Nutritional therapy in patients with inherited metabolic disorders
Authors:
M. Floriánková; Š. Bláhová; M. Pencová; T. Honzík; P. Ješina
Authors place of work:
Klinika dětského a dorostového lékařství 1. LF UK a VFN, Praha
Published in the journal:
Čes-slov Pediat 2018; 73 (6): 395-407.
Category:
Summary
Most of inherited metabolic disorders are rare, but a number of these disorders increases and so does a number of diagnosed patients due to a newborn screening and a development of new analytic methods. Inherited metabolic disorders are mostly serious. Nutrition management has a significant role in a therapy of a part of these disorders, especially in so called small molecules disorders. It involves a regulated intake of a substrate, which metabolism is limited in a patient due to an enzymatic or a protein deficiency or functional disruption. Supplementation of lacking metabolic products and essential nutrients is also a part of a nutrition management. The goals of a nutrition management are to reach and maintain a normal level of all metabolites and a normal growth and development of a patient.
This article reviews the basic principles of a nutrition management of most often occuring inherited metabolic disorders, which are managible by a diet.
KEY WORDS:
nutrition therapy, inherited metabolic disorders, low protein diet, low fat diet, antihypoglycemic regiment, lactose-free diet, low fructose diet, low cholesterol diet, low plant sterol diet, ketogenic diet, low purine diet
Zdroje
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Štítky
Neonatology Paediatrics General practitioner for children and adolescentsČlánok vyšiel v časopise
Czech-Slovak Pediatrics
2018 Číslo 6
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