidemiology in non-European peoples, but the CCFS statements are only one perspective on the issue, and should be taken in balance. 128.189.137.17 (talk) 17:44, 27 November 2008 (UTC)

I'm encouraged by your concession that the opinions of the Canadian Cystic Fibrosis Foundation "should be taken in balance". You're obviously a very important authority since, single-handedly, you have determined that "more research is needed". I presume that you mean both sides of the topic can be included and discussed, so I'm returning the quote to the article. Remember that the people contributing to Wikipedia are all the English-speaking peoples of the world, of all colours and nationalities. Your particular narrow elitist perspective (by your comments, you are an American socialist, perhaps?) is just one tiny POV amongst many. Oh, and BTW, the National Post is not "a right-leaning newspaper from Canada"; it is a liberal Jewish paper, featuring internationally known and respected Jewish journalists. Bushcutter (talk) 02:00, 5 December 2008 (UTC)

I've added some stats from the NIH that clearly show that CF occurs in non-caucasians in the US, although at a much lower rate than in caucasians. This is because the carrier frequency is lower in these other races than in caucasians (see reference that I provided; to calculate incidence rate from carrier frequency, you have to square the carrier frequency - calculating the probability that two carriers have children - and then divide by four - because according to Mendel only one in four children will have the disease, assuming that all the carriers are heterozygous.(134.225.154.77 (talk) 12:49, 18 March 2009 (UTC))

I question whether reports of the incidence of CF carriers that do not appear in good, peer-reviewed articles should be used. In the developed countries (where "caucasians" live) CF is detected at birth and CF patients live to become adults. In the developing world, CF patients die in infancy, and the true incidence of CF is not detected or reported. This is a case of ascertainment bias. Also, remember that those Mendelian calculations apply to peas, not to disease (hey, that rhymes!). When you talk about disease, it may be the case that not all homozygotes reach the age of zero (are born). Metzenberg (talk) 12:39, 22 April 2009 (UTC)

Page Vandalism by "Bushcutter"

The edit history shows that the section added by "Bushcutter" who makes political edits to pages added a reference about skin color which is inaccurate. This has been removed. 128.189.137.17 (talk) 17:54, 27 November 2008 (UTC)

Removed. It's a bunch of nonsense. Nishkid64 (Make articles, not wikidrama) 20:31, 27 November 2008 (UTC)
I have restored the vandalism urged by 128.189.137.17, and trust that he will leave it alone from now on. Nishkid64, please discuss before joining any actions with 128.189.137.17 in future. Remember that documented material from a reputable source should not be labelled as "nonsense" unless you can clearly show what you mean. This page is the considered work of many learned volunteers and it's not yours to do with whatever you and 128.189.137.17 wish. Thanks! Bushcutter (talk) 23:35, 15 February 2009 (UTC)

Problematic text removed

I removed the following paragraph for several reasons:

The impact of cystic fibrosis is limited by race. The Canadian Cystic Fibrosis Foundation (CCFF) which supports research into cystic fibrosis, says that CF only affects people of the caucasian racial group. However, the term caucasian includes people from South Asia, North Africa, the Persian Gulf and Israel, according to Cathleen Morrison, CEO of the CCFF. "[Although] these are Caucasian populations," Morrison, CEO of the CCFF told CTV [ctv.ca]. "[they] do not have white skin".[2]

First of all, it's a college newspaper. It's word should not be taken over organizations with more authority on the subject. Second of all, it doesn't even say that. The actual article says:

The website of the Cystic Fibrosis Foundation says the disease is most common in Caucasians but can affect all races. According to Wikipedia, "approximately 1 in 25 people of European descent and 1 in 22 people of Ashkenazi Jewish descent is a carrier of a cystic fibrosis mutation. Although CF is less common in these groups, approximately 1 in 46 Hispanics, 1 in 65 Africans and 1 in 90 Asians carry at least one abnormal CFTR gene."

So we're citing an article that not only doesn't say what we're citing it for, the only mention of racial prevalence in the article (whether sourced to the CCFF or not) is from Wikipedia itself.

I might support the inclusion of a very brief mention of the whole "supporting CF is racist" kerfuffle if it were properly cited and worded neutrally, but this isn't it. It's confusing and unnecessary, since the article generally uses the term "of European descent" anyways.--TexasDex 23:21, 6 December 2008 (UTC)

(Copied from my talk page)
In your response you referred to the source of the Cystic Fibrosis statement as a "college newspaper". The quote by the spokesperson for CCFF was broadcast by the CTV television network, and was quoted in the Financial Post. Neither of these resembles in any way a college newspaper. Perhaps you're confusing my sources with something in your distant past? I think you're being a little too quick with the hatchet since WP states quite clearly that sourced material stays and discussion follows if there's disagreement. The quote should stay in place for subsequent discussion. For all that anyone knows, you may simply be an angry vandal since you brought out the hatchet before discussing your concerns. You see, right away you were wrong about the source, the content, and the motives of all concerned, and that's reason enough to restore the edit for further discussion. Bushcutter (talk) 17:02, 8 December 2008 (UTC)
The only citation I removed was a college newspaper. If more reputable sources have said it, cite them instead. When you add a source that actually has the information you cited then I'll leave it and discussion will follow. However the source that you added to the CF article did not back up what you added. If I added a statement to the Abraham Lincoln article saying he was a KKK member and cited a source that only said he was suspected of being a freemason, then it would be removed, and rightly so. Material that says things the cited article doesn't is not sourced, and should be removed, so I removed it, and started this discussion on the talk page so other users could express their opinions as well.
Also, I made no assumptions about your motives as you accused me of doing, yet you are quick to accuse me of vandalism. I am simply following policy and trying to keep unsourced information out of Wikipedia. There are good reasons for these policies. Please assume good faith in your future edits.--TexasDex 17:52, 8 December 2008 (UTC)
I can't figure out if you're confused or intentionally dissembling. Please help us understand what you are trying to say. Let's work through this one item at a time to lessen the confusion. For example, you keep referring to a "college newspaper", as in "The only citation I removed was a college newspaper." What exactly do you mean by this? What "college newspaper" citation are you referring to? If you can explain this, it will be easier to understand your next move. Bushcutter (talk) 21:53, 4 January 2009 (UTC)

Median vs Mean (Average)

The article cites the Cystic Fibrosis Foundation when it mentions the average life expectancy of 37 years. The CFF actually says that the median expected age is 37. There is a difference and it could be significant. —Preceding unsigned comment added by 38.117.149.83 (talk) 18:19, 7 April 2009 (UTC)

Overhaul of the Article

This change was a response to the introduction cleanup warning.

After reading the article I saw many areas in which the layout could be improved. The main reason behind this edit was the warning banner advising a cleanup of the introduction.

Main changes: (these can be seen by viewing the history page attached to this article and comparing the two latest changes)

Condensing the introduction, much of the introduction was repeated in further sections. I have tried to make the introduction a brief outline of the disease while presenting most of the information that a person may need in the introduction. The paragraph regarding diagnosis is covered more completely in the diagnosis section and has been removed.

