Meconium Ileus in the Cystic Fibrosis Patients of Russia - Abstract
The influence of meconium ileus (MI) on cystic fibrosis (CF) progression attracts
steady interest due to high MI prevalence and widespread practice of neonatal
screening.
Study: The aim of this study was to investigate MI prevalence in the Russian
population and describe the clinical characteristics of patients with MI in the neonatal
period in different age groups based on the Russian Federal Registry data as of 2014.
One hundred forty two MI patients were analyzed. The patients were included in the
registry, which contains 2131 CF patients from Russia.
Results: In 2014, the registry contained 6.6% CF patients who had experienced
MI. Within the first year of life 22.1% of children were diagnosed, among them 10,7%
of the children aged between 1 year and 7 years old, 5,6% of the children from 7
to 18 years old, and 1,5% of patients over 18. The age of diagnosis in patients with
ileus was 5-fold lower than the age of diagnosis of the patients without ileus. It was
0.76±2.01 years old versus 3.72±6.16, p<0.0001. The sweat chlorides determined
by sweat test were significantly higher in the group with ileus than in the group without
ileus. Significant clinical differences were identified in the body mass index (BMI)
only. The BMI was higher in the group of patients without MI. Electrolyte impairments,
aspergillosis and hepatic cirrhosis were more frequent in MI group. The survival rate
and age of death were lower for the patients with MI.
Conclusion: The frequency of MI was 6,6% among the patients with CF according
to the Russian Federal Registry data (2014). The affect of MI on survival and quality
of life still requires further discussion.