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Cystic Fibrosis: Pathogenesis01:23

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Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
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Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
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Age-related pharmacokinetic changes are extensively documented, but understanding age-related pharmacodynamic alterations is relatively limited. This knowledge gap can be partly attributed to the complexity of developing appropriate measures of drug responses compared to bioanalytical methods for determining drug concentrations.Most information regarding age-related differences in human pharmacodynamics originates from cross-sectional studies. However, these studies assume that observed mean...
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Drug distribution in the human body is influenced by several factors, including plasma protein concentration, body composition, blood flow, tissue-protein concentration, and tissue fluid pH. Among these, changes in plasma protein concentration and body composition due to aging significantly affect how drugs are distributed within the body. Specifically, aging is associated with a decrease in albumin levels by about 10% and an increase in α1-acid glycoprotein levels. These alterations are...
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EVALUATION OF NUTRITIONAL STATUS IN PATIENTS WITH CYSTIC FIBROSIS ACCORDING TO AGE GROUP.

Lenycia de Cassya Lopes Neri1, Denise Pimentel Bergamaschi1, Luiz Vicente Ribeiro Ferreira da Silva Filho1

  • 1Universidade de São Paulo, São Paulo, SP, Brasil.

Revista Paulista De Pediatria : Orgao Oficial Da Sociedade De Pediatria De Sao Paulo
|August 16, 2018
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Summary

Most cystic fibrosis patients have good nutritional status and adequate nutrient intake, though some school-aged children are underweight. Further research on preschool children is recommended to reduce nutritional risks.

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Area of Science:

  • Pediatric Nutrition
  • Cystic Fibrosis Management
  • Public Health

Background:

  • Cystic Fibrosis (CF) requires careful nutritional monitoring due to malabsorption and increased energy needs.
  • Optimizing nutritional status is crucial for improving health outcomes and quality of life in CF patients.

Purpose of the Study:

  • To assess the nutritional profile of pediatric patients with cystic fibrosis.
  • To identify factors influencing nutritional status and dietary intake in this population.

Main Methods:

  • Cross-sectional study of 101 pediatric CF patients at a reference center in São Paulo, Brazil.
  • Data collection included dietary habits (24-hour recall), socioeconomic factors, anthropometrics, and pulmonary function.
  • Patients were stratified by age for statistical analysis.

Main Results:

  • 75% of patients were eutrophic; however, lower BMI Z-scores were noted in schoolchildren and adolescents.
  • 10% of preschoolers and 35% of school-aged children were underweight.
  • Adequate dietary intake was observed, with specific supplements linked to macronutrient adequacy.

Conclusions:

  • The majority of CF patients exhibited adequate nutritional status and nutrient intake, suggesting effective nutritional management.
  • Targeted studies on preschool children are needed to mitigate nutritional risks in older age groups.