New sections Cure and Cause have been added.

Cause: A paragraph from the introduction has been moved here and I have moved the graphic of the autosomal recessive pattern to here for relevance.

Cure: A paragraph from the introduction has been moved here and I have added information regarding foundations dedicated to finding a cure for this disease.

Diagnosis and Symptomatic diseases sections have been swapped around to improve the flow of the article, prognosis is now a subsection in treatment.

Rninne (talk) 07:16, 21 April 2009 (UTC) This webpage is confsuing me...im 12 and have CF...the life exoectancy may be 37 or watever now, but new things are coming out! they are working on tests now for the cure and by the time i am 20 there couldd be a cure!! also this is in an unhealthy cf patient, for you didnt say about a healthy patient like me...You scare me wiht the whole "TERRIBLE DEATH AT 37" or watever,...wcan u put it in a better way than that? You're scaring me and i must've cried 4 timess on this article...PLEASE VERIFY and dont jump to conclusiopn s and put things in a better way...thank you. —Preceding unsigned comment added by 209.198.81.224 (talk) 05:28, 27 November 2010 (UTC)

65 Roses

What does 65 Roses have to do with Cystic fibrosis? It is a redirect page. can someone explain it to me, Thanks. -- Danreilly123(talk) 09:18, 29 May 2009 (UTC)

I would think that the redirect should point to the Cystic Fibrosis Foundation, as it is one of their trademarks, and their page has an explanation of the phrase's origins. 75.80.187.10 (talk) 03:51, 1 June 2009 (UTC)

hey i was wondering, can a cf person reproduct? in other words cana cf person have kids? —Preceding unsigned comment added by 24.8.211.7 (talk) 03:26, 22 July 2009 (UTC)

Um, try reading the article... ~dom Kaos~ (talk) 10:55, 27 July 2009 (UTC)

IT's also the name of Cdn blog and documentary film, and a common malapropism for CF in Canada (the way kids often pronounce CF). I think linking here, or to the film, or to a disambiguation page makes sense. —Preceding unsigned comment added by 173.183.70.183 (talk) 23:43, 7 May 2010 (UTC)

ARTICLE HAS SOME DATA THAT CONFLICTING W/ OTHER ARTICLES IN WIKI

1) The non-passage of meconium in infants, according to the _linked_ article in this one on meconium ileus, is 20%. In this article it states that is an issue for only 10% of the infants. Why is this discrepancy? Is it related to statistics that come from one country or area related to another--does it have to do w/ misdiagnoses, or what?

2) The information for newborn testing in the US is *incorrect*--according to the US WEBSITE provided by the wiki article that this one links to on newborn testing, MOST & NEARLY ALL U.S. states test REGULARLY for Cystic Fibrosis.

CONSIDER REVISING THESE ISSUES Heathermadisonline (talk) 13:28, 2 August 2009 (UTC)HM

Information on Linda Posner

Hi, It appears as though a family member or friend of CF researcher Linda A. Posner has posted a sizeable amount of text about her in an almost obituary-like format. I suggest that this be removed from the article and moved to a separate page or be deleted altogether. I have no ill will towards the people who posted it, but I think it is inappropriate for Wikipedia.

Smcauley (talk) 10:26, 10 August 2009 (UTC)

Good pickup. I dealt with it in the article and I'd encourage you to delete the text from your discussion in order to discourage people from spamming Wikipedia. Novangelis (talk) 10:45, 10 August 2009 (UTC)

How are you going to cure people if you have no heart? Facts factsa facts but no heart. "If I have not love I am nothing." This site is therefore nothing. I will not be returniung to it thanks to people like you. Omit my Linda Posner's material if you like, she is too good for you. —Preceding unsigned comment added by Spacebusmatrix3000 (talkcontribs) 14:54, 10 August 2009 (UTC) Cystic fibrosis is commonly nowen as cf —Preceding unsigned comment added by 94.5.21.43 (talk) 17:48, 30 October 2009 (UTC)

Wiki is not a medical body set up to cure people. It's an encyclopedia. And how do you expect scientists to cure people if they have to read through mountains of personal information as opposed to the biological causes? Or parents to know how to better treat the illness if they can't find the information for personal messages? —Preceding unsigned comment added by 82.24.47.178 (talk) 02:35, 31 May 2010 (UTC)

World map

A world map giving rates of CF would be nice.Doc James (talk · contribs · email) 06:07, 12 December 2009 (UTC)

Headings

The headings of the management section are poor.Doc James (talk · contribs · email) 06:55, 12 December 2009 (UTC)

Article is critically incomplete

The article states at least twice that CFRD is the main secondary complication of CF, after lung issues. Incorrect. Digestive issues stemming from inability to create enzymes are the main secondary complication. CFRD is much further down the list, affecting only 1 in 4, ultimately.

The article needs a major section on Pancreatic Insufficiency. This can have two sub-sections: the first on enzyme insufficiency and enzyme replacement therapies and their history, and the second section on CFRD.

I'm willing to write/re-write the section, including citations, but as all of my Wikipedia edits have thus far been discarded or reversed, I don't know if it's worth my time. Crisdopher (talk) 23:28, 13 December 2009 (UTC) Cris Dopher

Willy Needs Lungs Foundation

This is a support group for Children with Cystic Fibrosis that would need a heart or Lung transplant. My late Son Willy R. Thompson passed on at St Pauls Hospital in Vancouver BC Canada due to a very servere lung infection before He could get His transplant in Toronto. His living out expenses would have amounted in the thousands of dollars but a lot of money was amassed for this. Please join and Please donate to the worthy cause.

Norm Meyer

[1] —Preceding unsigned comment added by 154.5.1.114 (talk) 00:00, 2 February 2010 (UTC)

Caucasians and Ashkenazi Jews

Ashkenazi Jews are Caucasoid, so why include both in the intro? I say we remove them. Busha5a5a5 (talk) 03:46, 2 March 2010 (UTC)

Cause

I think the Cause paragraph is not sufficiently explanatory. We should give the reade a chance to visualize how it should work and how it fails. I learnt from this article that there are several distinct functional problems. But still, there is one that is best known and most evident. Two clicks away, in the article about the protein (why separate into gene and protein, and why give priority to gene?), I found the following description which I think is rather helpful in providing help to understand:

The CFTR is found in the epithelial cells of many organs, including the lung, liver, pancreas, digestive tract, reproductive tract, and skin. Normally, the protein moves chloride and thiocyanate[3] ions (with a negative charge) out of an epithelial cell to the covering mucus. This results in an electrical gradient being formed and in the movement of (positively charged) sodium ions in the same direction as the chloride via a paracellular pathway. Due to this movement, the water potential of the mucus is reduced, resulting in the movement of water here by osmosis and a more fluid mucus.

