Please use this identifier to cite or link to this item: http://repositorio.unicamp.br/jspui/handle/REPOSIP/202111
Type: Artigo de periódico
Title: Nasal Potential Difference In Cystic Fibrosis Considering Severe Cftr Mutations.
Author: Ng, Ronny Tah Yen
Marson, Fernando Augusto de Lima
Ribeiro, Jose Dirceu
Ribeiro, Antonio Fernando
Bertuzzo, Carmen Silvia
Ribeiro, Maria Angela Gonçalves de Oliveira
Severino, Silvana Dalge
Sakano, Eulalia
Abstract: The gold standard for diagnosing cystic fibrosis (CF) is a sweat chloride value above 60 mEq/L. However, this historical and important tool has limitations; other techniques should be studied, including the nasal potential difference (NPD) test. CFTR gene sequencing can identify CFTR mutations, but this method is time-consuming and too expensive to be used in all CF centers. The present study compared CF patients with two classes I-III CFTR mutations (10 patients) (G1), CF patients with classes IV-VI CFTR mutations (five patients) (G2), and 21 healthy subjects (G3). The CF patients and healthy subjects also underwent the NPD test. A statistical analysis was performed using the Mann-Whitney, Kruskal-Wallis, χ(2), and Fisher's exact tests, α = 0.05. No differences were observed between the CF patients and healthy controls for the PDMax, Δamiloride, and Δchloride + free + amiloride markers from the NPD test. For the finger value, a difference between G2 and G3 was described. The Wilschanski index values were different between G1 and G3. In conclusion, our data showed that NPD is useful for CF diagnosis when classes I-III CFTR mutations are screened. However, if classes IV-VI are considered, the NPD test showed an overlap in values with healthy subjects.
Rights: fechado
Identifier DOI: 10.1155/2015/306825
Address: http://www.ncbi.nlm.nih.gov/pubmed/25667564
Date Issue: 2015
Appears in Collections:Artigos e Materiais de Revistas Científicas - Unicamp

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