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Lungs with cystic fibrosis fail to activate vitamin D (poor CYP27B) – Feb 2016

Cystic fibrosis bronchial epithelial cells have impaired ability to activate Vitamin D.

Acta Paediatr. 2016 Feb 10. Doi: 10.1111/apa.13361. [Epub ahead of print]
Pincikova T1,2,3, Svedin E3, Domsgen E3, Flodström-Tullberg M3, Hjelte L1,2.

  • 1 Stockholm CF Center, Karolinska University Hospital Huddinge, Stockholm, Sweden.
  • 2 Division of Pediatrics, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Stockholm, Sweden.
  • 3 Center for Infectious Medicine, Department of Medicine, Karolinska Institutet, Karolinska University Hospital Huddinge, Stockholm, Sweden.

The vitamin D receptor system is widely expressed in the human body, including immune system cells and epithelial cells (1). The enzyme responsible for the second hydroxylation step in the production of active vitamin D, 1alpha-hydroxylase (Cyp27B), is not just expressed in the kidneys, as previously thought (2).
Primary lung epithelial cells have been shown to activate vitamin D (3). The focus of recent vitamin D research has been to test the effect of the active form of vitamin D in vitro (4-6) and this has led to increased knowledge about the role of active vitamin D in the immune system and in antimicrobial defence. The picture that has emerged from studies strongly suggests that active vitamin D has an important function in regulating inflammation and infection, including in cystic fibrosis (5-6).

PMID: 26865115 PDF is available in DeepDyve.com

Reference #3

Respiratory Epithelial Cells Convert Inactive Vitamin D to its Active Form - 2008
 Download the PDF from VitaminDWiki

Respiratory epithelium Wikipedia

See also VitaminDWiki

Cystic Fibrosis category page in VitaminDWiki follows:

(Note: Must click here to see attachements

42 items below


See also VitaminDWiki

Breathing category listing has 397 items along with related searches

Inhaled Vitamin D looks like a possibility for CF
COPD Overview

See also web

  • Vitamin D supplementation in patients with cystic fibrosis:
    • A systematic review and meta-analysis - Dec 2020 No such attachment on this page
    • Mortality. 2 trials studed CF deaths in the 12-month follow-up: 1 intervention, 5 placebo; p=0.03
    • Meta-analysis said Vitamin D did not consistently help - mainly because trials had different outcome measures (other than death)
  • Costly Vertex Drug Is Denied, and Medicaid Patients Sue July 2014
    • Kalydeco, for cystic fibrosis costs $300,000 per year;
    • average lung function improvement of 10.1% after about 11 months, compared with a decline of 0.4% in patients taking placebo….”
    • Note by VitaminDWiki - Vitamin D appears to provide a much better improvement and is 30,000 X less expensive
  • CF trial to use 250,000 IU bolus dose of vitamin D July 2017
    To be followed by maintenance dose of 50,000 IU Vitamin D every 2 weeks
    No such attachment on this page
  • Patient Voices: Cystic Fibrosis New York Times Oct 2017
    Four CF youths are profiled
    Two are running, one is surfing,
    One stays indoors and takes 50 pills per day
    Another hint that Vitamin D helps (not mentioned by the article)

See also PubMed

See also WikiPedia

  • Cystic fibrosis (CF), also known as mucoviscidosis, is a genetic disorder that affects mostly the lungs but also the pancreas, liver, kidneys and intestine.[1][2] Long-term issues include difficulty breathing and coughing up sputum as a result of frequent lung infections.
  • Other symptoms include sinus infections, poor growth, fatty stool, clubbing of the finger and toes, and infertility in males among others.
  • Different people may have different degrees of symptoms.
  • Occurance: 1 in 30,000 in US, 1 in 1,300 in Ireland
  • Primarily genetic, incureable
  • Congenitally unable to process Vitamin K very well - wonder if vitamin K injections would help
  • "While infants born with it 70 years ago would have been unlikely to live beyond their first year, infants today are likely to live well into adulthood"
  • "Many people with CF are on one or more antibiotics at all times, . . . "


UK Cystic Fibrosis recommendations to be released in 2017

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It is interesting to note that the comments document is 2X larger than the draft document

2014 PHD dissertation on CF and Vitamin D

97 pages by Pincikova
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Average annual cost of CF $16,000

Understanding the costs of care for cystic fibrosis: an analysis by age and health state March 2013
US $ Costs for patients with mild =$10,151, moderate, =$25,647 and severe =$33,691
Costs: hospital inpatients (58%), followed by pharmaceuticals (29%)
 Download the PDF from VitaminDWiki
_"Cure" for Cystic Fibrosis - Trikafta - Costs $311,000 for a year
Washington Post Oct 2019 a tripple drug
improves lung function by about only 14%
unknonw how many years it needs to be take

Role of vitamin D on gut microbiota in cystic fibrosis - 2018

Journal of Steroid Biochemistry – Jan 2018 Pages 82-87
Mansi Kanhere, Benoit Chassaing, Andrew T. Gewirtz, Vin Tangpricha

  • Vitamin D plays a vital role in maintaining the integrity of the gut epithelium.
  • The mechanism underlying this association involves the reduction of pro-inflammatory cytokines such as IL-8 and inhibition of inflammation-induced apoptosis of intestinal epithelial cells by vitamin D.
  • Intestinal dysbiosis is prevalent in CF owing to multiple factors including malabsorption of nutrients and recurrent antibiotics.
  • VDR regulates the composition and functions of the gut microbiota and decreased expression of intestinal epithelial VDR disrupts microbial homeostasis.
  • Vitamin D could be a potential treatment option for altering dysbiosis in CF, but additional trials are needed to evaluate this.

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CF results in pancreatic insufficiency-induced malabsorption - which reduces the response to standard Vitamin D - Jan 2021

Factors Contributing to Vitamin D Status at Hospital Admission for Pulmonary Exacerbation in Adults With Cystic Fibrosis
Am J Med Sci . 2021 Jan;361(1):75-82. doi: 10.1016/j.amjms.2020.08.020
Should consider gut-friendly forms of Vitamin D, such as nanoemulsions which can be absorbed in the mouth, rather than the gut

Attached files

ID Name Comment Uploaded Size Downloads
7907 Respiratory Epithelial Cells 2008.pdf admin 10 Apr, 2017 16:36 1.49 Mb 421
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