Many couples - unspecific

I moved the following statement to here, because, although it is referenced, I think it should be specified with exactly what couples are offered screening. Many is a little to obvious. Mikael Häggström (talk) 04:27, 19 March 2010 (UTC)

Cystic fibrosis testing is offered to many couples in the US.[4]

References/Further reading

I moved the references without in-line citations to further reading, because it's very hard to couple them to their facts. Still, conversion to in-line citations is appreciated. Mikael Häggström (talk) 18:57, 19 March 2010 (UTC)

Talk:Cystic fibrosis/Archive 2/GA1

AJE Citation Verification

This “Cystic Fibrosis” wiki, while written very thoughtfully and thoroughly, relies too much on primary source information and thus has not passed the “secondary source requirement” test as per the wiki guidelines. Efforts should be made to find strong secondary source material to . Currently the Center for Disease Control and World Health Organization do not have separate Cystic Fibrosis subject material, and Google Books lack accessible resources from current published Cystic Fibrosis texts. The Cystic Fibrosis Foundation [2] seems to be the best secondary source authority on the web. All other issues with the wiki article are below and categorized according to the following headings:

a) PRIMARY SOURCES b) ADDITIONAL CITATIONS NEEDED C) REFERENCES NOT IN ENGLISH D) QUESTIONABLE SOURCE E) UNRETRIEVABLE LINKS

PRIMARY SOURCES The following references are primary source articles that should not be used as per the wiki guidelines. I have included the entire reference in case the numbers change in future edits. The numbers are current as of May-4-2010.

13. ^ Girón RM, Domingo D, Buendía B, Antón E, Ruiz-Velasco LM, Ancochea J (October 2005). "Nontuberculous mycobacteria in patients with cystic fibrosis" (in Spanish; Castilian). Arch. Bronconeumol. 41 (10): 560–5. PMID 16266669.

14. ^ Franco LP, Camargos PA, Becker HM, Guimarães RE (December 2009). "Nasal endoscopic evaluation of children and adolescents with cystic fibrosis". Braz J Otorhinolaryngol 75 (6): 806–13. PMID 20209279.

18. ^ Kulczycki LL, Shwachman H (August 1958). "Studies in cystic fibrosis of the pancreas; occurrence of rectal prolapse". N. Engl. J. Med. 259 (9): 409–12. PMID 13578072.


19. ^ Cohn JA, Friedman KJ, Noone PG, Knowles MR, Silverman LM, Jowell PS (September 1998). "Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis". N. Engl. J. Med. 339 (10): 653–8. doi:10.1056/NEJM199809033391002. PMID 9725922.

20. ^ Malfroot A, Dab I (November 1991). "New insights on gastro-oesophageal reflux in cystic fibrosis by longitudinal follow up". Arch. Dis. Child. 66 (11): 1339–45. doi:10.1136/adc.66.11.1339. PMID 1755649.

21. ^ Khoshoo V, Udall JN (February 1994). "Meconium ileus equivalent in children and adults". Am. J. Gastroenterol. 89 (2): 153–7. PMID 8304294. 24. ^ Moran A, Pyzdrowski KL, Weinreb J, et al. (August 1994). "Insulin sensitivity in cystic fibrosis". Diabetes 43 (8): 1020–6. doi:10.2337/diabetes.43.8.1020. PMID 8039595.

26. ^ Haworth CS, Selby PL, Webb AK, et al. (November 1999). "Low bone mineral density in adults with cystic fibrosis". Thorax 54 (11): 961–7. doi:10.1136/thx.54.11.961. PMID 10525552.

27. ^ Vandemergel X, Decaux G (April 2003). "[Review on hypertrophic osteoarthropathy and digital clubbing]" (in French). Rev Med Brux 24 (2): 88–94. PMID 12806875.

28. ^ Pitts-Tucker TJ, Miller MG, Littlewood JM (June 1986). "Finger clubbing in cystic fibrosis". Arch. Dis. Child. 61 (6): 576–9. PMID 3488032.

29. ^ McCallum TJ, Milunsky JM, Cunningham DL, Harris DH, Maher TA, Oates RD (October 2000). "Fertility in men with cystic fibrosis: an update on current surgical practices and outcomes". Chest 118 (4): 1059–62. doi:10.1378/chest.118.4.1059. PMID 11035677.

31. ^ Augarten A, Yahav Y, Kerem BS, et al. (November 1994). "Congenital bilateral absence of vas deferens in the absence of cystic fibrosis". Lancet 344 (8935): 1473–4. doi:10.1016/S0140-6736(94)90292-5. PMID 7968122. 32. ^ Gilljam M, Antoniou M, Shin J, Dupuis A, Corey M, Tullis DE (July 2000). "Pregnancy in cystic fibrosis. Fetal and maternal outcome". Chest 118 (1): 85–91. doi:10.1378/chest.118.1.85. PMID 10893364.

34. ^ Short DB, Trotter KW, Reczek D, et al. (July 1998). "An apical PDZ protein anchors the cystic fibrosis transmembrane conductance regulator to the cytoskeleton". J. Biol. Chem. 273 (31): 19797–801. doi:10.1074/jbc.273.31.19797. PMID 9677412.

36. ^ Xu Y, Szép S, Lu Z (December 2009). "The antioxidant role of thiocyanate in the pathogenesis of cystic fibrosis and other inflammation-related diseases". Proceedings of the National Academy of Sciences of the United States of America 106 (48): 20515–19. doi:10.1073/pnas.0911412106. PMID 19918082.

37. ^ Moskwa P, Lorentzen D, Excoffon KJ, et al. (January 2007). "A novel host defense system of airways is defective in cystic fibrosis". American Journal of Respiratory and Critical Care Medicine 175 (2): 174–83. doi:10.1164/rccm.200607-1029OC. PMID 17082494.

38. ^ Conner GE, Wijkstrom-Frei C, Randell SH, Fernandez VE, Salathe M (January 2007). "The lactoperoxidase system links anion transport to host defense in cystic fibrosis". FEBS Letters 581 (2): 271–78. doi:10.1016/j.febslet.2006.12.025. PMID 17204267.

39. ^ a b Saiman L (2004). "Microbiology of early CF lung disease". Paediatric Respiratory Reviews 5 (Suppl A): S367–69. doi:10.1016/S1526-0542(04)90065-6. PMID 14980298.

40. ^ Tümmler B, Koopmann U, Grothues D, Weissbrodt H, Steinkamp G, von der Hardt H (June 1991). "Nosocomial acquisition of Pseudomonas aeruginosa by cystic fibrosis patients". J. Clin. Microbiol. 29 (6): 1265–7. PMID 1907611.

42.^ Pegues DA, Carson LA, Tablan OC, et al. (May 1994). "Acquisition of Pseudomonas cepacia at summer camps for patients with cystic fibrosis. Summer Camp Study Group". J. Pediatr. 124 (5 Pt 1): 694–702. doi:10.1016/S0022-3476(05)81357-5. PMID 7513755.

43. ^ Pankhurst CL, Philpott-Howard J (April 1996). "The environmental risk factors associated with medical and dental equipment in the transmission of Burkholderia (Pseudomonas) cepacia in cystic fibrosis patients". J. Hosp. Infect. 32 (4): 249–55. doi:10.1016/S0195-6701(96)90035-3. PMID 8744509.

