Cystic fibrosis

Lack of applicability of the Enterocyte Chloride ion secretion paradigm to the Pathology of Cystic Fibrosis

Published on: 23rd October, 2017

OCLC Number/Unique Identifier: 7900080180

This review examines of the concept of a defective chloride channel in epithelial cells being a major cause of cystic fibrotic pathophysiology. The central concept of the defective chloride ion channel paradigm is that faulty CFTR protein or failed delivery of CFTR protein to the mucosal membrane of epithelial cells is the basis of cystic fibrosis. Defective placement or function of CFTR prevents hydration of bronchial mucus that is normally caused by epithelial cells; these are capable through chloride ion secretion of transporting fluid to the mucosal surface. This concept relies heavily on a paradigm taken from intestinal physiology-namely that the intestinal epithelial cell secretes chloride ion and fluid and that this has conferred heterozygote selective advantage in carriers of the cystic fibrosis gene. This present review examines the evidence for that hypothesis and assembles evidence from past studies that it is the smooth muscle cell that is of greater relevance. This review does not aim to provide an overview of current research into cystic fibrosis. The intention is to provide an overview of past research that led to the concept of a failure of epithelial cells to hydrate bronchial mucus because of compromised CFTR function. It is important to present all past evidence for aspects of the chloride secretion hypothesis and its associated heterozygote advantage concept so that the important evidential milestones can be re-assessed.
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Cystic fibrosis and congenital adrenal hyperplasia: A rare occurrence with diagnostic dilemmas, similarities and contradictions

Published on: 20th October, 2020

OCLC Number/Unique Identifier: 8686101406

Cystic fibrosis (CF) is a hereditary syndrome composed of exocrine gland dysfunction involving multiple systems which if untreated may result in chronic respiratory infections, pancreatic enzyme deficiency and failure to thrive. The association between CF and other inherited diseases or congenital anomalies is rare. We describe a rare case of CF with concomitant congenital adrenal hyperplasia (CAH). 21- Hydroxylase deficiency accounts for most CAH cases. Varity in clinical phenotypes depends on the amount of enzymatic activity which in turn depends on different combination of gene mutations. The genes of CAH and CF are located in different locations. The chance of these diseases coexisting in our patient would be a rare combination. However, such a case will be more frequent in our population than others because of consanguineous marriage and common ancestors. There are diagnostic difficulties, similarities and contradictions between two diseases and they are pointed out.
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Case reports of observed significant improvement in patients with ARDS due to COVID-19 and maximum ventilatory support after inhalation of sodium bicarbonate

Published on: 19th May, 2020

OCLC Number/Unique Identifier: 8599065026

The emergence of COVID-19 worldwide in an unprecedented pandemic. COVID-19 has a significant mortality, mostly from acute lung injury. We reviewed the available literature from China and Europe in regard to the behavior of SARS-Cov2 and ability to adhere to the cell wall [1,2]. The evidence based literature describes three component for the virus to grant entry to the target cells including Cathepsin B/L (the viral cap protein needed for initial connectivity to the cell wall), the angiotensin converting enzyme 2 and a low PH environment to allow the first connectivity of the virus to the cell wall [3]. The goal of our Case study was to prevent SARS- SARS-Cov2 from entering target cells by raising the airways PH using sodium bicarbonate inhalation. The sodium Bicarbonate inhalation (4.2% concentration) has been used safely in Cystic fibrosis (CF) patients with inspissated mucoid impaction [3,4] and in chloride inhalation toxicity by opposing the effect of the low PH induced by the insulting agent [4,5]. It has not been administered for COVID -19 patients particularly prior to this study. 
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Cystic fibrosis, the key link with airborne alpha emitting nanoparticulates

Published on: 18th June, 2021

OCLC Number/Unique Identifier: 9100376557

Cystic fibrosis is explained in this paper that suggests tackling the disease by elimination of the most significant sources of contamination.
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