Cystic Fibrosis

and, Neal S. Gould; Day, Brian J.

doi:10.1002/9781118355886.ch13

Cited by 1 publication

(3 citation statements)

References 124 publications

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“…When CFTR function is impaired, organ-specific pathology occurs in all but the kidneys. 2,9 The risk factor for developing CF has two parents who carry the abnormal gene, which will give birth to children with CF at 25%, healthy at 25%, and 50% are carriers, as seen in Figure 2. CF patients need to consume large amounts of calories to maintain their weight and growth.…”

Section: Etiology and Risk Factorsmentioning

confidence: 99%

“…These high doses have the potential to overcome the inflammation that occurs in CF. 9,15 Dornase alfa meghidrolysis deoxyribose nucleic acid (DNA), which is in mucus or phlegm and reduces the viscosity of mucus or phlegm so that it can increase airway clearance, thereby reducing exacerbations and improving lung function. 9,15 Inhalation therapy for CF uses hypertonic saline solution.…”

Section: Sweat Testmentioning

confidence: 99%

“…9,15 Dornase alfa meghidrolysis deoxyribose nucleic acid (DNA), which is in mucus or phlegm and reduces the viscosity of mucus or phlegm so that it can increase airway clearance, thereby reducing exacerbations and improving lung function. 9,15 Inhalation therapy for CF uses hypertonic saline solution. This therapy is recommended for patients aged 6 years and over.…”

Section: Sweat Testmentioning

confidence: 99%

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Cystic Fibrosis Lung Disease: A Narrative Literature Review

Putra,

Masrul Basyar,

Yessy Susanty Sabri

2024

Bioscmed

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Cystic fibrosis (CF) is caused by mutations in autosomal recessive genes that code for proteins cystic fibrosis transmembrane conductance regulator (CFTR) which is located on chromosome seven. The CFTR protein under normal conditions acts as a chloride channel and helps the movement of sufficient electrolytes and water across the membrane. Mutations in CFTR cause abnormalities in chloride ion transport through epithelial cells and impaired sodium and water transport resulting in viscous secretions with low water content. This thick and sticky secretion will inhibit the normal function of various organs, although pulmonary complications are the most common cause of death. Cystic fibrosis has wide genotypic and phenotypic variations. There are six categories of mutations based on their effect on the CFTR protein, where these categories are not only used to predict the phenotype but also to determine better therapeutic strategies based on the identified mutations.

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Section: Etiology and Risk Factorsmentioning

confidence: 99%