Cystic Fibrosis in Children—Monitoring, Complications and New Therapies

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Pulmonary and Sleep Medicine".

Deadline for manuscript submissions: closed (15 June 2024) | Viewed by 2892

Special Issue Editors


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Guest Editor
Pediatric Department, ‘Victor Babes’ University of Medicine and Pharmacy Timisoara, Eftimie Murgu Square No. 2, 300041 Timisoara, Romania
Interests: cystic fibrosis; CF lung disease; exacerbations therapy; CF-associated liver disease; CF diabetes; CF modulators; CF therapy; CF elastography

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Guest Editor
Pediatric Pulmonology and Cystic Fibrosis Unit, Hippokration Hospital, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece
Interests: cystic fibrosis; asthma; NIV – children with special care needs (technology dependent children)

Special Issue Information

Dear Colleagues,

Cystic fibrosis remains a challenging disease, whether in terms of diagnosis and treatment points of view, or regarding its multifaceted complications. Besides lung disease, the condition that dictates the disease outcome, more other comorbidities like liver disease and diabetes arise, complicating disease management, especially in children.

With the new use of modulators, the life expectancy is likely to upsurge. However, but further studies would be necessary in order to provide the best care for CF children. With increased life expectancy, more complications occur and several decisions must be taken in clinical practice.

As monitoring the disease is key to obtaining satisfactory outcome, new approaches like lung ultrasound, liver elastography or continuous glycemia monitoring requires appropriate evaluation and management. Deliver individual care to CF children is achievable and possible with the adaptation of new techniques and treatments to every patient. 

This Issue addresses the cystic fibrosis diagnosis, monitoring and management of disease’s complications like lung disease exacerbations, and their relation to other non-respiratory complications like liver disease, diabetes, bone disease, endocrine dysfunctions and new treatments.

Prof. Dr. Ioana-Mihaiela Ciuca
Prof. Dr. Elpis Hatziagorou
Guest Editors

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Keywords

  • cystic fibrosis
  • CF lung disease
  • children
  • CF modulators
  • CF complication
  • CF diabetes
  • CF liver disease
  • CF bone disease
  • CF respiratory exacerbations

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Published Papers (1 paper)

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Review

29 pages, 1049 KiB  
Review
An Update in Cystic Fibrosis-Related Diabetes in Children and Adolescents
by Dana-Teodora Anton-Păduraru, Alina Mariela Murgu, Mădălina Andreea Donos, Felicia Trofin, Alice Nicoleta Azoicăi, Paula Popovici, Aurelian Bogdan Stana, Ionela Gheorghiescu and Laura Mihaela Trandafir
Children 2023, 10(12), 1879; https://doi.org/10.3390/children10121879 - 30 Nov 2023
Cited by 1 | Viewed by 1883
Abstract
This paper delineates several aspects of cystic fibrosis-related diabetes (CFRD)—a common complication of cystic fibrosis (CF). CFRD exhibits a predilection for older individuals with CF, yet it also extends its influence on children and adolescents. Scientific insights postulate a potential link between CFRD [...] Read more.
This paper delineates several aspects of cystic fibrosis-related diabetes (CFRD)—a common complication of cystic fibrosis (CF). CFRD exhibits a predilection for older individuals with CF, yet it also extends its influence on children and adolescents. Scientific insights postulate a potential link between CFRD and the aberrant mucus production within the pancreas, thereby culminating in pancreatic insufficiency. This, in turn, perturbs the synthesis of insulin, a pivotal endocrine hormone responsible for the regulation of glycemic levels. Standardized protocols advocate for the systematic screening of CFRD among all individuals with CF, commencing at the age of 10 years using the oral glucose tolerance test (OGTT). Therapeutic modalities encompass insulin therapy, dietary adjustments, and the vigilant monitoring of glycemic parameters. The overarching objective is to maintain blood glucose levels within a targeted range to mitigate the advent of diabetic complications. Untreated or sub-optimally managed CFRD can precipitate a spectrum of deleterious health ramifications, encompassing cardiovascular afflictions, neuropathy, renal dysfunction, and ocular complications. Full article
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