44. ^ Jones AM, Govan JR, Doherty CJ, et al. (June 2003). "Identification of airborne dissemination of epidemic multiresistant strains of Pseudomonas aeruginosa at a CF centre during a cross infection outbreak". Thorax 58 (6): 525–27. doi:10.1136/thorax.58.6.525. PMID 12775867.

51. ^ Minarowski Ł, Sands D, Minarowska A, Karwowska A, Sulewska A, Gacko M, Chyczewska E. Thiocyanate concentration in saliva of cystic fibrosis patients. Folia Histochem Cytobiol. 2008;46(2):245-6. http://versita.metapress.com/content/12805r021413m867/fulltext.pdf

53 .^ Freudenheim, Milt (2009-12-22). "Tool in Cystic Fibrosis Fight: A Registry". New York Times: pp. D1. Retrieved 2009-12-21.

54. ^ American College of Obstetricians and Gynecologists and American College of Medical Genetics. Preconception and prenatal carrier screening for cystic fibrosis. Clinical and laboratory guidelines. American College of Obstetricians and Gynecologists, Washington, DC, October 2001.

56. ^ Tabor A, Philip J, Madsen M, Bang J, Obel EB, Nørgaard-Pedersen B (June 1986). "Randomised controlled trial of genetic amniocentesis in 4606 low-risk women". Lancet 1 (8493): 1287–93. PMID 2423826.

57. ^ Eddleman KA, Malone FD, Sullivan L, et al. (November 2006). "Pregnancy loss rates after midtrimester amniocentesis". Obstet Gynecol 108 (5): 1067–72. doi:10.1097/01.AOG.0000240135.13594.07. PMID 17077226.

58. ^ Davis LB, Champion SJ, Fair SO, Baker VL, Garber AM (April 2010). "A cost-benefit analysis of preimplantation genetic diagnosis for carrier couples of cystic fibrosis". Fertil. Steril. 93 (6): 1793–804. doi:10.1016/j.fertnstert.2008.12.053. PMID 19439290.

60. ^ Westerman EM, Le Brun PP, Touw DJ, Frijlink HW, Heijerman HG (March 2004). "Effect of nebulized colistin sulphate and colistin sulphomethate on lung function in patients with cystic fibrosis: a pilot study". J. Cyst. Fibros. 3 (1): 23–8. doi:10.1016/j.jcf.2003.12.005. PMID 15463883.

61. ^ McCoy KS, Quittner AL, Oermann CM, Gibson RL, Retsch-Bogart GZ, Montgomery AB (November 2008). "Inhaled aztreonam lysine for chronic airway Pseudomonas aeruginosa in cystic fibrosis". Am. J. Respir. Crit. Care Med. 178 (9): 921–8. doi:10.1164/rccm.200712-1804OC. PMID 18658109.

62. ^ Hansen CR, Pressler T, Koch C, Høiby N (March 2005). "Long-term azitromycin treatment of cystic fibrosis patients with chronic Pseudomonas aeruginosa infection; an observational cohort study". J. Cyst. Fibros. 4 (1): 35–40. doi:10.1016/j.jcf.2004.09.001. PMID 15752679.

65. ^ Kuver R, Lee SP (April 2006). "Hypertonic saline for cystic fibrosis". N. Engl. J. Med. 354 (17): 1848–51; author reply 1848–51. doi:10.1056/NEJMc060351. PMID 16642591.

66. ^ Lieberman J (July 1968). "Dornase aerosol effect on sputum viscosity in cases of cystic fibrosis". JAMA 205 (5): 312–3. doi:10.1001/jama.205.5.312. PMID 5694947.

68. ^ Fridell JA, Vianna R, Kwo PY, et al. (October 2005). "Simultaneous liver and pancreas transplantation in patients with cystic fibrosis". Transplant. Proc. 37 (8): 3567–9. doi:10.1016/j.transproceed.2005.09.091. PMID 16298663.

69. ^ Belkin RA, Henig NR, Singer LG, et al. (March 2006). "Risk factors for death of patients with cystic fibrosis awaiting lung transplantation". Am. J. Respir. Crit. Care Med. 173 (6): 659–66. doi:10.1164/rccm.200410-1369OC. PMID 16387803.

74. ^ Hardin DS, Rice J, Ahn C, et al. (March 2005). "Growth hormone treatment enhances nutrition and growth in children with cystic fibrosis receiving enteral nutrition". J. Pediatr. 146 (3): 324–8. doi:10.1016/j.jpeds.2004.10.037. PMID 15756212.

75. ^ Marks SC, Kissner DG (1997). "Management of sinusitis in adult cystic fibrosis". Am J Rhinol 11 (1): 11–4. doi:10.2500/105065897781446810. PMID 9065342.

76. ^ Phillipson GT, Petrucco OM, Matthews CD (February 2000). "Congenital bilateral absence of the vas deferens, cystic fibrosis mutation analysis and intracytoplasmic sperm injection". Hum. Reprod. 15 (2): 431–5. doi:10.1093/humrep/15.2.431. PMID 10655317.

79. ^ Araújo FG, Novaes FC, Santos NP, et al. (January 2005). "Prevalence of deltaF508, G551D, G542X, and R553X mutations among cystic fibrosis patients in the North of Brazil". Braz. J. Med. Biol. Res. 38 (1): 11–5. doi:10.1590/S0100-879X2005000100003. PMID 15665983.

83. ^ Hamosh A, FitzSimmons SC, Macek M, Knowles MR, Rosenstein BJ, Cutting GR (February 1998). "Comparison of the clinical manifestations of cystic fibrosis in black and white patients". J. Pediatr. 132 (2): 255–9. doi:10.1016/S0022-3476(98)70441-X. PMID 9506637.

84. ^ Hytönen M, Patjas M, Vento SI, et al. (December 2001). "Cystic fibrosis gene mutations deltaF508 and 394delTT in patients with chronic sinusitis in Finland". Acta Otolaryngol. 121 (8): 945–7. PMID 11813900.

87. ^ Rosenfeld M, Davis R, FitzSimmons S, Pepe M, Ramsey B (May 1997). "Gender gap in cystic fibrosis mortality". Am. J. Epidemiol. 145 (9): 794–803. PMID 9143209.

88. ^ Coakley RD, Sun H, Clunes LA, et al. (December 2008). "17beta-Estradiol inhibits Ca2+-dependent homeostasis of airway surface liquid volume in human cystic fibrosis airway epithelia". J. Clin. Invest. 118 (12): 4025–35. doi:10.1172/JCI33893. PMID 19033671.

89. ^ Verma N, Bush A, Buchdahl R (October 2005). "Is there still a gender gap in cystic fibrosis?". Chest 128 (4): 2824–34. doi:10.1378/chest.128.4.2824. PMID 16236961.

90. ^ Moran A, Dunitz J, Nathan B, Saeed A, Holme B, Thomas W (September 2009). "Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality". Diabetes Care 32 (9): 1626–31. doi:10.2337/dc09-0586. PMID 19542209.

