# Biochemical assessment in a cohort of pediatric patients with cystic fibrosis

**Authors:** Corina-Elena Anghel (Delia), Andreea-Mariana Negrescu, Iustina-Violeta Stan, Simona Raluca Iacoban, Elena-Simona Moiceanu, Geanina-Mirela Toma, Elena Ionica, Anisoara Cimpean

PMC · DOI: 10.25122/jml-2024-0288 · Journal of Medicine and Life · 2024-06-01

## TL;DR

This study examines the health and biochemical markers of children with cystic fibrosis to better understand their condition and improve care.

## Contribution

The study explores the relationship between biochemical parameters and clinical symptoms in pediatric cystic fibrosis patients.

## Key findings

- Patients with CF showed impaired lipid status and deficiencies in iron and vitamins A, D, and E.
- Chronic bacterial infections were common among the studied CF patients.
- The findings suggest personalized therapeutic strategies could improve CF patient management.

## Abstract

Cystic fibrosis (CF) is a recessive inherited disorder caused by genetic mutations in the CF transmembrane conductance regulator (CFTR) gene. It is a multisystem condition that primarily induces abnormal mucus accumulation in the respiratory system and obstructs the intrapancreatic common bile duct, causing a reduction in the delivery of digestive enzymes to the small intestine. Thus, patients with CF are characterized by maldigestion, malabsorption, and recurrent airway bacterial infections. Clinical monitoring of the health status of patients with CF is mandatory for increasing the patients’ lifespan. To assess the feasibility of monitoring life quality (LQ) in pediatric patients with cystic fibrosis (CF) and to explore the relationship between biochemical parameters and clinical symptoms, our study analyzed inflammatory responses related to CF, medication, and pulmonary bacterial infections in 52 patients diagnosed with CF. Blood, hypo-pharyngeal exudate, and fecal samples were analyzed using clinical biochemistry, hematology, and microbiology techniques at the Alessandrescu-Rusescu National Institute for Mother and Child Health central laboratory in Bucharest, Romania. All the participants adhered to their prescribed outpatient CF regimens and appeared clinically stable. The overall clinical status of patients with CF was observed and compared with that of a healthy control group, which consisted of individuals similar in number and age. The screened patients with CF presented an impaired lipid status and chronic infections with various bacteria, iron, and vitamin (A, D, and E) deficiencies. Our findings provide insights into the pathophysiological mechanisms of CF and suggest that tailored monitoring and personalized therapeutic strategies could improve patient management.

## Linked entities

- **Genes:** CFTR (CF transmembrane conductance regulator) [NCBI Gene 1080]
- **Chemicals:** iron (PubChem CID 23925), vitamin A (PubChem CID 445354), vitamin E (PubChem CID 14985)
- **Diseases:** cystic fibrosis (MONDO:0009061)

## Full-text entities

- **Genes:** CFTR (CF transmembrane conductance regulator) [NCBI Gene 1080] {aka ABC35, ABCC7, CF, CFTR/MRP, MRP7, TNR-CFTR}
- **Diseases:** inflammatory (MESH:D007249), lipid (MESH:D011017), infections (MESH:D007239), CF (MESH:D003550), recessive inherited disorder (MESH:D030342), airway (MESH:D000402), malabsorption (MESH:D008286), bacterial infections (MESH:D001424)
- **Chemicals:** iron, and vitamin (A, D, and E) deficiencies (-)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

8 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11407493/full.md

## References

46 references — full list in the complete paper: https://tomesphere.com/paper/PMC11407493/full.md

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Source: https://tomesphere.com/paper/PMC11407493