Cystic fibrosis presenting with recurrent vomiting and metabolic alkalosis

Mathew, P. M.; Hamdan, Jahed A.; Nazer, Hisham M.

doi:10.1007/bf01955527

Cited by 19 publications

(10 citation statements)

References 18 publications

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“…19 Again, a further 10 children from the Eastern Province were documented as having CF in 1991, and Mathew et al suggested an incidence of 1 in 4243 for Saudi children. 20 Again, as previously described in Kuwait, vomiting and metabolic alkalosis associated with the high ambient temperatures in the summer months were associated with the presentation in these patients. A review by Nazer and Rahbeeni of their experiences of CF included 36 patients seen by them from 1986 to 1992.…”

supporting

confidence: 69%

Cystic Fibrosis in the Middle East: The Historical Perspective

Dawson

Frossard

2000

Ann Saudi Med

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supporting

confidence: 69%

Cystic Fibrosis in the Middle East: The Historical Perspective

Dawson

Frossard

2000

Ann Saudi Med

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“…The incidence of CF in the United Arab Emirates and Saudi Arabia is 1:16,000 and 1:4,000, respectively [ 4 , 5 ], while in Caucasian populations it is approximately 1:2,500, with comparable carrier rates throughout North America, the UK and Europe [ 6 ]. Eight thousand people are currently living with CF in the UK, with a median predicted survival of 41.5 years [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

Cystic Fibrosis from Laboratory to Bedside: The Role of A20 in NF-κB-Mediated Inflammation

et al. 2015

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Cystic fibrosis (CF) is a lifelong, inflammatory multi-organ disease and the most common lethal, genetic condition in Caucasian populations, with a median survival rate of 41.5 years. Pulmonary disease, characterized by infective exacerbations, bronchiectasis and increasing airway insufficiency is the most serious manifestation of this disease process, currently responsible for over 80% of CF deaths. Chronic dysregulation of the innate immune and host inflammatory response has been proposed as a mechanism central to this genetic condition, primarily driven by the nuclear factor κB (NF-κB) pathway. Chronic activation of this transcription factor complex leads to the production of pro-inflammatory cytokines and mediators such as IL-6, IL-8 and TNF-α. A20 has been described as a central and inducible negative regulator of NF-κB. This intracellular molecule negatively regulates NF-κB-driven pro-inflammatory signalling upon toll-like receptor activation at the level of TRAF6 activation. Silencing of A20 increases cellular levels of p65 and induces a pro-inflammatory state. We have previously shown that A20 expression positively correlates with lung function (FEV₁%) in CF. Despite improvement in survival rates in recent years, advancements in available therapies have been incremental. We demonstrate that the experimental use of naturally occurring plant diterpenes such as gibberellin on lipopolysaccharide-stimulated cell lines reduces IL-8 release in an A20-dependent manner. We discuss how the use of a novel bio-informatics gene expression connectivity-mapping technique to identify small molecule compounds that similarly mimic the action of A20 may lead to the development of new therapeutic approaches capable of reducing chronic airway inflammation in CF.

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“…These cases are usually difficult to correct and treatment needs modified oral rehydration solution [13-15]. Patients with CF are also at risk of increased gastrointestinal and insensible fluid losses [16]. In addition, 5-17 % of CF patients are at risk of developing CFRD due to pancreatic endocinopathy [17-20].…”

Section: Discussionmentioning

confidence: 99%

Metabolic Alkalosis in Adults with Stable Cystic Fibrosis

Al-Ghimlas

Faughnan²,

Tullis³

2012

TORMJ

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Background: The frequency of metabolic alkalosis among adults with stable severe CF-lung disease is unknown.Methods: Retrospective chart review.Results: Fourteen CF and 6 COPD (controls) patients were included. FEV1 was similar between the two groups. PaO2 was significantly higher in the COPD (mean ± 2 SD is 72.0 ± 6.8 mmHg,) than in the CF group (56.1 ± 4.1 mmHg). The frequency of metabolic alkalosis in CF patients (12/14, 86%) was significantly greater (p=0.04) than in the COPD group (2/6, 33%). Mixed respiratory acidosis and metabolic alkalosis was evident in 4 CF and 1 COPD patients. Primary metabolic alkalosis was observed in 8 CF and none of the COPD patients. One COPD patient had respiratory and metabolic alkalosis.Conclusions: Metabolic alkalosis is more frequent in stable patients with CF lung disease than in COPD patients. This might be due to defective CFTR function with abnormal electrolyte transport within the kidney and/ or gastrointestinal tract.

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Cystic fibrosis presenting with recurrent vomiting and metabolic alkalosis

Cited by 19 publications

References 18 publications

Cystic Fibrosis in the Middle East: The Historical Perspective

Cystic Fibrosis in the Middle East: The Historical Perspective

Cystic Fibrosis from Laboratory to Bedside: The Role of A20 in NF-κB-Mediated Inflammation

Metabolic Alkalosis in Adults with Stable Cystic Fibrosis

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Cystic fibrosis presenting with recurrent vomiting and metabolic alkalosis

Cited by 19 publications

References 18 publications

Cystic Fibrosis in the Middle East: The Historical Perspective

Cystic Fibrosis in the Middle East: The Historical Perspective

Cystic Fibrosis from Laboratory to Bedside: The Role of A20 in NF-&#954;B-Mediated Inflammation

Metabolic Alkalosis in Adults with Stable Cystic Fibrosis

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Cystic Fibrosis from Laboratory to Bedside: The Role of A20 in NF-κB-Mediated Inflammation