91. ^ Wennberg C, Kucinskas V (1994). "Low frequency of the delta F508 mutation in Finno-Ugrian and Baltic populations". Hum. Hered. 44 (3): 169–71. doi:10.1159/000154210. PMID 8039801.

92. ^ Kere J, Savilahti E, Norio R, Estivill X, de la Chapelle A (September 1990). "Cystic fibrosis mutation delta F508 in Finland: other mutations predominate". Hum. Genet. 85 (4): 413–5. doi:10.1007/BF02428286. PMID 2210753.

93. ^ Wiuf C (August 2001). "Do delta F508 heterozygotes have a selective advantage?". Genet. Res. 78 (1): 41–7. PMID 11556136.

94. ^ Gabriel SE, Brigman KN, Koller BH, Boucher RC, Stutts MJ (October 1994). "Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model". Science 266 (5182): 107–9. doi:10.1126/science.7524148. PMID 7524148.

95. ^ Cuthbert AW, Halstead J, Ratcliff R, Colledge WH, Evans MJ (January 1995). "The genetic advantage hypothesis in cystic fibrosis heterozygotes: a murine study". J. Physiol. (Lond.) 482 ( Pt 2): 449–54. PMID 7714835.

96. ^ Högenauer C, Santa Ana CA, Porter JL, et al. (December 2000). "Active intestinal chloride secretion in human carriers of cystic fibrosis mutations: an evaluation of the hypothesis that heterozygotes have subnormal active intestinal chloride secretion". Am. J. Hum. Genet. 67 (6): 1422–7. doi:10.1086/316911. PMID 11055897.

97. ^ Pier GB, Grout M, Zaidi T, et al. (May 1998). "Salmonella typhi uses CFTR to enter intestinal epithelial cells". Nature 393 (6680): 79–82. doi:10.1038/30006. PMID 9590693.

99. ^ Poolman EM, Galvani AP (February 2007). "Evaluating candidate agents of selective pressure for cystic fibrosis". Journal of the Royal Society, Interface 4 (12): 91–8. doi:10.1098/rsif.2006.0154. PMID 17015291.

102. ^ G. Fanconi, E. Uehlinger, C. Knauer, "Das coeliakiesyndrom bei angeborener zysticher pankreasfibromatose und bronchiektasien," Wien. Med. Wschr., 1936, 86:753–756.

103. ^ Di Sant'Agnese PA, Darling RC, Perera GA, Shea E (November 1953). "Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas; clinical significance and relationship to the disease". Pediatrics 12 (5): 549–63. PMID 13111855.

104. ^ Riordan JR, Rommens JM, Kerem B, et al. (September 1989). "Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA". Science 245 (4922): 1066–73. doi:10.1126/science.2475911. PMID 2475911.

105. ^ Rommens JM, Iannuzzi MC, Kerem B, et al. (September 1989). "Identification of the cystic fibrosis gene: chromosome walking and jumping". Science 245 (4922): 1059–65. doi:10.1126/science.2772657. PMID 2772657.

106. ^ Ramalho AS, Beck S, Meyer M, Penque D, Cutting GR, Amaral MD (November 2002). "Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis". Am. J. Respir. Cell Mol. Biol. 27 (5): 619–27. PMID 12397022.


ADDITIONAL CITATIONS NEEDED: Background Section At the end of Paragraph 1, I suggest this citation: http://books.google.com/books?id=i9lXQ6xb-HsC&printsec=frontcover&dq=cystic+fibrosis&cd=2#v=onepage&q&f=false At the end of Paragraph 2, I suggest these 2 citations: http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_causes.html & http://www.cff.org/AboutCF/Faqs/ For the 1st “citation needed” in Paragraph 3, I suggest: http://www.ncbi.nlm.nih.gov/pubmed/11499059 PMID: 11499059 For the 2nd “citation needed” in Paragraph 3, I suggest: http://www.cff.org/AboutCF/Faqs/ (note: this is the same site as another citation above)

REFERENCES NOT IN ENGLISH 27. ^ Vandemergel X, Decaux G (April 2003). "[Review on hypertrophic osteoarthropathy and digital clubbing]" (in French). Rev Med Brux 24 (2): 88–94. PMID 12806875.

102. ^ G. Fanconi, E. Uehlinger, C. Knauer, "Das coeliakiesyndrom bei angeborener zysticher pankreasfibromatose und bronchiektasien," Wien. Med. Wschr., 1936, 86:753–756.

QUESTIONABLE SOURCE 53 .^ Freudenheim, Milt (2009-12-22). "Tool in Cystic Fibrosis Fight: A Registry". New York Times: pp. D1. Retrieved 2009-12-21.

80. ^[Unreliable medical source?]Ottawa university boots cystic fibrosis from charity drive, National Post, November 25, 2008

85. ^[Unreliable medical source?]About Cystic Fibrosis

UNRETRIEVABLE LINKS – i.e. you cannot access the source of the link via clicking or searching. 54. ^ American College of Obstetricians and Gynecologists and American College of Medical Genetics. Preconception and prenatal carrier screening for cystic fibrosis. Clinical and laboratory guidelines. American College of Obstetricians and Gynecologists, Washington, DC, October 2001.

102. ^ G. Fanconi, E. Uehlinger, C. Knauer, "Das coeliakiesyndrom bei angeborener zysticher pankreasfibromatose und bronchiektasien," Wien. Med. Wschr., 1936, 86:753–756.

Ggschwei (talk) 17:24, 4 May 2010 (UTC)

Image in lead

image preferred by Doc James image preferred by Badger Drink

I personally think the image of the girl on a nebulizer is better than that of a cell membrane to illustrate this condition. Opinions?Doc James (talk · contribs · email) 05:23, 29 April 2011 (UTC)

The problem with the girl on a nebulizer is that the image is such poor-quality (terrible lighting, poor resolution) that whatever subtle distinguishing elements that would render this as illustrative of CF (to the exclusion of all other respiratory conditions) are completely lost - it looks like a girl with a breathing mask, and while it could be for CF, it could just as easily be for chronic asthma, bronchitis, allergies, or maybe she just doesn't like the shampoo her mom used on the rug (:-)). Simply put, this image doesn't illustrate this condition - we cannot see sticky mucus, or anything that would allow even a trained medical professional to make the diagnosis of CF just from looking at it. It's a touching image, and I understand why some may be attached to it - but as an encyclopedia, we need to base our decisions on more logical, objective standards. The end result of using the nebulizer picture in the lede is to make the article look amateurish - a picture of someone missing an arm is good for an article about amputation, but a picture of someone in a knee brace isn't really such an iconic example of a ACL tear that it'd be fit for the lede there. It's like using a (blurry, poorly-lit) picture of someone with a mansion and a yacht they bought with lottery winnings in the Lottery article, rather than an image of a lottery ticket. Badger Drink (talk) 18:53, 29 April 2011 (UTC)
Response to Third Opinion Request:
Disclaimers: I am responding to a third opinion request made at WP:3O. I have made no previous edits on Cystic fibrosis/Archive 2 and have no known association with the editors involved in this discussion. The third opinion process (FAQ) is informal and I have no special powers or authority apart from being a fresh pair of eyes. Third opinions are not tiebreakers and should not be "counted" in determining whether or not consensus has been reached. My personal standards for issuing third opinions can be viewed here.

