Cystic fibrosis classification: Difference between revisions

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{{Cystic fibrosis}}
{{Cystic fibrosis}}


{{CMG}}; {{AE}}  
{{CMG}}; {{AE}} {{SHH}}
==Overview==
==Overview==
There is no established system for the classification of [disease name].
Cystic fibrosis may be classified according to [[Cystic fibrosis transmembrane conductance regulator|CFTR protein]] function abnormality into 6 classes and includes lack of production (class 1), failure to reach the site of action due to misfolding (class 2), defects in gating (class 3), reduced [[ion]] conductance (class 4), abnormally low [[Ion channel|channel]] numbers (class 5), and decreased [[half-life]] (class 6). Cystic fibrosis classes 1,2 and 3 are the most commonly associated with [[pancreatic insufficiency]].
 
OR
 
[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].
 
OR
 
[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3].
[Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].
 
OR
 
Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
 
OR
 
If the staging system involves specific and characteristic findings and features:
According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].
 
OR
 
The staging of [malignancy name] is based on the [staging system].
 
OR
 
There is no established system for the staging of [malignancy name].


==Classification==
==Classification==
Cystic fibrosis may be classified according to CFTR protein function abnormality:
Cystic fibrosis may be classified according to [[Cystic fibrosis transmembrane conductance regulator|CFTR]] [[mutation]] type:<ref name="pmid23776378">{{cite journal |vauthors=Burney TJ, Davies JC |title=Gene therapy for the treatment of cystic fibrosis |journal=Appl Clin Genet |volume=5 |issue= |pages=29–36 |year=2012 |pmid=23776378 |pmc=3681190 |doi=10.2147/TACG.S8873 |url=}}</ref><ref name="pmid12606185">{{cite journal |vauthors=Ratjen F, Döring G |title=Cystic fibrosis |journal=Lancet |volume=361 |issue=9358 |pages=681–9 |year=2003 |pmid=12606185 |doi=10.1016/S0140-6736(03)12567-6 |url=}}</ref><ref name="pmid27347364">{{cite journal |vauthors=Edmondson C, Davies JC |title=Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications |journal=Ther Adv Chronic Dis |volume=7 |issue=3 |pages=170–83 |year=2016 |pmid=27347364 |pmc=4907071 |doi=10.1177/2040622316641352 |url=}}</ref>
* '''Class 1:''' A lack of production
{|
* '''Class 2:''' Failure to reach its site of action due to misfolding (commonest Caucasian defect is Phe508Del)
! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Cystic fibrosis classification according to CFTR protein function abnormality
* '''Class 3:''' Defects in gating
|-
* '''Class 4:''' Conductance
!style="background:#4479BA; color: #FFFFFF;" align="center" | Class
* '''Class 5:''' Abnormally low channel numbers
!style="background:#4479BA; color: #FFFFFF;" align="center" | Type of abnormality
* '''Class 6:''' Decreased half-life
!style="background:#4479BA; color: #FFFFFF;" align="center" | Features
|-
| style="background:#DCDCDC;" align="center" + |Class 1
| style="background:#F5F5F5;" align="center" + |Total or partial lack of production of a functional [[Cystic fibrosis transmembrane conductance regulator|CFTR]]
| rowspan="3" style="background:#F5F5F5;" + |
*Most common [[Mutation|mutations]] (class 2 is the most common [[mutation]] worldwide)
*Associated with [[pancreatic insufficiency]]
|-
| style="background:#DCDCDC;" align="center" + |Class 2
| style="background:#F5F5F5;" align="center" + |Failure to reach the site of action on the cell surface
(due to misfolding of the [[protein]])
|-
| style="background:#DCDCDC;" align="center" + |Class 3
| style="background:#F5F5F5;" align="center" + |Defects in gating
(fail to open in response to [[intracellular]] signal)
|-
| style="background:#DCDCDC;" align="center" + |Class 4
| style="background:#F5F5F5;" align="center" + |Reduced [[ion]] conductance
| rowspan="3" style="background:#F5F5F5;" + |
*Rare [[Mutation|mutations]]
*Typically do not have [[pancreatic insufficiency]]
|-
| style="background:#DCDCDC;" align="center" + |Class 5
| style="background:#F5F5F5;" align="center" + |Abnormally low [[Ion channel|channel]] numbers
([[Splicing (genetics)|splicing]] [[Mutation|mutations]] resulting in reduced amounts of [[Cystic fibrosis transmembrane conductance regulator|CFTR protein]])
|-
| style="background:#DCDCDC;" align="center" + |Class 6
| style="background:#F5F5F5;" align="center" + |Decreased [[half-life]]
|}


==References==
==References==
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[[Category:Pediatrics]]
[[Category:Pulmonology]]

Latest revision as of 20:36, 29 March 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]

Overview

Cystic fibrosis may be classified according to CFTR protein function abnormality into 6 classes and includes lack of production (class 1), failure to reach the site of action due to misfolding (class 2), defects in gating (class 3), reduced ion conductance (class 4), abnormally low channel numbers (class 5), and decreased half-life (class 6). Cystic fibrosis classes 1,2 and 3 are the most commonly associated with pancreatic insufficiency.

Classification

Cystic fibrosis may be classified according to CFTR mutation type:[1][2][3]

Cystic fibrosis classification according to CFTR protein function abnormality
Class Type of abnormality Features
Class 1 Total or partial lack of production of a functional CFTR
Class 2 Failure to reach the site of action on the cell surface

(due to misfolding of the protein)

Class 3 Defects in gating

(fail to open in response to intracellular signal)

Class 4 Reduced ion conductance
Class 5 Abnormally low channel numbers

(splicing mutations resulting in reduced amounts of CFTR protein)

Class 6 Decreased half-life

References

  1. ↑ Burney TJ, Davies JC (2012). "Gene therapy for the treatment of cystic fibrosis". Appl Clin Genet. 5: 29–36. doi:10.2147/TACG.S8873. PMC 3681190. PMID 23776378.
  2. ↑ Ratjen F, Döring G (2003). "Cystic fibrosis". Lancet. 361 (9358): 681–9. doi:10.1016/S0140-6736(03)12567-6. PMID 12606185.
  3. ↑ Edmondson C, Davies JC (2016). "Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications". Ther Adv Chronic Dis. 7 (3): 170–83. doi:10.1177/2040622316641352. PMC 4907071. PMID 27347364.

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