Long Term Outcome of Cystic Fibrosis Patients with Multisystem Evaluation

Hassanzad, Maryam; Boloursaz, Mohammad Reza; Darougar, Sepideh; Nejad, Sabereh Tashayoie; Mohajerani, Seyed Amir; Baghaie, Nooshin; Hashemitari, Seyed Karen; Velayati, Ali Akbar

doi:10.5603/arm.2016.0040

Cited by 9 publications

(7 citation statements)

References 16 publications

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“…Cystic fibrosis (CF) is the most common genetic disease inherited in an autosomal recessive manner caused by mutations in the CFTR (CF transmembrane conductance regulator) gene located on the long arm of the seventh chromosome. The product of this gene is the CFTR protein present on the apical surface of the epithelial cells of the respiratory system, pancreas, intestine and sweat glands, where it acts as a chloride (Cl) channel [1,2]. The loss of functional CFTR Cl − channels disrupts Cl − transport across epithelia, which contributes to the production of thick, sticky mucus in secretory organs.…”

Section: Introductionmentioning

confidence: 99%

Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis

Drzymała-Czyż

Krzyżanowska-Jankowska

Dziedzic

et al. 2021

Biomolecules

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Background: Several factors could lead to lipid disturbances observed in cystic fibrosis (CF). This study aimed to assess sterol homeostasis in CF and define potential exogenous and endogenous determinants of lipid dysregulation. Methods: The study involved 55 CF patients and 45 healthy subjects (HS). Sterol concentrations (μg/dL) were measured by gas chromatography/mass spectrometry. CF was characterised by lung function, pancreatic status, liver disease and diabetes coexistence, Pseudomonas aeruginosa colonisation and BMI. CFTR genotypes were classified as severe or other. Results: Campesterol and β-sitosterol concentrations were lower (p = 0.0028 and p < 0.0001, respectively) and lathosterol levels (reflecting endogenous cholesterol biosynthesis) were higher (p = 0.0016) in CF patients than in HS. Campesterol and β-sitosterol concentrations were lower in patients with a severe CFTR genotype, pancreatic insufficiency and lower pancreatic enzyme dose (lipase units/gram of fat). In multiple regression analyses, β-sitosterol and campesterol concentrations were predicted by genotype and pancreatic insufficiency, whereas cholesterol and its fractions were predicted by phytosterol concentrations, age, dose of pancreatic enzymes, nutritional status and genotype. Conclusions: Independent determinants of lipid status suggest that malabsorption and pancreatic enzyme supplementation play a significant role in sterol abnormalities. The measurement of campesterol and β-sitosterol concentrations in CF patients may serve for the assessment of the effectiveness of pancreatic enzyme replacement therapy and/or compliance, but further research is required.

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Section: Introductionmentioning

confidence: 99%

Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis

Drzymała-Czyż

Krzyżanowska-Jankowska

Dziedzic

et al. 2021

Biomolecules

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“…5 In isolation of saliva cultures, some patients can be infected with Haemophilus influenza, Staphylococcus aureus, Pseudomonas aeruginosa, Burkholderia cepacia, Escherichia coli, and Klebsiella pneumonia. 6 Treatment of lung disease using antibiotics is to reduce infection and control inflammation to enhance drug efficacy and reduce the dose and side effects; microspheres are alternative to lung delivery system. 7 Chemical properties of forming polymer may provide potential efficacy for the antibiotic drug.…”

Section: Introductionmentioning

confidence: 99%

Optimization Performance and Physical Stability of Ciprofloxacin HCL-Ca Alginate Microspheres: Effect of Different Concentration of Alginate and CaCl2

Hariyadi¹,

Hendradi²

2020

ijddt

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Inhalation treatment using antibiotics is an alternative for lung delivery. However, the therapeutic efficacy of inhaled drugs is limited by their rapid clearance in the lungs. Sustained release systems in the lungs can improve therapeutic outcomes of drugs because they can retain the drug load within the lungs and progressively release the drug locally at therapeutic levels. This study presents the formulation strategies to control drug release in the lungs using an alginate polymer-based microspheres system. The microsphere’s composition can be adjusted to modulate release and can encapsulate compounds with high loading. The pulmonary route is commonly used and has been well accepted as a portal for non-invasive drug delivery for many lung diseases. It is explored for decades as an alternative for systemic as well as local drug delivery. The present study explored the in vitro benefits of ciprofloxacin encapsulated in alginate microspheres. The studies included size, morphology, yield, drug loading, and encapsulation efficiency as well as stability. Current results showed small, smooth, and spherical ciprofloxacin-alginate microspheres were produced using aerosolization techniques. Small particles of less than 5μm were formed, which suitable for inhalation particles for lung delivery. High entrapment efficiency up to 95%, loadings of 80%, and a yield of 89% were also showed from microspheres. It was confirmed that all microspheres were stably indicated by no significant changes in morphology, organoleptic, and drug content after 30 days of storage. The recent promising characteristics of microspheres for pulmonary delivery will need further evaluation of the potency against microorganisms in lung disease.

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“…This information should be helpful in developing practice guidelines in order to improve quality of inpatient care, decreasing iatrogenic complications, providing valuable prognostic information, and identifying new challenges in the management of the increasing population of individuals with CF. A second article by Hassanzad et al [5] from Iran gives us an opportunity to recognize another example of a CF national group. Clinical characteristics may be an important example.…”

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confidence: 99%

Living Better and Longer with Cystic Fibrosis

Cofta

2016

Advances in Respiratory Medicine

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Long Term Outcome of Cystic Fibrosis Patients with Multisystem Evaluation

Abstract: The clinical outcome of cystic fibrosis is variable in different countries which may reflect environmental influences and the role of early diagnosis on long term outcomes. However, the role of early diagnosis in long-term outcomes of the disease can not be ignored.

Cited by 9 publications

References 16 publications

Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis

Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis

Optimization Performance and Physical Stability of Ciprofloxacin HCL-Ca Alginate Microspheres: Effect of Different Concentration of Alginate and CaCl2

Living Better and Longer with Cystic Fibrosis

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