Opinion: One particularly wise Third Opinion Wikipedian, RegentsPark, once succinctly put the purpose of Third Opinions like this, "It's sort of like if you're having an argument on the street in front of City Hall and turn to a passer-by to ask 'hey, is it true that the Brooklyn Bridge is for sale?'." This opinion is one of that kind. Frankly, I don't much care for either image and think that the article might be better off without either in the lede. I don't like the picture of the girl for the reasons set out by Badger, but I don't like the protein diagram because it's incomprehensible to the average layperson, even with the caption "Molecular structure of the CFTR protein" ("Protein? What do proteins have to do with disease? If I don't get enough protein in my diet will I get CF?"); moreover, the article says that the CFTR protein may be deformed by the mutation in several different ways and I, as a layman, am not sure whether this diagram illustrates one, several, or none of those deformations. If I were forced to pick one or the other, I'd probably choose the girl because I think the protein picture does more good placed later in the article but I have to reiterate that for me that would be a far distant second choice over removing the image altogether until a better one comes along.

What's next: Once you've considered this opinion click here to see what happens next.—TransporterMan (TALK) 13:59, 19 May 2011 (UTC)

Image has been in place a long time. This is what some people with CF look like. Much of their time is spent in health on breathing treatments. Doc James (talk · contribs · email) 20:44, 27 May 2011 (UTC)
For most readers, I would expect an image of an affected human to be a good lead-in to the article; a membrane cartoon, not so much. That said, I think it's likely one could improve on the image of the girl. -- Scray (talk) 02:52, 28 May 2011 (UTC)
Cystic fibrosis is a channelopathy caused by a mutation at Phe 508 (labeled in green above) in the Cystic fibrosis transmembrane conductance regulator protein. This mutation causes the Chloride channel to become ineffective.
Here's an image from the French wiki page. I think it looks better at reduced size than the existing diagram. The captions are in French, but because the file is in SVG format, the captions could be changed into English using Inkscape or a similar tool. Another option could be to build a new image with four quadrants, each containing one of the four most common pathogens in CF. --Arcadian (talk) 04:05, 28 May 2011 (UTC)
I prefered the old image but I guess we could put the image of clubbing in the lead.Doc James (talk · contribs · email) 23:19, 28 May 2011 (UTC)
As I suggested before I think an image of a human would be preferred, though it's hard to identify a classic and specific image. A child receiving chest percussion might fit the bill; digital clubbing would also be relevant; the child connected to the nebulizer (until recently the longstanding lead image) was fine if not "perfect" (always elusive); a collage of images (for this multisystem disease) would also seem appropriate. What I don't like is a cell membrane cartoon as the lead image when the human connection is so much more accessible to WP's primary audience. -- Scray (talk) 04:31, 29 May 2011 (UTC)
I like the French image. Again, just to sorta re-state where I'm coming from, even if the nebulizer girl was perfectly lit in high resolution, the main trouble is that this doesn't "look" like cystic fibrosis. There's absolutely nothing in it to the layman which says "this is what cystic fibrosis, and cystic fibrosis alone, looks like" - it's a girl with a breathing mask. The image could be used in the treatment subsection (where I originally placed it) or somewhere else in the article, but the top image should be as singularly "cystic fibrosis" as the title itself. For diseases like, off the top of my head, lupus, clubfoot, ALS, spina bifida, or Downs syndrome, images of people could be very suitable for the lede. But for other diseases, a picture of a person suffering from the disease really doesn't work - a person with a runny nose could have the flu, or could just have allergies. There's nothing so strikingly visual about how the flu manifests itself in a human being that justifies a picture of a person with the flu in the lede when a picture of the bug itself can be used. Same with Tay-Sachs, or Alzheimers, or AIDS - none of these articles have a picture of a human being in their lede, and for good reason. It's an article about cystic fibrosis, not "living with" cystic fibrosis, and as such, we should strive to present readers with an image that's as singularly CF as possible. The image of the girl is cute and touching and is suitable enough for the "treatment" section, but is unfitting of being the lead image in the article. Badger Drink (talk) 03:46, 30 May 2011 (UTC)
The requirement is that the image look like (some aspect of) CF, not that it look specifically and exclusively like CF, in a way that visually distinguishes CF from all other conditions. To use your example, a photo of a person with a runny nose is perfectly fine at multiple articles, including common cold, influenza, allergies, and anything else that involves a runny nose. It's not necessary to figure out exactly what triggered the runny nose, so long as it looks like a runny nose. You might like to read Wikipedia:Images#Pertinence_and_encyclopedic_nature.
Additionally, the image is not just "a girl with a breathing mask"; it's a girl with a breathing mask and a breathing vest. The most common use of a breathing vest is for CF. A child with a breathing vest is far more specific for CF than a person with clubbed fingers.
On the broader issue, I think that, when available, a photograph of a person is usually a better introductory image for medical conditions than technical diagrams. Such images aren't always available (e.g., Commons has no images of babies with Tay Sachs) and may not be illustrative (because conditions like gastric ulcers don't show up on the outside of the body, and the most common treatment is to swallow a few pills, which is not very photogenic), but when they exist, and when they show something about the condition or the treatment, then I think we should use them more prominently than pictures of drugs or technical diagrams of pathophysiology. Think of it as my version of "person first" writing. WhatamIdoing (talk) 17:53, 30 May 2011 (UTC)
This is not a photo of a girl with a mask, this is a photo of a CF affected, clearly sick girl with a mask wearing a percussive vest. This is a great photo representing how cystic fibrosis looks. These people (CF affected people) do these hour long therapies upwards to 4+ times per day every day of their entire lives, a photo of a membrane channel doesn't give an overview image of CF like the photo of the girl does. subsequent scientific diagrams would be more than appropriate throughout the article, but I agree that the photo of the girl mid-therapy is a great introductory image, even if its not stock-photo quality. Je.rrt (talk) 20:41, 11 September 2011 (UTC)

One in 25

The article states, "CF is most common among Caucasians; one in 25 people of European descent carry one allele for CF." This is uncited, and does not match up with the cited stats given below. If 1 in 25 people of European descent carry the recessive gene, 1 in 625 people who reproduce would have the recessive gene and reproduce with someone else with the recessive gene. One in 4 of their offspring would have CF, for a prevalence of 4 in 10,000. However, the cited statistics list Ireland as having the highest carrier rate in the world, but only 2.98 in 10,000 children have CF. What accounts for this discrepancy? Or is the uncited claim of one in 25 incorrect? --Yamla (talk) 01:47, 8 July 2011 (UTC)

