High ambient temperature and risk of intestinal obstruction in cystic fibrosis

Ooi, Chee Y.; Jeyaruban, Christina; Lau, Jasmine; Katz, Tamarah; Matson, A.; Bell, Scott C.; Adams, Susan; Krishnan, Usha

doi:10.1111/jpc.13096

Cited by 18 publications

(11 citation statements)

References 23 publications

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“…Increased insensible fluid losses, sometimes brought about by concomitant illness or environmental factors (i.e. CF pulmonary exacerbation, other febrile illness, increased ambient temperature), and relative dehydration may contribute to DIOS [10,19]; the impacts of these factors may be overestimated, however, as inadequate fluid intake may be a more likely contributing cause when prospectively assessed [11]. CF-related Diabetes Mellitus (CFRD) may be a possible risk factor for DIOS, but this has not yet been objectively corroborated [11].…”

Section: Risk Factors For Diosmentioning

confidence: 99%

Cystic Fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer

Abraham

Taylor

2017

Journal of Cystic Fibrosis

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Since 1966 when the Cystic Fibrosis Foundation Patient Registry (CFFPR) was founded, clinicians have witnessed significant advances in both the quality and quantity of life for patients living with Cystic Fibrosis (CF). As patients with CF live longer and fuller lives, increasing encumbrances from gastrointestinal manifestations of CF will be observed. This article serves to discuss "below the diaphragm" concerns involving the large intestine (Distal Intestinal Obstruction Syndrome, Constipation, and Colorectal Cancer). Avenues for development and implementation of clinical care protocols, particularly regarding proactive management of known associated conditions and cancer screening, will continue to be refined in the coming years. It falls to the multidisciplinary CF care team to be actively engaged in addressing these concerns effectively as priority shifts from relative acuity (typically related to early nutrition and lung function) to the travails of longevity as the CF population continues to age.

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Section: Risk Factors For Diosmentioning

confidence: 99%

Cystic Fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer

Abraham

Taylor

2017

Journal of Cystic Fibrosis

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“…Such fluctuations might be expected to be mirrored in a hard outcome such as lung function, but this link has also turned out not to be direct [24]. Dehydration during warm weeks is a risk factor for obstipation, but not for lung function [25]. Altered clinic opening hours during vacation periods, patient travel patterns during the calendar year, and respiratory outbreaks during seasonal CF community events [26,27] could all effect groups of patients, but the effect on a population level is uncertain.…”

Section: Discussionmentioning

confidence: 99%

Seasonal fluctuation of lung function in cystic fibrosis: A national register-based study in two northern European populations

Qvist

Schlüter

Rajabzadeh

et al. 2019

Journal of Cystic Fibrosis

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Background Many risk factors for lung disease in cystic fibrosis (CF) display a seasonal pattern yet it is unclear whether this is reflected in seasonal fluctuations in lung function. Methods We conducted a longitudinal study using CF registries in Denmark and the UK. 471 individuals with a median of 104 FEV 1 measurements per person and 7586 individuals with a median of nine FEV 1 measures per person were included from Denmark and the UK respectively. We estimated the effect of seasonality on percent predicted FEV 1 trajectories using mixed effects models whilst adjusting for clinically important covariates. Results We found no significant cyclical seasonal variation in lung function in either country. The maximum variation in percent predicted FEV 1 around the yearly average was estimated to be 0.1 percentage points (95%CI 0 to 0.21) and 0.14 percentage points (95%CI 0 to 0.29) in Denmark and the UK, respectively. When considering possible step-like changes between the four seasons, we found that lung function was higher in spring compared to winter in the UK (0.34 percentage points, 95%CI 0.1 to 0.59) though the difference was not of clinical significance. Conclusion In both the UK and Denmark there may be small seasonal changes in lung function but this effect is not of clinical importance.

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“…Additional file 3: Table S3. Genotypes and genetic backgrounds of CF patients with the p.Ser4Ter, p.Gln1100Pro or c.120del23, found in the literature and in the ClinVar database [27][28][29][30][31][32][33][34][35].…”

Section: Supplementary Informationmentioning

confidence: 99%

A comprehensive overview of the cystic fibrosis on the island of São Miguel (Azores, Portugal)

et al. 2020

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Background: Early diagnosis and treatment are improving significantly the quality of life of patients with cystic fibrosis (CF). This recessive disease is caused by a great variability of mutations in the CF transmembrane conductance (CFTR) gene, whose spectrum and frequency can be different across populations. Methods: We performed a retrospective cross-sectional study of CF patients from the island of São Miguel (Azores, Portugal) through a clinical, genealogical, genetic and epidemiological investigation. The clinical course of patients was analyzed as a whole and according to their genotype. Results: We identified 14 CF patients within a 23-year period, corresponding to a cumulative incidence of 1:3012 births, being three of them born from consanguineous unions. Genetic analysis revealed three CFTR genotypes: p.[Ser4Ter];[Gln1100Pro] was present in one patient with a less severe phenotype (1/14); c.[120del23];p.[Phe508del], a very rare one (2/14); and p.[Phe508del];[Phe508del] in the remaining patients (11/14). Clinically, respiratory infections (8/ 14) and growth failure (6/14) were the most common initial manifestations. All patients presented pancreatic dysfunction, with 21.4 and 100% of them showing endocrine and exocrine insufficiency, respectively. As expected, patients with severe phenotype were homozygous for p.Phe508del and had the lowest value of body mass index. Conclusions: The present study demonstrated that São Miguel Island has an increased incidence of CF when compared to recent Portuguese data (1:7500 live births). It also allowed a comprehensive overview of CF in São Miguel, improving medical practice along with genetic counselling and creating opportunities for genotype-targeted therapies.

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High ambient temperature and risk of intestinal obstruction in cystic fibrosis

Abstract: Relatively high ambient temperature may play a role in the pathogenesis of DIOS and constipation in CF.

Cited by 18 publications

References 23 publications

Cystic Fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer

Cystic Fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer

Seasonal fluctuation of lung function in cystic fibrosis: A national register-based study in two northern European populations

A comprehensive overview of the cystic fibrosis on the island of São Miguel (Azores, Portugal)

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