Malnutrition in cystic fi brosis – beyond pancreatic insuffi ciency

Neishranjenost u cističnoj fi brozi posljedica je nerazmjera između povećanih potreba za energijom, povećanih gubitaka, manjeg iskorištavanja hranjivih tvari s jedne strane i nedostatnog unosa hrane s druge. Vodeći uzrok maldigestije i malapsorpcije je insufi - cijencija pankreasa, no ni optimalna nadoknada enzima ne može ispraviti neishranjenost, jer ne djeluje na niz drugih čimbenika koji pridonose energetsko-prehrambenoj neravnoteži i trijadi infekcije, upale i opstrukcije u probavnom sustavu. Oni uključuju promijenjene karakteristike crijevnih sokova, disbiozu, nedostatak žučnih soli i neadekvatnu lipolizu, promjenu motiliteta, jetrenu bolest i drugo. Energetske potrebe veće su nego u zdravih osoba, zbog kronične infekcije i upale te dodatno rastu s propadanjem plućne funkcije. Dostatan unos hrane sprječavaju gastrointestinalne komplikacije, narušen tek, a često i psihosocijalni čimbenici. Važan je anticipirajući i aktivan stav liječnika i nutriciologa prema problemu uhranjenosti, čak i prije nego je ona narušena.Malnutrition in cystic fi brosis is a result of imbalance between increased energy requirement, increased losses and poorer nutrient utilization on the one hand, and inadequate food intake on the other hand. The most important cause of maldigestion and malabsorption is pancreatic insuffi ciency. However, even optimal enzyme replacement cannot correct malnutrition as it does not target many other causes adding to the nutritional imbalance and the pathophysiological triad of infection, infl ammation and obstruction. Contributing factors are abnormal intestinal juices, dysbiosis, bile salt abnormalities and altered lipolysis, altered motility, liver disease, etc. Energy requirements are increased in comparison to healthy subjects due to chronic infection and infl ammation, and additionally rise as pulmonary function deteriorates. Energy intake may be inadequate due to gastrointestinal complications, poor nutrient utilization, or psychosocial issues. An anticipatory and active attitude of gastroenterologists and nutritionists regarding nutritional problems is mandatory even before malnutrition emerges