Given these numbers are clearly rounded estimates, the fact that the 1/25 figure used in a simplified calculation produces the same order of magnitude as the other statistic means they are in agreement. You can't expect statistical averages for prevalence to be accurate to the level of a few people in ten thousand. However you are right that there should be a citation so I will find one and add it in.Anonymous watcher (talk) 12:14, 3 November 2011 (UTC)

Quality of life

Chronic illnesses are very difficult to manage. Cystic fibrosis (CF) is a chronic illness that affects the “digestive and respiratory tracts resulting in generalized malnutrition and chronic respiratory infections”. The thick secretions clog the airways in the lungs, which often causes inflammation and severe lung infections. Therefore, mucus makes it challenging to breathe. Breathing is an essential part of life. If it is compromised, it will affect your quality of life, such as everyday chores. It is important for CF patients to understand the detrimental relationship that chronic illnesses place on the quality of life. Schmitz and Goldbeck, underline the fact that Cystic Fibrosis significantly increases emotional stress on both the individual and the family, “and the necessary time-consuming daily treatment routine may have further negative effects on quality of life (QOL)”. However, Havermans and colleagues (2006) have shown that young outpatients with CF that have participated in the CFQ-R (Cystic Fibrosis Questionnaire-Revised) “rated some QOL domains higher than did their parents”. Consequently, outpatients with CF have a more positive outlook for themselves. Furthermore, there are many ways to improve the QOL in CF patients. Exercise is promoted to increase lung function. The fact of integrating an exercise regime into the CF patient’s daily routine can significantly improve the quality of life. There is no definitive cure for Cystic Fibrosis. However, there are diverse medications used such as, mucolytics, bronchodilators, steroids and antibiotics that have the purpose of loosening mucus, expanding airways, decreasing inflammation and fighting lung infections.

This doesn't really fit in the treatment section. Would need to fill in the references also. Doc James (talk · contribs · email) 16:46, 30 September 2011 (UTC)

Ireland

I'm going to remove from the lede the assertion that Ireland's incidence of CF is 2.98 per 10,000. The fact that Ireland has the highest incidence of CF in the world seems indeed well sourced, but the 2.98 per 10,000 figure is very dubious and is contradicted by many sources. This study for instance says "The incidence of cystic fibrosis (CF) at birth in Ireland is 1/1461" [3], and this figure is also cited here [4]. This other study says: "We estimate the incidence of cystic fibrosis in Ireland to be at least 1 case per 1838 live births". [5] You'll also have to take into account that the WHO says that "In the European Union 1 in 2000-3000 newborns is found to be affected by CF"[6]

188.25.160.155 (talk) 22:36, 20 December 2011 (UTC)

the "at least" portion of the second article does not contradict the first, it supports the first. The information is factual and well sourced. Your removal of the information has been undone. Pulmonological (talk) 00:44, 22 December 2011 (UTC)
I think you don't understand what I mean. I was talking about the 2.98 per 10,000 which is contradicted by two studies: one which says 1/1461 and second which says at least 1 case per 1838.

188.25.162.97 (talk) 01:14, 22 December 2011 (UTC)

Then fix the number and add a citation, don't delete the entire mentioning of the Irish incidence since its ~ 1/3000 instead of 1/2000. Pulmonological (talk) 08:05, 22 December 2011 (UTC)
Instead, your citation is all of Europe and the section you're contesting is specific to Ireland, with reputable citations. Instead of changing irelands rate, include the rate for all of Europe which is not Ireland alone. Pulmonological (talk) 08:09, 22 December 2011 (UTC)

Please use reliable sources per WP:MEDRS Doc James (talk · contribs · email) 08:18, 22 December 2011 (UTC)

None of these refs are appropriate http://www.breakingnews.ie/ireland/scientists-make-major-cystic-fibrosis-breakthrough-468625.html http://www.independent.ie/health/we-have-the-highest-rate-of-cystic-fibrosis-in-the-world-84572.html http://www.rte.ie/tv/theafternoonshow/2010/0408/cysticfibrosis899.html Doc James (talk · contribs · email) 08:25, 22 December 2011 (UTC)
I did not add this information, but I reverted the deletion of cited content by [User talk:188.25.162.97|188.25.162.97]]. Then you reverted back to his deletion because the information that has been there for however long wasn't cited well enough. The citations I thought were citing this information were:
I did notice the website citations after you posted on my talk. So it needs to be re-written but not completely removed without correcting it. I am still new to how the history works so I thought the citations being argued were the citations being deleted Pulmonological (talk) 08:30, 22 December 2011 (UTC)
No worries and again welcome. Wikipedia takes time to learn. We try to use review articles in the last 5 years especially for major topics such as this.Doc James (talk · contribs · email) 08:32, 22 December 2011 (UTC)

Incidence

Incidence of cystic fibrosis: (my citation was not the one I was using, I will have to find it)

  • Finland 1 in 25000
  • Mexico 1 in 8500
  • Sweden 1 in 7300
  • Poland 1 in 6000
  • North Ireland 1 in 5350
  • Russia 1 in 4900
  • Denmark 1 in 4700
  • Norway 1 in 4500
  • Netherlands 1 in 3650
  • Spain 1 in 3500
  • Greece 1 in 3500
  • Germany 1 in 3300
  • United States 1 in 2835
  • Czech Republic 1 in 2833
  • United Kingdom 1 in 2600
  • Australia 1 in 2500
  • Italy 1 in 2438
  • France 1 in 2350
  • Switzerland 1 in 2000
  • Ireland 1 in 1800
  1. ^ Waknine, Yael (2005-07-27). "Orphan Drug Approvals: Bronchitol, Prestara, GTI-2040". Medscape today for WebMD. Retrieved 2007-06-22. {{cite web}}: Check date values in: |date= (help); Cite has empty unknown parameter: |coauthors= (help)
  2. ^ http://oncampus.macleans.ca/education/2008/11/25/carleton-cancels-shinerama-says-disease-only-affects-%E2%80%9Cwhite-people%E2%80%9D-2/
  3. ^ Moskwa P, Lorentzen D, Excoffon KJ, Zabner J, McCray PB, Nauseef WM, Dupuy C, Bánfi B (2007). "A novel host defense system of airways is defective in cystic fibrosis". Am. J. Respir. Crit. Care Med. 175 (2): 174–83. doi:10.1164/rccm.200607-1029OC. PMC 2720149. PMID 17082494. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  4. ^ Committee on Genetics, American College of Obstetricians and Gynecologists (2005). "ACOG Committee Opinion. Number 325, December 2005. Update on carrier screening for cystic fibrosis". Obstet Gynecol. 106 (6): 1465–8. PMID 16319281. {{cite journal}}: Unknown parameter |month= ignored (help)

potential ivacaftor aka Kalydeco resource

"Gaining Time: Cystic Fibrosis Drug Shows Rapid Benefits A rigorous study concludes that experimental medication improved lung function and weight gain" by Christine Gorman Scientific American November 2, 2011

99.181.147.68 (talk) 06:16, 4 January 2012 (UTC)

This drug (trade name Kalydeco) was approved today to treat a subgroup of CF victims. This should probably get worked into the article to mention this by someone more familiar with the finer points of CF medically than I am. Here's a good starting article [[7]] Tseliotwave (talk) 04:13, 1 February 2012 (UTC)

Contradictory?

