Pancreatitis in Cystic Fibrosis and CFTR-Related Disorder

Coffey, Michael J.; Ooi, Chee Y.

doi:10.5772/27861

Cited by 2 publications

(1 citation statement)

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“…Иммуногистологические исследования указывают на локализацию CFTR в апикальной части ацинарных клеток. Установлена четкая зависимость между недостаточностью внешнесекреторной функции ПЖ у больных МВ и мутациями в гене CFTR [4]. Мутации разделены на «тяжелые» и «мягкие» в соответствии со степенью нарушения внешнесекреторной функции ПЖ у больных МВ [5].…”

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Functional insufficiency of the pancreas and the metabolic activity of the microbiota in cystic fibrosis adults patients

Vinokurova

Баймаканова

Krasovsky³

et al. 2018

Terapevticheskii arkhiv

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In order to optimize the therapy, the functional state of the pancreas (P) and the peculiarities of metabolic activity of intestinal microbiota in adults with cystic fibrosis (CF) were assessed. Materials and methods. 14 CF patients (20-34 years, 7 men, 7 women) were enrolled. In 8 patients, the diagnosis was confirmed in the first year of life on the basis of clinical data, positive sweat test, 5 had genetic confirmation. In 4 patients, the diagnosis was confirmed at the age of 8-13 years and 2 patients aged 18, 27 years. In this group, genetic confirmation was in 4 subjects. In addition to general clinical studies, the level of C-peptide in blood, elastase and the concentration of short chain fatty acids in feces was determined. Results and discussion. Of elastase feces in 9 patients was 5.5±4.7 icg/g, that is revealed severe exocrine insufficiency of the pancreas and in 5 patients the elastase level was normal and amounted to 402±124 icg/g. Deployed the clinical picture of diabetes mellitus was observed in 3 patients. Metabolic activity of the colon microflora as a whole was reduced, the sum of the concentration of short-chain fatty acids (ΣCn) was 6.03±4.11 mg/g at a rate of 10.61±5.11 (p

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