"The ΔF508 mutation is estimated to be up to 52,000 years old."

"It is supposed that CF appeared about 3,000 BC because of migration of peoples, gene mutations, and new conditions in nourishment."

If the ΔF508 mutation is thought to be 52,000 years old, CF must also have existed 52,000 years ago. If CF appeared in 3,000 BC, the mutation must not have existed before 3,000 BC. These two sentences are contradictory.

Also, what do "new conditions in nourishment" and "migration of peoples" have to do with the expression of a genetic disease? --NellieBly (talk) 05:15, 20 May 2012 (UTC)

On a different note, in the overview section it says that CF is caused by a frameshift mutation. However, further down the page it says the most common cause of it is a deletion of the 3 base pairs that code for phenylalanine (the ΔF508 mutation). However, according to my knowledge, a deletion of one codon does not cause a frameshift, as it affects only the codon that is deleted. Which one is it, a frameshift or a deletion?

(Although it has nothing to do with NellieBly's comment, it also seems contradictory, so I put it here)

Wertyu739 (talk) 16:18, 7 April 2014 (UTC)

Epidemiology

"Frequency worldwide" is misleading. Cited source names it "in the worldwide populations studied to date" and doesn't give a source. Maybe we can find something better (through I doubt that there are any population independent numbers out there) Yak90 (talk) 20:59, 13 December 2013 (UTC)

There is no know global registry for CF. The population to date study is [1] This study was published in 2002. --Ckarsiyaka (talk) 07:31, 3 May 2014 (UTC)

Lead Image

The lead image was deleted. It should be restored but I don't know how to petition for this to be fixed. Rivard.M (talk) 04:26, 12 December 2012 (UTC)

The image was deleted because it was a copyvio, see the discussion on commons. The previous image was low resolution and out of focus, a better image can definitely be found/taken to replace it. --ImGz (t/c) 07:01, 13 December 2012 (UTC)

Curcumin

I support the removal of the primary sourced material.[8] Treatments do not appear overnight because of new medical research. It takes time for things to be tested, approved, etc. If this is a main line of research inquiry, then one should be able to find a secondary WP:MEDRS stating so an it can be incorporated into a Research directions section per Wikipedia:Manual_of_Style/Medicine-related_articles#Diseases_or_disorders_or_syndromes. Biosthmors (talk) 00:17, 16 December 2012 (UTC)

Page semiprotected for 2 days until the new editor understands why primary sources won't work here. JFW | T@lk 00:31, 16 December 2012 (UTC)

Localising gene

There is a statement that the gene was difficult to locate because the mutation was small.

A very large number (~1000) of human autosomal genes which involved SNIPs were mapped using classical linkage study techniques and somatic cell genetics prior to widespread availability of DNA-based techniques. The size of the mutation is irrelevant: it is merely important that other known genes are relatively close on the chromosome and that the allele being studied has a readily observed phenotype. It therefore seems to me that this statement is misleading, furthermore the reference provided is to the sequencing study rather than to the paper which located the gene to Ch7.

The failure to locate the gene underlying Cystic Fibrosis was a puzzle to geneticists for many years. I think there were many papers which offered suggested linkages none of which stood up to further data. It was known that the disease was caused by autosomal recessive mutations. I think a better quality explanation is needed in this section.

I also find some of the following sentences a bit confusing. All-in-all section could do with a more studied re-write. 109.144.166.225 (talk) 10:20, 20 January 2014 (UTC)

Information on milder forms, lung transplant, infertility issues

This New York Times interview with Dr. Preston W. Campbell III covers these points, among others. At the time (maybe still - I didn't check), he was executive vice president for medical affairs at the Cystic Fibrosis Foundation and a pediatric pulmonologist at Johns Hopkins Hospital in Baltimore:

  • 1,400 gene changes known
  • Some people aren't diagnosed until age 40 (= milder forms, underdiagnosed) ". . .we are not really sure we are going to call them cystic fibrosis patients yet."
  • Importance of carefully timing a lung transplant
  • Infertile men can bear children by having sperm taken from their testes and utilizing in vitro fertilization

"Expert Q & A: Cystic Fibrosis, Complicated and Variable" by Carolyn Sayre, published 24 April 2009 - http://www.nytimes.com/ref/health/healthguide/esn-cystic-fibrosis-expert.html Thank you, Wordreader (talk) 06:23, 16 August 2014 (UTC)

More interesting online resources

From the site: "The Cochrane Cystic Fibrosis & Genetic Disorders Review Group comprises an international network of health care professionals, researchers and consumers preparing, maintaining, and disseminating systematic reviews of randomised control trials in the treatment of cystic fibrosis and other genetic disorders."
Cochrane Cystic Fibrosis and Genetic Disorders Group
http://cfgd.cochrane.org/

This site has an excellent History section that chronologically includes numerous journal reports. The wording is mostly quite accessible to the casual, but interested reader:
Cystic Fibrosis Medicine
http://www.cfmedicine.com/Index.aspx

This is a long article that summarizes the various aspects of CF, including research on the mild forms, and historical high points of research. It cites numerous journal reports:
OMIM: Online Mendelian Inheritance in Man
http://omim.org/
"Article #219700: Cystic Fibrosis; CF"
http://omim.org/entry/219700

PDF from Chest, the journal of the American College of Chest Physicians
"Cystic Fibrosis Adult Care"
http://www.readbag.com/cff-uploadedfiles-treatments-cfcareguidelines-agespecificcare-cf-adult-care-chest-2004

Thank you, Wordreader (talk) 01:22, 17 August 2014 (UTC)

Restoring the function of the chloride pump, Thoracentesis

In a recent article about Crohn’s Disease they were able to restore the function of molecular receptor causing the disease. I have have not heard any attempts to restore preceptor function in cystic fibrosis. In addition, why Thoracentesis-like procedure is not used in cystic fibrosis, it is all about draining fluid/mucus build up in the lungs. Or the use of net of artificial-micro-tubules inserted into the lungs to pump the mucus/fluid out with appropriate solution. Taissirn (talk) 17:17, 16 September 2014 (UTC) https://biobeat.nigms.nih.gov/2014/08/restoring-the-function-of-an-immune-receptor-involved-in-crohns-disease/ Taissirn (talk) 17:18, 16 September 2014 (UTC) May be we can think of drowning person with fluid in the lungs. We have mechanical techniques to pump the fluid out. Taissirn (talk) 17:38, 16 September 2014 (UTC)

Constructive comment regarding the current infobox image

Finger clubbing is not pathognomonic of this condition. I suggest an image of a person receiving chest physiotherapy would be iconic of this condition, since it is likely a daily ritual. Matthew Ferguson (talk) 16:55, 29 October 2015 (UTC)

  1. ^ Cite error: The named reference http://www.who.int/genomics/publications/en/HGN_WB_04.02_TOC.pdf was invoked but never defined (see the help page).